Antibodies against hair follicles are associated with alopecia totalis in autoimmune polyendocrine syndrome type I

In the autosomal recessively inherited autoimmune polyendocrine syndrome ty pe I (APS I) patients have autoantibodies directed against several endocrin e and nonendocrine organs. Alopecia areata is present in about one-third of the patients and usually in the more severe forms, alopecia universalis or totalis. Sera from 39 patients with APS I, diluted 1:150, were used in ind irect immunofluorescence staining of cryo-sections from normal human scalp. Two hair follicle staining patterns were observed. A cytoplasmic staining of the differentiating matrix, cuticle, and cortex keratinocytes in the ana gen hair follicle was seen in five (13%) APS I sera. All these five patient s had alopecia totalis, representing 63% of the eight patients with alopeci a totalis (p<0.0001). Furthermore, four (10%) of the APS I sera stained the nuclei of the melanocytes in the hair follicle. Two of these patients had vitiligo. None of 20 healthy control sera stained the keratinocyte cells or the melanocyte nuclei. These data show that many patients with APS I have high-titer autoantibodies directed against the anagen matrix, cuticle, and cortex keratinocytes and a melanocyte nuclear antigen, and also that the ha ir follicle keratinocyte staining is associated with alopecia, especially a lopecia totalis. This study emphasizes the role of the differentiating anag en keratinocytes as an important structure in the autoimmune etiology of al opecia, both in APS I and at least in a subgroup of patients with alopecia areata unrelated to APS I.