A slowly growing, highly vascularized papule in a 4‐year‐old girl

A 4-year-old Caucasian girl was referred to our Paediatric Dermatology Unit for evaluation of a dome-shaped papular lesion on the columella. The papule had appeared 8 months earlier and been reported as progressively growing, but completely asymptomatic. On examination, 4 mm in diameter elastic hard palpation (Figure 1a). Noncontact polarised dermoscopy showed highly vascularises pattern, with out-of-focus arboriform telangiectasias pinkish background. There no pigment network or other dermoscopic signs suggestive melanocytic 1b). Given nonspecificity clinical findings progressive enlargement over time, surgically removed histological examination carried out 2). Cutaneous myoepithelioma. Histopathology well-circumscribed neoplasm centred within dermis lobulated architecture 2a), characterised by proliferation ovoid spindle histiocytoid cells pale eosinophilic cytoplasm, organised cords nests myxoid matrix 2b). Tumour were significant nuclear pleomorphism, inconspicuous nucleoli, mitoses; glandular ductal differentiation evident. express several neuroid markers (S-100 protein [Figure 3a], glial fibrillary acidic protein, SRY-related HMG-box 10 [SOX10] protein), one epithelial marker (epithelial membrane antigen [EMA]) 3b), focal expression smooth muscle actin. coexpression immunohistochemical markers, association absence any differentiation, led histopathological diagnosis cutaneous syncytial cytologic atypia made it possible exclude malignancy. myoepithelioma is rare composed primarily myoepithelial cells, represents end spectrum neoplasms skin soft tissue also including mixed tumours/chondroid syringomas carcinomas. These tumours display wide immunophenotypical profile.1, 2 Mixed typically show tubuloductal which present salivary gland pleomorphic adenoma (benign glands). myoepithelioma, instead, lacks predominantly spindled cells. While myoepithilal malignancy defined architectural features, their counterpart currently only known predictor an aggressive behaviour.2, 3 Only 20% occur children, they are malignant up 65% cases. They usually subcutaneous nodules extremities proximal limb girdles, affect males females equally.1, 2, From genetic point view, EWSR1 gene rearrangement found 45% tissue, thus supporting idea neoplasms. Several fusion partners have detected so far; however, significance, if any, products not well understood.2, 5 Differential diagnoses include epithelioid fibrous histiocytoma, early juvenile xanthogranuloma, Spitz naevus, ossifying fibromyxoid tumour sarcoma.6 In particular, from child should raise suspicion spitzoid neoplasm.7 To best knowledge, features described. Further studies this may provide additional tool differential these uncommon tumours. Although benign behaviour, there risk local recurrence (up 20%), especially case incomplete resection; therefore, regular follow-up after surgical removal recommended.8 case, visits scheduled every 6 years, observed. All authors contributed designing conducting work, draughting revising manuscript approved final version submission. declare conflict interest. parents/guardians minor patient included provided written informed consent child's participation study, use deidentified, anonymized, aggregated data, details (including photographs) publication. data that support study available corresponding author upon reasonable request.