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A benign skin appendage tumor frequently involving the skin of the head, neck, and upper extremities in young children.

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Epithelioma Calcificans of Malherbe; Pilomatricoma Malherbe Epithelioma; Malherbe Tumor; Calcifying Epitheliomia of Malherbe.

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Isolated lesions are not rare in children with antecedent trauma, which has been described in 30% of cases. Pilomatrical neoplasms are considered the most common solid cutaneous tumors in patients 20 years of age or younger. In the United Kingdom, pilomatrixomas account for 1 in 500 histologic specimens. Cases have been reported in Japan (37), Turkey (15) and France (33) up to year 2000. The female-to-male ratio is 1.5:1.

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Isolated lesions are sporadic. The presence of multiple lesions may be suggestive of an autosomal dominant transmission. In this case, it may be associated with myotonic dystrophy.

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The tumor arises from hair matrix cells. The cause has been suggested as the result of a mutation in the beta-catenin gene.

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Pathologically, the tumors were situated in the dermis or subcutaneous tissue. The histopathologic findings of viable basaloid cells in the periphery, shadow cells in the central part, and foci of calcification are characteristic of pilomatrixoma. Computed tomography scans clearly demonstrated a well-defined, subcutaneous mass with amorphous calcifications. Lesions are most often discovered in the first 2 years of life.

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This benign lesion is a firm, circumscribed tumor, 1 to 2 cm (0.4 to 0.8 inches) in diameter, usually in the head and neck area, attached to the subcutaneous tissue and overlying skin. Usually it is an asymptomatic superficial mass that increased slowly in size. Multiple lesions occur in 2 to 3% of reported series, in some cases in association with myotonic dystrophy. Complete surgical excision is the best management.

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Presence of myotonic dystrophy should be sought in any patient with more than one pilomatrixoma and in any patient with a family history of pilomatrixoma.

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The presence of myotonic dystrophy dictates the choice and conduct of the anesthesia. Isolated pilomatrixoma does not have any special anesthetic or pharmacological implications.

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The use of neuromuscular blocking agents should be used with cautions.

Baltogiannis N, Faviou E, Cigliano B, et al: Diagnosis of pilomatrixoma in childhood. Plast Reconst Surg 115:1783, 2005.  [PubMed: 15861101]
Demircan M, Balik E: Pilomatricoma in children: A prospective study. Pediatr Dermatol 14:430, 1997.  [PubMed: 9436837]
Wells NJ, Blair GK, Magee JF, Whiteman DM: Pilomatrixoma: A common, benign childhood skin tumour. Can J Surg 7:483, 1994.

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