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PubMed1994

Acrodermatitis chronica atrophicans: a case report and review of the literature.

We report a 55-year-old woman with acrodermatitis chronica atrophicans (ACA) and a peripheral sensory neuropathy. ACA is an uncommon late cutaneous manifestation of Lyme disease, which follows disseminated Borrelia burgdorferi infection. This is the second published case from the U.K. since serological diagnosis has been available. In this patient the diagnosis was confirmed by serology using a sensitive enzyme-linked immunosorbent assay and immunoblotting techniques. B. burgdorferi DNA was demonstrated in the affected skin using the polymerase chain reaction, although staining and cultures for the organism were negative. Recommended treatment of ACA is with oral doxycycline 100 mg twice daily for 28 days, but our patient did not respond well to this regimen. She was therefore treated with ceftriaxone intravenously for 21 days, which resulted in a rapid symptomatic and clinical response.

Acrodermatitis↗
PubMed1998

Acrodermatitis chronica atrophicans: histopathologic findings and clinical correlations in 111 cases.

We studied 111 consecutive, untreated and serologically confirmed patients with acrodermatitis chronica atrophicans. Emphasis was on the histopathologic patterns of erythematous and fibrous lesions, and on an assay used to correlate histopathologic findings with such clinical features as fibrous nodules, ulnar bands and the pain reaction allodynia. There was a significant correlation between allodynia and signs of marked inflammation, but not between allodynia and neural and perineural cell infiltrates or fibrosis. Moreover, there was no significant correlation between serum IgG titers to Borrelia and the density of inflammatory cell infiltrates or the proportion of plasma cells in tissue. Histopathologic examination did not reveal any important differences between fibrous nodules, ulnar bands and sclerodermatous lesions. The histopathologic pattern is not diagnostic per se, but characteristic enough to alert the experienced pathologist.

Acrodermatitis↗
PubMed1999

Joint and bone involvement in Dutch patients with Lyme borreliosis presenting with acrodermatitis chronica atrophicans.

We report on the radiological abnormalities of bones and joints in Dutch patients suffering from Lyme borreliosis presenting with acrodermatitis chronica atrophicans (A.C.A.). In a highly endemic area of the Netherlands rheumatic complaints were mentioned by 26 out of 60 patients suffering from a late stage of Lyme disease. Radiological findings in our group of patients were subluxation of the toe joint and periostitis of the bones of the lower limb.

Acrodermatitis↗
PubMed1995

Acrodermatitis chronica atrophicans: a light and electron microscopic study.

Degeneration of the elastica and collagen fibres in skin biopsies from patients with acrodermatitis chronica atrophicans was studied with light and electron microscopy. Elastic fibres were involved in the infiltrative stage while the elastin plexus was still present. In the atrophic phase, only fragments of elastic and oxytalan fibres were seen and the elaunin plexus was absent. Some collagen fibres were surrounded by osmiophilic material. In all biopsies, myelin sheaths were collapsed without axon structures. Spirochetes could be demonstrated in 69% of the biopsies and were most numerous in infiltrative and nodular lesions. The loss of elasticity of the skin in the atrophic phase may be caused by the destruction of both elastic and elastin fibres.

Acrodermatitis↗
PubMed1987

Acrodermatitis chronica atrophicans in association with lichen sclerosus et atrophicans: tubulo-interstitial nephritis and urinary excretion of spirochete-like organisms.

We report about a 38-year-old male patient with coexisting acrodermatitis chronica atrophicans, lichen sclerosus et atrophicans and recurrent diabetic metabolic disorders since 9 years. Serologically IgG antibodies against Borrelia burgdorferi could be detected. Moveless winded structures, morphologically resembling borreliae could be demonstrated in the urine sediment by dark field microscopy. Additionally a tubulo-interstitial nephritis was diagnosed by the presence of a dysmorphic hematuria, a pathological polyacrylamide gel electrophoresis and raised alpha 1- and beta 2-microglobulin in the urine. We suggest that the excreted spirochete-like structures are borreliae. They may be the putative infectious agent for the development of lichen sclerosus et atrophicans in the genital area.

Acrodermatitis↗
PubMed1993

Acrodermatitis chronic atrophicans: a chronic T-cell-mediated immune reaction against Borrelia burgdorferi? Clinical, histologic, and immunohistochemical study of five cases.

BACKGROUND: Acrodermatitis chronica atrophicans (ACA) is a late manifestation of infection caused by Borrelia burgdorferi. OBJECTIVE: Our purpose was to study the clinical, histopathologic, and immunohistochemical findings in patients with ACA to understand better the pathogenesis of the disease. METHODS: Five patients were studied. Skin biopsy specimens were obtained from active lesions for histologic and immunohistochemical studies. RESULTS: Clinical lesions included an initial erythematous discoloration in one patient and violaceous infiltrated plaques and nodules in four patients, three of whom also had late atrophic lesions. Biopsy specimens showed a dermal perivascular and interstitial lymphocytic infiltrate with plasma cells. There was a predominance of CD3+, CD4+ T cells in the dermal infiltrate. B cells were present in three patients. The dermal infiltrate showed an intense expression of lymphocyte function-associated antigen. The intercellular adhesion molecule type 1 was expressed on endothelial cells, perivascular mononuclear cells, and focally on basal keratinocytes. CONCLUSION: Our findings suggest that a chronic, T-cell-mediated immune reaction against B. burgdorferi is involved in the pathogenesis of ACA.

Acrodermatitis↗
PubMed1997

Detection of Borrelia afzelii, Borrelia burgdorferi sensu stricto, Borrelia garinii and group VS116 by PCR in skin biopsies of patients with erythema migrans and acrodermatitis chronica atrophicans.

OBJECTIVE: To evaluate the diagnostic performance of two polymerase chain reaction (PCR) procedures using skin biopsies of 20 erythema migrans (EM) and 24 acrodermatitis chronica atrophicans (ACA) patients. METHODS: One assay amplified a fragment of the outer surface protein (Osp) A gene. The second method amplified the spacer region between the 5S and 23S rRNA genes; hybridization of this fragment allowed identification of Borrelia burgdorferi sensu lato species. RESULTS: Among EM patients, both assays detected Borrelia DNA in 15 samples. Among ACA patients, the ospA PCR detected 15 positives and 10 samples were positive by 5S-23S PCR. In 19 samples one species was detected, 15 skin biopsies contained Borrelia afzelii, and Borrelia garinii was found in two patients. Group VS116 was detected in two EM patients, and therefore this group has pathogenic potential. Mixed infections of B. afzelii and B. garinii, group VS116 or B. burgdorferi sensu stricto were found in three EM and three ACA patients. CONCLUSIONS: Diagnosis of EM and ACA by PCR is useful and knowledge of the presence of species may be used to predict the course of disease or the need for further antibiotics.

Journal Article↗
PubMed1997

Peripheral neuropathy in acrodermatitis chronica atrophicans - a late Borrelia manifestation.

Clinical and/or neurophysiological signs of peripheral neuropathy were found in 64% of 63 consecutive untreated patients with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA). The neuropathy frequency was significantly higher in the patients than in 30 age- and sex-matched control persons of whom 27% had neuropathy findings. The most common neuropathy in ACA was a symmetric distal sensory polyneuropathy. In a subgroup of patients with localized or asymmetric neuropathy, the changes were found more often in extremities with than without visible ACA lesions. Neuropathy symptoms, most often pain and/or paresthesia, were present in 64% of the patients, compared to in 13% of the control persons. Thus, both symptoms and signs of neuropathy were significantly more frequent in patients with untreated ACA than in control subjects.

Acrodermatitis↗
PubMed1985

Successful cultivation of spirochetes from skin lesions of patients with erythema chronicum migrans Afzelius and acrodermatitis chronica atrophicans.

Spirochetes could be cultivated from 9 out of 13 skin biopsies from patients with erythema chronicum migrans Afzelius (ECMA) and from 2 out of 5 biopsies from patients with acrodermatitis chronica atrophicans (ACA) by using a newly modified serumless Kelly's medium. The different spirochete strains grew best at a low oxygen tension. Attempts to grow spirochetes from blood and cerebrospinal fluid failed. The cultivation of spirochetes from secondary ECMA lesions favours the presumption that a spirochetemia may occur in ECMA. The isolation of spirochetes from an ACA patient who had a disease duration of greater than 10 years proves that the spirochetes may survive in the human body for a considerable time.

Acrodermatitis↗