FAMILIAL PEELING SKIN SYNDROME WITH EOSINOPHILIA - CLINICAL, HISTOLOGIC, AND ULTRASTRUCTURAL-STUDY OF 3 CASES

Objective.--The clinicopathologic features of a rare familial form of congenital blister are analyzed to assess what factors could lead to t he cutaneous split. Methods.--Three consanguinous newborn babies had a congenital ichthyosis with eosinophilia and elevated total immunoglob ulin 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 w as located within the corneocytes. Isolated eosinophilic granules were found at this site. Desmosomes and the dermoepidermal junction were i ntact. Blisters and eosinophilia resolved in 3 weeks, and there was no recurrence during 4 and 6 years of follow-up in two of the patients. Conclusion.--Peeling skin syndrome is a rare blistering disorder of th e newborn that should be recognized because it has a good prognosis. E osinophils may play an important role in the cutaneous split of this c ongenital ichthyosis.