Dermatology by unknow

Author:unknow
Language: eng
Format: epub, pdf
Publisher: CRC Press
Published: 2016-04-16T04:00:00+00:00

CASE 54

Trichoepithelioma

QUESTION 54

A 54-year-old woman presents to the dermatology clinic as a referral from her otolaryngologist for the evaluation of a growing tumour on and around the left ear. She is unsure of how long the tumour has been present. On examination, there is a pink nodular plaque involving the tragus and spanning from the antitragus to the crus of the helix (54). On histology, there are basaloid islands in a sclerotic stroma with concentric collagen and many fibroblasts.

i. What is the diagnosis?

ii. What is the inheritance pattern of this condition? What syndrome(s) is/are associated with this growth?

iii. What is the other variant of this tumour, and what is the pitfall in its diagnosis and management?

Answer 54

i. The patient presents with clinical and histological findings of a trichoepithelioma, a benign neoplasm of follicular germinative differentiation. Classic trichoepitheliomas present as skin-coloured papules or nodules on the face or upper trunk.

ii. Multiple trichoepitheliomas, known as epithelioma adenoids cysticum, can be seen associated with familial cylindromatosis, an autosomal dominant condition caused by a mutation in the cylindromatosis (CYLD) gene. In addition, there are several syndromes associated with multiple trichoepitheliomas, listed below.

syndrome

Cutaneous findings

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