Neuropsychiatric manifestations in an adolescent male with Rowell syndromeRA Kadiru1, SP Hegde1, HK Mithun2, AC Rao3
1 Department of Dermatology, Venereology and Leprosy, Yenepoya Medical College, Yenepoya (Deemed to be University), Mangalore, Karnataka, India
2 Department of Paediatrics, Yenepoya Medical College, Yenepoya (Deemed to be University), Mangalore, Karnataka, India
3 Department of Pathology, Yenepoya Medical College, Yenepoya (Deemed to be University), Mangalore, Karnataka, India
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpgm.JPGM_879_20
Source of Support: None, Conflict of Interest: None
Keywords: Erythema multiforme, neuropsychiatric systemic lupus erythematosus, Rowell syndrome
Rowell syndrome (RS) is a unique and rare disorder characterized clinically by lupus erythematosus (LE) and erythema multiforme (EM) like lesions. In 1963, Rowell et al. described the presence of erythema multiforme and discoid LE (DLE) in female patients that were associated with a speckled pattern of antinuclear antibodies (ANA), positive Rheumatoid Factor (RF), and precipitating antibodies to a saline extract of human tissues (anti-SjT). Neurological involvement in cases of Systemic lupus erythematosus (SLE) presenting as Rowell syndrome is very uncommon and has been rarely reported in literature. We report a case of Rowell syndrome in a 15-year-old boy with occurrence of neuropsychiatric manifestations.
A 15-year-old boy presented with a 4-week history of low-grade fever, oral ulcers, and widespread pruritic and painful rash over the body. He had asymptomatic red lesions over the palms and soles. He gave a history of photosensitivity, and Raynaud's phenomenon. There was no history of joint pain. He denied any history of drug intake in the recent past. Dermatological examination revealed multiple erythematous, annular, scaly plaques, and few crusted plaques distributed bilaterally and symmetrically over the malar region (sparing the nasolabial folds), perioral area, chest, extensor aspect of the elbows, upper back, and pinna of the ears [Figure 1]a and [Figure 1]b. Multiple erosions were present over the buccal mucosa, hard and soft palate [Figure 1]c. There were targetoid lesions distributed symmetrically over the palms and soles [Figure 2]a and [Figure 2]b. His vital parameters and systemic examination were normal.
Hemogram revealed hemoglobin of 11.4 g/dL, leukopenia 1400 cells/mm3 (normal value 4000-11000 cells/mm3), with lymphopenia 588 cells/mm3 (1000-4400 cells/mm3) and neutropenia 686 cells/mm3 (1600-8260 cells/mm3), thrombocytopenia 124,000 cells/mm3 (150,000-450,000 cells/mm3), and erythrocyte sedimentation rate of 40 mm at 1 h. Peripheral smear suggested evidence of hemolysis. His liver enzymes were elevated. (SGOT 671 U/L, SGPT 355 U/L). His urine analysis, renal functions, and serum electrolytes were normal. Serology revealed positive ANA with a speckled pattern, and ANA profile was strongly positive for RNP/Sm, Sm, SS-A (anti Ro). Complement (C3) levels were low and he tested negative for Rheumatoid factor. Histopathology from the skin lesions over the trunk showed epidermal thinning with hydropic degeneration of the basal cell layer with subepidermal clefts [Figure 3]a There was dermal edema with perivascular and peri adnexal dermal lymphocytic infiltrate and mucin deposits around eccrine glands [Figure 3]b. These features were suggestive of subacute cutaneous lupus erythematosus. Enzyme-linked Immunosorbent Assay (ELISA) for Human immunodeficiency virus (HIV) and serology for herpes viral infection were negative. On the basis of the Systemic Lupus International Collaborating Clinics (SLICC) criteria, a diagnosis of systemic lupus erythematous was arrived at. The patient also satisfied the criteria of Rowell syndrome. The patient was administered injection dexamethasone 8 mg and hydroxychloroquine 400 mg daily following which the skin lesions began to improve.
Two weeks later, the patient returned with urinary and fecal incontinence. His parents noticed that he was unresponsive to commands. He was conscious, oriented to time, place, and person, but was not responding verbally to oral commands and was able to walk only with support. His prothrombin time and activated partial thromboplastin time were normal. His antiphospholipid antibodies (APLA) were negative. A 3 Tesla Magnetic Resonance imaging (3T MRI) brain plain study showed multiple hemorrhagic foci and micro-hemorrhages in bilateral basal ganglia and corona radiata and white matter of fronto-parietal lobe, suggestive of ischemia [Figure 4]. The opinions of neurologists and psychiatrists were sought and a diagnosis of neuropsychiatric SLE with adjustment disorder with anxious or depressive reactions was arrived at. He was started on intravenous methyl prednisolone pulse therapy, cyclophosphamide pulse therapy, aspirin, and benzodiazepines. Improvement was noted with respect to response to oral commands and ability to walk. His blood parameters also showed improvement (Total leucocyte count 8900 cells/mm3 and platelet count 291,000 cells/mm3).
Antiga et al. compiled the characteristics of 71 cases of Rowell syndrome reported in literature in which there are very few reports of its presentation in pediatric population and in males. The diagnostic criteria for RS was revised by Zeitouni et al. in 2000. Major criteria include SLE, DLE, or subacute cutaneous LE (SCLE); EM-like lesions (with or without mucosal involvement); and ANA with a speckled pattern. Minor criteria include chilblains, anti-Ro/anti-La antibody, and a positive RF. All three major criteria and at least one minor criterion is required for a diagnosis of RS. With the presence of lesions suggestive of SCLE and EM, a speckled ANA pattern, and Anti- Ro antibody, our patient satisfied the criteria for the diagnosis of Rowell syndrome.
Speckled ANA pattern is the most preserved feature of RS occurring in about 88% of the cases, whereas a positive RF is the least consistent feature, present in only 41% of cases., Our patient showed a speckled pattern of ANA on immunofluorescence and had a negative rheumatoid factor.
Despite the refined diagnostic criteria, recent literature has debated on the existence of Rowell syndrome. Some authors have proposed that Rowell syndrome is a distinct clinical entity with specific diagnostic features. Whereas, other authors opine that it is a subset of subacute lupus erythematosus with targetoid lesions, a different variant of cutaneous LE, a subtype of chronic LE or an independent LE subtype.,,
Good therapeutic results have been achieved with azathioprine, antimalarials, prednisone, and cyclosporine.,, Our patient responded well to prednisolone, and hydroxychloroquine.
The prevalence of neuropsychiatric SLE (NPSLE) varies from 6% to 91%. Neuropsychiatric symptoms may be the initial presentation of SLE in children. Approximately 25% of children with SLE have neuropsychiatric manifestations which are a major cause of morbidity and mortality. NPSLE occurs more frequently when the SLE is active clinically and serologically as in our case. The involvement of nervous system in SLE may show a wide variety of neurological and psychiatric manifestations which could be focal or diffuse. These include headaches, seizures, cognitive dysfunction, cerebrovascular disease, mood disorders, depressions and rarely, transverse myelitis., A frequent association has been found with the presence of APL antibodies and cognitive impairment and cerebrovascular disease in patients with NPSLE. Early recognition of symptoms is crucial in prevention of permanent neurological sequel and improvement in patients' quality of life. Diagnosing NPSLE is especially difficult in pediatrics; many current diagnostic tools are invasive and/or costly, and there are no current accepted screening mechanisms.
Our case of RS was unique due to the central nervous involvement which, to the best of our knowledge, has not been reported in the literature so far. Kim et al. reported a case of SLE with RS who had a history of neuropsychiatric manifestations 4 months prior to the onset of RS whereas our patient developed neuropsychiatric manifestations 6 weeks after the onset of RS. The presence of neuropsychiatric manifestations in RS appears to be an incidental finding in our case, but it could emerge as an important association of RS in the future as more data on RS becomes available.
The addition of new cases like the present one to the literature further strengthens the opinion that Rowell 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. We report this case for the unusual occurrence of a rare entity like Rowell Syndrome in an adolescent male. The co- existence of neuropsychiatric SLE in our patient is a novel finding in the setting of RS.
Declaration of patient consent
The authors certify that appropriate patient consent was obtained.
We thank Dr Malcolm Pinto, Associate Professor of Dermatology, for his valuable inputs in preparation of this manuscript.
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Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]