Case Report

Utilization of Rituximab for Refractory Rowell Syndrome

Table 1

Summary of proposed diagnostic criteria for Rowell syndrome by different authors.

Diagnosis

Rowell et al. [5]All features required
 LE
 EM-like lesions (with the absence of any known precipitating factors)
 Speckled pattern of ANA
 Anti-SJT antibody (anti-La/SSB)
 Positive rheumatoid factor

Lee et al. [10]All features required
 LE
 EM-like lesions (with the absence of any known precipitating factors)
 Chilblains
 Speckled pattern ANA
 Anti-La/SSB antibody
 Positive rheumatoid factor

Zeitouni et al. [1]3 major + 1 minor criteria
 Major criteria
  SLE, DLE, or SCLE
  EM-like lesions (with/without involvement of mucous membranes)
  Speckled pattern ANA
 Minor criteria
  Chilblains
  Anti-Ro/SSA or anti-La/SSB
  Positive rheumatoid factor

Torchia et al. [8]Presence of all 4 major + 1 minor criteria
 Major criteria
  Presence of CCLE (DLE and/or chilblain)
  Presence of EM-like lesions (typical or atypical targets)
  At least one positivity among speckled ANA, anti-Ro/SSA, and anti-La/SSB antibodies
  Negative DIF on lesional EM-like lesions
 Minor criteria
  Absence of infectious or pharmacologic triggers
  Absence of typical EM location (acral and mucosal)
  Presence of at least one additional ARA criterion for diagnosis of SLE besides discoid rash and ANA and excluding photosensitivity, malar rash, and oral ulcers