Connective tissue nevi: an entity revisited.

Abstract:

BACKGROUND:Connective tissue nevi (CTN) may be isolated, either sporadic or hereditary, or syndromic as in the Buschke-Ollendorff syndrome. Few publications have addressed the variable clinical and histopathologic expression of these benign hamartomas. OBJECTIVE:We sought to characterize the clinical and histopathologic features of CTN and to highlight a spectrum of clinical disease. METHODS:We carried out a retrospective study of cases selected after strict clinical and histopathologic confirmation of the diagnosis. RESULTS:A total of 33 patients with CTN were included. The average age of onset was 2 years. Three clinical forms were distinguished: type A with lesions at a single site, with one case presenting as an ulcerated infiltrated plaque; type B with two or more sites of involvement; and type C with unusually severe infiltration with functional impairment of a limb. Histopathologic examination of lesional biopsy specimens showed 10 collagenomas, one elastoma, 18 mixed CTN, and an increased number of fibroblasts in 4 cases. No correlation between clinical type and histopathologic findings was observed. LIMITATION:This was a descriptive case series. CONCLUSIONS:CTN comprise a clinical spectrum ranging from isolated papules to unusually severe aggressive plaques with monomelic involvement. The histopathologic features are heterogeneous and include a newly described variant, which we name "cellular CTN" because of the increased number of fibroblasts.

journal_name

J Am Acad Dermatol

authors

Saussine A,Marrou K,Delanoé P,Bodak N,Hamel D,Picard A,Sassolas B,de Prost Y,Lemerrer M,Fraitag S,Bodemer C

doi

10.1016/j.jaad.2011.08.008

subject

Has Abstract

pub_date

2012-08-01 00:00:00

pages

233-9

issue

2

eissn

0190-9622

issn

1097-6787

pii

S0190-9622(11)00910-8

journal_volume

67

pub_type

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