Congenital Atrophic Plaque: Fibroblastic Connective Tissue Nevus

Abstract

Fibroblastic connective tissue nevus (FCTN) is a rare, benign, dermal mesenchymal hamartoma that affects children. We report a 15-year-old boy with a congenital FCTN and describe the clinical, dermatoscopic, and histopathologic features.

Congenital fibroblastic connective tissue nevi: Unusual and misleading presentations in three infantile cases

“…In patient 1 there was an uncommon infiltration of the entire thickness of the subcutis with focal infiltration of the periosteum, which has never been reported. In patients 1 and 2, we report a superficial infiltration into the subcutis; this infiltration is also reported in 31 (67%) cases from the literature . In addition to CD34 positivity of the fibroblastic lineage, all other markers for spindle cells such as S100P, SMA, desmin, and myogenin were negative, except for a moderate focal SMA positivity in patient 3.…”

“…The overlying epidermis was normal histologically in all the cases we report, as opposed to the papillomatous epidermic pattern described in several cases . As in all cases reported (n = 46) in the literature, we did not find cellular atypias or mitoses; adnexal structures were entrapped but always spared . In patient 1 there was an uncommon infiltration of the entire thickness of the subcutis with focal infiltration of the periosteum, which has never been reported.…”

“…FCTN present as slowly growing, painless plaques and nodules of the trunk, head, and neck . In our cases, no family history of CTN, osteopoikilosis, cardiovascular disease, or any other syndrome was reported .…”

“…In the second largest series available in the literature (14 patients), age ranged from 1 to 56 years . Other smaller series and case reports have described patients at various ages: one case at 1 month old; one case at 5 months old; one case at 9 years old; three cases at 10, 24, and 32 years old; and one case at 15 years old . FCTN may be more frequent in women (n = 25 cases) than men (n = 20 cases) …”

“…The clinical and histologic differential diagnosis with FCTN may be difficult. Even if entire dermis involvement is in favor of FCTN, there may be overlaps between these entities. Molecular analyses (in situ hybridization) are recommended in all cases …”