Huriez syndrome with superadded dermatophyte infection

Palmoplantarkeratodermas (PPKs) are a heterogeneous group of hereditary and acquired disorders with underlying gene defects, and characterized by hyperkeratosis of palms and soles with or without other ectodermal and systemic abnormalities. Huriez syndrome is a rare autosomal dominant transgradient type of PPK with high frequency of squamous cell carcinoma in the affected skin. We hereby describe a case of a very rare autosomal dominant PPK in a 40-year-old male patient presenting since birth with PPK extending onto the dorsal aspects of hands and feet with peeling of the skin. The complaints were associated with sclerodactyly, hyperhidrosis, and nail abnormalities. Also superadded dermatophyte infection was observed involving abdomen. No history of loss of any digit. No mucosal, dental, or any systemic involvement was present. No sign of malignancy was noted. Baseline investigations, including ultrasonography of abdomen were normal. Histological findings were nonspecific with only orthohyperkeratosis and acanthosis. Diagnosis was mainly done on clinical grounds. The patient is better with oral retinoids and topical emollients and keratolytics along with antifungal treatment for dermatophyte infection. He is under follow up.