A 6-year-old boy presented with multiple asymptomatic papules on the face noticed since 8 months, which first developed around nasolabial folds and later involved periocular area and forehead. There were no other cutaneous or systemic complaints. There was history of similar lesions on the face of mother. General and systemic examination was normal. Cutaneous examination revealed multiple dome-shaped, skin colored pearly papules of size 1-2 mm, distributed in a centrofacial pattern around the nose and the eyes (Fig.1). The mother had similar papules coalescing to form plaques present all over the face, neck and retroauricular areas. A clinical diagnosis of multiple familial trichoepitheliomas (MFT) was considered and biopsy from both the mother and the child confirmed the diagnosis.

Fig.1. Multiple dome-shaped, skin colored pearly papules distributed in centro-facial pattern in MFT.

Trichoepitheliomas (syn: Brooke’s tumor) are benign adnexal tumors, possibly of folliculosebaceous-apocrine origin. They are slow growing and may be solitary or multiple. MFT is a rare autosomal dominant genodermatosis, linked to chromosome 9p21. The tumors appear in childhood and gradually increase in number. The diagnosis is based on the family history, clinical and histological findings. The clinical differentials include colloid milium, milia, pilar cyst, syringomas, steatocystoma multiplex and angiofibromas of tuberous sclerosis.

Solitary lesions can be excised. Multiple lesions usually lead to marked disfigurement and the treatment options include electrosurgery, cryotherapy. dermabrasion, ablative lasers (such as erbium: Yag and CO2 laser) and split-thickness skin grafting. Such procedures result in flattening of lesions and good cosmetic outcome but recurrence may occur. Recently, topical therapy with a combination of imiquimod and tretinoin, has been reported to be effective.

Amit Kumar Malhotra,
Neena Khanna,
Department of Dermatology and Venereology,
All India Institute of Medical Sciences,
New Delhi 110 029, India.
E-mail: [email protected]