Clinical analysis of 69 patients with familial benign chronic pemphigus

Objective To analyze the clinical feature, efficacy of treatment and prognosis in familial benign chronic pemphigus (FBCP) Methods Sixty nine cases of FBCP were retrospectively analyzed Results The ratio of male to female is 3 93:1 in 69 patients (55 males, 14 females) The mean age at the onset was 29 09 years (3-60 years) There was familial history in 27 families in all of the cases The lesion usually involved in genital area, neck, axillae and popliteal fossa Erythemas and vesicles on the soles were seen only in 1 case Histopathologically 44 cases had special features of FBCP, and immunopathologically 8 cases were direct immunofluorescence (DIF) negative, in which one case had C3 linear deposition along dermoepidermal junction The combined regimen was more effective The low dose X ray could improve the effect Conclusion The disease is transmitted as an irregular autosomal dominant trait The condition in males is more frequent than that in females, probably owing to the different level of female hormone in both sexes Our patients have the same clinical features as those reported in the literature, but the erythema, vesicle lesions on sole have not been documented in the literature The combined therapy should be adopted in this condition