Familial peeling skin syndrome with eosinophilia: clinical, histologic, and ultrastructural study of three cases.

. Objective.-The clinicopathologic features of a rare familial form of congenital blister are analyzed to assess what factors could lead to the cutaneous split. Methods.-Three consanguinous newborn babies had a congenital ichthyosis with eosinophilia and elevated total immunoglobulin E. The type and level of the split were studied on skin biopsies performed on the first day of life. Results.-The level of the split was located within the corneocytes. Isolated eosinophilic granules were found at this site. Desmosomes and the dermoepidermal junction were intact. Blisters and eosinophilia resolved in 3 weeks, and there was no reccurrence during 4 and 6 years of follow-up in two of the patients. Conclusion.-Peeling skin syndrome is a rare blistering disorder of the newborn that should be recognized because it has a good prognosis. Eosinophils may play an important role in the cutaneous split of this congenital ichthyosis.