Clinical and Laboratory Features of 6 Cases of Pemphigoid Nodularis / 中国医学科学院学报

To investigate the clinical manifestations and laboratory characteristics of 6 cases of pemphigoid nodularis (PN). The clinical and laboratory data of 6 patients with PN admitted to the Department of Dermatology,Peking Union Medical College Hospital from January 2016 to August 2019 were retrospectively analyzed. PN mainly occurred in middle-aged and elderly people,with an average age of (58±16) years. Eosinophils were elevated in 4 patients. Immunoglobulin E (IgE) level was (530±672) kU/L in five patients. Direct immunofluorescence showed IgG and/or C3 deposition on basal membrane zone. Indirect immunofluorescence showed positive IgG anti-basement membrane zone,with a titer of 1:40-1:320. Enzyme-linked immunosorbent assay showed the anti-BP180 antibodies were positive [24-85 U/ml,average(43±26) U/ml] in 5 patients. None of the patients had neurological disorders. One patient was lost to follow-up. The disease recurred in 3 of 5 patients during the follow-up,and two patients still received maintenance corticosteroids. PN mainly occurs in middle-aged and elderly individuals. It is featured by elevated eosinophils and total IgE and relatively low anti-BP180 antibody titers. Recurrence is common but PN is less likely to be associated with neurological diseases.

Clinicopathological analysis of seven cases of pemphigoid nodularis / 中华皮肤科杂志

Objective To analyze clinical characteristics of pemphigoid nodularis. Methods Seven cases of pemphigoid nodularis were included in this retrospective study. The clinicopathological features of pemphigoid nodularis were retrospectively analyzed, including patients′gender, age at onset, clinical manifestations, treatment and follow?up. Results Of the 7 patients, 4 were female and 3 were male, with the median age at onset being 59 years. Clinical manifestations mainly included markedly pruritic prurigo?like lesions and nodular lesions with or without blisters. The most common misdiagnoses were nodular prurigo and eczema. Pathology showed epidermal hyperplasia and subepidermal clefts, collagen hyperplasia in the papillary dermis, and perivascular infiltration of lymphocytes and eosinophils in the superficial dermis in all the patients. Direct immunofluorescence assay showed linear deposition of IgG and C3 in the basement membrane zone, and indirect immunofluorescence was positive in 2 patients. Glucocorticoids combined with immunosuppressants were effective in all the patients. Conclusions Pemphigoid nodularis is easy to be misdiagnosed, and immunopathology is helpful for its diagnosis. Glucocorticoids combined with immunosuppressants appear to be effective for its treatment.