A case of anti-laminin gamma-1 (p200) pemphigoid with bullous lesions on the lips, hands and feet showing antibodies to desmoglein 1

Abstract

An 87-years-old-man presented with bullous and erosive lesions exclusively on the lips, hands and feet. Histopathological and direct immunofluorescence studies suggested the diagnosis of bullous pemphigoid, but the serum anti-BP180 antibodies were negative. Strangely, anti-desmoglein 1 antibodies were positive, although no pemphigus-like features were found. Indirect immunofluorescence showed IgG reactivity with dermal side of 1 M NaCl-split skin. Immunoblotting showed positive reactivity with the 200 kDa laminin gamma-1 but not with type VII collagen. After the administration of prednisolone 20 mg/day, bullous lesions cleared within 2 weeks.

FIGURE 1

Clinical features (a) on the lips, (b) on the dorsal hands, and (c) soles

FIGURE 2

The results of (a) histopathology and (b) indirect immunofluorescence using 1 M NaCl‐split skin at titre 1:40

FIGURE 3

Immunoblotting analysis of the patient's serum. In immunoblotting of normal human dermal extract (Dermal), the patient serum reacted with the 200 kDa laminin gamma‐1, but not with the 290 kDa type VII collagen (left). No positive reactivity was seen in immunoblotting using purified human laminin‐332 (light). (Performed by the Study Group of Kurume University)