Cowden's Disease Associated With Immunodeficiency

• Cowden's disease is an autosomal dominant disease characterized by verrucous lesions, mucosal papules, and trichilemmomas associated with gastrointestinal tract, thyroid, CNS, and musculoskeletal abnormalities. In addition, our patient exhibited a deficiency of T-lymphocyte function, with recurrent cellulitis and abscess formation, and the eventual development of acute myelogenous leukemia. The defect in T-lymphocyte function may help to explain the higher incidence of malignant neoplasms in patients with Cowden's disease. We recommend complete immunologic examination of patients with this disorder. (Arch Dermatol1981;117:573-575)