TY - JOUR
T1 - Scarring autoimmune bullous disease in a Ugandan patient with autoantibodies to BP180, BP230, and laminin 5
AU - Mulyowa, Grace K.
AU - Jaeger, Gerold
AU - Sitaru, Cassian
AU - Bröcker, Eva B.
AU - Zillikens, Detlef
AU - Schmidt, Enno
N1 - Funding Information:
Supported by Stiefel Laboratories.
Funding Information:
Supported by grants from the Germany Academic Exchange Service (DAAD A/01/20559 to G. K. M.), Wilhelm Sander-Stiftung (98. 078. 2 to D. Z.), and the German Society for Dermatology in the Tropics (to E. S.).
PY - 2006/2
Y1 - 2006/2
N2 - 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 α3 and γ2 chains of laminin 5. No reactivity was detected with type VII collagen, α6β4 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.
AB - 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 α3 and γ2 chains of laminin 5. No reactivity was detected with type VII collagen, α6β4 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.
UR - http://www.scopus.com/inward/record.url?scp=30944470243&partnerID=8YFLogxK
U2 - 10.1016/j.jaad.2005.03.054
DO - 10.1016/j.jaad.2005.03.054
M3 - Journal articles
C2 - 16427991
AN - SCOPUS:30944470243
VL - 54
SP - S43-S46
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
IS - 2 SUPPL.
ER -