Etiology • A complex mucocutaneous disease affecting two or more
mucosal sites simultaneously • Most common trigger: antecedent recurrent herpes simplex
infection • Infection with Mycoplasma also may serve as a trigger. • Medications may serve as initiators in some cases. • Sometimes referred to as “erythema multiforme major”
Clinical Presentation • Labial vermilion and anterior portion of oral cavity usually
affected initially • Early phase is macular followed by erosion, sloughing, and
mucosal sites simultaneously • Most common trigger: antecedent recurrent herpes simplex
infection • Infection with Mycoplasma also may serve as a trigger. • Medications may serve as initiators in some cases. • Sometimes referred to as “erythema multiforme major”
Clinical Presentation • Labial vermilion and anterior portion of oral cavity usually
affected initially • Early phase is macular followed by erosion, sloughing, and
painful ulceration • Lip ulcers appear crusted and hemorrhagic. • Pseudomembrane; foul-smelling presentation as bacterial
colonization supervenes • Posterior oral cavity and oropharyngeal involvement leads to
odynophagia, sialorrhea, drooling • Eye (conjunctival) involvement may occur. • Genital involvement may occur. • Cutaneous involvement may become bullous. • Iris or target lesions are characteristic on skin.
Microscopic Findings • Subepithelial separation with basal cell liquefaction • Intraepithelial neutrophils • Epithelial and connective tissue edema • Perivascular lymphocytic infiltrate
Diagnosis • Usually made on clinical grounds • Histopathology is not diagnostic.
Differential Diagnosis • Pemphigus vulgaris • Paraneoplastic pemphigus • Mucous membrane (cicatricial) pemphigoid
• Bullous pemphigoid • Acute herpetic gingivostomatitis • Stomatitis medicamentosa
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Treatment • Hydration and local symptomatic measures • Topical compounded oral rinses • Systemic corticosteroid use controversial • Recurrent, virally associated cases may be reduced in frequency
with use of daily, low-dose antiviral prophylactic therapy (acyclovir, famciclovir, valacyclovir).
• May require admission to hospital burn unit
Prognosis • Good; self-limiting usually • Recurrences not uncommon