Clinical Case Vignettes

Rowell’s syndrome: A rare presentation of childhood lupus

Sham Santhanam1*, Madeshwaran M2, Rajeswari Sankaralingam3


Author Affiliations

1Postgraduate, Postgraduate, Department Of Rheumatology, Madras Medical College, Chennai, India

2Department of Rheumatology, Madras Medical College, Chennai, India

3Professor & HOD, Department of Rheumatology, Madras Medical College, Chennai, India


* Correspondence: Dr. Sham S


IJRCI. 2014;2(1):V4.

Received: 3 December 2014, Accepted: 16 December 2014, Published: 24 December 2014



A 11-year-old girl presented to the clinic with intermittent fever, multiple erythematous maculopapular lesions (erythema multiforme (EM) like lesions) of targetoid appearance over face, trunk, and limbs (Fig. 1), and a large palatal ulcer (Fig. 2). She was tested positive for ANA by Hep-2 (speckled pattern) and for anti-Ro, anti-nucleosome, anti-histone, and anti-dsDNA antibodies by immunoblot. She had reduced complement levels (C3, C4) and tested negative for rheumatoid factor and viral markers. Further evaluation has concluded that the patient met the diagnostic criteria for Rowell’s syndrome.


In 1963, Rowell et al. described the syndrome as the presence of discoid lupus erythematosus with erythema multiforme and with a positive serology for rheumatoid factor, speckled ANA, and precipitating antibodies to saline extract of human tissue (anti-SjT identical to anti-Ro and  anti-La antibodies).1 Lee et al. (1995) have included chilblains as one of the characteristic features of Rowell’s syndrome.2 In 1997, Zeitouni et al. redefined the major and minor diagnostic criteria for Rowell’s syndrome as given below:3

The major criteria include

•             Systemic lupus erythematosus (SLE), discoid LE (DLE) or subacute cutaneous LE (SCLE)

•             EM-like lesions with or without mucosal involvement

•             Speckled pattern of ANA


The minor criteria are:

•             Chilblains

•             Positive anti-Ro or anti-La antibodies

•             A reactive rheumatoid factor


Fig 1: Targetoid lesion over right cheek



Fig 2: Palatal ulcer



Competing interests

The authors declare that they have no competing interests.



1.     Manzi Rowell NR, Beck NJ, Anderson JR. Lupus erythematosus and erythema multiforme-like  lesions. Arch Dermatol1963; 88: 176–80.

2.     Lee S, Schloss E, Kowichi J. Rowell’s syndrome: a case report of subacute cutaneous lupus erythematosus and erythema multiforme. Can J Dermatol 1995; 7: 807–10.

3.     Zeitouni NC, Funaro D, Cloutier RA, Cloutier RA, Gagne E, Claveau J. Redefining Rowell’s syndrome. Br J Dermatol 2000; 142: 343–6.