Connective tissue nevi: an entity revisited.
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.


CONCLUSIONS
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.
Department of Pediatric Dermatology, Hôpital Necker-Enfants Malades (AP-HP), Paris, France.
Anne
A
Saussine
Karine
K
Marrou
Phillippe
P
Delanoé
Nathalie
N
Bodak
Dominique
D
Hamel
Arnaud
A
Picard
Bruno
B
Sassolas
Yves
Y
de Prost
Martine
M
Lemerrer
Sylvie
S
Fraitag
Christine
C
Bodemer
D000328:Adult; D017668:Age of Onset; D001706:Biopsy; D002675:Child, Preschool; D003238:Connective Tissue / Q000473:pathology; D003240:Connective Tissue Diseases / Q000473:pathology*; D020405:Dermis; D003937:Diagnosis, Differential; D005260:Female; D005347:Fibroblasts / Q000473:pathology; D006222:Hamartoma / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D009506:Nevus / Q000473:pathology*; D010023:Osteopoikilosis / Q000473:pathology*; D012189:Retrospective Studies; D012873:Skin Diseases, Genetic / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*; D012883:Skin Ulcer / Q000473:pathology