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June 2006

Treatment of Multiple Familial Trichoepitheliomas With a Combination of Aspirin and a Neutralizing Antibody to Tumor Necrosis Factor α: A Case Report and Hypothesis of Mechanism

Arch Dermatol. 2006;142(6):781-798. doi:10.1001/archderm.142.6.782

The condition known as multiple familial trichoepitheliomas (MFT) is an autosomal dominantly inherited genodermatosis. Patients with MFT usually present between the ages of 10 and 20 years with multiple flesh-colored centrofacial papules.1 The area of involvement is usually limited to the face and scalp. The facial papules can be quite dense and disfiguring. A subset of patients with MFT have mutations in the same gene, CYLD, that is responsible for familial cylindromatosis and Brooke-Spiegler syndrome. CYLD encodes a deubiquitinating enzyme that acts as a negative regulator of tumor necrosis factor (TNF)-induced NF-kB (nuclear factor-κB) activation.2,3 The current methods of treatment are surgical and include excision, electrocautery, and ablative resurfacing.

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