Substance P in Keratosis Follicularis Spinulosa Decalvans

The study described a 10-year-old Brazilian girl with keratosis follicularis spinulosa decalvans (KFSD), a rare inherited disorder characterized by scarring alopecia and keratosis pilaris. The patient exhibited severe pruritus and increased levels of Substance P (SP) in the scalp, particularly in the most affected areas. Histopathologic analysis showed perifollicular lymphocytic inflammation and fibrosis. The findings suggested that SP might play a role in the pruritus and inflammation associated with KFSD, indicating a need for further research into neurogenic inflammation and potential treatments targeting SP, such as NK-1R antagonists.