Case of pemphigoid vegetans with autoantibodies against the BP180 C‐terminal domain and BP230 antigen

Abstract

Pemphigoid vegetans is a rare variant of bullous pemphigoid characterized by vegetative and purulent lesions of the groin, axillae, thighs, hands, eyelids, and perioral regions. 1 The clinical features and histological findings of pemphigus vegetans and immunohistochemical characteristics of bullous pemphigoid are shown. Only 11 cases have been reported in the English-language literature to date. [1][2][3][4][5][6][7][8][9][10][11] Here, we describe a case of pemphigoid vegetans in which autoantibodies against both BP180 C-terminal domain and BP230 were detected. | C A S E REP ORTAn 86-year-old woman presented with erosions on the head and oral mucosa, along with blisters on the axillae and thighs. Physical examination revealed vegetative plaques on the groin and vulva (Figure 1a,b). Blisters and erosions were abundant on the head, neck, axillae, and thighs (Figure 1c). Crusts were observed around the eyes and lips, and erosions of the oral mucosa were detected (Figure 1df). The patient had no relevant family history.Laboratory findings established a white blood cell count of 14 200/μL and an increased eosinophil count of 1988/μL. Chemiluminescent enzyme immunoassay showed positive results for BP180 (12.3 U/mL; normal, <9) and enzyme-linked immunosorbent assay (ELISA) was positive for BP230 (index, 74.8; normal, <9), but negative for desmoglein (Dsg)1 and 3.Histopathology of an erosive lesion on the left axillary revealed irregular epidermal acanthosis, subepidermal edema, and infiltration of many inflammatory cells in the upper dermis (Figure 2a). In some areas, there were subepidermal clefts with eosinophil infiltration (Figure 2b) and eosinophilic pustules in the epidermis (Figure 2c). In addition, biopsy of vegetating plaque on the left groin showed striking epidermal hyperplasia, papillomatosis, subepidermal edema, and

Management of pemphigoid vegetans with mycophenolate mofetil: A case report

“…In our case, a linear IgA deposit at the dermo-epidermal junction was found by direct immunofluorescence, as reported by Ogasawara et al 11 Indirect immunofluorescence was positive in nine cases. 1 – 3 , 5 , 6 , 8 , 10 , 12 , 13 Immunoblotting analysis has been reported in five cases. 3 , 5 , 6 , 10 , 12 BP230 autoantibodies were positive in every case, and three cases had BP180 autoantibodies.…”

“… 1 – 3 , 5 , 6 , 8 , 10 , 12 , 13 Immunoblotting analysis has been reported in five cases. 3 , 5 , 6 , 10 , 12 BP230 autoantibodies were positive in every case, and three cases had BP180 autoantibodies. 5 , 8 , 12 …”

“…The age of presentation ranged from 9 to 89 years. 1 – 13 All patients presented with intertriginous vegetative lesions, mainly in the inguinal area ( Table 1 ). Some had mucosal lesions ( n = 8) and a few had vesicles or pustules ( n = 7 vs 6).…”

“…Only six cases had eosinophilia. 1 , 5 , 7 , 8 , 10 , 13 The most common pathological features were epidermal hyperplasia, subepidermal blister and eosinophilic infiltrate. Our patient had the same clinical and histopathological features as the patients described in the literature.…”

A Case of Eosinophilic Pustular Folliculitis with Vegetating Lesions on the Lower Legs and Feet Resembling Pemphigus Vegetans

“…may contribute to the development of vegetating lesions because Staphylococcus spp. has been detected in the vegetative lesions of pemphigus vegetans and pemphigoid vegetans patients (7)(8)(9). Several studies revealed that S. aureus can modify the immunological milieu in the colonization region (10,11).…”