Intertriginöse Vesikel und Pusteln bei einem 36-Jährigen

Abstract

Abb. 1a,b ▲ Pusteln, Erosionen und seröse Krusten auf gerötetem Grund; zusätzlich multiple postinflammatorisch hyperpigmentierte Maculae in der linken Axilla (a); Ausschnittsvergröße-rung (b) AnamneseBei einem 36-jährigen Patienten traten seit mehreren Jahren rezidivierend juckende Bläschen und Pusteln auf, die vor allem axillär,inguinal,am Rücken und den Oberarmen imponierten. Die Läsio-nen heilten unter Hypo-bzw. Hyperpigmentierungen ab. Die histologische Untersuchung einer Hautbiopsie zeigte eine subkorneale Pustel. Unter dem Verdacht einer subkornealen Pustulosis SneddonWilkinson wurde ein Therapieversuch mit Dapson eingeleitet (täglich 2 mg/kg Kör-pergewicht). Der Patient setzte diese Behandlung trotz Besserung des Hautbefundes wegen subjektiv empfundener Belastungsdyspnoe ab. In der Folgezeit wurde eine 12-monatige Therapie mit Azathioprin (Imurek®, täglich 2 mg/kg Körper-gewicht) und Prednison (Decortin®, 5 mg/d) durchgeführt, die jedoch keine Besserung bewirkte. Liebe Kolleginnen, liebe Kollegen

IgA pemphigus – Occurrence of anti‐Desmocollin 1 and anti‐Desmoglein 1 antibody reactivity in an individual patient

“…By immunoblot analysis using dermal and epidermal extracts, no specific IgA and IgG reactivity, including the 105 kDa and 115 kDa desmocollin doublet, the 160 kDa Dsg1 and the 130 kDa Dsg3 proteins, could be demonstrated. Based on the clinical appearance and the immunopathology results, IgA pemphigus with dual antibody reactivity was diagnosed and therapy with dapsone 50 mg twice daily was instituted [1, 12, 16]. Additional therapy included intermittent systemic antibiotic therapy with cefaman‐dole and topical sulfadiazine ointment.…”

“…To detect IgA anti‐Dsc1 autoantibodies, the patient's serum was tested using COS7 cells transfected with human Dsc1 cDNA as described [7, 8, 12, 17]. Sera of a patient with known IgA anti‐Dsc1 reactivity and of healthy persons were used as positive and negative controls, respectively.…”

“…IgA pemphigus should be distinguished from subcorneal pustulosis Sneddon‐Wilkinson, pemphigus foliaceus, pustular psoriasis, bullous impetigo, dermatitis herpetiformis, linear IgA dermatosis and necrolytic migratory erythema [12]. Cases of SPD type IgA pemphigus associated with IgA gammopathy have been reported, some of which were benign while others occurred in the context of B‐cell lymphoma or myeloma [12–15]. IgA pemphigus responds usually well to dapsone, either alone or in combination with corticosteroids [12, 16].…”

“…Cases of SPD type IgA pemphigus associated with IgA gammopathy have been reported, some of which were benign while others occurred in the context of B‐cell lymphoma or myeloma [12–15]. IgA pemphigus responds usually well to dapsone, either alone or in combination with corticosteroids [12, 16]. Other therapeutic options include retinoids, sulfapyridine, colchicine and PUVA [1, 3, 12].…”

“…IgA pemphigus responds usually well to dapsone, either alone or in combination with corticosteroids [12, 16]. Other therapeutic options include retinoids, sulfapyridine, colchicine and PUVA [1, 3, 12].…”