Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 1537  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Articlesmenu-bullet Search Instructions Online Submission Subscribe Etcetera Contact
 
  NAVIGATE Here 
  Search
 
  
 RESOURCE Links
 ::  Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::Related articles
 ::  Article in PDF (1,235 KB)
 ::  Citation Manager
 ::  Access Statistics
 ::  Reader Comments
 ::  Email Alert *
 ::  Add to My List *
* Registration required (free) 

  IN THIS Article
 ::  Abstract
  ::  Introduction
  ::  Case History
  ::  Discussion
 ::  References
 ::  Article Figures

 Article Access Statistics
    Viewed828    
    Printed14    
    Emailed0    
    PDF Downloaded7    
    Comments [Add]    

Recommend this journal


 


 
  Table of Contents     
CASE REPORT
Year : 2021  |  Volume : 67  |  Issue : 2  |  Page : 109-112

Neuropsychiatric manifestations in an adolescent male with Rowell syndrome


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

Date of Submission25-Jul-2020
Date of Decision08-Oct-2020
Date of Acceptance10-Dec-2020
Date of Web Publication07-Apr-2021

Correspondence Address:
S P Hegde
Department of Dermatology, Venereology and Leprosy, Yenepoya Medical College, Yenepoya (Deemed to be University), Mangalore, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpgm.JPGM_879_20

Rights and Permissions


 :: Abstract 


A 15-year-old boy presented with fever, skin, and oral lesions for 4 weeks. The cutaneous lesions were suggestive of subacute cutaneous lupus erythematosus and erythema multiforme. His clinical, histopathological, and immunological features were indicative of Rowell syndrome and he satisfied the diagnostic criteria of Rowell syndrome proposed by Zeitouni et al. He subsequently developed neurological manifestations and was diagnosed to have neuropsychiatric systemic lupus erythematosus. We report this case for the unusual occurrence of a rare entity like Rowell syndrome in an adolescent male with co-existence of neuropsychiatric systemic lupus erythematosus.


Keywords: Erythema multiforme, neuropsychiatric systemic lupus erythematosus, Rowell syndrome


How to cite this article:
Kadiru R A, Hegde S P, Mithun H K, Rao A C. Neuropsychiatric manifestations in an adolescent male with Rowell syndrome. J Postgrad Med 2021;67:109-12

How to cite this URL:
Kadiru R A, Hegde S P, Mithun H K, Rao A C. Neuropsychiatric manifestations in an adolescent male with Rowell syndrome. J Postgrad Med [serial online] 2021 [cited 2021 Aug 1];67:109-12. Available from: https://www.jpgmonline.com/text.asp?2021/67/2/109/313348





 :: Introduction Top


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).[1] 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.


 :: Case History Top


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.
Figure 1: Erythematous, annular, scaly plaques, and few crusted plaques over the (a) malar region (sparing the nasolabial folds), perioral area, chest; (b) ear pinna; and (c) palatal erosions

Click here to view
Figure 2: Targetoid lesions over (a) palms (black arrows); and (b) soles

Click here to view


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.
Figure 3: Histopathology from the trunk lesion showing (a) epidermal thinning, vacuolar basal degeneration, and subepidermal clefts (H and E stain, 20×); (b) dermal edema, papillary dermal peri adnexal and perivascular infiltrate, and extravasation of RBC's (H and E stain, 20×)

Click here to view


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).
Figure 4: A 3 Tesla magnetic resonance imaging brain plain study showing multiple hemorrhagic foci in bilateral basal ganglia

Click here to view



 :: Discussion Top


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.[2] The diagnostic criteria for RS was revised by Zeitouni et al. in 2000.[3] 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.[3] 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.[3],[4] 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.[4] 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.[2],[5],[6]

Good therapeutic results have been achieved with azathioprine, antimalarials, prednisone, and cyclosporine.[4],[5],[7] Our patient responded well to prednisolone, and hydroxychloroquine.

The prevalence of neuropsychiatric SLE (NPSLE) varies from 6% to 91%.[8] Neuropsychiatric symptoms may be the initial presentation of SLE in children.[9] Approximately 25% of children with SLE have neuropsychiatric manifestations which are a major cause of morbidity and mortality.[9] 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.[8] These include headaches, seizures, cognitive dysfunction, cerebrovascular disease, mood disorders, depressions and rarely, transverse myelitis.[9],[10] A frequent association has been found with the presence of APL antibodies and cognitive impairment and cerebrovascular disease in patients with NPSLE.[8] 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.[9]

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.[11] 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.

Acknowledgements

We thank Dr Malcolm Pinto, Associate Professor of Dermatology, for his valuable inputs in preparation of this manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 :: References Top

1.
Rowell NR, Beck JS, Anderson JR. Lupus erythematosus and erythema multiforme-like lesions. A syndrome with characteristic immunological abnormalities. Arch Dermatol 1963;88:176-80.  Back to cited text no. 1
    
2.
Antiga E, Caproni M, Bonciani D, Bonciolini V, Fabbri P. The last word on the so-called 'Rowell's syndrome'? Lupus 2012;21:577-85.  Back to cited text no. 2
    
3.
Zeitouni NC, Funaro D, Cloutier RA, Gagné E, Claveau J. Redefining Rowell's syndrome. Br J Dermatol 2000;142:343-6.  Back to cited text no. 3
    
4.
Khandpur S, Das S, Singh MK. Rowell's syndrome revisited: Report of two cases from India. Int J Dermatol 2005;44:545-9.  Back to cited text no. 4
    
5.
Aydogan K, Karadogan S, Balaban Adim S, Tunali S. Lupus erythematosus associated with erythema multiforme: Report of two cases and review of the literature. J Eur Acad Dermatol Venereol 2005;19:621-7.  Back to cited text no. 5
    
6.
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. 6
    
7.
Bhat RY, Varma C, Bhatt S, Balachandran C. Rowell syndrome. Indian Dermatol Online J 2014;5(Suppl 1):S33-5.  Back to cited text no. 7
    
8.
Zhang Y, Han H, Chu L. Neuropsychiatric lupus erythematosus: Future directions and challenges; a systematic review and survey. Clinics (San Paulo) 2020;75:e1515.  Back to cited text no. 8
    
9.
Soybilgic A. Neuropsychiatric systemic lupus erythematosus in children. Pediatr Ann 2015;44;e153-8.  Back to cited text no. 9
    
10.
Hanly JG. Diagnosis and management of neuropsychiatric SLE. Nat Rev Rheumatol 2014;10;338-47.  Back to cited text no. 10
    
11.
Kim SS, Magro C, Granstein RD, Bass A, Erkan D. Systemic lupus erythematosus associated with Rowell's syndrome: A clinical pathology conference held by the division of rheumatology at hospital for special surgery. HSS J 2013;9:289-92.  Back to cited text no. 11
    


    Figures

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



 

Top
Print this article  Email this article
 
Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow