Abstract

Vogt-Koyanagi-Harada (VKH) disease is a rare multisystem autoimmune disorder affecting melanin-containing tissues.  Clinical manifestations of VKH are caused by autoimmune response directed against melanocytes in target tissues with well-documented ophthalmologic, neurologic, and cutaneous findings. We present a 20 year old male patient with a known history of VHK syndrome with associated uveitis, vitiligo, and alopecia, who presented with a new rash to the bilateral shins and dorsal forearms. Clinically the rash was described as erythematous to violaceous, shiny, and scaly, composed of 6-8 mm papules coalescing into plaques. Punch biopsy revealed a non caseating granulomatous reaction distinctively abutting the dermal-epidermal junction and focally clustering around adnexal structures in the dermis. Additionally, a mild associated lymphocytic infiltrate was noted. Previously described cutaneous manifestations of VKH disease have been mostly limited to the finding of a mild lymphocytic infiltrate in the dermis. We describe here a distinct cutaneous granulomatous dermatitis that is similar to the granulomatous inflammation classically found in the chronic recurrent stage of uveitis in VKH disease.