A rare case of pemphigoid vegetans / 中华皮肤科杂志

ABSTRACT

A 89-year-old female patient presented with skin lesions of the groin, vulva and intergluteal sulcus for 10 months, and blisters for 3 weeks. Skin examination revealed the red-white hyperplastic plaques on the groin, vulva and intergluteal sulcus, on which mung-bean- to pea-sized erosions and blisters scattered, and several similar blisters were scattered on the right axilla and right leg, some of which were broken and covered with crusts. Histopathological examination of the skin lesion on the intergluteal sulcus showed thickened spinous layer without acantholysis, subepidermal fissures and blisters in some areas, focal papillary dermal edema with eosinophil infiltration, and perivascular infiltration composed mainly of lymphocytes in the superficial dermis. Direct immunofluorescence assay showed linear deposition of IgG and C3 at the epidermal basement membrane zone, clustered deposition of IgM in the dermis, but no IgA deposition. Indirect immunofluorescence on salt-split skin revealed that IgG and C3 were deposited on the epidermal side. Enzyme linked immunosorbent assay (ELISA) showed that serum levels of anti-BP180 and anti-BP230 antibodies were 26.92 U/ml and 68.17 U/ml respectively, and those of anti-desmoglein 1 (Dsg1) and anti-Dsg3 antibodies were normal. The patient was diagnosed with pemphigoid vegetans. After the treatment with oral methylprednisolone combined with topical halometasone ointment and tacrolimus 0.03% ointment, the skin lesions gradually subsided.