|Year : 2017 | Volume
| Issue : 1 | Page : 60-63
An interesting case of systemic lupus erythematosus – Rowell's syndrome
Chandrasekhar Siddhavatam, Vidyasagar Kekathi, Quraishi Sayed Mohammad Saifullah, Saikiran Kakarla
Department of Medicine, Kurnool Medical College, Kurnool, Andhra Pradesh, India
|Date of Web Publication||20-Mar-2017|
Flat No. 207, Dheeraj Enclave, Saptagiri Nagar, Kurnool - 518 002, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
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 2020 Nov 24];6:60-3. Available from: https://www.jdrntruhs.org/text.asp?2017/6/1/60/202579
| Introduction|| |
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. The first described association between LE and erythema multiforme was made by Scholtz in 1922. 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).,, However, at the present time, there seems to be enough evidence to classify Rowell's syndrome within the subacute cutaneous lupus erythematosus (SCLE) subset. 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|| |
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.
| Discussion|| |
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. 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, further cases with different variants of cutaneous LE such as systemic (SLE) and subacute cutaneous (SCLE) were reported. 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.,,,,,, Erythema multiforme is not associated with any specific autoimmune serological abnormality. There was no identifiable precipitating factor for erythema multiforme in this case. Clinical and histological differentiation of erythema multiforme from SCLE may be difficult.,, Early SCLE lesions with annular-polycyclic pattern may resemble EM. Necrotic keratinocytes may be found in SCLE lesions  as in erythema multiforme.
In fact, Herrero et al. recently described the presence of necrotic keratinocytes in 6 of 13 (54%) SCLE patients. 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.,, Although this syndrome was originally described in DLE patients, some of these patients developed SLE years after the onset of DLE. 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].
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.,, 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%.
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).
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].
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
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.
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.
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.
Mandelcorn R, Shear N. Lupus associated toxic epidermal necrolysis. A novel manifestation of lupus? J Am Acad Dermatol 2003;48:525-9.
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.
Zeitouni NC, Funaro D, Cloutier RA, Gagne E, Claveau J. Redefining Rowell's syndrome. Br J Dermatol 2000;142:343-6.
Shteyngarts AR, Warner MR, Camisa C. Lupus erythematosus associated with erythema multiforme: Does Rowell syndrome exist? J Am Acad Dermatol 1999;40(5Pt1):77.
Child FJ, Kapur N, Creamer D, Kobza Black A. Rowell syndrome. Clin Exp Dermatol 1999;24:74-7.
Mendonça R. Lupus erythematosus and erythema multiforme-like lesions. Rowell syndrome. Dermatol Online J 1997;3:4.
Fitzgerald E, Purcell SM, Kantor GR, Goldman HM. Rowell syndrome: Report of case. J Am Acad Dermatol 1996;35(5Pt2):801-3.
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.
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.
Millard LG, Rowell NR. Chilblain lupus erythematosus (Hutchinson). A clinical and laboratory study of 17 patients. Br J Dermatol 1978;98:497-506.
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.
Rowell NR, Beck S, Anderson JR. Lupus erythematosus and erythema multiforme-likelesions. Arc Dermatol 1963;88:176-80.
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.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]