Acrodermatitis chronica atrophicans
Encyclopedia
Acrodermatitis chronica atrophicans (ACA) (also known as "Herxheimer disease" and "Primary diffuse atrophy") is a skin rash indicative of the third or late stage of European Lyme borreliosis
.
ACA is a dermatological
condition that takes a chronically progressive course and finally leads to a widespread atrophy
of the skin. Involvement of the peripheral nervous system
is often observed, specifically polyneuropathy
.
This progressive skin process is due to the effect of continuing active infection with the spirochete Borrelia afzelii
. B afzelii is the predominant pathophysiology, but may not be the exclusive, etiologic
agent of ACA. Borrelia garinii, has also been detected.
, where a physician named Alfred Buchwald first delineated it.
Herxheimer
and Hartmann described it in 1902 as a "tissue paper" like cutaneous atrophy.
stage with bluish red discoloration and cutaneous swelling
and concluding several months or years later with an atrophic phase. Sclerotic
skin plaques may also develop.
As ACA progresses the skin begins to wrinkle
.
mobility.
The outlook is good if the acute inflammatory stage of ACA is treated adequately. The therapeutic outcome is difficult to assess in patients with the chronic atrophic phase, in which many changes are only partially reversible.
Physicians should use serologic
and histologic
examination to confirm the diagnosis of ACA. Treatment consists of antibiotics including doxycycline
and penicillin
for up to four weeks in the acute case.
Lyme disease
Lyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
.
ACA is a dermatological
Dermatology
Dermatology is the branch of medicine dealing with the skin and its diseases, a unique specialty with both medical and surgical aspects. A dermatologist takes care of diseases, in the widest sense, and some cosmetic problems of the skin, scalp, hair, and nails....
condition that takes a chronically progressive course and finally leads to a widespread atrophy
Atrophy
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations , poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ, disuse or lack of exercise or disease intrinsic to the tissue itself...
of the skin. Involvement of the peripheral nervous system
Peripheral nervous system
The peripheral nervous system consists of the nerves and ganglia outside of the brain and spinal cord. The main function of the PNS is to connect the central nervous system to the limbs and organs. Unlike the CNS, the PNS is not protected by the bone of spine and skull, or by the blood–brain...
is often observed, specifically polyneuropathy
Polyneuropathy
Polyneuropathy is a neurological disorder that occurs when many peripheral nerves throughout the body malfunction simultaneously. It may be acute and appear without warning, or chronic and develop gradually over a longer period of time. Many polyneuropathies have both motor and sensory...
.
This progressive skin process is due to the effect of continuing active infection with the spirochete Borrelia afzelii
Lyme disease microbiology
Lyme disease, or borreliosis, is caused by Gram negative spirochetal bacteria from the genus Borrelia, which has at least 37 known species, 12 of which are Lyme related, and an unknown number of genomic strains...
. B afzelii is the predominant pathophysiology, but may not be the exclusive, etiologic
Etiology
Etiology is the study of causation, or origination. The word is derived from the Greek , aitiologia, "giving a reason for" ....
agent of ACA. Borrelia garinii, has also been detected.
History
The first record of ACA was made in 1883 in Breslau, PolandPoland
Poland , officially the Republic of Poland , is a country in Central Europe bordered by Germany to the west; the Czech Republic and Slovakia to the south; Ukraine, Belarus and Lithuania to the east; and the Baltic Sea and Kaliningrad Oblast, a Russian exclave, to the north...
, where a physician named Alfred Buchwald first delineated it.
Herxheimer
Herxheimer reaction
The Herxheimer reaction resembles bacterial sepsis and can occur after initiation of antibiotic therapy or treatment of tick-borne relapsing fever. An association has been found between the release of heat-stable proteins from spirochetes and the reaction. The same can be true for candida die-off...
and Hartmann described it in 1902 as a "tissue paper" like cutaneous atrophy.
Presentation
The rash caused by ACA is most evident on the extremities or limbs beginning with an inflammatoryInflammation
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...
stage with bluish red discoloration and cutaneous swelling
Swelling (medical)
In medical parlance, swelling is the transient enlargement or protuberance in the body and may include tumors. According to cause, it may be congenital, traumatic, inflammatory, neoplastic or miscellaneous....
and concluding several months or years later with an atrophic phase. Sclerotic
Sclerosis (medicine)
In medicine, sclerosis refers to the stiffening of a structure, usually caused by a replacement of the normal organ-specific tissue with connective tissue.Types include:...
skin plaques may also develop.
As ACA progresses the skin begins to wrinkle
Wrinkle
A wrinkle is a fold, ridge or crease in the skin. Skin wrinkles typically appear as a result of aging processes such as glycation or, temporarily, as the result of prolonged immersion in water. Wrinkling in the skin is caused by habitual facial expressions, aging, sun damage, smoking, poor...
.
Prognosis and Treatment
The course of ACA is long-standing, from a few to several years, and it leads to extensive atrophy of the skin and, in some patients, to the limitation of upper and lower limb jointJoint
A joint is the location at which two or more bones make contact. They are constructed to allow movement and provide mechanical support, and are classified structurally and functionally.-Classification:...
mobility.
The outlook is good if the acute inflammatory stage of ACA is treated adequately. The therapeutic outcome is difficult to assess in patients with the chronic atrophic phase, in which many changes are only partially reversible.
Physicians should use serologic
Serology
Serology is the scientific study of blood serum and other bodily fluids. In practice, the term usually refers to the diagnostic identification of antibodies in the serum...
and histologic
Histology
Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...
examination to confirm the diagnosis of ACA. Treatment consists of antibiotics including doxycycline
Doxycycline
Doxycycline INN is a member of the tetracycline antibiotics group, and is commonly used to treat a variety of infections. Doxycycline is a semisynthetic tetracycline invented and clinically developed in the early 1960s by Pfizer Inc. and marketed under the brand name Vibramycin. Vibramycin...
and penicillin
Penicillin
Penicillin is a group of antibiotics derived from Penicillium fungi. They include penicillin G, procaine penicillin, benzathine penicillin, and penicillin V....
for up to four weeks in the acute case.
See also
- Lyme DiseaseLyme diseaseLyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
- Erythema MigransErythema migransErythema chronicum migrans refers to the rash often seen in the early stage of Lyme disease. It can appear anywhere from one day to one month after a tick bite. This rash does not represent an allergic reaction to the bite, but rather an actual skin infection with the Lyme bacteria, Borrelia...
- List of cutaneous conditions