Azam Jah Samdani.
Ectodermal Dysplasia with Acanthosis Nigricans (Lelis syndrome)
J Coll Physicians Surg Pak Oct 2004;14(10):626-7.
King Abdul Aziz Hospital, Makkah, Saudi Arabia

A 32-year-old Saudi male was referred with a long-standing history of abnormal hair of the scalp, dystrophic nails and dry skin with palmoplantar hyperkeratosis. He had a twin brother with similar but milder manifestations on clinical examination who refused to be photographed. The parents were not related to each other but belonged to the same tribe. General physical examination showed a normal height and weight, low-set ears, and narrow face with prominent forehead and lips. He had saddle nose with an increased nose width. Cutaneous examination revealed dry, hypochromic wrinkled skin with thick lips. The scalp hairs were sparse, dry and lusterless, beard and pubic hairs were also scanty. There was hyperkeratosis of the skin of the hands and feet. The patient also had acanthosis nigricans in the axillary region. Nails were thick, yellow in color, dystrophic and hyper convex. Oral examination revealed hypodontia with peg-shaped anterior teeth. The patient reported fragile temporary teeth with early loss. There was delayed neurological development and the patient had mild mental retardation. Investigations including blood counts, chemistry, thyroid studies, testosterone and estradiol were all within normal limits. Chromosomal analysis on peripheral blood lymphocytes with G–banding technique showed a normal male constituency. Skeletal survey and examination of the hair from the scalp under the light microscope were normal. Skin from the palm was taken for histopathology which showed reduction in number of eccrine sweat glands, sebaceous glands and hair follicles, also the epidermis was thin and flattened. Biopsy taken from the axilla revealed acanthosis nigricans.

Category: Case Reports
Keywords: Ectodermal Dysplasia. Acanthosis Nigricans. Lelis Syndrome. Hyperkeratosis.

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