Acrodermatitis chronica atrophicans: Case report

Abstract

Introduction: Acrodermatitis chronica atrophicans (ACA) is a rare late chronic cutaneous manifestation of Lyme disease, which follows disseminated Borrelia burgdorferi infection. As ACA imitates various other skin diseases (as lupus erythematosus tumidus, intravascular histiocytosis, erysipelas, livedo reticularis and racemosa, etc.) the diagnosis is challenging and often delayed leading to a cutaneous inflammation progression to a chronic atrophy. Case description: A 74-year-old male admitted for a complaint of a unilateral painful edema and erythema of the right upper limb for 6 months, followed by myalgias and arthralgias of the lower limbs and a general muscle weakness for 1 month. The symptoms started in an early spring after hitting the right elbow. The clinical examination showed edema with subcutaneous infiltration of the right hand and forearm, purple-violet discoloration and nodules on the dorsal surface of the right hand and elbow. After the laboratory blood tests indicated slightly increased inflammatory markers (raised C-reactive protein – 11,06 mg/l and erythrocyte sedimentation rate – 32 mm/h) the suspicion of Borrelia burgdorferi infection occurred, though the anamnesis of a tick bite was negative. Following the suspicion, Lyme borreliosis was confirmed by two-tier serology using a sensitive enzyme-linked immunosorbent assay (ELISA) and immunoblotting techniques, as well as histological examination of the affected skin (morphological changes compatible with the diagnosis of ACA inflammatory stage). Based on the clinical, serological and histopathological findings the diagnosis of Lyme disease stage III, acrodermatitis chronica atrophicans was made. The patient was prescribed the standard antibiotic therapy byoral Doxycycline 100 mg two times a day for 30 days followed by a significant clinical cutaneous improvement. After the successful treatment course the disease was considered to be cured. Further serological tests for Borrelia antibodies were not repeated as they continue to persist and are informative for the disease follow-up. Conclusions: The diagnosis of ACA should be first suspected clinically and confirmed by positive Borrelia serology and supporting histopathology. One course of perioral recommended antibiotic therapy in the case of ACA is usually enough to achieve clinical improvement (in the case of inflammatory rather than late atrophy stage), though persistent symptoms as fatigue, arthralgia, pain, neurocognitive disorders can continue as posttreatment Lyme disease syndrome over the years.