# Skin Fragility-Woolly Hair Syndrome (SFWHS): A Case Report

## TL;DR

This case report describes a patient with Skin Fragility-Woolly Hair Syndrome, a rare inherited disorder affecting skin and hair structure.

## Contribution

The report adds a new clinical case to the limited literature on this extremely rare desmosomal disorder.

## Key findings

- The patient exhibited skin fragility, woolly hair, and palmoplantar keratoderma consistent with SFWHS.
- The case highlights the early childhood onset of symptoms including fragile skin and nail abnormalities.

## Abstract

Skin fragility, palmoplantar keratoderma, and characteristic woolly hair are defining features of skin fragility-woolly hair syndrome (SFWHS), an extremely rare inherited disorder. This condition is classified within the group of desmosomal disorders, which are associated with compromised structural integrity of the skin and hair. Clinically, affected individuals often present with hair fragility, fragile skin, recurrent erosions, nail abnormalities, and varying degrees of palmoplantar thickening beginning in early childhood. In this case report, we describe a patient with this extremely rare syndrome.