Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Print this page Email this page Users Online: 1879

 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 60-63

An interesting case of systemic lupus erythematosus – Rowell's syndrome


Department of Medicine, Kurnool Medical College, Kurnool, Andhra Pradesh, India

Date of Web Publication20-Mar-2017

Correspondence Address:
Vidyasagar Kekathi
Flat No. 207, Dheeraj Enclave, Saptagiri Nagar, Kurnool - 518 002, Andhra Pradesh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_127_16

Rights and Permissions
  Abstract 

Rowell's syndrome is a rare disease consisting of systemic lupus erythematosus associated with erythema multiforme-like lesions. Here, we are reporting a patient who presented with a history of erythema multiforme-like eruption with systemic lupus erythematosus in the form of a malar rash with unusual laboratory and immunological findings consistent with Rowell's syndrome.

Keywords: Anti La antibodies, anti Ro antibodies, erythema multiforme, Rowell's syndrome, systemic lupus erythematosus


How to cite this article:
Siddhavatam C, Kekathi V, Saifullah QS, Kakarla S. An interesting case of systemic lupus erythematosus – Rowell's syndrome. J NTR Univ Health Sci 2017;6:60-3

How to cite this URL:
Siddhavatam C, Kekathi V, Saifullah QS, Kakarla S. An interesting case of systemic lupus erythematosus – Rowell's syndrome. J NTR Univ Health Sci [serial online] 2017 [cited 2019 Nov 15];6:60-3. Available from: http://www.jdrntruhs.org/text.asp?2017/6/1/60/202579




  Introduction Top


Rowell's syndrome is a rare presentation of lupus erythematosus (LE) with erythema multiforme-like lesions associated with antinuclear antibody (ANA), anti-La (SSB)/anti-Ro (SS-A) antibodies and rheumatoid factor (RF) positivity.[1] The first described association between LE and erythema multiforme was made by Scholtz in 1922.[2] In 1963, Rowell et al. reported a new syndrome characterized by LE, erythema multiforme-like lesions, a positive test for RF, speckled ANA, and a saline extract of human tissue (antiSJT) which is now regarded to be similar to Ro (SSA).[1],[2],[7] However, at the present time, there seems to be enough evidence to classify Rowell's syndrome within the subacute cutaneous lupus erythematosus (SCLE) subset.[1] Till now, approximately 71 cases have been reported. Nevertheless, we describe a patient whose clinical picture was consistent with the so-called Rowell's syndrome with secondary Sjogrens syndrome.


  Case History Top


A 23-year-old unmarried female was admitted to our ward with a 3-month history of fever with arthritis, myalgias, and rash over the face with photosensitivity, as well 3-day history with high-grade fever and bilateral paotid swellings [Figure 1], oral ulcers, and a spreading rash over the trunk, forearms, hands, and feet. There was altered sensorium since 1 day. She had a similar episode of fever with myalgias and arthritis 6 years ago. Physical examination revealed a malar rash which was an erythematous edematous lesion present over malar eminences and bridge of the nose, sparing the nasolabial folds with few erythematous papules over the forehead. Bilateral parotid swelling was present. Multiple discrete ill-defined to well-defined targetoid lesions were present predominantly over sun exposed areas, i.e., on the neck, anterior part of the trunk, extensor aspects of upper and lower extremities, with few erythematous papules over the palms and soles [Figure 2]. Patient also had ulceration on the hard palate [Figure 3]. Ophthalmic examination revealed normal fundus with positive schrimmers-I test with 8 mm in the right eye and 7 mm in the left eye, and tear film break up time (TBUT) of 8 s in the right and 9 s in the left eye. During admission, laboratory parameters showed normocytic normochromic anemia with thrombocytopenia. The patient exhibited normal liver function tests and renal function tests and electrolytes. Serological markers for hepatitis B and Hepatitis C and human immunodeficiency virus (HIV) are negative. Cerebrospinal fluid (CSF) analysis was normal, urine routines within normal limits, and erythrocyte sedimentation rate (ESR) was 55 mm/h. Autoimmune screening – ANA 1:160 with speckled pattern anti smith D1 and Anti ss-A (Anti -Ro) was positive, and all the remaining antibodies were negative; RA factor was also negative. Lupus anticoagulant was 52 s, hence positive. However, other APLAS antibodies were negative. There was no evidence suggestive of other system involvement such as arthritis, serositis, and glomerulonephritis. Two-dimensional echo was normal, and thyroid profile was also normal. Patient was treated with topical and intravenous steroids and emollients. After 10 days, the lesions subsided with hyperpigmentation [Figure 4]; the patient was discharged with an advice of steroids and hydroxychloroquine. Hence, we believe that our patient met the criteria for this rarely reported entity of Rowell's syndrome with secondary Sjogren's syndrome.
Figure 1: At the time of admission

Click here to view
Figure 2: Erythema multiforme like lesions

Click here to view
Figure 3: Ulcers over hard palate

Click here to view
Figure 4: At the time of discharge

Click here to view



  Discussion Top


Since the first report of Rowell's syndrome, not more than 71 cases have been reported in the English literature in which the presence of erythema multiforme-like lesions was associated with LE. However, a recent review demonstrated that most of the reported cases did not fulfill all the diagnostic criteria of Rowell's original description, especially the presence of rheumatoid factor and anti-La antibody.[1] Clinical lesions of RS include LE (systemic, discoid, or subacute), erythema multiforme-like lesions and chilblains. Although this syndrome was originally described in a discoid lupus erythematous (DLE) patient by Rowell,[15] further cases with different variants of cutaneous LE such as systemic (SLE) and subacute cutaneous (SCLE) were reported.[1] On admission, a diagnosis of SCLE was considered in our patient due to the presence of classical malar rash, polyarthritis, and oral ulcers, with laboratory investigations showing thrombocytopenia, anemia, ANA positivity with speckled pattern, and positive anti Smith antibody. Classical erythema multiforme is precipitated by trigger factors such as infectious agents, mainly Mycoplasma pneumonia, and HSV or drugs such as antibiotics, nonsteroid anti-inflammatories and anti-convulsants, although other causes including malignant conditions and connective tissue disease have been implicated.[1],[4],[7],[9],[12],[14],[15] Erythema multiforme is not associated with any specific autoimmune serological abnormality.[15] There was no identifiable precipitating factor for erythema multiforme in this case. Clinical and histological differentiation of erythema multiforme from SCLE may be difficult.[1],[10],[12] Early SCLE lesions with annular-polycyclic pattern may resemble EM. Necrotic keratinocytes may be found in SCLE lesions [1] as in erythema multiforme.

In fact, Herrero et al. recently described the presence of necrotic keratinocytes in 6 of 13 (54%) SCLE patients.[11] As some clinical, histological, and immunological findings seems to overlap with RS and SCLE; it has been suggested by some that LE with erythema multiforme-like rashes designated as RS represent a subset of SCLE with targetoid lesions, rather than a distinct entity.[1],[5],[8] Although this syndrome was originally described in DLE patients, some of these patients developed SLE years after the onset of DLE.[13] In 1995, Lee et al. reaffirmed the existence of Rowell's syndrome and suggested the inclusion of chilblains to the diagnostic criteria [Table 1]. Zeitouni et al. redefined the criteria in 2000 Rowell's syndrome [Table 1].[6]
TABLE 1: ZEITOUNI ET AL. CRITERIA (2000)

Click here to view


Three major and one minor criterion required diagnosed Rowell's syndrome. Our case fulfils all the major criteria and 1 minor criteria, i.e., the presence of anti-Ro and anti-La antibodies. However, at the present time there seems to be enough evidence to classify Rowell's syndrome within SCLE subset rather than accepting it as a separate entity. The immunologic abnormalities described in Rowell's syndrome may also associate with SCLE.[1],[2],[3] Speckled ANA pattern is the most consistent feature of RS occurring in approximately 88% of the cases, whereas RF is the least preserved feature, present in only 41%.[6]

However, more recent criteria from 2012 was proposed by Torchia et al., based on an extensive literature review identifying 95 cases of reported RS of lupus with erythema multiforme lesion morphology with an addition of histopathology of lesions and direct immunoflorescence (DIF).[16]

Erythema multiforme rarely has positive DIF, though when present, is typically IgM or C3 involving the basement membrane zone and superficial blood vessels. This set of guidelines requires all four of the major criteria and one minor criteria to be called as Rowell's syndrome [Table 2].
TABLE 2: TORCHIA ET AL. CRITERIA (2012)

Click here to view


Because of various reasons we could not perform DIF and histopathology for this patient otherwise our patient met three major and three minor of these newer criteria.

However, patients with these characteristic clinical and immunological features have been very rarely reported in the literature; we have described a patient whose clinical picture and immunological profile consistent with Rowell's syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Aydoğan K, Karadoğan SK, BalabanAdım S, TunalıŞ. Lupus erythematosus associated with erythemamultiforme: Report of two cases and review of theliterature. J Eur Acad Dermatol Venereol 2005;19:621-7.  Back to cited text no. 1
    
2.
Shteyngarts AR, Warner MR, Camisa C. Lupus erythematosus associated with erythema multiforme: Does Rowell's syndrome exist? J Am Acad Dermatol 1999;40:773-7.  Back to cited text no. 2
    
3.
Sontheimer RD. Subacute cutaneous lupus erythematosus: 25-year evolution of a prototypic subset (subphenotype) of lupus erythematosus defined by characteristic cutaneous, pathological, immunological, and genetic findings. Autoimmun Rev 2005;4:253-63.  Back to cited text no. 3
    
4.
Mandelcorn R, Shear N. Lupus associated toxic epidermal necrolysis. A novel manifestation of lupus? J Am Acad Dermatol 2003;48:525-9.  Back to cited text no. 4
    
5.
Roustan G, Salas C, Barbadillo C, Sánchez Yus E, Mulero J, Simón A. Lupus erythematosus with an erythema multiforme-like lesion. Eur J Dermatol 2000;10:459-62.  Back to cited text no. 5
    
6.
Zeitouni NC, Funaro D, Cloutier RA, Gagne E, Claveau J. Redefining Rowell's syndrome. Br J Dermatol 2000;142:343-6.  Back to cited text no. 6
    
7.
Shteyngarts AR, Warner MR, Camisa C. Lupus erythematosus associated with erythema multiforme: Does Rowell syndrome exist? J Am Acad Dermatol 1999;40(5Pt1):77.  Back to cited text no. 7
    
8.
Child FJ, Kapur N, Creamer D, Kobza Black A. Rowell syndrome. Clin Exp Dermatol 1999;24:74-7.  Back to cited text no. 8
    
9.
Mendonça R. Lupus erythematosus and erythema multiforme-like lesions. Rowell syndrome. Dermatol Online J 1997;3:4.  Back to cited text no. 9
    
10.
Fitzgerald E, Purcell SM, Kantor GR, Goldman HM. Rowell syndrome: Report of case. J Am Acad Dermatol 1996;35(5Pt2):801-3.  Back to cited text no. 10
    
11.
Herrero C, Bielsa I, Font J, Lozano F, Ercilla G, Lecha M, Ingelmo M, Mascaro JM. Subacute cutaneous lupus erythematosus: Clinicopathologic findings in thirteen cases. J Am Acad Dermatol 1988;19:1057-62.  Back to cited text no. 11
    
12.
Huff JC, Weston WL, Tonnesen MG. Erythema multiforme: A critical review of characteristics, diagnostic criteria and causes. J Am Acad Dermatol 1983;8:763-75.  Back to cited text no. 12
    
13.
Millard LG, Rowell NR. Chilblain lupus erythematosus (Hutchinson). A clinical and laboratory study of 17 patients. Br J Dermatol 1978;98:497-506.  Back to cited text no. 13
    
14.
Sanches M, Cruces J, Fonseca A, Taboada M, Cacharron J, Peteiro C, Robledo A. Systemic lupus erythematosus with skin manifestations of exsudative erythema multiforme. Actas Dermatosifiliogr 1975;66:631-8.  Back to cited text no. 14
    
15.
Rowell NR, Beck S, Anderson JR. Lupus erythematosus and erythema multiforme-likelesions. Arc Dermatol 1963;88:176-80.  Back to cited text no. 15
    
16.
Torchia D, Romanellie P, Kerdel FA. Erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis associated lupus erythematosus. J Am Acad Dermatol 2012;63:417-23.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case History
Discussion
References
Article Figures
Article Tables

 Article Access Statistics
    Viewed2556    
    Printed16    
    Emailed0    
    PDF Downloaded185    
    Comments [Add]    

Recommend this journal