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A 64-year-old woman presents with a request for the removal of a lesion on her right cheek. She first noticed the growth approximately 18 months ago, at which time she sought an evaluation. At the time the site was injected with triamcinolone. However, over the past 3 months she reports a marked increase in the lesion’s size. Upon manipulation, a whitish discharge was observed. Physical examination revealed a 1.8cm firm, flesh-colored nodule with 2 whitish papules within the medial border.
Pilomatrixoma, also referred to as calcifying epithelioma of Malherbe, is a benign tumor that develops within the hair follicle matrix.1 The tumor is thought to arise from a mutation that upregulates the WNT-signaling pathway. The tumor can appear either sporadically or...
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Pilomatrixoma, also referred to as calcifying epithelioma of Malherbe, is a benign tumor that develops within the hair follicle matrix.1 The tumor is thought to arise from a mutation that upregulates the WNT-signaling pathway. The tumor can appear either sporadically or accompany conditions such as Gardner syndrome, myotonic dystrophy, and Rubinstein-Taybi syndrome.1,2
Clinical manifestations of pilomatrixoma are diverse and some lesions may display a “tent” sign when skin over the tumor is stretched.3 A definitive diagnosis is made by biopsy, which characteristically shows epithelial islands of basaloid cells along with shadow or so-called “ghost” cells. Imaging studies (high-frequency ultrasound) have been used to ascertain lesion depth.4
Pilomatrixomas have a bimodal distribution of occurrence, with a peak incidence between birth to under 30 years and a second peak incidence between 50 and 65 years.5 The tumors are most frequently found on the head and neck and are more common in women.2 Lesions rarely exceed 3.0 cm.5 Surgical excision is curative although adequate margins are required to prevent recurrence.3
Sidney Lampert is a medical student at the Drexel University College of Medicine, in Philadelphia. Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
References
1. Jones CD, Ho W, Robertson BF, Gunn E, Morley S. Pilomatrixoma: a comprehensive review of the literature. Am J Dermatopathol. 2018;40(9):631-641. doi:10.1097/DAD.0000000000001118
2. Hu JL, Yoo H, Kwon ST, et al. Clinical analysis and review of literature on pilomatrixoma in pediatric patients. Arch Craniofac Surg. 2020;21(5):288-293. doi:10.7181/acfs.2020.00528
3. Le C, Bedocs PM. Calcifying epithelioma of Malherbe. In: StatPearls [Internet]. StatPearls Publishing; 2023 Jan-. Updated June 12, 2023.
4. Li L, Xu J, Wang S, Yang J. Ultra-high-frequency ultrasound in the evaluation of paediatric pilomatricoma based on the histopathologic classification. Front Med (Lausanne). 2021;8:673861. doi:10.3389/fmed.2021.673861
5. Beattie G, Tai C, Pinar Karakas S, Cham E, Idowu O, Kim S. Colossal pilomatrixoma. Ann R Coll Surg Engl. 2018;100(2):e38-e40. doi:10.1308/rcsann.2017.0196
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