Clinical Images: Targeting Rowell syndrome in systemic lupus erythematosus

Rowell syndrome was originally proposed as a unique clinical pattern by Rowell et al in 1963.1 It describes the development of erythema multiforme (EM)–like eruptions in individuals with underlying lupus erythematosus (LE), representing an uncommon but characteristic overlap of these two conditions. Over the decades, several diagnostic criteria have been proposed to better characterize this association between lupus and EM-like eruptions. Although its definition remains debated, the revised criteria proposed by Zeitouni et al are commonly referenced.2 These criteria require the presence of all three major components (LE, EM-like lesions, and a speckled antinuclear antibody pattern) together with at least one minor criterion, including anti-Ro/SSA or anti-La/SSB positivity, rheumatoid factor, or chilblain-like lesions. A previously healthy 60-year-old man developed a two-week generalized, pruritic, painful skin rash. Physical examination revealed well-demarcated, edematous, and erythematous macules, papules, and plaques, with typical/atypical targetoid lesions over the face (A), trunk (B), and limbs, along with acral violaceous papules (C). Palms, soles, and mucosa were spared. The presence of targetoid lesions was highly suggestive of EM, prompting an investigation into its common triggers. However, a thorough investigation for its common triggers proved unrevealing. A comprehensive review excluded typical infectious etiologies, particularly recent herpes simplex virus or Mycoplasma pneumoniae infection. Furthermore, there was no history of exposure to culprit medications, such as nonsteroidal anti-inflammatory drugs, antibiotics, or anticonvulsants. With no identifiable cause, the possibility of an autoimmune etiology was raised. Evaluation for a systemic autoimmune disease revealed speckled antinuclear antibody (1:1,280), anti–double-stranded DNA antibody, anti-Ro-52, and rheumatoid factor positivity; low complement levels; and low-level proteinuria. Histopathologic analysis of skin from the upper back demonstrated vacuolar interface dermatitis with dyskeratosis and subepidermal separation. CD123 immunohistochemical staining revealed plasmacytoid dendritic cell infiltration in the dermal, intraepidermal, and perieccrine regions.3, 4 A diagnosis of systemic LE presenting with features of Rowell syndrome was established.5 This rare entity is defined by the development of EM-like lesions in patients with lupus. The acral violaceous papules were identified as chilblain lupus (perniosis), a cutaneous finding frequently associated with this syndrome. Treatment with hydroxychloroquine (400 mg/day, approximately 5 mg/kg/day), methotrexate (15 mg/wk), and prednisolone (initiated at 0.5 mg/kg/day and subsequently tapered to a maintenance dose of 5 mg/day) led to complete clinical resolution of the cutaneous lesions within four months. Given its rarity, Rowell syndrome carries important clinical significance, as EM-like eruptions in lupus may be misinterpreted as infectious or drug-induced EM. Awareness of this association helps prevent diagnostic delay and underscores the educational value of this case. The data that support the findings of this study are available from the corresponding author upon reasonable request. Disclosure Form: Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.