CHAPTER 47 UVEODERMATOLOGIC SYNDROME 693
Ophthalmic signs: photophobia, diminished or absent papillary light reflexes, ble-
pharospasm, anterior uveitis, hyphema, chorioretinitis, conjunctivitis, retinal detach-
ment, iris bombe, glaucoma, blindness.Dermatologic signs: symmetric (often striking) depigmentation of the planum nasale,
perioral/oral (lips and oral mucosa), and periocular tissue; less frequently, scrotum,
vulva, anus, and footpads may be depigmented (Figures 47.1–47.4).Rarely, leukoderma and leukotrichia may become generalized.
Skin lesions may ulcerate and become crusted.
Meningoencephalitis is reported rarely.
DIFFERENTIAL DIAGNOSIS
Immune-mediated diseases: pemphigus complex, systemic lupus erythemato-
sus, cutaneous lupus erythematosus, bullous pemphigoid, vitiligo (idiopathic
leukoderma/leukotrichia), vasculitis.Neoplasia (cutaneous lymphoma) and numerous other inflammatory and infectious
skin diseases that can cause depigmentation.
DIAGNOSTICS
Biopsy: lichenoid interface granulomatous pattern with large histiocytes and pro-
nounced pigmentary incontinence; hydropic degeneration of the epidermal basal cell
layer rare.Evaluate the retina; often the first abnormal clinical feature (granulomatous panu-
veitis).
THERAPEUTICS
Aggressive and rapid initiation of immunosuppressive therapy is recommended to
prevent formation of posterior synechiae and secondary glaucoma, cataracts, or
blindness.Subconjunctival or topical glucocorticoids (i.e., 0.1% dexamethasone ophthalmic
solution q4h, dexamethasone 1–2 mg subconjunctivally) until the uveitis has
resolved.Cycloplegics: 1% atropine ophthalmic solution q6–24h.
Retinal examinations: most important means of monitoring progress; improvement
in dermatologic lesions may not reflect the retinal pathology.
Enucleation: sometimes recommended because of pain.
Drugs of Choice
Corticosteroids: initial high doses of prednisolone 1.1–2.2 mg/kg PO BID to q24h.
Azathioprine 1–2 mg/kg or 50 mg/m2 PO q24h.