Clinical case: Hailey-Hailey disease or Darier’s disease?

We present the clinical observation of a patient whose final diagnosis establishing has caused certain difficulties. Patient B., 31 years old, admitted to the hospital with complaints of copious rash on the skin of the face, neck, axillary and inguinal regions, intense itching in the foci, oozing, painful rhagades. According to the patient, he has been sick since childhood. He associates the disease onset with a negative family history. Similar rash is observed in relatives of the father’s side (grandmother, father, sister), father underwent a histological examination that confirmed the diagnosis of Darier’s disease. The rest of the family did not undergo biopsy and were diagnosed on the basis of the family history. Differential diagnosis was performed between two autosomal dominant genodermatoses, namely Hailey-Hailey disease and Darier’s disease. Despite the availability of information about the negative family history, the clinical picture of dermatosis was more attributable to the familial benign chronic pemphigus. The patient underwent additional methods of investigation and, despite atypical manifestations of dermatosis, histological examination allowed to verify the diagnosis, which emphasizes the necessity of its application in patients with suspicion of Hailey-Hailey disease.