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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.