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 recurrence 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.