Scarring autoimmune bullous disease in a Ugandan patient with autoantibodies to BP180, BP230, and laminin 5.
We report on a 24-year-old, male Ugandan patient with a 2-week history of itchy papules, vesicles, erosions, and crusts distributed on the entire body, accompanied by minor erosions on the palate, tongue, and lower lip. Conjunctivae and genital mucosa were not involved. Circulating IgG and IgA autoantibodies were found against recombinant full-length BP180, BP180 4575, and the C-terminus of BP230. In addition, IgG reactivity was observed against the 16th noncollagenous region of the BP180 ectodomain, the cell-derived soluble ectodomain of BP180 (linear IgA disease antigen 1), and the alpha3 and gamma2 chains of laminin 5. No reactivity was detected with type VII collagen, alpha6beta4 integrin, and the p200 protein. Oral prednisolone and dapsone led to clearance of lesions that mostly healed with scarring and milia formation. Here, we describe a scarring mucocutaneous variant of an autoimmune blistering skin disorder that extends the current clinical and immunopathologic spectrum of this group of diseases.
Mulyowa
Grace K
GK
Department of Dermatology, University of Würzburg, Würzburg, Germany.
Jaeger
Gerold
G
Sitaru
Cassian
C
Bröcker
Eva-B
EB
Zillikens
Detlef
D
Schmidt
Enno
E
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology; D002352:Carrier Proteins / Q000276:immunology*; D015815:Cell Adhesion Molecules / Q000276:immunology*; D002921:Cicatrix / Q000276:immunology / Q000473:pathology; D003598:Cytoskeletal Proteins / Q000276:immunology*; D000071821:Dystonin; D006801:Humans; D008297:Male; D009419:Nerve Tissue Proteins / Q000276:immunology*; D024041:Non-Fibrillar Collagens; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology* / Q000473:pathology; D014454:Uganda; D000097794:Kalinin; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D015815:Cell Adhesion Molecules; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097794:Kalinin; D000097805:Collagen Type XVII; C486268:DST protein, human