Case Studies
Rowell Syndrome: A case report and review of literature
Anuj Sharma1*, Reena Sharma2
Author Affiliations
1, 2 Regional Hospital, Bilaspur, Himachal Pradesh, India
*Correspondence: Dr. Anuj Sharma
IJRCI. 2016;4(1):CS6
Submitted: 3 July 2016, Accepted: 28 July 2016, Published: 1 September 2016
© IJRCI
Abstract
Rowell syndrome is a rare disease consisting of lupus erythematosus associated with erythema multiforme-like lesions. The present case study discusses the occurrence of this syndrome in a 38-year-old female who presented with erythematous scaly lesions on face, neck and back, along with the targetoid lesions on upper arms and back of the neck, with oral erosions. Laboratory investigations revealed speckled pattern of anti-nuclear antibody (ANA) with a titer of 1:640, and anti-dsDNA and anti-La (SS-B) positivity. Histology of the targetoid lesion was consistent with erythema multiforme. Significant improvement within four weeks was noted with the use of oral steroids and anti-malarials.
Keywords: Rowell syndrome, Lupus erythematosus, Erythema multiformae, Speckled ANA
Introduction
Rowell syndrome, described in 1960s, is a rare presentation of lupus erythematosus (LE) with erythema multiforme (EM)-like lesions associated with antinuclear antibody (ANA), anti-La (SS-B)/anti-Ro (SS-A) antibodies and rheumatoid factor (RF) positivity.1, 2
Case report
A 38-year-old married female presented with a one-month history of multiple reddish colored lesions on various body parts, low-grade fever, generalized body ache and lethargy. The patient had reddish, painful lesions over the back of neck and upper arms and painful oral lesions for past 1 week. The patient history also revealed photosensitivity, chilblains and pain in the limb joints. She did not have decreased urination, hematuria, chest pain, cough, dyspnea, palpitations or spontaneous bleeding tendencies. Detailed cutaneous examination revealed that the patient had multiple, well-defined, erythematous, scaly plaques approximately 2x1 cm in size over face (predominantly nasal area and forehead), neck, pre-sternal area, trunk and back (Fig. 1 and 3). There was hemorrhagic crusting on the lips, and well-defined painful erosions over the palatal surface (Fig. 2). A few well-defined erythematous papules with central duskiness giving targetoid morphology were present over upper arms and back of the neck (Fig. 4). Other systemic examinations were normal. Due to the concurrent presence of lupus eryththematosus and EM-like lesions, the possibility of Rowell syndrome was considered and the patient was subjected to baseline investigations. Complete hemogram was normal except anemia (Hb:8.6 g/dl) and raised TLC (12,680 /ul). Serum chemistry and urine analysis were also normal. ANA by immunofluorescent assay (IFA) was positive with a titer of 1:640 and speckled pattern. Anti-dsDNA done by IFA was also positive (+2). Anti SSB-LA performed by enzyme immunoassay (EIA) was also raised with a value of 59.51U (normal<20.00U). RF (nephelometric method) was found to be negative. Histologic examination of lesional skin of the upper arm revealed hyperkeratosis, epidermal necrosis, vacuolar degeneration of the dermal-epidermal junction, and papillary dermal edema suggestive of EM.
Diagnosis of Rowell syndrome was concluded and the patient was started on oral prednisolone 60 mg/ day, hydroxycholoroquine 200 mg twice daily and other supportive medications. Significant improvement was seen within 4 weeks, steroids were gradually tapered off and hydroxychloroquine was continued with other topical agents.
Fig. 1, 2, 3 and 4: Skin and oral mucosal lesions
Discussion
The association of LE with EM was first reported by Shlotz in 1922.3 In 1963, Rowell defined this association as a distinct entity upon discovering different clinical and immunologic findings in four patients with discoid lupus erythematosus (DLE).2 The original criteria of Rowell’s syndrome consist of LE, EM-like lesions and immunological abnormalities such as speckled pattern of ANA, RF and saline extract of human tissue (anti-SJT) positivity, which is now regarded as similar to anti-Ro.1, 2, 4 In 2000, Zeitouni et al. proposed the revised diagnostic criteria for Rowell syndrome (Table 1). Three major criteria and at least one minor criterion are required for diagnosing the syndrome.4 Speckled ANA pattern is the most consistent feature of RS occurring in about 88% of the cases, whereas RF is the least preserved feature, present in only 41%.2, 5 Khandpur et al. have reported two cases of RS in SLE who were negative for anti-Ro/SSA and anti-La/SSB. The present patient had characteristic lesions of sub-acute cutaneous LE, lesions of EM, speckled pattern of ANA, positive anti-dsDNA and anti-La/SSB, and history of chill blains, but negative RF. The patient fulfilled the required criteria of Rowell’s syndrome.
Recent evidence has indicated that viral infections may be triggering factors for both SLE and EM. Unidentified Herpes simplex virus, Epstein–Barr virus or other viral infections may cross-react with lupus autoantigens and may initiate the immunologic response, thereby triggering the EM-like lesions in SLE. Dysregulated apoptosis may be the cause for many of the major manifestations of LE and EM skin lesions. Similar immunopathogenetic mechanisms described in both diseases may be responsible for the concurrence of these two diseases.6 Before making a diagnosis of RS, it is important to rule out other mimics of EM (Table 2) and common causes of EM such as herpes and drugs.7
Table 1: Revised diagnostic criteria for Rowell’s syndrome by Zeitouni et al.
Table 2: Differential diagnosis of erythema multiforme
The present case did not have any identifiable precipitating factor for EM. Rowell should be suspected in patients with EM of longer duration or those not responding to a course of oral acyclovir.8 The treatment of the syndrome is similar to that of SLE. Oral steroids in tapering doses, azathioprine, antimalarials like hydroxychloroquine or chloroquine, dapsone, cyclosporine and cyclophosphamide have been used with good results.1, 5, 9, 10
Despite the refined diagnostic criteria, recent literature has debated on whether RS is an overlap syndrome or co-occurrence of DLE and EM. Some studies have proposed that the RS is a subentity of subacute LE with EM while others have variously suggested that RS is a different variant of cutaneous LE, a subtype of chronic LE or an independent LE subtype.1, 9 The addition of new cases (like the present one) to the literature further strengthens the opinion that Rowell’s syndrome is a distinct clinical entity with specific diagnostic features. Although it is not so common, a diagnostic suspicion should always be kept in mind while dealing with patients of LE with EM-like lesions.
Competing interests
The authors declare that they have no competing interest
References
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