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Presentation

Rowell Syndrome vs. Erythema Multiforme: A Lupus-Associated Diagnostic Debate

Description

This talk presents a diagnostic debate about a 72-year-old woman with recurrent painful targetoid, crusted, and ulcerated plaques on the face, trunk, and extremities, with no mucosal involvement. Her eruption had been recurring since 2022, repeatedly improving with prednisone, and she also had arthralgia, synovitis, and arthritis. Initial concern included a phototoxic drug eruption or erythema multiforme, but labs showed a high ANA, positive SSA/Ro, low complement, and elevated CRP, supporting subacute cutaneous lupus. Biopsy showed interface dermatitis with marked epidermal necrosis, peri-adnexal lymphocytic inflammation, and rare eosinophils; direct immunofluorescence was negative, and pathology was read as most compatible with erythema multiforme. Review of prior photos and biopsies suggested EM-like lesions superimposed on lupus, raising the question of Rowell syndrome versus SCLE with EM-like lesions versus true erythema multiforme. The speaker explains that older Rowell criteria are restrictive, newer criteria and literature support grouping these lupus-associated EM-like eruptions together, and clinical pattern, serology, triggers, and histology help distinguish them from true EM. The patient improved rapidly with corticosteroids, hydroxychloroquine, and photoprotection, later requiring anifrolumab, and has remained clear for over a year. The main take-home message is that these entities may overlap, but recognizing underlying lupus is essential for treatment and follow-up.

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Conclusions

The patient’s recurrent targetoid and bullous eruption was ultimately most consistent with lupus-related erythema multiforme-like disease rather than a phototoxic drug eruption or classic erythema multiforme.
Rowell syndrome and related entities such as SCLE/ACLE with EM-like lesions may represent overlapping manifestations of cutaneous lupus rather than completely separate diseases.
Clinical morphology, serologies, and histopathology together are more useful than eosinophils alone for distinguishing lupus-related EM-like eruptions from true erythema multiforme.
A speckled ANA, anti-Ro/SSA positivity, low complement, and absence of infectious or pharmacologic triggers support an underlying lupus driver.
Biopsies showing interface dermatitis with epidermal necrosis and periadnexal lymphocytic inflammation favor lupus-related disease, while direct immunofluorescence and classic EM patterns help separate it from true erythema multiforme.
Because the terminology is debated, the practical management focus should be on recognizing cutaneous lupus and treating it appropriately rather than fixating on the label.
Corticosteroids, hydroxychloroquine, photoprotection, and escalation to additional lupus-directed therapy can lead to substantial improvement and long-term control.
The case suggests that some eruptions previously called drug reactions may actually be lupus-associated flares, underscoring the need for careful longitudinal review and follow-up.

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Torchia D et al. J Am Acad Dermatol. 2012.
Source: N Engl J Med. 2012
Herold M et al. J Am Acad Dermatol. 2019.