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 1a], trunk [Figure 1b], 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 2a]. Direct immunofluorescence of skin samples showed granular deposits of C3 (++), but not of IgG, IgM, and IgA deposits, along basement membrane zone [Figure 2b], 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)
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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References
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