Poikiloderma vasculare atrophicans
Encyclopedia
Poikiloderma vasculare atrophicans (PVA), sometimes referred to as parapsoriasis variegata or parapsoriasis lichenoides is a cutaneous condition (skin disease) characterized by hypo- or hyperpigmentation (diminished or heightened skin pigment
ation, respectively), telangiectasia
and skin atrophy. Other names for the condition include prereticulotic poikiloderma and atrophic parapsoriasis. The condition was first described by pioneer American pediatrician Abraham Jacobi
in 1906. PVA causes areas of affected skin to appear speckled red and inflamed, yellowish and/or brown, gray or grayish-black, with scaling and a thinness that may be described as "cigarette paper". On the surface of the skin, these areas may range in size from small patches, to plaques (larger, raised areas), to neoplasms (spreading, tumor-like growths on the skin).
Mycosis fungoides, a type of skin lymphoma
, may be a cause of PVA. The condition may also be caused by, associated with or accompany any of the following conditions or disorders: other skin lymphoma
s, dermatomyositis
, lupus erythematosus
, Rothmund-Thompson syndrome, Kindler syndrome
, dyskeratosis congenita
, and chronic radiodermatitis
. Rare causes include arsenic
ingestion, and the condition can also be idiopathic
.
PVA may be considered a rare variant of cutaneous T-cell lymphoma, a non-Hodgkin's
form of lymphoma
affecting the skin. It may also be included among a number of similar conditions that are considered as precursors to mycosis fungoides
. PVA is believed to be a syndrome closely associated with large-plaque parapsoriasis
and its cohort retiform parapsoriasis
; including PVA, all three conditions fit within an updated view of the once ambiguous classification scheme known as parapsoriasis
.
") is occurring, associated with heightened visibity of capillaries
("vasculare", referring to telangiectasia) under the skin, related to thinning and wasting away ("atrophicans") of the skin and its tissue
. Telangiectasia is an enlargement of capillaries underneath the skin.
PVA also has common names that include parapsoriasis-related terminology (i.e. parapsoriasis variagata, or "variegated" parapsoriasis). Parapsoriasis is a term first used by Brocq in 1902, intended to represent a group comprising a number of uncommon skin disorders, under a once used, now antiquated classification scheme for all inflammatory dermatoses (skin diseases known to be associated with or cause inflammation). Brocq chose the term "parapsoriasis" to illustrate that the dermatoses placed in this group had or would have commonalities with psoriasiasis, including appearance and chronicity (lifelong or indefinite duration). This poorly designated grouping has led to confusion in establishing a nosology (a method of classifying diseases and disorders) that associated or distinguished these disorders, and through the years differing opinions and uses regarding parapsoriasis by both authors and physicians has caused further confusion. In more recent times, after much discussion and growing consensus, parapsoriasis and its terminology has been revisited and re-examined often. Newer thought on parapsoriasis, such as by Sutton (1956) all the way to that by Sehgal, et al. (2007) has cleared much of the confusion and has sparked increased understanding of parapsoriasis and its constituents.
PVA fits within this updated view of parapsoriasis as a syndrome often associated with large plaque parapsoriasis and, or including its variant form, retiform parapsoriasis. Additionally, it may be considered a precursor or variant of the lymphomatous skin disorder mycosis fungoides, which is also associated with large plaque parapsoriasis. Large plaque parapsoriasis consists of inflamed, oddly discolored (such as yellow or blue), web-patterned and scaling plaques on the skin, 10 cm (3.9 in) or larger in diameter. When the condition of the skin encompassed by these plaques worsens and becomes atrophic, it is typically considered retiform parapsoriasis. PVA can occur in either the large plaque or retiform stage, but it can only be considered PVA when its three constituents (poikiloderma, telangiectasia, atrophy) are present. PVA is therefore considered an independent syndrome identified by its constituents, wherever it occurs.
In modern consideration and usage, the solitary term "poikiloderma" has also come to represent all three elements of PVA. When skin diseases and disorders or skin conditions described as dermatoses contain the term poikiloderma in their assessment or diagnosis (such as with Bloom syndrome
), this can sometimes be an erroneous usage of the term. Discretion has been advised. Usage of the entire term "poikiloderma vasculare atrophicans" may also be reserved to indicate it as the primary condition affecting the skin in cases where the disorder associated with it is secondary.
from melanocyte
s into the dermal
skin layer below. Inflammation of the skin and cutaneous tissue, common with PVA, also contributes to color changes in the skin, typified by redness. Telangiectasia, the visible "vascular"
element of PVA, is the dilation of small blood vessels near the skin surface. Skin atrophy, a wasting-away of the tissue comprising the skin, is a prominent part of PVA and effects the dermal, and particularly the epidermal layer. This, in part, is the result of degenerative liquefaction of the stratum basale
(bottom cell-layer) of the epidermis. Atrophy of the skin gives it a thin, dry and wrinkled appearance, which in PVA-affected individuals has been described as "cigarette paper". Hyperkeratosis
, a thickening of the stratum corneum
(top cell-layer of the epidermis), has also been reported.
(an inflammatory disorder of the skin and muscle) are other common causes of PVA. Less common causes include autoimmune-related connective tissue disease
s such as lupus
and scleroderma
. Dermatoses and those that are genetically inspired, called genodermatoses
, may also be an underlying cause of PVA. Among them, xeroderma pigmentosum
and Rothmund-Thomson syndrome
(poikiloderma congenita) are thought to be the most prominent. Ingestion of substances containing arsenic
, such as arsphenamine
, has also been suggested as a least common cause. PVA can also be idiopathic
(of unknown cause), as seen in a small number of cases.
Pigment
A pigment is a material that changes the color of reflected or transmitted light as the result of wavelength-selective absorption. This physical process differs from fluorescence, phosphorescence, and other forms of luminescence, in which a material emits light.Many materials selectively absorb...
ation, respectively), telangiectasia
Telangiectasia
Telangiectasias /tɛ.læn.dʒiː'ɛk.teɪ.ʃi:ə/ are small dilated blood vessels near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. They can develop anywhere on the body but are commonly seen on the face around the nose, cheeks, and chin...
and skin atrophy. Other names for the condition include prereticulotic poikiloderma and atrophic parapsoriasis. The condition was first described by pioneer American pediatrician Abraham Jacobi
Abraham Jacobi
Abraham Jacobi was a pioneer of pediatrics, opening the first children's clinic in the United States. To date, he is the only foreign born president of the American Medical Association.-Biography:...
in 1906. PVA causes areas of affected skin to appear speckled red and inflamed, yellowish and/or brown, gray or grayish-black, with scaling and a thinness that may be described as "cigarette paper". On the surface of the skin, these areas may range in size from small patches, to plaques (larger, raised areas), to neoplasms (spreading, tumor-like growths on the skin).
Mycosis fungoides, a type of skin lymphoma
Lymphoma
Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
, may be a cause of PVA. The condition may also be caused by, associated with or accompany any of the following conditions or disorders: other skin lymphoma
Lymphoma
Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
s, dermatomyositis
Dermatomyositis
Dermatomyositis is a connective-tissue disease related to polymyositis and Bramaticosis that is characterized by inflammation of the muscles and the skin.- Causes :...
, lupus erythematosus
Lupus erythematosus
Lupus erythematosus is a category for a collection of diseases with similar underlying problems with immunity . Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs...
, Rothmund-Thompson syndrome, Kindler syndrome
Kindler syndrome
Kindler syndrome is a rare congenital...
, dyskeratosis congenita
Dyskeratosis congenita
Dyskeratosis congenita , also called Zinsser-Cole-Engman syndrome, is a rare progressive congenital disorder which results in what in some ways resembles premature aging...
, and chronic radiodermatitis
Chronic radiodermatitis
Chronic radiodermatitis occurs with chronic exposure to "sub-erythema" doses of ionizing radiation over a prolonged period, producing varying degrees of damage to the skin and its underlying parts after a variable latent period of several months to several decades...
. Rare causes include arsenic
Arsenic
Arsenic is a chemical element with the symbol As, atomic number 33 and relative atomic mass 74.92. Arsenic occurs in many minerals, usually in conjunction with sulfur and metals, and also as a pure elemental crystal. It was first documented by Albertus Magnus in 1250.Arsenic is a metalloid...
ingestion, and the condition can also be idiopathic
Idiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
.
PVA may be considered a rare variant of cutaneous T-cell lymphoma, a non-Hodgkin's
Non-Hodgkin lymphoma
The non-Hodgkin lymphomas are a diverse group of blood cancers that include any kind of lymphoma except Hodgkin's lymphomas. Types of NHL vary significantly in their severity, from indolent to very aggressive....
form of lymphoma
Lymphoma
Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
affecting the skin. It may also be included among a number of similar conditions that are considered as precursors to mycosis fungoides
Mycosis fungoides
-External links:* * *...
. PVA is believed to be a syndrome closely associated with large-plaque parapsoriasis
Large-plaque parapsoriasis
Large plaque parapsoriasis are skin lesions that may be included in the modern scheme of cutaneous conditions described as parapsoriasis. These lesions, called plaques, may be irregularly round-shaped to oval and are or larger in diameter. They can be very thin plaques that are symptomatic or...
and its cohort retiform parapsoriasis
Retiform parapsoriasis
Retiform parapsoriasis is a cutaneous condition, considered to be a type of large-plaque parapsoriasis. It is characterized by widespread, ill-defined plaques on the skin, that have a net-like or zebra-striped pattern...
; including PVA, all three conditions fit within an updated view of the once ambiguous classification scheme known as parapsoriasis
Parapsoriasis
Parapsoriasis refers to one of a group of skin disorders that are characterized primarily by their resemblance to psoriasis , rather than by their underlying etiology.Neoplasms can develop from parapsoriasis...
.
Classification
Poikiloderma vasculare atrophicans, or PVA, indicates that extra or altered skin pigmentation ("poikilodermaPoikiloderma
Poikiloderma is a skin condition that "consists of areas of increased and decreased pigmentation, prominent blood vessels, and thinning of the skin."...
") is occurring, associated with heightened visibity of capillaries
Capillary
Capillaries are the smallest of a body's blood vessels and are parts of the microcirculation. They are only 1 cell thick. These microvessels, measuring 5-10 μm in diameter, connect arterioles and venules, and enable the exchange of water, oxygen, carbon dioxide, and many other nutrient and waste...
("vasculare", referring to telangiectasia) under the skin, related to thinning and wasting away ("atrophicans") of the skin and its 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...
. Telangiectasia is an enlargement of capillaries underneath the skin.
PVA also has common names that include parapsoriasis-related terminology (i.e. parapsoriasis variagata, or "variegated" parapsoriasis). Parapsoriasis is a term first used by Brocq in 1902, intended to represent a group comprising a number of uncommon skin disorders, under a once used, now antiquated classification scheme for all inflammatory dermatoses (skin diseases known to be associated with or cause inflammation). Brocq chose the term "parapsoriasis" to illustrate that the dermatoses placed in this group had or would have commonalities with psoriasiasis, including appearance and chronicity (lifelong or indefinite duration). This poorly designated grouping has led to confusion in establishing a nosology (a method of classifying diseases and disorders) that associated or distinguished these disorders, and through the years differing opinions and uses regarding parapsoriasis by both authors and physicians has caused further confusion. In more recent times, after much discussion and growing consensus, parapsoriasis and its terminology has been revisited and re-examined often. Newer thought on parapsoriasis, such as by Sutton (1956) all the way to that by Sehgal, et al. (2007) has cleared much of the confusion and has sparked increased understanding of parapsoriasis and its constituents.
PVA fits within this updated view of parapsoriasis as a syndrome often associated with large plaque parapsoriasis and, or including its variant form, retiform parapsoriasis. Additionally, it may be considered a precursor or variant of the lymphomatous skin disorder mycosis fungoides, which is also associated with large plaque parapsoriasis. Large plaque parapsoriasis consists of inflamed, oddly discolored (such as yellow or blue), web-patterned and scaling plaques on the skin, 10 cm (3.9 in) or larger in diameter. When the condition of the skin encompassed by these plaques worsens and becomes atrophic, it is typically considered retiform parapsoriasis. PVA can occur in either the large plaque or retiform stage, but it can only be considered PVA when its three constituents (poikiloderma, telangiectasia, atrophy) are present. PVA is therefore considered an independent syndrome identified by its constituents, wherever it occurs.
In modern consideration and usage, the solitary term "poikiloderma" has also come to represent all three elements of PVA. When skin diseases and disorders or skin conditions described as dermatoses contain the term poikiloderma in their assessment or diagnosis (such as with Bloom syndrome
Bloom syndrome
Bloom's syndrome , also known as Bloom–Torre–Machacek syndrome, is a rare autosomal recessive chromosomal disorder characterized by a high frequency of breaks and rearrangements in an affected person's chromosomes. The condition was discovered and first described by dermatologist Dr...
), this can sometimes be an erroneous usage of the term. Discretion has been advised. Usage of the entire term "poikiloderma vasculare atrophicans" may also be reserved to indicate it as the primary condition affecting the skin in cases where the disorder associated with it is secondary.
Characteristics
PVA can be characterized by speckled, combined hyper- and hypopigmentation in the plaques or patches of affected skin. Hyperpigmentation is excess coloration, or darkening of the skin, while hypopigmentation is a diminished or palid coloring to the skin. Pigmentation changes in PVA, apparent in the epidermal (outermost) skin layer, may be attributed to incontinence (leaking out) of melaninMelanin
Melanin is a pigment that is ubiquitous in nature, being found in most organisms . In animals melanin pigments are derivatives of the amino acid tyrosine. The most common form of biological melanin is eumelanin, a brown-black polymer of dihydroxyindole carboxylic acids, and their reduced forms...
from melanocyte
Melanocyte
-External links: - "Eye: fovea, RPE" - "Integument: pigmented skin"...
s into the dermal
Dermis
The dermis is a layer of skin between the epidermis and subcutaneous tissues, and is composed of two layers, the papillary and reticular dermis...
skin layer below. Inflammation of the skin and cutaneous tissue, common with PVA, also contributes to color changes in the skin, typified by redness. Telangiectasia, the visible "vascular"
Circulatory system
The circulatory system is an organ system that passes nutrients , gases, hormones, blood cells, etc...
element of PVA, is the dilation of small blood vessels near the skin surface. Skin atrophy, a wasting-away of the tissue comprising the skin, is a prominent part of PVA and effects the dermal, and particularly the epidermal layer. This, in part, is the result of degenerative liquefaction of the stratum basale
Stratum germinativum
The stratum basale is the deepest layer of the five layers of the epidermis, which is the outer covering of skin in mammals. The stratum basale is a continuous layer of cells...
(bottom cell-layer) of the epidermis. Atrophy of the skin gives it a thin, dry and wrinkled appearance, which in PVA-affected individuals has been described as "cigarette paper". 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...
, a thickening of the stratum corneum
Stratum corneum
The stratum corneum is the outermost layer of the epidermis, consisting of dead cells that lack nuclei and organelles. The purpose of the stratum corneum is to form a barrier to protect underlying tissue from infection, dehydration, chemicals and mechanical stress...
(top cell-layer of the epidermis), has also been reported.
Cause
PVA usually always has an underlying cause, attributed to existing skin diseases and disorders associated with a cutaneous lymphoma or inflammation. Mycosis fungoides is the common lymphoma believed to cause PVA, although it may be considered a precursor when the lymphoma is occult (hidden) and undiagnosed. Large plaque parapsoriasis and dermatomyositisDermatomyositis
Dermatomyositis is a connective-tissue disease related to polymyositis and Bramaticosis that is characterized by inflammation of the muscles and the skin.- Causes :...
(an inflammatory disorder of the skin and muscle) are other common causes of PVA. Less common causes include autoimmune-related connective tissue disease
Connective tissue disease
A connective tissue disease is any disease that has the connective tissues of the body as a target of pathology. Connective tissue is any type of biological tissue with an extensive extracellular matrix that supports, binds together, and protects organs...
s such as lupus
Lupus erythematosus
Lupus erythematosus is a category for a collection of diseases with similar underlying problems with immunity . Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs...
and scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
. Dermatoses and those that are genetically inspired, called genodermatoses
Genodermatosis
Genodermatoses are inherited genetic skin conditions often grouped into three categories: chromosomal, single gene, and polygenetic....
, may also be an underlying cause of PVA. Among them, xeroderma pigmentosum
Xeroderma pigmentosum
Xeroderma pigmentosum, or XP, is an autosomal recessive genetic disorder of DNA repair in which the ability to repair damage caused by ultraviolet light is deficient. In extreme cases, all exposure to sunlight must be forbidden, no matter how small. Multiple basal cell carcinomas and other skin...
and Rothmund-Thomson syndrome
Rothmund-Thomson syndrome
Rothmund–Thomson syndrome , also known as poikiloderma atrophicans with cataract or poikiloderma congenitale, is a rare autosomal recessive skin condition originally described by August von Rothmund in 1868. Matthew Sydney Thomson published further descriptions in 1936.There have been several...
(poikiloderma congenita) are thought to be the most prominent. Ingestion of substances containing arsenic
Arsenic
Arsenic is a chemical element with the symbol As, atomic number 33 and relative atomic mass 74.92. Arsenic occurs in many minerals, usually in conjunction with sulfur and metals, and also as a pure elemental crystal. It was first documented by Albertus Magnus in 1250.Arsenic is a metalloid...
, such as arsphenamine
Arsphenamine
Arsphenamine, also known as Salvarsan and 606, is a drug that was used beginning in the 1910s to treat syphilis and trypanosomiasis...
, has also been suggested as a least common cause. PVA can also be idiopathic
Idiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
(of unknown cause), as seen in a small number of cases.