Acrodermatitis chronica atrophicans

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2] ; Raviteja Guddeti, M.B.B.S. [3]

First record of acrodermatitis chronica atrophicans was made in 1883 in Breslau, Germany, where a physician named Alfred Buchwald first delineated it. Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis with Borrelia afzelii known as the most predominant responsible microorganism. Transmission of this infection probably occur via ixodes tick (such as Ixodes ricinus), mosquito and horsefly bite. These vectors themselves get infected by feeding on an infected animal reservoir. Immune reaction against borrelia leads to infiltration of CD3+ and CD4+ cells in the dermis. Some conditions such as lymphocytic meningoradiculitis, lichen sclerosus et atrophicus, morphea and other tick borne diseases have been associated with acrodermatitis chronica atrophicans. Thinning of skin, visible veins, swelling and wrinkles are some of the features can be noticed on gross pathology. Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers. Acrodermatitis chronica atrophicans must be differentiated from chronic venous insufficiency, chronic arterial insufficiency, superficial thrombophlebitis, frostbite, morphea, and granuloma annulare. Acrodermatitis chronica atrophicans is a rare disease. The prevalence of acrodermatitis chronica atrophicans is estimated to include 10% of cases with lyme disease in Europe. The incidence of acrodermatitis chronica atrophicans increases with age. Acrodermatitis chronica atrophicans affects women more than men and the majority of acrodermatitis chronica atrophicans cases are reported in northern, central and eastern Europe. Common risk factors in the development of acrodermatitis chronica atrophicans include tick exposure, female gender and residents of northern, central and eastern Europe. The course of acrodermatitis chronica atrophicans is chronic and could lasts for several years and it can progress slowly overtime. In first phase (the inflammatory phase) skin changes appear as blue and red discoloration with boggy infiltration. These inflammatory skin lesions can become atrophic without treatment (atrophic phase). Superimposed bacterial infection, sclerotic skin changes, malignancies, arthropathy and peripheral neuropathy are some of the common complications of acrodermatitis chronica atrophicansis. The general pognosis is good with proper and rapid treatment in acute inflammatory stage of acrodermatitis chronica atrophicans, nevertheless late treatment can cause some irreversible changes. Skin examination of acrodermatitis chronica atrophicans‘s patients include blue, red or brown discoloration, hypopigmentation, indurated plaques and wrinkles. High anti-spirochetal antibody levels (such as IgG, IgM and IgA) has been detected at indirect immunofluorescence and enzyme linked immunosorbent assay (ELISA). Antibiotic therapy is recommended in patients with acrodermatitis chronica atrophicans. Up to four weeks treatment with antibiotics such as amoxicillin, doxycycline, ceftriaxone, cefotaxime and penicillin G has been recommended for acrodermatitis chronica atrophicans‘s treatment. Since transmission of borrelia infection occurs by ticks, mosquitos and horse flies bites, primary prevention could be achieved by bite avoidance.

• The First record of acrodermatitis chronica atrophicans was made in 1883 in Breslau, Germany, where a physician named Alfred Buchwald first delineated it.^[1]
• Later in 1902 Herxheimer and Hartmann described it as a “tissue paper” like cutaneous atrophy and there were first physicians that came up with acrodermatitis chronica atrophicans‘s name. They described the biphasic manner of this disease by demonstrating both inflammatory and atrophic phases of it.^[2]
• In 1950s the possibility of human to human transmission was discussed. For the first time in 1984, borrelia was discovered as the responsible etiology of acrodermatitis chronica atrophicans.

• Acrodermatitis chronica atrophicans is one of the tertiary presentations of European Lyme borreliosis with Borrelia afzelii known as the most predominant responsible microorganism.
• Nevertheless other borrelia species such as borrelia garinii and borrelia burgdorferi (B. burgdorferi sensu lato) have been also detected in acrodermatitis chronica atrophicans patients.
• Transmission of this infection probably occur via ixodes tick (such as Ixodes ricinus), mosquito and horsefly bite. These vectors themselves get infected by feeding on an infected animal reservoir.
• Development of various symptoms in this disease is a result of chronic T cell mediated reaction of immune system against borrelia.^[3]
• This immune reaction leads to infiltration of CD3+ and CD4+ cells in the dermis. Borrelia is capable of attaching to the extracellular matrix proteins (such as glycosaminoglycan, fibronectin and decorin proteoglycan) which eventually leads to metalloproteases activation and extracellular matrix degradation. Pro-inflammatory cytokines, such as tumor necrosis factor alpha and interleukin-4, have been detected in skin biopsies.^[4]
• There is no known gene responsible in pathophysiology of acrodermatitis chronica atrophicans disease. Some conditions such as lymphocytic meningoradiculitis, lichen sclerosus et atrophicus, morphea and other tick borne diseases have been associated with acrodermatitis chronica atrophicans. Thinning of skin, visible veins, swelling and wrinkles are some of the features can be noticed on gross pathology.
• Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers. Thinning of dermis and epidermis, pigmented stratum germinativum, dermal blood vessels dilation and perivascular plasma cell infiltration are some of the findings on microscopic pathology.^[5]

• This progressive skin disorder is due to the effect of chronic infection with the spirochete borrelia afzelii, which is the predominant cause of acrodermatitis chronica atrophicans.
• However borrelia afzelii is not the exclusive etiologic agent of acrodermatitis chronica atrophicans and other microorganisms such as borrelia garinii and borrelia burgdorferi have also been detected.

• Acrodermatitis chronica atrophicans must be differentiated from chronic venous insufficiency, chronic arterial insufficiency, superficial thrombophlebitis, frostbite, morphea, erysipelas, acrocyanosis and granuloma annulare.

• Acrodermatitis chronica atrophicans is a rare disease. The prevalence of acrodermatitis chronica atrophicans is estimated to include 10% of cases with lyme disease in Europe.^[6][7]
• The incidence of acrodermatitis chronica atrophicans increases with age and commonly affects individuals in range of 40 to 70 years old with a median of 64 years old. However there are few case reports on children who are diagnosed with acrodermatitis chronica atrophicans.
• Acrodermatitis chronica atrophicans affects women more than men.
• The majority of acrodermatitis chronica atrophicans cases are reported in northern, central and eastern Europe (most commonly in countries bordering the Baltic Sea). Lately few cases of acrodermatitis chronica atrophicans have been reported in the United States and Canada.^[8]

• Common risk factors in the development of acrodermatitis chronica atrophicans include:^[9]
  • Tick exposure
  • Female gender and residents of northern, central and eastern Europe.

• The course of acrodermatitis chronica atrophicans is chronic and could last for several years and it can progress slowly over time. It has been estimated that mean duration of acrodermatitis chronica atrophicans before diagnosis is approximately 12 months, based on a study. It usually start on one extremity and can spread and involve extensor surfaces of the acral regions of limbs^[10].
• Acrodermatitis chronica atrophicans has a biphasic manner. In the first phase (the inflammatory phase) skin changes appear as blue and red discoloration with boggy infiltration. These inflammatory skin lesions can become atrophic without treatment (atrophic phase).
• Based on two studies, 55% and 66% of patients with acrodermatitis chronica atrophicans have at least one history of tick bite, while others may never remember such an accident. One fifth of patients in a study experienced erythema migrans 6 months to 8 years before acrodermatitis chronica atrophicans development.
• Superimposed bacterial infection, sclerotic skin changes, malignancies, arthropathy and peripheral neuropathy are some of the common complications of acrodermatitis chronica atrophicansis. In contrast to other skin manifestations of borrelia infection, acrodermatitis chronica atrophicans doesn’t heal without treatment and can lead to extensive atrophy of skin and limitations of upper and lower limb joint mobility.
• The general pognosis is good with proper and rapid treatment in acute inflammatory stage of acrodermatitis chronica atrophicans. Nevertheless late treatment can cause some irreversible changes.^[11]

• History of tick bite, erythema migrans or other symptoms of lyme disease, and rheumatological symptoms have been presented in patients with acrodermatitis chronica atrophicans. Since there could be several years between the tick bite and development of skin lesions, absence of tick bite in patients‘ history never exclude the diagnosis.
• Symptoms and different forms of skin involvement in acrodermatitis chronica atrophicans are dependent to duration of the disease. Symptoms, such as sclerotic skin changes, pain and burning, edema and constitutional symptoms have been observed in acrodermatitis chronica atrophicans.^[12]
• Half of patients with acrodermatitis chronica atrophicans experience symptoms of peripheral neuropathy, such as paresthesia and hypesthesia. Symptoms of peripheral neuropathy can occur at the exact site of acrodermatitis chronica atrophicans‘s lesion or at other sites.^[13]
• Involvement of lower limb is more common compared to the upper limb. In some cases episodic knee joint effusion has been observed.

• Skin examination of acrodermatitis chronica atrophicans‘s patients include blue, red or brown discoloration, hypopigmentation, indurated plaques and wrinkles, thinning and shining of involved skin.^[14]
• Readily visible veins, edema, ulcers and peeling are usually found. Although the most common location of these skin changes are observed on limbs, there are some cases with facial and abdominal involvement.
• Peripheral neuropathy develops in 50% of patients. Physical examination of some patients may reveal ulnar bands. Moreover fibrotic nodules could be seen on bony prominences, such as tibia or ulna.

• High anti-spirochetal antibody levels (such as IgG, IgM and IgA) has been detected at indirect immunofluorescence and enzyme linked immunosorbent assay (ELISA).^[15]
• Among various antigens in borrelia burgdorferi, flagellum antigen is one of the recommended serologic evaluation in acrodermatitis chronica atrophicans patients. Diagnosis of acrodermatitis chronica atrophicans can be excluded if the serologic evaluataion is negative.^[16][17]
• Borrelia itself has been found in some of the skin samples. When clinical presentations are not clear enough, biopsy and histological evaluation can assist. Findings such as plasma cells, histiocytes and lymphocytic infiltration plus telangiectasia and thinning of dermis and epidermis are commonly found in skin biopsies.^[18]

• There are no other diagnostic studies associated with acrodermatitis chronica atrophicans.

• Antibiotic therapy is recommended in patients with acrodermatitis chronica atrophicans.
• Up to four weeks treatment with antibiotics such as amoxicillin, doxycycline, ceftriaxone, cefotaxime and penicillin G has been recommended for acrodermatitis chronica atrophicans‘s treatment.
  • Preferred regimen (1): Amoxicillin 500 to 1000 mg three times daily for 14 to 28 days^[19][20]
  • Preferred regimen (2): Doxycycline 100 mg twice daily or 200 mg once daily for 14 to 28 days
  • Preferred regimen (3): Ceftriaxone (IV) 2000 mg every 24 hours for 14 to 28 days
  • Preferred regimen (4): Cefotaxime (IV) 2000 mg every 8 hours for 14 to 28 days
  • Preferred regimen (5): Penicillin G (IV) 3 to 4 MU every 4 hours for 14 to 28 days

• Since transmission of borrelia infection occurs by ticks, mosquitos and horse flies bites, primary prevention could be achieved by bite avoidance.
• Instructions such as using insect repellants, avoiding tick-infested regions or wearing long sleeves and pants when necessary can help.

• Proper antibiotic treatment of a patient who has been diagnosed with lyme disease can reduce the probability of acrodermatitis chronica atrophicans.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2] Raviteja Guddeti, M.B.B.S. [3]

In 1883, acrodermatitis chronica atrophicans was described for the first time by a German physician named Alfred Buchwald. In 1902 two physicians, called Herxheimer and Hartmann, described the condition as a “tissue paper” like cutaneous atrophy and the current name was suggested. They described the biphasic manner of this disease by demonstrating both inflammatory and atrophic phases of it. In 1950s the possibility of human to human transmission was discussed.For the first time in 1984, borrelia was discovered as the responsible etiology of acrodermatitis chronica atrophicans.

• In 1883, acrodermatitis chronica atrophicans was described for the first time by a German physician named Alfred Buchwald.
• In 1895, Pick reported the inflammatory phase as a new disease and he named it eythromelie.^[1]
• In 1902, two physicians, called Herxheimer and Hartmann, described the condition as a “tissue paper” like cutaneous atrophy and the current name was suggested..^[1]
• In 1946, penicillin has been recommended as an effective treatment.
• The possibility of human to human transmission was first discovered in 1950s.
• For the first time in 1984, borrelia was discovered as the responsible etiology of acrodermatitis chronica atrophicans. Before that conditions such as elastin weakness, trophic disturbances or vascular diseases were believed to be the etiologies of acrodermatitis chronica atrophicans. ^[2][1]
• First case of acrodermatitis chronica atrophicans in North America was reported in 1895.^[3]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2] Raviteja Guddeti, M.B.B.S. [3]

Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis. Borrelia afzelii is known as the most predominant responsible microorganism. Nevertheless, other borrelia species such as borrelia garinii and borrelia burgdorferi (B. burgdorferi sensu lato) have been also detected in acrodermatitis chronica atrophicans patients. Transmission of this infection probably occur via ixodes tick (such as Ixodes ricinus), mosquito and horsefly bite. These vectors themselves get infected by feeding on an infected animal reservoir. Development of various symptoms in this disease is a result of chronic T cell mediated reaction of immune system against borrelia. This immune reaction leads to infiltration of CD3+ and CD4+ cells in the dermis. Borrelia is capable of attaching to the extracellular matrix proteins (such as glycosaminoglycan, fibronectin and decorin proteoglycan) which eventually leads to metalloproteases activation and extracellular matrix degradation. Pro-inflammatory cytokines, such as tumor necrosis factor alpha and interleukin-4, have been detected in skin biopsies. There is no known gene responsible in pathophysiology of acrodermatitis chronica atrophicans disease. Some conditions such as lymphocytic meningoradiculitis, lichen sclerosus et atrophicus, morphea and other tick borne diseases have been associated with acrodermatitis chronica atrophicans. Thinning of skin, visible veins, swelling and wrinkles are some of the features that can be noticed on gross pathology. Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers. Thinning of dermis and epidermis, pigmented stratum germinativum, dermal blood vessels dilation and perivascular plasma cell infiltration are some of the findings on microscopic pathology.

• Acrodermatitis chronica atrophicans is one of the tertiary presentations of European lyme borreliosis.^[1][2]
• This progressive skin process is due to the effect of chronic infection with the spirochete borrelia burgdorferi. Borrelia afzelii is the predominant pathophysiology, but may not be the exclusive, etiology of acrodermatitis chronica atrophicans.^{[3][4][5][6][1][2]}
• Borrelia garinii, borrelia afzelii and borrelia burgdorferi (B. burgdorferi sensu lato), are all responsible and have been detected in acrodermatitis chronica atrophicans patients.^[7][8]
• Based on numerous studies, majority of skin biopsies from acrodermatitis chronica atrophicans patients demonstrated borrelia afzelii.^[8][7]
• Transmission of this infection probably occur via ixodes tick (such as Ixodes ricinus), mosquito and horsefly bite. These vectors themselves get infected by feeding on an infected animal reservoir.^[9][3]
• Acrodermatitis chronica atrophicans development is the result of chronic T cell mediated reaction of immune system against borrelia. This immune reaction leads to infiltration of CD3+ and CD4+ cells in the dermis.^[9]
• Borrelia is capable of attaching to the extracellular matrix proteins (such as glycosaminoglycan, fibronectin and decorin proteoglycan) which eventually leads to metalloproteases activation. When metalloproteases activate they degrade extracellular matrix.^[9][10]
• High affinity of borrelia to collagen fibers explains how damage of connective tissue, fibrosis and dermal atrophy occur.^[9][11][12]
• Fibrosis and collagen accumulation lead to formation of band-like hardness on extremities and may cause joint movement reduction as a consequent.^[12]
• Based on a study which included acrodermatitis chronica atrophicans patients, expression of pro-inflammatory cytokines (such as tumor necrosis factor alpha and interleukin-4) have been detected in skin biopsies. In contrast to skin biopsies of erythema migrans which demonstrate high expression of interferon-gamma, skin biopsies of acrodermatitis chronica atrophicans lack this interferon. Since presence of interferon-gamma is related to control of the spirochetal infection, it’s absence in acrodermatitis chronica atrophicans could explain the chronic manner of this disorder.^[13]

There is no known gene responsible in pathophysiology of acrodermatitis chronica atrophicans disease.

Conditions associated include:^{[14][4][15][16][17]}

• Lymphocytic meningoradiculitis:
  • Also known as bannwarth syndrome.
  • Lymphocytic meningoradiculitis is a neurological disease which is also due to Borrelia burgdorferi infection and subsequent lyme disease.
  • Symptoms such as radicular pain in cervical or lumbar regions and cranial nerve palsy (such as facial palsy) are common among patients.
• Lichen sclerosus et atrophicus
  • Also called Lichen sclerosus.
  • It appears as scleroderma-like skin lesions.
  • It has been reported in 12% patients of a study done on 50 patients with acrodermatitis chronica atrophicans.
• Morphea
• Other tick borne diseases

• In the atrophy phase of the acrodermatitis chronica atrophicans skin appears transparent with easily seen veins on gross pathology.^[8][18]
• The following list are some of the findings on gross pathology:^[2]
  • Thinning of skin
  • Visible veins
  • Swelling
  • Wrinkles

• Light and electron microscopic study of the skin biopsy shows degeneration of the elastica and collagen fibers.^[19]
• A study that investigated skin biopsies under light and electron microscopes demonstrated osmiophilic materials around collagen fibres.^[19]
• Findings from biopsies exhibit leukocytic infiltrations, plasma cells, histiocytes and telangiectasia. ^[20][4][9]
• Thinning of skin layers such as dermis and epidermis has been seen in atrophic phase. ^[8][20][9]
• Pigmented stratum germinativum also has been reported in some biopsies.^[20]
• The following are list of pathognomonic microscopic findings when atrophic phase starts:^[20][9][19]
  • Epidermal atrophy
  • Elastin and collagen damage
  • Dermal blood vessels dilation
  • Perivascular plasma cell infiltration
  • Particles of elastic and oxytalan fibres

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ogheneochuko Ajari, MB.BS, MS [2] Anahita Deylamsalehi, M.D.[3]

This progressive skin disorder is due to the effect of chronic infection with the spirochete borrelia afzelii, which is the predominant cause of acrodermatitis chronica atrophicans. However borrelia afzelii is not the exclusive etiologic agent of acrodermatitis chronica atrophicans and other microorganisms such as borrelia garinii and borrelia burgdorferi have also been detected.

• The following are lists of common microorganisms that cause acrodermatitis chronica atrophicans:^{[1][2][3][4][5][6]}
  • Borrelia afzelii
  • Borrelia burgdorferi
  • Borrelia garinii
• Based on numerous studies, majority of skin biopsies from acrodermatitis chronica atrophicans patients demonstrated borrelia afzelii.^[7][8]

• Borrelia afzelii
• Borrelia burgdorferi
• Borrelia garinii

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Acrodermatitis chronica atrophicans must be differentiated from chronic venous insufficiency, chronic arterial insufficiency, superficial thrombophlebitis, frostbite, morphea, erysipelas, acrocyanosis and granuloma annulare.

• Based on the main presentation of a patient with acrodermatitis chronica atrophicans there are different differential diagnosis. The following are the most common differential diagnosis of acrodermatitis chronica atrophicans:^[1][2][3][4]
  • Chronic venous insufficiency
  • Chronic arterial insufficiency
  • Superficial thrombophlebitis
  • Cold injury (Frostbite)
  • Livedoid vasculitis
  • Localized scleroderma (also known as morphea)
  • Erysipelas
  • Erysipeloid
  • Lymphedema
  • Chronic potent topical corticosteroids use
  • Acrocyanosis
  • Aging
  • Chilblain
  • Granuloma annulare
  • Systemic sclerosis
• Misdiagnosis of rheumatoid arthritis may occur due to joint deformities and fibrotic nodules.^[2]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Acrodermatitis chronica atrophicans is a rare disease. The prevalence of acrodermatitis chronica atrophicans is estimated to include 10% of cases with lyme disease in Europe. The incidence of acrodermatitis chronica atrophicans increases with age and commonly affects individuals in range of 40 to 70 years old with a median of 64 years old. However there are few case reports on children who are diagnosed with acrodermatitis chronica atrophicans. Acrodermatitis chronica atrophicans affects women more than men. The majority of acrodermatitis chronica atrophicans cases are reported in northern, central and eastern Europe (most commonly in countries bordering the Baltic Sea). Lately few cases of acrodermatitis chronica atrophicans have been reported in the United States and Canada.

• Only 10% of lyme disease cases in European population have been presented with arodermatitis chronica atrophicans. ^[1]

• The prevalence of acrodermatitis chronica atrophicans is estimated to include 10% of cases with lyme disease in Europe.^[2]

• The incidence of acrodermatitis chronica atrophicans increases with age. ^[3]
• Acrodermatitis chronica atrophicans commonly affects individuals in range of 40 to 70 years old.^[3]
• There are few case reports on children who are diagnosed with acrodermatitis chronica atrophicans.^[4]
• In a study done on 693 patients with acrodermatitis chronica atrophicans median age of disease has been estimated 64 years old. ^[5]

• Acrodermatitis chronica atrophicans affects women more than men.^[3][2]
• Based on several studies female predominance has been reported approximately 65-80 % in acrodermatitis chronica atrophicans patient.^[6][7][8]

• The majority of acrodermatitis chronica atrophicans cases are reported in northern, central and eastern Europe (most commonly in countries bordering the Baltic Sea).^[3][9][10]
• Lately few cases of acrodermatitis chronica atrophicans have been reported in the United States which were associated to Lyme disease (besides European immigrants). ^[3][5]
• Few cases have been observed in Canada recently.^[10]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Common risk factors in the development of acrodermatitis chronica atrophicans include tick exposure, female gender and residents of northern, central and eastern Europe.

Common risk factors in the development of acrodermatitis chronica atrophicans include:^{[1][1][2][3][4]}

• Exposure to ticks
  • Frequent exposure to dogs, deers and other animals which may have ticks
  • Living near wooded areas or areas with high grass
• Female gender
• Residents of northern, central and eastern Europe

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2] Raviteja Guddeti, M.B.B.S. [3]

The course of acrodermatitis chronica atrophicans is chronic and could lasts for several years and it can progress slowly overtime. It has been estimated that mean duration of acrodermatitis chronica atrophicans before diagnosis is approximately 12 months, based on a study. It usually start on one extremity and can spread and involve extensor surfaces of the acral regions of limbs. Acrodermatitis chronica atrophicans has a biphasic manner. In first phase (the inflammatory phase) skin changes appear as blue and red discoloration with boggy infiltration. These inflammatory skin lesions can become atrophic without treatment (atrophic phase). Based on two studies, 55% and 66% of patients with acrodermatitis chronica atrophicans have at least one history of tick bite, while others may never remember such an accident. One fifth of patients in a study experienced erythema migrans 6 months to 8 years before acrodermatitis chronica atrophicans development. Superimposed bacterial infection, sclerotic skin changes, malignancies, arthropathy and peripheral neuropathy are some of the common complications of acrodermatitis chronica atrophicansis. In contrast to other skin manifestations of borrelia infection, acrodermatitis chronica atrophicans doesn’t heal without treatment and can lead to extensive atrophy of skin and limitations of upper and lower limb joint mobility. The general pognosis is good with proper and rapid treatment in acute inflammatory stage of acrodermatitis chronica atrophicans. Nevertheless late treatment can cause some irreversible changes.

• The course of acrodermatitis chronica atrophicans is chronic and could lasts for several years.^[1]
• Mean duration of the disease before diagnosis was approximately 12 months among 693 patients.^[2]
• It can progress slowly overtime.^[1]
• It usually start on one extremity and can spread and involve extensor surfaces of the acral regions of limbs.^[1][3][4]
• Skin changes first appear as blue and red discoloration with boggy infiltration (inflammatory phase). These inflammatory skin lesions can become atrophic later without treatment (atrophic phase), which resembles the biphasic manner of the disease.^[3]
• Based on two studies, 55% and 66% of patients with acrodermatitis chronica atrophicans have at least one history of tick bite, while others may never remember such an accident. Since there could be several years between the tick bite and development of skin lesions, absence of tick bite in patients‘ history is not meaningful alone.^[5]
• One fifth of patients in a study experienced erythema migrans 6 months to 8 years before acrodermatitis chronica atrophicans development.^[5][6][4]
• Some of patients reported previous neurological or rheumatological symptoms. Sensory polyneuropathy is the common form of neuropathy in these patients.^[2][7]
• Although symptoms of acrodermatitis chronica atrophicansis commonly present as a tertiary manifestation of lyme disease, it could be the first presentation of it in some cases.^[8]

• Common complications of acrodermatitis chronica atrophicansis include:^{[1][2][9][4][10]}
  • Superimposed bacterial infection
  • Sclerotic skin changes
  • Malignancy
    • Basal cell carcinoma
    • Squamous cell carcinoma
    • B-cell lymphoma
  • Peripheral neuropathy
    • May occur at the site of skin involvement or even at other sites.
    • The most common form of peripheral neuropathy reported in these patients was sensory polyneuropathy.
    • Based on a study done on 47 patients, the recommended antibiotic treatments for acrodermatitis chronica atrophicans are not effective against neuropathy.
  • Arthropathy
    • May occur at the site of skin involvement or even at other sites.

• Acrodermatitis chronica atrophicans can lead to extensive atrophy of skin and, in some patients it can cause limitation of upper and lower limb joint mobility.^[4]
• In contrast to other skin manifestations of borrelia infection, acrodermatitis chronica atrophicans doesn’t heal without treatment and is capable of becoming chronic.^[11][12]
• The inflammatory phase can last for years and the atrophic phase can last for decades Without appropriate treatment.
• The general pognosis is good with proper and rapid treatment in acute inflammatory stage of acrodermatitis chronica atrophicans.^[4]
• The therapeutic outcome is difficult to assess in patients with the chronic atrophic phase because most changes are only partially reversible. For instance there are numerous evidences on irreversibility of peripheral neuropathy in acrodermatitis chronica atrophicans patients with antibiotic treatments.^[4]

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