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Pilomatrixoma : Intro & Clinical Features

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Pilomatrixoma (also known as pilomatricoma and calcifying epithelioma of Malherbe) is a benign tumor of the hair matrix.

It mainly affects children and young adults with a female predominance. The majority of patients are under 10 years of age. It usually presents as a solitary, painless, slow-growing, firm to hard nodule on head, face, upper extremities, and neck. It is small ranging in size from 0.5 to 3 cm. Yellow-white chalky deposits are sometimes extruded through the stretched skin. Imaging studies frequently reveal calcification within the lesion.

Multiple lesions may occur as a part of genetic syndromes or as a skin manifestation of systemic diseases. Associated conditions include myotonic dystrophy, Gardner syndrome, MYH-associated polyposis, Turner syndrome, trisomy 9, Rubinstein-Taybi syndrome, and Soto syndrome.

The image shows a pilomatrixoma above the right eye of a young female. Image courtesy of: Dermatopathology Program, CES University, Medellin, Colombia; Used with permission.

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