On the basis of the histologic findings and clinical history a diagnosis of connective nevus was made. As far as we are aware, this is the first report of the onset of naevus mucinosus in an adult. 1 , 2 They are often solitary but may be multiple. Start studying Histology Lippincott Chapter 3 (Connective Tissue). The latter have been reported as dermatofibrosis lenticularis disseminata, juvenile elastoma, and connective-tissue nevus. Histologically, large amounts of acid mucoploysaccharides (proteoglycans) were demonstrated in the superficial dermis. The occurrence of this syndrome in three members of a family is used to demonstrate the salient features of familial incidence, characteristic distribution patterns, … They could present as part of systemic diseases or inherited disorders. Each subtype can present as a solitary lesion or multiple lesions. 1.: Punch biopsy showed hyperpigmented epidermis and very bland, spindle cells in the dermis, few of them vertically oriented around an adnexal structure. Cesinaro AM (2003) "Connective tissue nevus" and a serendipitous S-100 discovery. 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 FIG. They are described as benign, firm, asymptomatic, skin-colored plaques composed of closely grouped papules with an orange peel–like surface texture. 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. On J Dermatopathol 25: 86-87 el Fekih N et al (1993) A case for diagnosis: connective tissue nevi of the skin (hamartoma)]. The findings are very distinctive and most compatible with a fibroblastic connective tissue nevus. Histology is highly characteristic, with thick interwoven elastic fiber tracts between normal collagen bundles. The lesions typically present in the first Connective tissue nevi (CTN) are hamartomas of the dermis, with the 3 main components being collagen, elastin, and proteoglycans. The term “connective tissue nevus” was coined by Gutmann in 1926. 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. 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