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Introduction Demographics Causes Clinical features Complications Diagnosis Differential diagnoses Treatment Outcome
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Rowell syndrome is a rare entity consisting of erythema multiforme-like lesions coexisting with lupus erythematosus (LE) along with characteristic immunological findings. The acceptance of Rowell syndrome as a distinct entity is controversial and not widely acknowledged.
The syndrome was first described in 1963 by Rowell, Beck, and Anderson who reported four out of 120 patients with chronic discoid LE with erythema multiforme-like lesions on the arms, legs, face, and chest, coinciding with a speckled pattern of antinuclear antibody (ANA), positive rheumatoid factor, and a precipitating antibody to saline extract from human tissue (anti-Sj-T) [1].
Rowell syndrome is currently classified as a subtype of chronic cutaneous lupus erythematosus [2].
Rowell syndrome has been reported in patients with systemic and discoid lupus erythematosus. Of the 96 reported cases, 18 are from India [3].
The pathogenesis of LE involves genetics and environmental factors [7].
Rowell syndrome is characterised by erythema multiforme-like lesions in a patient with LE.
Complications are mostly due to systemic LE, such as haematological and renal involvement, or other conditions [3,19]. Stevens Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) has been reported [20].
Rowell syndrome has also been associated with:
The specific features of Rowell syndrome have been controversial [21,25-35].
It is currently considered to be a distinct type of chronic cutaneous LE with the following criteria [2].
1. Major criteria (all required)
a. Presence of chronic cutaneous LE
b. Erythema multiforme-like lesions (typical or atypical target lesions)
c. ≥1 positive speckled ANA (present in >90% [27,28]), anti-Ro/SSA, or anti-La/SSB antibodies
d. Negative direct immunofluorescence.
2. Minor criteria (one required)
a. Absence of infectious or pharmacological triggers
b. Lesions are not in acral or mucosal areas (the typical sites of erythema multiforme)
c. Presence of at least one additional criterion for systemic LE.
Histology shows a periadnexal lymphocytic infiltrate and periadnexal CD123+ plasmacytoid dendritic cells [32].
The differential diagnosis for Rowell syndrome includes [2,21,31,32,34]:
The treatment of Rowell syndrome includes the range of medicines used to treat LE, including:
Most patients with Rowell syndrome respond well to therapy.
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