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September 1981

Cowden's Disease Associated With Immunodeficiency

Author Affiliations

From the Department of Dermatology, Thomas Jefferson University, Jefferson Medical College, Philadelphia.

Arch Dermatol. 1981;117(9):573-575. doi:10.1001/archderm.1981.01650090055027

• 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 Dermatol 1981;117:573-575)