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Year : 2020  |  Volume : 65  |  Issue : 6  |  Page : 549-550
Rowell's syndrome with lupus hepatitis: A case report from China


Department of Dermatology, The Fifth People's Hospital of Hainan Province, Haikou, China

Date of Web Publication23-Oct-2020

Correspondence Address:
Weiwei Wu
Department of Dermatology, The Fifth People's Hospital of Hainan Province, Haikou
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_222_19

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How to cite this article:
Zhang M, Lu J, Zhang B, Li Z, Wu W. Rowell's syndrome with lupus hepatitis: A case report from China. Indian J Dermatol 2020;65:549-50

How to cite this URL:
Zhang M, Lu J, Zhang B, Li Z, Wu W. Rowell's syndrome with lupus hepatitis: A case report from China. Indian J Dermatol [serial online] 2020 [cited 2020 Dec 4];65:549-50. Available from: https://www.e-ijd.org/text.asp?2020/65/6/549/298903




Sir,

The constellation of lupus erythematosus (LE) with erythema multiforme (EM) and specific immunological laboratory changes is known as Rowell's syndrome (RS).[1] We report a Chinese adolescent boy with extensive erythematous and target-like lesions, who fulfilled the criteria of RS and systemic lupus erythematosus (SLE). A 15-year-old Chinese adolescent boy visited our department with generalized erythema and blister with fever for 1 month. On physical examination, there were numerous erythematous and target-like lesions on the face [Figure 1]a, trunk [Figure 1]b, and limbs with negative Nikolsky's sign. Dark-red patches were seen around the end of the fingers and toes and crusted erosions on the lips. There was palpable regional lymphadenopathy upon examination on the neck and axilla. Leucopenia (white blood cell count, 2.95 × 109/L), neutropenia (neutrophil count, 0.75 × 109/L), and anemia (hemoglobin, 114 g/L) were found. The erythrocyte sedimentation rate (45 mm/h) and C-reactive protein (12 mg/L) were increased. Rheumatoid factor (32 IU/mL) was positive. The serum activity of aminotransferases (alanine aminotransferase, 86U/L and aspartate aminotransferase, 147U/L) and creatine kinase (lactate dehydrogenase [LDH], 1287U/L and alpha-hydroxybutyrate dehydrogenase, 1107 U/L) was increased. Serum immunofluorescence revealed a speckled pattern of antinuclear antibodies (ANA, 1:1000 dilution). Immunoenzymatic assay showed positive for nRNP/Sm, Sm, SS-A, SSB, and anti-ribosomal P protein antibodies. Histopathological examination of erythematous targetoid lesions on the chest revealed features consistent with LE [Figure 2]a. Direct immunofluorescence of skin samples showed granular deposits of C3 (++), but not of IgG, IgM, and IgA deposits, along basement membrane zone [Figure 2]b, which accorded with the diagnosis of LE. In 2000, Zeitouni et al . redefined the diagnostic criteria of RS,[2] which included three major criteria: (a) LE (systemic, discoid, or subacute); (b) EM (with or without mucosal involvement); and (c) speckled ANA; and three minor criteria: chilblains, anti-Ro or anti-La antibodies, and positive rheumatoid factor. All three major and at least one minor criteria should be fulfilled. According to the above diagnostic criteria, the diagnosis of RS is established in this case. At the same time, the patient had elevated transaminase and LDH. We excluded liver damage caused by virus or other common factors, and considered the combination of lupus hepatitis, which was rarely reported in previous cases.[3],[4] The treatment and prognosis are often referred to as SLE, SCLE, and DLE. Oral steroids, immunosuppressive agents (azathioprine and cyclophosphamide), and antimalarial-like chloroquine or hydroxychloroquine are reported to be effective drugs.[3],[5] Response to treatment is very variable with frequent episodes of clinical exacerbation. This patient was treated with methylprednisolone 60 mg/day intravenously, combined with hydroxychloroquine 200 mg twice daily orally. After 14 days of treatment, disease activity improved significantly. After a gradual reduction of glucocorticoid, the patient remained free of lesions on a regimen of prednisolone 15 mg/day till reporting. In summary, we reported a unique case of RS with lupus hepatitis from China with a distinct clinical and immunologic entity.
Figure 1: Clinical features of Rowell's syndrome in our case. A 15-year-old Chinese adolescent boy presented extensive erythematous and target-like lesions on the face (a) and trunk (b)

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Figure 2: Histopathological findings of skin lesion in our case. (a) The histopathological examination of erythematous targetoid lesions on the chest revealed hyperkeratosis, slight hyperplasia of spinous layer, liquefaction degeneration of basal cells, lymphocyte infiltration around superficial dermal vessels, and appendages (H and E, ×200). (b) Direct immunofluorescence of skin samples showed granular deposits of C3 (++) along basement membrane zone (IHC, ×100)

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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.
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.
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. 2
    
3.
Madke B, Khopkar U. Rowell syndrome in an Indian male and review of the literature. Indian Dermatol Online J 2015;6:S12-6.  Back to cited text no. 3
    
4.
Mittal RR, Shivall G. Rowell's syndrome. Indian J Dermatol 1997;42:28-9.  Back to cited text no. 4
  [Full text]  
5.
Roy M, Ghosh JB, Bala AK, Chatterjee S. Rowell's syndrome: Presenting features of systemic lupus erythematosus. Rheumatol Int 2013;33:1075-7.  Back to cited text no. 5
    


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