Rowell syndrome complicated with macrophage activation syndrome in a child

Author:

Aguirre-Martinez I1,Vélez-Tirado N2,García-Romero M T1,Rodríguez-Lozano A L2,Corcuera-Delgado C T3,Yamazaki-Nakashimada M2,Rivas-Larrauri F2ORCID

Affiliation:

1. Dermatology Department, Instituto Nacional de Pediatría, Mexico City, Mexico

2. Clinical Immunology Department, Instituto Nacional de Pediatría, Mexico City, Mexico

3. Pathology Department, Instituto Nacional de Pediatría, Mexico City, Mexico

Abstract

Rowell syndrome (RS) is a rare disease characterized by the association of systemic lupus erythematosus (SLE) or cutaneous lupus with lesions similar to erythema multiforme and the presence of autoantibodies including ANA, SSA, SSB, or rheumatoid factor. Due to the low incidence of this disease, the epidemiology of RS is not clear. So far there are 95 cases reported in the literature; of these, only seven cases are pediatric patients. Macrophage activation syndrome (MAS) is an increasingly recognized complication of SLE, although its true prevalence in childhood is still unknown. We describe a unique pediatric patient with RS who developed MAS.

Publisher

SAGE Publications

Subject

Rheumatology

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