Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease
characterized by linear deposition of IgA along the basement membrane zone. In
the last three decades, many different drugs have been associated with the
drug-induced form of the disease, especially vancomycin. We report a case of
vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. The
aim of this work is to emphasize the need to include this differential diagnosis
in cases of epidermal detachment and to review the literature on the subject and
this specific clinical presentation.
A 21-year-old man presented with a 4-year history of seizures, visual hallucinations, cognitive decline, and gait impairment. Neurologic examination revealed myoclonic jerks, ataxia, and retinitis pigmentosa. Axillary skin biopsy showed Lafora bodies (figure). Lafora disease, the most common progressive myoclonic epilepsy with adolescent onset, is characterized by cognitive decline, visual hallucinations, myoclonus, generalized seizures, and pathognomonic inclusion bodies of polyglucosan found in cells of the skeletal muscle, skin, and brain.
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