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March 21, 2011

Multiple Congenital Facial Papules—Diagnosis

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Arch Dermatol. 2011;147(3):345-350. doi:10.1001/archdermatol.2011.36-b

Histopathologic findings from a facial papule showed a polypoid fragment of skin with numerous villous hair follicles set in a background of fibroadipose tissue, consistent with an accessory tragus.

The presence of multiple accessory tragi in a newborn is often the first indicator of a congenital syndrome. The differential diagnosis of syndromes featuring accessory tragi include oculocerebrocutaneous (Delleman syndrome), oculoauriculovertebral (Goldenhar syndrome), encephalocraniocutaneous lipomatosis (Haberland syndrome), and Townes-Brock syndrome.1 Colobomas also occur in all of these syndromes along with a variety of neurologic abnormalities. Aplasia cutis (as noted in this case), lack of auricular abnormalities, and the presence of neurologic abnormalities usually distinguish Delleman syndrome from the closely related Goldenhar syndrome.2 Aplasia cutis may be seen in both, but it is more common in Delleman syndrome. Haberland syndrome has a characteristic cutaneous finding of nevus psiloliparus or hairy fatty nevus. Although focal dermal hypoplasia (Goltz syndrome) features aplasia cutis and raspberry papillomas, it lacks auricular tags and is associated with the distinctive finding of ectrodactyly.

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