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.
Fisher GH, Geronemus RG. 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