Fibroelastic connective tissue nevus D48.-

Feraudy and Fletcher 2012

Rare, slow-growing, nodular or plate-like connective tissue hamartoma that can become clinically evident as early as infancy.

w:m=6:4 (not representative for n=25)

A larger series (n=25) showed an age distribution between 1.5 months and 58 years. In the meantime 3 cases with congenital manifestation have been described (Bouaoud J et al. 2018).

Trunk, head, neck, extremities (Pennacchia I et al. 2017).

Symptomless, moderately firm, skin-coloured or brownish, plate-like or nodular neoplasm, which can be displaced over the base and whose size ranges from 0.3-2.0 cm.

Below a papillomatous epidermis in the reticular dermis there are fuzzy, fascicularly arranged proliferates of myofibroblasts (the cells stain positively for CD34 and focally for smooth muscle actin; no increase of proliferation factors). The proliferates spread to the subcutaneous fatty tissue (de Feraudy S, Fletcher CD 2012). There are no indications of malignancy.

Hypertrophic scar, dermatofibroma, dermatomyofibroma, piloleiomyoma, plaque-like CD34-positive dermal fibroma, lipofibromatosis, superficial desmoid fibromatosis.

The cases observed so far showed neither recurrence nor metastasis (Pennacchia I et al. 2017).

1. Bouaoud J et al (2018) Congenital fibroblastic connective tissue nevi: Unusual and misleading presentations in three infantile cases. Pediatric Dermatol 35:644-650.
2. de Feraudy S, Fletcher CD (2012) Fibroblastic connective tissue nevus: a rare cutaneous lesion analyzed in a series of 25 cases. On J Surg Pathol 36:1509-155.
3. Pennacchia I et al (2017) Fibroblastic connective tissue nevus: Clinicopathological and immunohistochemical study of 14 cases. J Cutan Pathol 44:827-834.