Rowell's syndrome: a rare but distinct entity in rheumatology

BMJ Case Rep. 2020 Sep 23;13(9):e235173. doi: 10.1136/bcr-2020-235173.

Abstract

Rowell's syndrome is a rare disorder characterised by an association of lupus erythematosus with erythema multiforme (EM)-like skin lesions. EM as the initial clinical presentation of systemic lupus erythematosus is also atypical and even rarer. We report the case of an 18-year-old girl admitted to our hospital with fever and polyarthralgia along with multiple discrete ill-defined target lesions with crust formation over forehead, cheek, external ears, scalp, upper chest and back (predominantly over sun-exposed areas) with ulceration over hard palate. Investigations revealed pancytopaenia, a positive rheumatoid factor, positive antinuclear antibody with a speckled pattern, anti-Smith antibody and strongly positive anti-Ro. Patient was diagnosed with Rowell's syndrome as per clinical and laboratory features. Majority of skin lesions including oral ulcerations subsided gradually after treatment with steroids and hydroxychloroquine.

Keywords: dermatology; rheumatology; systemic lupus erythematosus.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Antibodies, Antinuclear / blood
  • Antibodies, Antinuclear / immunology
  • Antipyretics / therapeutic use
  • Arthralgia / blood
  • Arthralgia / drug therapy
  • Arthralgia / immunology*
  • Drug Therapy, Combination / methods
  • Erythema Multiforme / blood
  • Erythema Multiforme / drug therapy
  • Erythema Multiforme / immunology*
  • Female
  • Fever / blood
  • Fever / drug therapy
  • Fever / immunology*
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / diagnosis*
  • Lupus Erythematosus, Systemic / drug therapy
  • Lupus Erythematosus, Systemic / immunology
  • Pancytopenia / blood
  • Pancytopenia / diagnosis
  • Pancytopenia / drug therapy
  • Pancytopenia / immunology*
  • Prednisolone / therapeutic use
  • Rheumatoid Factor / blood
  • Rheumatoid Factor / immunology
  • Syndrome
  • Treatment Outcome

Substances

  • Antibodies, Antinuclear
  • Antipyretics
  • Hydroxychloroquine
  • Rheumatoid Factor
  • Prednisolone