Development of sinus tracts within a connective tissue nevus

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Conference Proceeding

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J Am Acad Dermatol


Background: Connective tissue nevi (CTN) are dermal hamartomas characterized by abnormal proliferation of components of the extracellular dermal matrix, specifically collagen and elastin and/or proteoglycans. CTN present as firm, flesh-colored papules or nodules that coalesce into plaques. They can be sporadic or can be seen in genetic disorders including Buschke-Ollendorff syndrome, Proteus syndrome, tuberous sclerosis, and multiple endocrine neoplasia type 1. We report a case of development of multiple sinus tracts within a connective tissue nevus and our treatment approach. Case report: A 13-year-old Caucasian male with velocardiofacial syndrome presented for evaluation of a lesion on his right posterior neck of approximately 6 years duration. Our patient had a history of cleft palate repair, myringotomy tubes, failure to thrive and reflux but was now in generally good health. On exam, he had a rugated, palm-sized, skin colored to slightly orange plaque on the posterior neck. A punch biopsy demonstrated papillomatosis, mild hyperkeratosis with thickened collagen bundles, and increased numbers of fibroblasts in the dermis. Elastic tissue stain showed decreased staining of lesional skin compared to normal skin, consistent with a diagnosis of CTN. Four months later, several draining pustules and a tender thumb print sized area developed within the CTN. Wound cultures ×2 were negative. The patient remained afebrile throughout and demonstrated no improvement with oral antibiotics and warm soaks. During a subsequent clinic visit, a sterile probe was used to explore the lesion and we determined that several interconnecting sinus tracts had developed within the CTN (see Fig). Treatment consisted of surgical deroofing of the sinus tracts followed by scarification with 100% trichloroacetic acid. Discussion: CTN are considered benign lesions however there are cases in the literature of extensive presentations demonstrating confluence of papules resulting in involvement of an entire limb and ulcerative lesions. To our knowledge there have been no reports of sinus tract development within CTN and this is the first report of sinus tract development within a connective tissue nevus in a patient with velocardiofacial syndrome.





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