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A Comprehensive Review of the Literature

Jones, Christopher D. MBBS, MRCS*; Ho, Weiguang MBChB, MRCS; Robertson, Bernard F. MBChB, MRCS; Gunn, Eilidh MBChB, MRCS*; Morley, Stephen MBChB, FRCS (Plast)

The American Journal of Dermatopathology: September 2018 - Volume 40 - Issue 9 - p 631–641
doi: 10.1097/DAD.0000000000001118
CME Article

Introduction: Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a superficial benign skin tumor that arises from hair follicle matrix cells. Although pilomatrixomas are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. By reviewing all the literature over the past 10 years, the aims of this article are to analyze the cause, clinical presentation, management, and outcome of pilomatrixoma among children and adults to gain a more complete understanding of this lesion in today's clinical context.

Methods: A MEDLINE and EMBASE search was conducted from January 2005 to February 2015 using a combination of the terms: “child,” “childhood,” “adult,” and keywords: “pilomatrixoma,” “pilomatricoma,” and “calcifying epithelioma of Malherbe.” A total of 150 articles were reviewed.

Results: The lesions occurred most commonly in the first and second decades (mean age 16 years and 7 months). The commonest presentation was of an asymptomatic, firm, slowly growing, mobile nodule. Only 16% were accurately diagnosed on clinical examination. Imaging in the form of ultrasound, computed tomography, and magnetic resonance imaging has been reported. Pathological diagnosis was achieved through incision, punch, and shave biopsies. Pathological findings are discussed and summarized in this review.

Conclusion: Pilomatrixomas are thought to arise from mutation in the Wnt pathway and has been linked to several genetic conditions. It is commonly misdiagnosed preoperatively; however, with better awareness of the lesion, it can be appropriately treated while avoiding unnecessary diagnostic tests. Complete surgical excision with clear margins is almost always curative.

*Department of Plastic Surgery, St John's Hospital, Livingstone, Edinburgh, United Kingdom;

Department of Plastic Surgery, Ulster Hospital, Dundonald, Belfast, United Kingdom; and

Canniesburn Plastic Surgery Unit, Glasgow Royal Infirmary, Glasgow, United Kingdom.

Correspondence: Christopher D. Jones, MBBS, MRCS, Department of Plastic Surgery, St John's Hospital, Ward 18, Howden Road West, Howden, Livingston EH54 6PP, United Kingdom (e-mail:

All authors and staff in a position to control the content of this CME activity and their spouses/life partners (if any) have disclosed that they have no financial relationships with, or financial interests in, any commercial organizations pertaining to this educational activity.

Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.