DOI: 10.36347/sasjm.2024.v10i04.007

Pages: 246-249

Progressive erythrokeratodermia (PEK) is a rare génodermatose associated with disorders of keratinization. Mutations are found in genes encoding connexins 31 and 30.3 mapped to chromosome 1 p34-35. This disease is characterized by fixed hyperkeratotic plaques and transient erythematous plaques. Approximately 50% of affected individuals develop palmoplantar keratoderma. Connexins are components of gap junctions, which are intercellular channels present in almost all tissues, including the skin. A 15-year-old Guinean female, a seamstress, presented with asymptomatic black patches on her feet that had been evolving continuously for 5 years, initially starting on the feet and spreading elsewhere, gradually increasing in size with a transient tingling sensation. Previous treatment involved topical phytotherapy. She had no significant medical history, no similar skin conditions in the family, and no history of consanguinity between her parents. On examination, erythematous and keratotic plaques of variable shapes and sizes were noted on the backs of the hands, the posterior surfaces of the forearms, both knees, the lower thirds of the legs, and the backs of the feet, giving a sock-like appearance to the latter location. The complete blood count, lipid profile, and fasting blood glucose were normal. Histopathology was not performed. The diagnosis of progressive erythrokeratodermia of Gottron was considered based on the age of onset, clinical characteristics, and location of the lesions. The patient was prescribed topical keratolytics, oral Acitretin 10 mg, and psychotherapy for both the patient and her parents. It often constitutes an entity encountered in a syndromic context, highlighting the necessity for surveillance.