Substance P in Keratosis Follicularis Spinulosa Decalvans

    September 2015 in “ JAAD case reports
    Isabella Doche, Maria Hordinsky, George L. Wilcox, Neusa Yuriko Sakai Valente, Ricardo Romiti
    Studysummary This article reviews the characteristics and inheritance patterns of keratosis follicularis spinulosa decalvans, but it does not present new clinical findings, noting the disease's complex and poorly understood pathogenesis.
    Our plain-language summary. Not medical advice or a treatment recommendation. Consult a qualified healthcare professional before changing treatment. Full disclaimer
    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.
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