Abstract

Rowell syndrome is characterized by erythema multiforme (EM)-like cutaneous lesions and lupus erythematosus (LE). The classification of Rowell syndrome remains controversial, due to different diagnostic criteria and overlapping clinical and histologic findings between EM and cutaneous LE. We report 3 cases of Rowell syndrome and discuss unifying features that may help clarify this diagnosis. A retrospective review was conducted over 5 years to identify patients with Rowell syndrome. Demographic, clinical, serologic, and histologic information were obtained. A total of 4 biopsies from 3 patients were identified. All patients were female and of Black or Asian race, age ranging 34-55 years. Two of 3 patients had a known history of systemic LE. All patients presented with targetoid, dusky erythematous papules coalescing into plaques with superficial erosions. Two patients had mucosal involvement. All patients had positive serology for ANA (1:2560) in either speckled (66%) or homogenous (33%) patterns. The most common serologic abnormalities were positive SSA/La (66%), SSB/Ro (66%), anti-smith (66%), and anti-RNP (66%) antibodies. Complement levels were low in only 1 patient, and proteinuria was present in all patients. All patients underwent biopsy and direct immunofluorescence (DIF). The unifying histologic features were epidermal necrosis, dyskeratotic keratinocytes, and parakeratosis. Distinct features of LE, including dermal mucin and periadnexal lymphocytic inflammation and/or CD123+ plasmacytoid dendritic cells, were not consistently observed. Direct immunofluorescence studies were negative in all patients. Dermatopathologists must be aware of the combination of histologic, immunofluorescence, clinical, and serologic features that can lead to the diagnosis of Rowell syndrome.

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