Similar to a postinfectious Stevens-Johnson syndrome, though more common in younger patients with notable mucosal findings and absent or minimal cutaneous findings
Clinical Manifestations
Prodrome of fever, cough, and malaise lasting 3 to 13 days (median 8 days)
Usually two or more mucosal surfaces involved, including oral (most common, in 95% of cases), ocular (next most common, in around 90%), urogenital (about 60%), and anal or esophageal surfaces (least common)
Oral: erosions, ulcers, vesicular or vesiculobullous lesions, or desquamation
Ocular: bilateral conjunctivitis (often purulent), conjunctival injection, or hyperemia
Urogenital: more commonly described in female patients
May have skin involvement, though absent in 1/3 and sparse in 1/2 of cases
Vesiculobullous lesions are most common, followed by targetoid, papular, macular, and morbilliform
May need biopsy, though on biopsy it is indistinguishable from SJS/TEN
Diagnosis
Evidence of an infectious trigger, with at least 2 of:
Non-contributory medication history
Erosive mucositis affecting 2 or more sites
Vesiculobullous lesions or atypical targets affecting less than 10% of body surface area
Evidence of an infectious trigger can include history of cough, fever, malaise, and arthralgias in the preceding 7 to 10 days, or signs of respiratory infection on clinical examination
Supporting features include prodromal symptoms in the preceding 7 to 10 days, or histology ruling out other diagnoses
Management
Not well studied
Supportive care: fluids, nutrition, and pain management
Low threshold for Dermatology consult to help with diagnosis and treatment
Consider Ophthalmology consult for patients with ocular involvement
Often receive antibiotics, particularly when considering a bacterial infectious trigger
