Alopecia areata in a patient with candidiasis-endocrinopathy syndrome: unsuccessful treatment trial with diphenylcyclopropenone.

The association of chronic mucocutaneous candidiasis with endocrine dysfunction (candidiasis-endocrinopathy syndrome, CES) is a complex immune disorder, in which autoimmunity is found besides selective impairment of cellular immunity. We report a case of CES associated with alopecia areata. The histology of a scalp biopsy demonstrated minor inflammation with profound atrophy of the hair follicles and sclerotic whorls in the region of the hair papillae. A treatment trial with topical immunotherapy using diphenylcyclopropenone was unsuccessful, which might be explained by both long-standing alopecia areata and the underlying immune deficiency with an impaired delayed hypersensitivity reaction. Furthermore, the patient presented with agenesis of the spleen. Atrophy of the spleen in patients suffering from CES has formerly been described in the literature, but not asplenia. Its significance in the setting of the syndrome is discussed.