UNCORRECTED PROOF. Congenital smooth muscle hamartoma on the scalp. Case report

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1 I J D Operat: Lin Li Dispatch: PE: Eugene Lim Journal Name Manuscript No. Proofreader: Zhang Yeping No. of Pages: 2 Copy-edit: Sue Daniels Blackwell Oxfd, IJD International XXX 2008 The UK Publishing International Journal Ltd of Dermatology Society of Dermatology Case rept Günlemez Case reptet smooth al. muscle hamartoma Congenital smooth muscle hamartoma on the scalp doi: /j x Ayla Günlemez, MD, Dilek Bayramgürler, MD, M. Cengiz Erçin, MD, Aysun Aktürk, MD, and A. Engin Arısoy, MD From the Section of Neonatology, Department of Pediatrics, Department of Dermatology, and Department of Pathology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey Crespondence Ayla Günlemez, MD Cumhuriyet Mahallesi Sahil Caddesi Deniz Sokagı B Blok D 6 ;zmit, Kocaeli Turkey aylagunlemez@yahoo.com Case Rept An 8-day-old male infant was referred to our neonatal unit f prematurity. It was observed that his scalp col was darker than the rest of his skin col when the scalp hair was partially shaved f intravenous access. Dermatologic examination revealed a slightly elevated, hyperpigmented plaque confined to the whole scalp. The margin of the lesion did not extend to the face and neck. His hair was abundant and dark (Fig. 1). There were no areas of alopecia and no overlying folds of skin. There were no dysmphic features, such as Michelin tire baby. Pseudo-Darier s sign was negative. A punch biopsy specimen was obtained from the lesion with a diagnosis of congenital melanocytic nevus congenital blue nevus. The result of histopathologic examination showed increased smooth muscle bundles in the reticular dermis running in various directions under a nmal epidermis (Fig. 2a); immunohistochemical stain revealed desminpositive (Fig. 2b) and S100-negative proliferation. Based on these clinical and histopathologic findings, a diagnosis of congenital smooth muscle hamartoma was made. No changes in this lesion were observed during a 9-month follow-up period. UNCORRECTED PROOF Discussion Congenital smooth muscle hamartoma (CSMH) is a rare cutaneous dysembryoplasia characterized by a disganized proliferation of nmal muscle fibers of arrect pili. The disease usually presents as a localized, skin-coled mildly hyperpigmented, irregularly shaped patch plaque with prominent vellus hairs located on the lumbosacral area. Figure 1 Congenital smooth muscle hamartoma on the scalp of an 8-day-old premature newbn 1 Col image 2008 The International Society of Dermatology International Journal of Dermatology 2008

2 2 Case rept Congenital smooth muscle hamartoma Günlemez et al. Col image Figure 2 (a) Increased smooth muscle bundles in the reticular dermis running in various directions under the epidermis (hematoxylin and eosin; magnification, 100). (b) Desmin positivity (immunohistochemistry, 200) UNCORRECTED PROOF Malignant transfmation has not been repted and pigmentation diminishes with time. 1,2 The prevalence of CSMH has been estimated to be 1 in 2600 live births, with a slight male predominance; 3 however, recent repts have suggested that CSMH is probably me prevalent than indicated by the small number of cases repted in the literature. 2 Although excessive hairiness is the most frequent sign of CSMH, lesions in the head and neck region have rarely been repted in the literature. Only two repts have described CSMH on the head, one of which was located on the frontal scalp and the other on the face. 4,5 In our case, the lesion appeared over the whole scalp and, interestingly, the margin of the lesion did not cross the line of the scalp hair. We noticed the lesion during shaving f intravenous access, otherwise it might have remained unrecognized. The diagnosis of CSMH is based on histopathologic examination. Histology is characterized by the presence of numerous smooth muscle fibers disseminated in the dermis and diversely iented, sometimes in contact with hair follicles which retain their nmal mphology. The clinical differential diagnosis of CSMH includes congenital melanocytic nevus, Becker s nevus, solitary mastocytoma, piloleiomyoma, café-au-lait spots, nevus pilosus, and occult spinal dysraphism. 2,5 Distinguishing CSMH from congenital melanocytic nevus may be difficult on clinical grounds. Congenital melanocytic nevi are differentiated from CSMH by histopathologic examination. The differentiation of these two clinical pictures is very imptant f the follow-up of patients, as congenital melanocytic nevi carry the risk of malignant transfmation. Becker s nevus is another imptant entity in the differential diagnosis of CSMH. It resembles CSMH both clinically and histopathologically. In contrast with CSMH, Becker s nevus is usually an acquired lesion, although congenital cases have been repted very rarely. 2,6 We also considered giant congenital blue nevus in the clinical differential diagnosis of our patient. Giant congenital blue nevus of the scalp presents with local invasion of muscle, bone, and meninges in most cases, with a risk of malignant transfmation. We differentiated this clinical entity from CSMH by histopathologic examination, which revealed no spindle-shaped dendritic cells with melanin granules in the dermis, typical of blue nevus. Immunohistochemical staining of the biopsy specimen from our patient was also S100 negative. The recognition and differentiation of CSMH from congenital melanocytic nevus is particularly fundamental to avoid unnecessary excision of this benign lesion. It is imptant to emphasize that giant congenital blue nevus of the scalp should also be included in the differential diagnosis of CSMH when the lesion is located on the scalp. CSMH lesions located on the scalp may be igned and thus their frequency may be underestimated. References 1 de la Espriella J, Grossin M, Marinho E, et al. Smooth muscle hamartoma: anatomoclinical characteristics and nosological limits. Ann Dermatol Venereol 1993; 120: International Journal of Dermatology The International Society of Dermatology

3 Günlemez et al. Congenital smooth muscle hamartoma Case rept 3 2 Schmidt CS, Bentz ML. Congenital smooth muscle hamartoma: the imptance of differentiation from melanocytic nevi. J Craniofac Surg 2005; 16: Zvulunov A, Rotem A, Merlob P, et al. Congenital smooth muscle hamartoma. Am J Dis Child 1990; 144: Jhonson MD, Jacobs AH. Congenital smooth muscle hamartoma. Arch Dermatol 1989; 125: Knable A, Treadwell P. Pigmented plaque with hypertrichosis on the scalp of an infant. Pediatr Dermatol 1996; 13: Holst VA, Junkins-Hopkins JM, Elenitsas R. Cutaneous smooth muscle neoplasms: clinical features, histologic findings, and treatment options. J Am Acad Dematol 2002; 46: UNCORRECTED PROOF 2008 The International Society of Dermatology International Journal of Dermatology 2008

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