Histopathologic findings in the alopecia associated with Vogt-Koyanagi-Harada disease

Vogt-Koyanagi-Harada (VKH) disease is a rare autoimmune disease primarily involving pigmented structures. The commonly associated alopecia usually precedes leukodermic changes seen in the hair and skin.

We present a 46-year-old oriental woman with VKH disease who developed diffuse alopecia followed by the regrowth of nonpigmented hairs. The biopsy specimens showed a peribulbar mononuclear infiltrate with increased telogen/catagen:anagen follicles. The most prominent additional histologic finding was melanin pigment release from the matrix into the dermal papillae, fibrous tracks, and surrounding perifollicular sheaths. The peribulbar mononuclear cells showed diffuse immunohistochemical staining of approximately 80 to 90% of the cells for CD3, CD45RO. More than 50% of the mononuclear cells showed positive staining for CD4, whereas approximately 10 to 20% showed staining for TIA with TIA+ cells within the follicular epithelium.

Although the histologic features seen in the alopecia associated with VKH are consistent with alopecia areata, the prominent pigment release does suggest that the prime target are the melanocytes and that keratinocytes may be secondarily involved.