Manifestation and diagnosis of Hailey-Hailey's disease in the Moscow region

A retrospective analysis of 36 case histories ofpatients with Hailey-Hailey disease, residents of the Moscow Region, is presented. The disease manifested in residents of region at the age of 34.5±14.2 years and was characterized by involvement of only the skin integument. Imitation of clinical manifestations of other diseases of the skin prevented its early diagnosis. Only after years the clinical picture aquired a pronounced polymorphism with manifestation of the pathognomonic sign of the disease. Uncommon manifestations, no data on the hereditary liability in 58.3% patients impeded the diagnosis of Hailey-Hailey disease. Autoimmune pemphigus was suspected most often, other more rare erroneous diagnoses were Duhring’s herpetiform dermatitis, streptostaphylodermia and toxicoderma. The clinical picture was augmented by the lasting chronic process with involvement of the entire skin integument, in some cases of the mucosal membranes, with unpleasant subjective sensations and deterioration of the general condition. All these events led to hospitalization, the patients were aged 47.5±12.4 years by this time. This cohort of patients were in need of life-time observations. The studies should be carried out at the molecular biological level. A stable clinical remission for many years, allowing patient’s professional and social activity, can be attained only in response to personified therapy.

Hailey-Hailey’s disease, clinical manifestations, diagnosis