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May 1978

Keratosis Follicularis Spinulosa Decalvans: An Infant With Failure to Thrive, Deafness, and Recurrent Infections

Author Affiliations

From the Departments of Pediatrics (Drs Britton, Lustig, and Thompson) and Ophthalmology (Dr Meyer), University of Chicago Hospitals and Clinics, and the Division of Dermatology, Department of Pediatrics (Dr Esterly), Michael Reese Hospital and Medical Center, Chicago.

Arch Dermatol. 1978;114(5):761-764. doi:10.1001/archderm.1978.01640170061012

† A 10-month-old male infant had keratosis follicularis spinulosa decalvans, an X-linked dominant disorder. His cutaneous abnormalities consisted of generalized hyperkeratosis, spiny follicular papular lesions, universal alopecia, and hypoplastic nails. Ocular changes characteristic of the disease were also present. Unusual findings included deafness, failure to thrive, predisposition to bacterial infections without demonstrable immune defect, and transient hepatomegaly with abnormal liver function studies.

(Arch Dermatol 114:761-764, 1978)