Document Type

Conference Proceeding

Publication Date


Publication Title

J Am Acad Dermatol


Patient history and physical: A 25-year-old African American female was admitted for painful oral erosions and widespread blistering lesions on the trunk and extremities which had been waxing and waning since age 12 years old. Disease flares requiring hospitalization had been continuing to occur multiple times annually in spite of numerous systemic immunosuppressive agents including chronic corticosteroids (resulting in Cushingoid features and insulin-dependent diabetes) in combination with cyclosporine, dapsone, tacrolimus, anakinra, rituximab, adalimumab, apremilast, mycophenolate mofetil, and intravenous immunoglobulin (IVIG) at various times. Adjunctive therapies including acyclovir and leuprolide had also proven ineffective. Physical examination revealed well-defined, targetoid macules with central vesiculation scattered upon all four extremities. Ill-defined, annular-appearing, hyperpigmented macules were observed on the trunk. Scattered, erythematous erosions were seen on the hard palate and buccal mucosa; in addition, the lips featured hemorrhagic crusts.

Biopsies: Punch biopsies of lesions obtained from the left arm revealed a brisk lymphocytic infiltrate at the dermoepidermal junction with necrotic keratinocytes observed at all levels of the epidermis.

Laboratory Data: Further work-up revealed negative PCR for EBV & CMV, negative ANA, negative HIV, negative Mycoplasma antibodies, and negative HSV-2 & HSV-2 antibodies.

Diagnosis: Bullous erythema multiforme. Clinical Course and Treatment: Patient was transitioned to oral tofacitinib 5 mg twice daily with corresponding complete resolution of active lesions. Disease activity has remained quiescent for over two years, which represents the longest period of time without a disease flare since the patient’s initial diagnosis during childhood.





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