Acrodermatitis Chronica Atrophicans Herxheimer (ACA) is a tick-born disease due to infection by Borrelia afzelii, the major vector organism is Ixodes rhicinus. We report on a 48-year-old male patient who developed extensive livid-erythematous fibrosclerotic symmetric plaques associated with hyperpigmented widely distributed lesions within the tension lines, and acrocyanosis. The diagnosis of ACA has been confirmed by histopathologic examination of a skin biopsy and laboratory investigations with positive IgG and IgM immunoblots. The patient was treated by intravenous ceftriaxone resulting in partial remission of cutaneous and extracutaneous symptoms.Keywords: Acrodermatitis chronic atrophicans Herxheimer; Borreliosis; Cutaneous manifestations; Treatment Sensitivity and specificity of enzyme immuno assay and immune blot are 95% and 80-95% for ACA [4]. Polymerase chain reaction (PCR) of skin biopsies was positive in up to 88% on fresh-frozen tissue but only in 44-52% using paraffin-embedded tissue [5].
Case ReportA 48-year-old male patient was referred to our hospital because of large livid-erythematous fibrosclerotic plaques on his trunk and extremities which developed within half a year. He suffered from arterial hypertension and had a penicillin allergy. He had no memory of any tick bite.On examination we observed symmetric large lividerythematous fibrosclerotic plaques on his upper back. Erythematous to brownish lesions along the tension lines of skin were found on the lower back, abdominal, on the shoulders and proximal extremities. On this hands, a livid erythema was noted. During inspiration the lower thoracic aperture had a decreased elongation. On the hands there was an incomplete fist circuit notable. No other clinical symptoms were noted.We performed a skin biopsy. Histologic evaluation disclosed dermal changes including broadened and homogenized collagen bundles, perivascular and periadnexal lymphocytic infiltrate with some mast cells and plasma cells intermingled. PCR for Borrelia remained negative.
Laboratory investigationsLeukocytes 12.4 (normal range: 3.8-11.0 Gpt/l); neutrophils 9.4 (1.8-7.6 Gpt/l); C-reactive protein 17.6 (< 5 mg/dl); total IgE 269 (0-100kU/l); rheumatoid factor 38 (< 14 IU/ml); Borrelia IgG-antibodies [Enzyme immune assay] >200 (<16 RE/ml), IgM-antibodies 19.2 (<16 RE/ml), IgG-immunoblot: positive; IgM-immunoblot positive; serum albumin 47.6 (60,3-71,4 %), γ-globulin 30.0 (8.7-16.0 %). Antinuclear antibodies (ANA) and antibodies against extractable nuclear antigens (ENA): negative.
Imaging diagnosticsUltrasound -neck, axillaries, groins, abdomen -unremarkable. Thoracic X-ray and body plethysmography-unremarkable.