Image Quiz: Dermatology—The case of the blind Akita
Uveodermatologic syndrome is correct!
Also known as Vogt-Koyanagi-Harada-like syndrome (VKH), this disorder is associated with autoantibodies that target melanin and related proteins, resulting in granulomatous uveitis and depigmentation of hair and skin, most commonly of the nose, lips, and eyelids. Lesions may also occur on the scrotum, vulva, perianal area, footpads, and oral cavity. Lesions rarely become ulcerated and crusted, and, in this case, cytologic examination of crusted lesions demonstrated that the autoimmune inflammation was complicated by a severe secondary bacterial infection. Histologic examination of skin biopsy samples confirmed the characteristic VKH features of a lichenoid interface dermatitis composed primarily of macrophages with fewer neutrophils, lymphocytes, and plasma cells.To prevent blindness, early and aggressive therapy for the uveitis is needed, and consultation with a veterinary ophthalmologist is recommended. In addition to topical ophthalmic steroidal medications and subconjunctival depot corticosteroid injections, systemic immunosuppressive therapy with a combination of prednisone and azathioprine is usually prescribed. Therapy is required lifelong to maintain remission, and the prognosis is fair to guarded; if the uveitis is not treated early or aggressively enough, then glaucoma, permanent blindness, or both, can result.