Hidradenitis suppurativa
Encyclopedia
Hidradenitis suppurativa is a skin disease that most commonly affects areas bearing apocrine sweat glands
Apocrine sweat glands
Apocrine sweat glands are sweat glands composed of a coiled secretory portion located at the junction of the dermis and subcutaneous fat, from which a straight portion inserts and secretes into the infundibular portion of the hair follicle...

 or sebaceous glands, such as the underarms, breasts, inner thighs, groin and buttocks.

Overview

The non-contagious disease manifests as clusters of chronic abscess
Abscess
An abscess is a collection of pus that has accumulated in a cavity formed by the tissue in which the pus resides due to an infectious process or other foreign materials...

es, epidermoid cysts, sebaceous cysts, pilonidal cyst
Pilonidal cyst
A pilonidal cyst, also referred to as a pilonidal abscess, pilonidal sinus or sacrococcygeal fistula, is a cyst or abscess near or on the natal cleft of the buttocks that often contains hair and skin debris.-Etymology:...

 or multilocalised infections, which can be as large as baseballs or as small as a pea, that are extremely painful to the touch and may persist for years with occasional to frequent periods of inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

, culminating in incision and drainage
Incision and drainage
Incision and drainage and clinical lancing are minor surgical procedures to release pus or pressure built up under the skin, such as from an abscess, boil, or infected paranasal sinus...

 of pus
Pus
Pus is a viscous exudate, typically whitish-yellow, yellow, or yellow-brown, formed at the site of inflammatory during infection. An accumulation of pus in an enclosed tissue space is known as an abscess, whereas a visible collection of pus within or beneath the epidermis is known as a pustule or...

, often leaving open wounds that will not heal. For unknown reasons, people with hidradenitis develop plugging or clogging of their apocrine glands. It causes chronic scarring and pus formation of the underarms (axilla) and groin/inner thigh areas. The simple procedure of incision and drainage provides some relief from severe, often debilitating, pressure pain. Flare-ups may be triggered by perspiration, hormonal
Hormone
A hormone is a chemical released by a cell or a gland in one part of the body that sends out messages that affect cells in other parts of the organism. Only a small amount of hormone is required to alter cell metabolism. In essence, it is a chemical messenger that transports a signal from one...

 changes (such as monthly cycles in women), humidity and heat, and clothing friction. Persistent lesions may lead to scarring and the formation of sinus tracts, or tunnels connecting the abscesses or infections under the skin. At this stage, complete healing is usually not possible, and progression varies from person to person, with some experiencing remission anywhere from months to years at a time, while others may worsen and require multiple surgeries in order to live comfortably. Wound dehiscence
Wound dehiscence
Wound dehiscence is a surgical complication in which a wound breaks open along surgical suture. Risk factors are age, diabetes, obesity, poor knotting or grabbing of stitches, and trauma to the wound after surgery.-Symptoms:...

, a premature "bursting" open of a wound, often complicates the healing process. Occurrences of bacterial infections and cellulitis
Cellulitis
Cellulitis is a diffuse inflammation of connective tissue with severe inflammation of dermal and subcutaneous layers of the skin. Cellulitis can be caused by normal skin flora or by exogenous bacteria, and often occurs where the skin has previously been broken: cracks in the skin, cuts, blisters,...

 (deep tissue inflammation) may occur at these sites. HS pain and depression
Depression (mood)
Depression is a state of low mood and aversion to activity that can affect a person's thoughts, behaviour, feelings and physical well-being. Depressed people may feel sad, anxious, empty, hopeless, helpless, worthless, guilty, irritable, or restless...

 can be difficult to manage.

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone. When they do see a doctor or medical practitioner, the disease is frequently misdiagnosed or prescribed treatments are ineffective, temporary and sometimes even harmful. There is no known cure nor any consistently effective treatment. Carbon dioxide laser surgery is currently considered the last resort for those who have advanced to its highest stage, where the affected areas are excised, and the skin is grafted. Surgery does not always alleviate the condition, however, and can be very expensive.

Several articles and clinics consider this disease as widely misdiagnosed, due to the misunderstanding of the causes and progression of the disease. HS is neither the biblical stigmata
Stigmata
Stigmata are bodily marks, sores, or sensations of pain in locations corresponding to the crucifixion wounds of Jesus, such as the hands and feet...

, leprosy
Leprosy
Leprosy or Hansen's disease is a chronic disease caused by the bacteria Mycobacterium leprae and Mycobacterium lepromatosis. Named after physician Gerhard Armauer Hansen, leprosy is primarily a granulomatous disease of the peripheral nerves and mucosa of the upper respiratory tract; skin lesions...

 nor caused by poor hygiene. HS is often called an 'orphan illness', due to little research being conducted on the disease at this time. Because HS is considered a rare disease, its incidence rate is not well known, but has been estimated as being between 1:24 (4.1%) and 1:600 (0.2%).

Areas of involvement with hidradenitis suppurativa

HS patients (n=164, 121 females, 43 males) Female Male p (statistical significance)
Axillae 70 (58%) 30 (70%) NS (not significant)
Mammary and inter-mammary 31 (26%) 2 (5%) 0.006
Inguino-femoral 111 (92%) 32 (74%) 0.007
Perianal and Perineal 40 (33%) 24 (56%) 0.01
Buttocks 30 (25%) 21 (50%) 0.006

  • Note that for the study involved in the table hereinbefore was a personal series of 164 patients. Of the 164 patients with HS, 76% were in Hurley's stage I, 20% were in Hurley's stage II, and 4% were in the final phase of the disease, Hurley's stage III.


Other names for HS
Hidradenitis suppurativa has been referred to by multiple names in the literature, as well as in various cultures. Some of these are also used to describe different diseases, or specific instances of this disease.
  • Acne conglobata
    Acne conglobata
    Acne conglobata is a highly inflammatory disease presenting with comedones, nodules, abscesses, and draining sinus tracts.This condition generally begins between the ages of 18 and 30. It usually persists for a very long time, and often until the patient is around 40 years old...

     – not really a synonym
    Synonym
    Synonyms are different words with almost identical or similar meanings. Words that are synonyms are said to be synonymous, and the state of being a synonym is called synonymy. The word comes from Ancient Greek syn and onoma . The words car and automobile are synonyms...

     – this is a similar process but in classic acne
    Acne
    Acne is a general term used for acneiform eruptions. It is usually used as a synonym for acne vulgaris, but may also refer to:*Acne aestivalis*Acne conglobata*Acne cosmetica*Acne fulminans*Acne keloidalis nuchae*Acne mechanica...

     areas of chest and back
  • Acne Inversa (AI) – a proposed new term which has not gained widespread favour.
  • Apocrine
    Apocrine
    Apocrine is a term used to classify exocrine glands in the study of histology. Cells which are classified as apocrine bud their secretions off through the plasma membrane producing membrane-bound vesicles in the lumen. This method is also called decapitation secretion...

     Acne
    Acne
    Acne is a general term used for acneiform eruptions. It is usually used as a synonym for acne vulgaris, but may also refer to:*Acne aestivalis*Acne conglobata*Acne cosmetica*Acne fulminans*Acne keloidalis nuchae*Acne mechanica...

     – a misnomer
    Misnomer
    A misnomer is a term which suggests an interpretation that is known to be untrue. Such incorrect terms sometimes derive their names because of the form, action, or origin of the subject becoming named popularly or widely referenced—long before their true natures were known.- Sources of misnomers...

    , out-dated, based on the disproven concept that apocrine glands are primarily involved. Though many do suffer with apocrine gland infection
    Infection
    An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...

    , so thought should be given to using HS with subtext re: glands involved.
  • Apocrinitis – another misnomer, out-dated, based on the disproven concept that apocrine glands are primarily involved
  • Fox-den disease – a catchy term not used in medical literature, based on the deep fox den / burrow – like sinuses
  • Hidradenitis Supportiva – a misspelling
  • Pyodermia fistulans sinifica – an older term, considered archaic now
  • Velpeau's disease – commemorating the French
    France
    The French Republic , The French Republic , The French Republic , (commonly known as France , is a unitary semi-presidential republic in Western Europe with several overseas territories and islands located on other continents and in the Indian, Pacific, and Atlantic oceans. Metropolitan France...

     surgeon who first described the disease
    Disease
    A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...

     in 1833
  • Verneuil's disease – recognizing the French surgeon
    Surgeon
    In medicine, a surgeon is a specialist in surgery. Surgery is a broad category of invasive medical treatment that involves the cutting of a body, whether human or animal, for a specific reason such as the removal of diseased tissue or to repair a tear or breakage...

     whose name is most often associated with the disorder as a result of his 1854–1865 studies

Historical overview of hidradenitis suppurativa

  • In 1839, Velpeau identified and described hidradenitis suppurativa.
  • In 1854, Verneuil described hidradenitis suppurativa as "Hidrosadénite Phlegmoneuse". This is how HS obtained its alternate name "Verneuil's disease".
  • In 1922, Schiefferdecker hypothesized a pathogenic link between "Acne inversa" and human apocrine
    Apocrine
    Apocrine is a term used to classify exocrine glands in the study of histology. Cells which are classified as apocrine bud their secretions off through the plasma membrane producing membrane-bound vesicles in the lumen. This method is also called decapitation secretion...

     sweat glands.
  • In 1956, Pillsbury wrote and published a medical journal article discussing hidradenitis suppurativa, describing the disease's main characteristics, dubbing them the "Acne triad: hidradenitis suppurativa,perifolliculitis capitis abscendens et suffodiens". Pillsbury's research study was one of the first peer-reviewed journal articles to appear publicly with many details of hidradenitis suppurativa, which are still used and relied on today in the medical realm of research on this disease.
  • In 1975, Plewig and Kligman, following Pillsbury's research path, modified the "Acne triad", replacing it with the "Acne tetrad: acne triad, plus pilonidal sinus". Plewig and Kligman's research follows in Pillsbury's footsteps, offering explanations of the symptoms associated with hidradenitis suppurativa.
  • Finally in 1989, Plewig and Steger's research led them to rename hidradenitis suppurativa, calling it "Acne Inversa" – which not still used today in medical terminology
    Medical terminology
    Medical terminology is a vocabulary for accurately describing the human body and associated components, conditions, processes and process in a science-based manner. Some examples are: R.I.C.E., trapezius, and latissimus dorsi. It is to be used in the medical and nursing fields...

    ; although some individuals still use this outdated term.




A surgeon from Paris
Paris
Paris is the capital and largest city in France, situated on the river Seine, in northern France, at the heart of the Île-de-France region...

, Velpeau described an unusual inflammatory process with formation of superficial axillary, sub-mammary and perianal abscesses in 1839. One of his colleagues also located in Paris, named Verneuil, coined the term “hidrosadénite phlegmoneuse” approximately 15 years later. This name for the disease reflects the former pathogenetic model of acne inversa, which is considered inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

 of sweat glands as the primary cause of hidradenitis suppurativa. In 1922 Schiefferdecker suspected a pathogenic association between acne inversa and apocrine sweat glands. In 1956 Pillsbury postulated follicular occlusion as the cause of acne inversa, which they grouped together with acne conglobata
Acne conglobata
Acne conglobata is a highly inflammatory disease presenting with comedones, nodules, abscesses, and draining sinus tracts.This condition generally begins between the ages of 18 and 30. It usually persists for a very long time, and often until the patient is around 40 years old...

 and perifolliculitis capitis abscendens et suffodiens (dissecting cellulitis of the scalp) as the "acne triad". Plewig and Kligman added another element to their acne triad, pilonidal sinus. Plewig et al. noted that this new "acne tetrad" includes all the elements found in the original "acne triad", in addition to a fourth element, pilonidal sinus. In 1989 Plewig and Steger introduced the term "acne inversa", indicating a follicular source of the disease and replacing older terms such as "Verneuil disease".

Historical view of hidradenitis suppurativa

Author Year Findings
Valpeau 1839 First description of the hidradenitis suppurativa
Verneuil 1854 "Hidrosadénite phlegmoneuse"
Pillsbury 1956 Acne triad (hidradenitis suppurativa, perifolliculitis capitis abscendens et suffodiens)
Plewig & Kligman 1975 Acne tetrad (acne triad + pilonidal sinus)
Plewig & Steger 1989 Acne inversa

Dermatohistological view of hidradenitis suppurativa

Author Year Major Features
Plewig & Steger 1989 Initial hyperkeratosis
Hyperkeratosis
Hyperkeratosis is thickening of the stratum corneum, often associated with a qualitative abnormality of the keratin, and also usually accompanied by an increase also in the granular layer...

 of the follicular
Hair follicle
A hair follicle is a skin organ that produces hair. Hair production occurs in phases, including a growth phase , and cessation phase , and a rest phase . Stem cells are principally responsible for the production of hair....

 infundibulum. Bacterial super-infection
Infection
An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...

 and follicle rupture. Granulomatous inflammatory reaction of the connective tissue. Apocrine
Apocrine
Apocrine is a term used to classify exocrine glands in the study of histology. Cells which are classified as apocrine bud their secretions off through the plasma membrane producing membrane-bound vesicles in the lumen. This method is also called decapitation secretion...

 and eccrine sweat glands secondarily involved.
Yu & Cook 1990 Cysts and sinus tracts lined with epithelium
Epithelium
Epithelium is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. Epithelial tissues line the cavities and surfaces of structures throughout the body, and also form many glands. Functions of epithelial cells include secretion, selective...

, in part with hair
Hair
Hair is a filamentous biomaterial, that grows from follicles found in the dermis. Found exclusively in mammals, hair is one of the defining characteristics of the mammalian class....

 shafts. Inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

 of apocrine
Apocrine
Apocrine is a term used to classify exocrine glands in the study of histology. Cells which are classified as apocrine bud their secretions off through the plasma membrane producing membrane-bound vesicles in the lumen. This method is also called decapitation secretion...

 sweat glands only if eccrine sweat glands and hair follicles are also inflamed.
Boer & Weltevreden 1996 Primary inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

 of the follicular infundibulum. Apocrine
Apocrine
Apocrine is a term used to classify exocrine glands in the study of histology. Cells which are classified as apocrine bud their secretions off through the plasma membrane producing membrane-bound vesicles in the lumen. This method is also called decapitation secretion...

 sweat glands are secondarily involved.

Stages

HS presents itself in three stages. Due to the large spectrum
Spectrum
A spectrum is a condition that is not limited to a specific set of values but can vary infinitely within a continuum. The word saw its first scientific use within the field of optics to describe the rainbow of colors in visible light when separated using a prism; it has since been applied by...

 of clinical severity, to severe repercussions on quality of life
Quality of life
The term quality of life is used to evaluate the general well-being of individuals and societies. The term is used in a wide range of contexts, including the fields of international development, healthcare, and politics. Quality of life should not be confused with the concept of standard of...

 and to the variety of available, a reliable method for evaluating HS severity is required. It should take into account the number, type and size of lesions, evolution, pain
Pain
Pain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...

 and repercussions for the quality of life
Quality of life
The term quality of life is used to evaluate the general well-being of individuals and societies. The term is used in a wide range of contexts, including the fields of international development, healthcare, and politics. Quality of life should not be confused with the concept of standard of...

 of the patient
Patient
A patient is any recipient of healthcare services. The patient is most often ill or injured and in need of treatment by a physician, advanced practice registered nurse, veterinarian, or other health care provider....

. Such a comprehensive instrument does not yet exist, but there have been two successful attempts proposed to classify patients with HS according to the severity of their disease
Disease
A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...

.

Hurley's staging system

This is historically the first classification system proposed, and is still in use for the classification of patients with skin/dermatologic diseases (i.e. psoriasis
Psoriasis
Psoriasis is an autoimmune disease that appears on the skin. It occurs when the immune system mistakes the skin cells as a pathogen, and sends out faulty signals that speed up the growth cycle of skin cells. Psoriasis is not contagious. However, psoriasis has been linked to an increased risk of...

, HS, acne
Acne
Acne is a general term used for acneiform eruptions. It is usually used as a synonym for acne vulgaris, but may also refer to:*Acne aestivalis*Acne conglobata*Acne cosmetica*Acne fulminans*Acne keloidalis nuchae*Acne mechanica...

). Hurley separated patients into three groups based largely on the presence and extent of cicatrization
Cicatrization
Cicatrization can mean:* the process of a wound healing to produce scar tissue* scarification, a form of body modification that uses cicatrization to create patterns on the skin....

 and sinuses. It has been used as a basis for clinical trials in the past and is a useful basis to approach therapy
Therapy
This is a list of types of therapy .* Adventure therapy* Animal-assisted therapy* Aquatic therapy* Aromatherapy* Art and dementia* Art therapy* Authentic Movement* Behavioral therapy* Bibliotherapy* Buteyko Method* Chemotherapy...

 for patients. These three stages are based on Hurley's staging system, which is simple and relies on the subjective
Subjectivity
Subjectivity refers to the subject and his or her perspective, feelings, beliefs, and desires. In philosophy, the term is usually contrasted with objectivity.-Qualia:...

 extent of the diseased tissue
Tissue (biology)
Tissue is a cellular organizational level intermediate between cells and a complete organism. A tissue is an ensemble of cells, not necessarily identical, but from the same origin, that together carry out a specific function. These are called tissues because of their identical functioning...

 the patient has. Hurley's three stages of hidradenitis suppurativa are as follows:
Stage Characteristics
I Solitary or multiple isolated abscess
Abscess
An abscess is a collection of pus that has accumulated in a cavity formed by the tissue in which the pus resides due to an infectious process or other foreign materials...

 formation without scarring or sinus tracts. (A few minor sites with rare inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

; may be mistaken for acne
Acne vulgaris
Acne vulgaris is a common human skin disease, characterized by areas of skin with seborrhea , comedones , papules , pustules , Nodules and possibly scarring...

.)
II Recurrent abscesses, single or multiple widely separated lesions, with sinus tract formation. (Frequent inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

 restrict movement and may require minor surgery
Surgery
Surgery is an ancient medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...

 such as incision and drainage
Incision and drainage
Incision and drainage and clinical lancing are minor surgical procedures to release pus or pressure built up under the skin, such as from an abscess, boil, or infected paranasal sinus...

.)
III Diffuse or broad involvement across a regional area with multiple interconnected sinus tracts and abscesses. (Inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

 of sites to the size of golf balls, or sometimes baseballs; scarring develops, including subcutaneous tracts of infection
Infection
An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...

 – see fistula
Fistula
In medicine, a fistula is an abnormal connection or passageway between two epithelium-lined organs or vessels that normally do not connect. It is generally a disease condition, but a fistula may be surgically created for therapeutic reasons.-Locations:Fistulas can develop in various parts of the...

. Obviously, patients at this stage may be unable to function.)

Sartorious staging system

The Sartorious staging system is more sophisticated than Hurley's, and is likely to supplant it as a means for conducting clinical trials during research. Sartorius et al. have suggested that the Hurley system is not sophisticated enough to assess treatment
Therapy
This is a list of types of therapy .* Adventure therapy* Animal-assisted therapy* Aquatic therapy* Aromatherapy* Art and dementia* Art therapy* Authentic Movement* Behavioral therapy* Bibliotherapy* Buteyko Method* Chemotherapy...

 effects in clinical trials. The need for uniform outcome variables when reporting treatment effects has led to the proposition of a score by Sartorious and colleagues. This classification allows for better dynamic monitoring of the disease
Disease
A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...

 severity in individual patients and therefore forms a complimentary system to the Hurley classification. They suggest a system that incorporates several of the involved areas of consideration:
  • Anatomic regions involved (axilla, groin gluteal or other region or infra-mammary region left or right)
  • Number and types of lesions involved (abscesses, nodules
    Nodule (medicine)
    For use of the term nodule in dermatology, see Nodule In medicine, a nodule refers to a relatively hard, roughly spherical abnormal structure....

    , fistulas, scars, points for lesions of all regions involved)
  • The distance between lesions, in particular the longest distance between two relevant lesions (i.e. nodules
    Nodule (medicine)
    For use of the term nodule in dermatology, see Nodule In medicine, a nodule refers to a relatively hard, roughly spherical abnormal structure....

     and fistulas in each region or size if only one lesion present)
  • The presence of normal skin in between lesions (i.e. are all lesions clearly separated by normal skin?)

Points are accumulated in each of the mentioned categories hereinbefore, and added to give both a regional and total score. In addition, the authors recommend adding a visual analog scale for pain or using the dermatology life quality index (DLQI, or the Skindex) when assessing HS. This system will likely be the basis of most future clinical trials and research studies.

Causes

As this disease
Disease
A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...

 is poorly studied, the causes are controversial and experts disagree.

Hydradenitis suppurativa occurs when apocrine glands become plugged. Lesions occur in areas of the body with numerous apocrine glands such as the axilla, groin, and perianal region. This theory includes most of the following potentials indicators:
  • Post-pubescent individuals are more likely to exhibit HS.
  • Females are more likely than males.
  • Genetic predisposition
    Genetic predisposition
    A genetic predisposition is a genetic affectation which influences the phenotype of an individual organism within a species or population but by definition that phenotype can also be modified by the environmental conditions. In the rest of the population, conditions cannot have that effect...

     among families of Sephardic Jewish, Italian, Greek, Middle Eastern and Northern African ancestry.
  • Research is assessing possible relations with Hashimoto's thyroiditis
    Hashimoto's thyroiditis
    Hashimoto's thyroiditis or chronic lymphocytic thyroiditis is an autoimmune disease in which the thyroid gland is gradually destroyed by a variety of cell- and antibody-mediated immune processes. It was the first disease to be recognized as an autoimmune disease...

    , Crohn's disease
    Crohn's disease
    Crohn's disease, also known as regional enteritis, is a type of inflammatory bowel disease that may affect any part of the gastrointestinal tract from mouth to anus, causing a wide variety of symptoms...

    , rheumatoid arthritis
    Rheumatoid arthritis
    Rheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development...

    , and squamous cell carcinoma
    Squamous cell carcinoma
    Squamous cell carcinoma , occasionally rendered as "squamous-cell carcinoma", is a histologically distinct form of cancer. It arises from the uncontrolled multiplication of malignant cells deriving from epithelium, or showing particular cytological or tissue architectural characteristics of...

    .
  • Plugged apocrine
    Apocrine
    Apocrine is a term used to classify exocrine glands in the study of histology. Cells which are classified as apocrine bud their secretions off through the plasma membrane producing membrane-bound vesicles in the lumen. This method is also called decapitation secretion...

     (sweat) gland or hair follicle
    Hair follicle
    A hair follicle is a skin organ that produces hair. Hair production occurs in phases, including a growth phase , and cessation phase , and a rest phase . Stem cells are principally responsible for the production of hair....

    .
  • Excessive sweating.
  • Bacteria
    Bacteria
    Bacteria are a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria have a wide range of shapes, ranging from spheres to rods and spirals...

    l infection
    Infection
    An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...

    .
  • Sometimes linked with other auto-immune conditions.
  • Androgen
    Androgen
    Androgen, also called androgenic hormone or testoid, is the generic term for any natural or synthetic compound, usually a steroid hormone, that stimulates or controls the development and maintenance of male characteristics in vertebrates by binding to androgen receptors...

     dysfunction.
  • Genetic disorder
    Genetic disorder
    A genetic disorder is an illness caused by abnormalities in genes or chromosomes, especially a condition that is present from before birth. Most genetic disorders are quite rare and affect one person in every several thousands or millions....

    s that alter cell
    Cell (biology)
    The cell is the basic structural and functional unit of all known living organisms. It is the smallest unit of life that is classified as a living thing, and is often called the building block of life. The Alberts text discusses how the "cellular building blocks" move to shape developing embryos....

     structure.
  • Being overweight
    Overweight
    Overweight is generally defined as having more body fat than is optimally healthy. Being overweight is a common condition, especially where food supplies are plentiful and lifestyles are sedentary...

     makes it worse, however this condition is not caused by obesity
    Obesity
    Obesity is a medical condition in which excess body fat has accumulated to the extent that it may have an adverse effect on health, leading to reduced life expectancy and/or increased health problems...

     and weight loss will improve but not cure it. Patients with more advanced cases may find exercise intolerably painful, which may increase the rate of obesity among sufferers.


The historical understanding of the disease is that there are dysfunctional apocrine glands or dysfunctional hair follicle
Hair follicle
A hair follicle is a skin organ that produces hair. Hair production occurs in phases, including a growth phase , and cessation phase , and a rest phase . Stem cells are principally responsible for the production of hair....

s, possibly triggered by a blocked gland, creating inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

, pain
Pain
Pain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...

, and a swollen lesion
Lesion
A lesion is any abnormality in the tissue of an organism , usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.- Types :...

. More recent studies imply there is an autoimmune component.

HS is not caused by any bacterial elements.

Triggering factors

There are a number of triggering factors that should be taken into consideration
Consideration
Consideration is the central concept in the common law of contracts and is required, in most cases, for a contract to be enforceable. Consideration is the price one pays for another's promise. It can take a number of forms: money, property, a promise, the doing of an act, or even refraining from...

, as it is advisable to avoid such triggers.
  • Obesity
    Obesity
    Obesity is a medical condition in which excess body fat has accumulated to the extent that it may have an adverse effect on health, leading to reduced life expectancy and/or increased health problems...

     is an exacerbating rather than a triggering factor, through mechanical irritation
    Irritation
    Irritation or exacerbation, in biology and physiology, is a state of inflammation or painful reaction to allergy or cell-lining damage. A stimulus or agent which induces the state of irritation is an irritant...

    , occlusion
    Occlusion
    Occlusion may refer to:* Occlusion , the manner in which the upper and lower teeth come together when the mouth is closed* Occlusion effect, an audio phenomenon that occurs when one closes the opening into the ear canal and the loudness of low pitched sounds increases* Occlusion miliaria, a skin...

    , and maceration.
  • Tight clothing, and clothing made of heavy, non-breathable materials.
  • Smoking
    Smoking
    Smoking is a practice in which a substance, most commonly tobacco or cannabis, is burned and the smoke is tasted or inhaled. This is primarily practised as a route of administration for recreational drug use, as combustion releases the active substances in drugs such as nicotine and makes them...

     tobacco
    Tobacco
    Tobacco is an agricultural product processed from the leaves of plants in the genus Nicotiana. It can be consumed, used as a pesticide and, in the form of nicotine tartrate, used in some medicines...

     products.
  • Deodorants, depilation products, shaving
    Shaving
    Shaving is the removal of hair, by using a razor or any other kind of bladed implement, to slice it down to the level of the skin. Shaving is most commonly practiced by men to remove their facial hair and by women to remove their leg and underarm hair...

     of the affected area – their association with hidradenitis suppurativa is still an ongoing debate
    Debate
    Debate or debating is a method of interactive and representational argument. Debate is a broader form of argument than logical argument, which only examines consistency from axiom, and factual argument, which only examines what is or isn't the case or rhetoric which is a technique of persuasion...

     amongst researchers.
  • Drugs
    DRUGS
    Destroy Rebuild Until God Shows are an American post-hardcore band formed in 2010. They released their debut self-titled album on February 22, 2011.- Formation :...

    , in particular oral contraceptives (i.e. oral hormonal birth control; "the pill") and lithium
    Lithium
    Lithium is a soft, silver-white metal that belongs to the alkali metal group of chemical elements. It is represented by the symbol Li, and it has the atomic number 3. Under standard conditions it is the lightest metal and the least dense solid element. Like all alkali metals, lithium is highly...

    .
  • Hot and especially humid
    Humidity
    Humidity is a term for the amount of water vapor in the air, and can refer to any one of several measurements of humidity. Formally, humid air is not "moist air" but a mixture of water vapor and other constituents of air, and humidity is defined in terms of the water content of this mixture,...

     climates (dry/arid climates often cause remission).

Predisposing factors

  • Genetic
    Genetics
    Genetics , a discipline of biology, is the science of genes, heredity, and variation in living organisms....

     factors: an autosomal dominant inheritance
    Inheritance
    Inheritance is the practice of passing on property, titles, debts, rights and obligations upon the death of an individual. It has long played an important role in human societies...

     pattern has been postulated.
  • Endocrine factors: sex hormones, principally an excess of androgens, are thought to be involved, although the apocrine glands are not sensitive to these hormones. Women often have outbreaks before menstruation
    Menstruation
    Menstruation is the shedding of the uterine lining . It occurs on a regular basis in sexually reproductive-age females of certain mammal species. This article focuses on human menstruation.-Overview:...

     and post-pregnancy
    Pregnancy
    Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...

    , and the disease
    Disease
    A disease is an abnormal condition affecting the body of an organism. It is often construed to be a medical condition associated with specific symptoms and signs. It may be caused by external factors, such as infectious disease, or it may be caused by internal dysfunctions, such as autoimmune...

     usually remits during pregnancy and after menopause
    Menopause
    Menopause is a term used to describe the permanent cessation of the primary functions of the human ovaries: the ripening and release of ova and the release of hormones that cause both the creation of the uterine lining and the subsequent shedding of the uterine lining...

    .

Severe complications

In disease stage III, fistulas left undiscovered, undiagnosed, or untreated, can lead to the development of squamous cell carcinoma
Squamous cell carcinoma
Squamous cell carcinoma , occasionally rendered as "squamous-cell carcinoma", is a histologically distinct form of cancer. It arises from the uncontrolled multiplication of malignant cells deriving from epithelium, or showing particular cytological or tissue architectural characteristics of...

, a rare cancer
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...

, in the anus
Anus
The anus is an opening at the opposite end of an animal's digestive tract from the mouth. Its function is to control the expulsion of feces, unwanted semi-solid matter produced during digestion, which, depending on the type of animal, may be one or more of: matter which the animal cannot digest,...

 or other affected areas. Other stage III chronic sequelae may also include anemia
Anemia
Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...

, multilocalised infections, amyloidosis
Amyloidosis
In medicine, amyloidosis refers to a variety of conditions whereby the body produces "bad proteins", denoted as amyloid proteins, which are abnormally deposited in organs and/or tissues and cause harm. A protein is described as being amyloid if, due to an alteration in its secondary structure, it...

, and arthropathy
Arthropathy
-Scope:Arthritis is a form of arthropathy that involves inflammation of one or more joints, while the term arthropathy may be used regardless of whether there is inflammation or not.Spondylarthropathy is any form of arthropathy of the vertebral column....

. Stage III complications have been known to lead to death
Death
Death is the permanent termination of the biological functions that sustain a living organism. Phenomena which commonly bring about death include old age, predation, malnutrition, disease, and accidents or trauma resulting in terminal injury....

, but clinical data is still uncertain.

Potential complications

  • Contractures and reduced mobility of the lower limbs and axillae due to fibrosis
    Fibrosis
    Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process. This is as opposed to formation of fibrous tissue as a normal constituent of an organ or tissue...

     and scarring. Severe lymphedema
    Lymphedema
    Lymphedema , also known as lymphatic obstruction, is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system....

     may develop in the lower limbs.
  • Local and systemic infections (meningitis
    Meningitis
    Meningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...

    , bronchitis
    Bronchitis
    Acute bronchitis is an inflammation of the large bronchi in the lungs that is usually caused by viruses or bacteria and may last several days or weeks. Characteristic symptoms include cough, sputum production, and shortness of breath and wheezing related to the obstruction of the inflamed airways...

    , pneumonia
    Pneumonia
    Pneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...

    , etc.), which may even progress to sepsis
    Sepsis
    Sepsis is a potentially deadly medical condition that is characterized by a whole-body inflammatory state and the presence of a known or suspected infection. The body may develop this inflammatory response by the immune system to microbes in the blood, urine, lungs, skin, or other tissues...

    .
  • Interstitial keratitis
    Interstitial keratitis
    Interstitial Keratitis basically means corneal scarring due to chronic inflammation of the corneal stroma. Interstitial means space between cells i.e corneal stroma which lies between the epithelium and the endothelium...

    .
  • Anal, rectal, or urethral fistulas in anogenital hidradenitis
    Hidradenitis
    Hidradenitis is a term used to describe diseases in which the histologic abnormality is primarily an inflammatory infiltrate around the eccrine glands...

     suppurativa.
  • Normochromic or hypochromic anemia
    Anemia
    Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...

    .
  • Squamous cell carcinoma
    Squamous cell carcinoma
    Squamous cell carcinoma , occasionally rendered as "squamous-cell carcinoma", is a histologically distinct form of cancer. It arises from the uncontrolled multiplication of malignant cells deriving from epithelium, or showing particular cytological or tissue architectural characteristics of...

    : this has been found on rare occasions in chronic hidradenitis suppurativa of the anogenital region. The mean time to the onset of this type of lesion
    Lesion
    A lesion is any abnormality in the tissue of an organism , usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.- Types :...

     is 10 years or more and the tumors are usually highly aggressive.
  • Tumors of the lung
    Lung
    The lung is the essential respiration organ in many air-breathing animals, including most tetrapods, a few fish and a few snails. In mammals and the more complex life forms, the two lungs are located near the backbone on either side of the heart...

     and oral cavity, probably related to the high level of smoking
    Smoking
    Smoking is a practice in which a substance, most commonly tobacco or cannabis, is burned and the smoke is tasted or inhaled. This is primarily practised as a route of administration for recreational drug use, as combustion releases the active substances in drugs such as nicotine and makes them...

     among these patients, and liver cancer
    Liver cancer
    Liver tumors or hepatic tumors are tumors or growths on or in the liver . Several distinct types of tumors can develop in the liver because the liver is made up of various cell types. These growths can be benign or malignant...

    .
  • Hypoproteinemia
    Hypoproteinemia
    Hypoproteinemia is a condition where there is an abnormally low level of protein in the blood.One common cause is due to excess protein in the urine , which can be a medical sign of nephrotic syndrome....

     and amyloidosis
    Amyloidosis
    In medicine, amyloidosis refers to a variety of conditions whereby the body produces "bad proteins", denoted as amyloid proteins, which are abnormally deposited in organs and/or tissues and cause harm. A protein is described as being amyloid if, due to an alteration in its secondary structure, it...

    , which can lead to renal failure
    Renal failure
    Renal failure or kidney failure describes a medical condition in which the kidneys fail to adequately filter toxins and waste products from the blood...

     and death
    Death
    Death is the permanent termination of the biological functions that sustain a living organism. Phenomena which commonly bring about death include old age, predation, malnutrition, disease, and accidents or trauma resulting in terminal injury....

    .
  • Seronegative and usually asymmetric arthropathy
    Arthropathy
    -Scope:Arthritis is a form of arthropathy that involves inflammation of one or more joints, while the term arthropathy may be used regardless of whether there is inflammation or not.Spondylarthropathy is any form of arthropathy of the vertebral column....

    : pauciarticular arthritis
    Arthritis
    Arthritis is a form of joint disorder that involves inflammation of one or more joints....

    , polyarthritis
    Polyarthritis
    Polyarthritis is any type of arthritis which involves 5 or more joints simultaneously. It is usually associated with autoimmune conditions.Polyarthritis may be experienced at any age and is not gender specific.-Causes:...

    /polyarthralgia syndrome.

Treatments

Treatments may vary depending upon presentation and severity of the disease. Due to the poorly studied nature of this disease, the effectiveness of the drugs and therapies listed below is not yet clear, and patients should discuss all options with their doctor or dermatologist. Nearly a quarter of patients state that nothing relieves their symptoms. A list of treatments that are possible treatments for some patients is as follows.
  • Lifestyle
    • Changes in diet
      Diet (nutrition)
      In nutrition, diet is the sum of food consumed by a person or other organism. Dietary habits are the habitual decisions an individual or culture makes when choosing what foods to eat. With the word diet, it is often implied the use of specific intake of nutrition for health or weight-management...

       avoiding inflammatory foods, foods high in refined carbohydrates.
    • Warm compresses, hydrotherapy
      Hydrotherapy
      Hydrotherapy, formerly called hydropathy, involves the use of water for pain-relief and treating illness. The term hydrotherapy itself is synonymous with the term water cure as it was originally marketed by practitioners and promoters in the 19th century...

      , balneotherapy
      Balneotherapy
      Balneotherapy is the treatment of disease by bathing, usually practiced at spas. While it is considered distinct from hydrotherapy, there are some overlaps in practice and in underlying principles. Balneotherapy may involve hot or cold water, massage through moving water, relaxation or stimulation...

      .
    • Icing the inflamed area daily until pain reduction is noticed.
    • Weight loss in overweight and obese patients, as well as smoking cessation can improve or even alleviate many symptoms of Hidradenitis suppurativa: Obese and overweight patients should be helped to lose weight in all cases of HS regardless of which stage patient may be in.

  • Medication
    • Antibiotics Taken orally, these are used for their anti-inflammatory properties rather than to treat infection. The most effective is a combination of Rifampicin [300 mg twice a day] and Clindamycin [300 mg twice a day] given concurrently for 2–3 months. This brings about remission in around three quarters of cases. A few popular antibiotics used to treat HS include tetracycline, minocycline, and clindamycin.
    • Corticosteroid
      Corticosteroid
      Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...

       injections. Also known as intralesional steroids: can be particularly useful for localized disease, if the drug can be prevented from escaping via the sinuses.
    • Vitamin A
      Vitamin A
      Vitamin A is a vitamin that is needed by the retina of the eye in the form of a specific metabolite, the light-absorbing molecule retinal, that is necessary for both low-light and color vision...

       supplementation
    • Anti-androgen therapy: hormonal therapy with cyproterone acetate and ethinyloestadiol proved effective in randomized control trials. It is of note that dosages reported have been very high.
    • IV
      Intravenous therapy
      Intravenous therapy or IV therapy is the infusion of liquid substances directly into a vein. The word intravenous simply means "within a vein". Therapies administered intravenously are often called specialty pharmaceuticals...

       or subcutaneous infusion of anti-inflammatory (anti-TNF-alpha) drugs such as infliximab
      Infliximab
      Infliximab is a monoclonal antibody against tumour necrosis factor alpha . It is used to treat autoimmune diseases. Remicade is marketed by Janssen Biotech, Inc...

       (Remicade), etanercept
      Etanercept
      Etanercept is a drug that treats autoimmune diseases by interfering with the tumor necrosis factor by acting as a TNF inhibitor. Pfizer describes in a SEC filing that the drug is used to treat rheumatoid, juvenile rheumatoid and psoriatic arthritis, plaque psoriasis and ankylosing spondylitis...

       (Enbrel), and adalimumab
      Adalimumab
      Adalimumab is the third TNF inhibitor, after infliximab and etanercept, to be approved in the United States. Like infliximab and etanercept, adalimumab binds to TNFα, preventing it from activating TNF receptors; adalimumab was constructed from a fully human monoclonal antibody, while infliximab...

      . This use of the drugs is not currently Food and Drug Administration
      Food and Drug Administration
      The Food and Drug Administration is an agency of the United States Department of Health and Human Services, one of the United States federal executive departments...

       (FDA) approved and is somewhat controversial, and therefore may not be covered by insurance.
    • Zinc gluconate
      Zinc gluconate
      Zinc gluconate is the zinc salt of gluconic acid. It is an ionic compound consisting of two moles of gluconate for each mole of zinc...

       taken orally has been shown to induce remission. Recommended dose is at least 30 mg taken 3 times daily (90 mg/day). Toxicity is known to occur at doses exceeding 1000 mg/day.
    • Chlorhexidine (Hibiclens) plus an antibiotic soap for cleansing the skin surface. Hexachlorophene shower with liquid soap like Phisohex, covering sores with Metrolotion after medicated showers. These are considered to be general measures, and are the foundation of any good medical treatment and management plans for HS.
    • Turmeric
      Turmeric
      Turmeric is a rhizomatous herbaceous perennial plant of the ginger family, Zingiberaceae. It is native to tropical South Asia and needs temperatures between 20 °C and 30 °C and a considerable amount of annual rainfall to thrive...

       capsules orally or through topical application. The active ingredient in the tumeric spice is curcumin.
    • Infliximab
      Infliximab
      Infliximab is a monoclonal antibody against tumour necrosis factor alpha . It is used to treat autoimmune diseases. Remicade is marketed by Janssen Biotech, Inc...

      : a chimeric monoclonal anti-TNF antibody has demonstrated efficacy in treating HS.
    • Topical clindamycin
      Clindamycin
      Clindamycin rINN is a lincosamide antibiotic. It is usually used to treat infections with anaerobic bacteria but can also be used to treat some protozoal diseases, such as malaria...

      has been shown to have an effect in double-blind placebo controlled studies.

  • Surgical therapy

When the process becomes chronic, wide surgical excision is the procedure of choice. Wounds in the affected area do not heal by secondary intention, and immediate application of a split thickness skin graft is more appropriate.
  • Radiation

Electron beam radiotherapy has been a successful treatment of hidradenitis, especially in Europe; it is not a common treatment option in most of the United States, as radiation oncologists generally refuse to treat patients with non-malignant diseases because of the potential for secondary radiation-induced tumors in the long term.

External links

The source of this article is wikipedia, the free encyclopedia.  The text of this article is licensed under the GFDL.
 
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