Comèl-Netherton syndrome and peeling skin syndrome type B: overlapping syndromes or one entity?

Abstract

Background: A 42-year-old male patient suffering from the rare Comèl-Netherton syndrome is reported, and the symptoms are discussed together with the results of light and electron microscopic examinations.

Case report: Our case presented peeling skin syndrome type B with spontaneous, lifelong skin shedding with underlying erythema; however, with more careful studies, the hidden features of Comèl-Netherton syndrome were identified.

Conclusion: This is an example of a condition where there is no clear border between Comèl-Netherton syndrome and peeling skin syndrome type B, lending credence to a close relationship between the two syndromes.