On the basis of the histologic findings and clinical history a diagnosis of connective nevus was made. The occurrence of this syndrome in three members of a family is used to demonstrate the salient features of familial incidence, characteristic distribution patterns, … If concern for systemic associations, then need appropriate workup and referral. The term “connective tissue nevus” was coined by Gutmann in 1926. 1 , 2 They are often solitary but may be multiple. Histologically, large amounts of acid mucoploysaccharides (proteoglycans) were demonstrated in the superficial dermis. As far as we are aware, this is the first report of the onset of naevus mucinosus in an adult. Cesinaro AM (2003) "Connective tissue nevus" and a serendipitous S-100 discovery. The findings are very distinctive and most compatible with a fibroblastic connective tissue nevus. On J Dermatopathol 25: 86-87 el Fekih N et al (1993) A case for diagnosis: connective tissue nevi of the skin (hamartoma)]. Often incorrectly spelled “nevus sebaceous” Congenital, organoid epidermal nevus on scalp and face Malformed adnexal structures, increased risk of trichoblastomas, basal cell carcinoma, squamous cell carcinoma Sex hormone responsive: need prophylactic excision before puberty The entity was recognized by Sebastian de Feraudy and Christopher Fletcher where they reported a series of twenty-five cases of a seemingly distinctive lesion for which they use the appellation fibroblastic connective tissue nevus. They could present as part of systemic diseases or inherited disorders. Especially if concern for tuberous sclerosis, a Wood’s lamp may be helpful in screening for hypopigmented macules or patches. They are described as benign, firm, asymptomatic, skin-colored plaques composed of closely grouped papules with an orange peel–like surface texture. Naevus mucinosus appears to be a distinct type of connective tissue naevus which is characterized by an increase in proteoglycans. Each subtype can present as a solitary lesion or multiple lesions. Biopsies are suggested to be into subcutaneous tissue and include adjacent normal skin to compare with the suspected connective tissue nevus. ... nevus) removed from her left upper thigh under ... rich connective tissue is important in transmitting Fibroblastic connective tissue naevus typically presents in the first decade of life, often as a poorly defined plaque-like cutaneous thickening arising most commonly on the trunk and head/neck of girls.. Histology of fibroblastic connective tissue naevus. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Connective tissue nevi (CTN) are hamartomas of the dermis, with the 3 main components being collagen, elastin, and proteoglycans. FIG. 1.: Punch biopsy showed hyperpigmented epidermis and very bland, spindle cells in the dermis, few of them vertically oriented around an adnexal structure. 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