Connective tissue nevi: An entity revisited - 13/07/12
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.
Le texte complet de cet article est disponible en PDF.Key words : Buschke-Ollendorff syndrome, collagenoma, connective tissue nevi, elastoma, monomelic infiltration, osteopoikilosis
Plan
Funding sources: None. |
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Conflicts of interest: None declared. |
Vol 67 - N° 2
P. 233-239 - août 2012 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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