Atypical presentation of cutaneous lupus mucinosis.

OBJECTIVE: To describe the epidemiological, clinical, and serological features of cutaneous lupus mucinosis and its relationship to systemic lupus erythematosus as well as elucidate the histopathological features of cutaneous lupus mucinosis and describe how these features differ from tumid lupus erythematosus. DESIGN: Case review and assessment of the literature. SETTING: University academic setting. PARTICIPANT: One patient. RESULTS: The authors report a case of antinuclear antibody negative cutaneous lupus mucinosis in a patient without systemic involvement who responded to hydroxychloroquine and intralesional triamcinolone. A review of the literature found 30 reported cases of cutaneous lupus mucinosis, three of which were antinuclear antibody negative and the majority had systemic lupus erythematosus. The most common therapy reported is systemic corticosteroids; however, the patient described in this case had significant improvement of the lesions with only intralesional steroid injections and hydroxychloroquine. Considering the proportion of patients with cutaneous lupus mucinosis who progress to systemic lupus is uncertain, the authors suggest following these patients closely for evidence of multisystem disease. CONCLUSION: The authors report a case of antinuclear antibody-negative cutaneous lupus mucinosis in a patient without systemic lupus erythematosus who responded to hydroxychloroquine and intralesional triamcinolone. Given the rarity of this condition and reported association with systemic lupus erythematosus, it is important to follow these patients clinically for any signs or symptoms of systemic involvement.