Darier's Disease

Fifteen cases of Darier's disease were studied. Males and females were in the ratio of 3:2. The disease appeared between the ages of 5 and 16 in majority (80%) of the patients. Family history was positive in only 60% cases. Itching was the main symptom in 2/3rd (66.6%) of cases. Seven cases experienced aggravation of the disease in summer. Recurrent skin infection and eczematisation were present in 5 cases. Majority of patients had typical lesions. Severity of the disease was directly related to its duration. Acrokeratosis verruciformis like lesions over the hands and feet were present in all the cases and 86.6% of cases had palmoplantar keratoderma. Mucosal lesions were seen in 73.3 % of cases. These unusual findings are not often stressed in clinical practice and need to be highlighted. Leucoderma guttate yet another unusual finding was seen in 20% of cases.