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The pathophysiologic features of lupus erythematosus (LE) are complex, and multiple factors have been suggested, such as genetics, drugs, and infections. Although an association of hepatitis C virus (HCV) infection with LE has not been clearly established, a higher proportion of HCV infection among patients with LE has been reported.1,2 In this observation, we report a case of refractory hypertrophic discoid cutaneous LE with complete remission after the treatment of HCV with direct-acting antivirals (DAAs).
Report of a Case
A 42-year-old woman with chronic HCV genotype 1B infection since childhood (viral load of 764 000 IU/mL) was diagnosed as having systemic LE in 2010 when she was admitted to the hospital with toxic epidermal necrolysis–like subacute cutaneous LE. She fulfilled 7 of 11 of the American College of Rheumatologists criteria for systemic LE (malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, antinuclear antibody positivity [>1/640], and anti–double-stranded DNA antibody positivity [16 IU/mL]). At a follow-up examination 6 years later, the patient presented with alopecic patches on the scalp; erythematous, scaly plaques on the face; oral lesions; and verrucous, hyperkeratotic plaques on both hands (Figure, A).
A, Roundish, verrucous, hyperkeratotic plaques on both hands. B, Complete clinical response after treatment of hepatitis C virus infection with direct-acting antivirals (sofosbuvir/velpatasvir combination therapy).
A biopsy specimen from the hand showed hyperkeratosis, interface dermatitis, a dense perivascular and periadnexal lymphohistiocytic infiltrate, and dermal mucin deposits. Clusters of CD123-positive cells were observed. A periodic acid–Schiff stain demonstrated thickening of the basement membrane. On the basis of clinical and histologic findings, a diagnosis of hypertrophic discoid cutaneous LE was made. Mycophenolic acid and prednisone were prescribed because the patient had previously experienced a cutaneous adverse reaction to hydroxychloroquine, and acitretin was not well tolerated. She had been treated with mycophenolic acid (720 mg twice a day), prednisone (2.5-20.0 mg/d), and 0.1% tacrolimus ointment for more than 2 years with only a partial response. The patient was evaluated at the hepatology department for chronic HCV infection. Laboratory tests revealed a viral load of 776 000 IU/mL; transaminase levels were normal, and results of ultrasonography and transient elastography ruled out hepatic cirrhosis.
The patient received DAA therapy with sofosbuvir/velpatasvir (400 mg/100 mg once daily) combination therapy. Four weeks later, a marked improvement in mucocutaneous lesions was noticed, and the prednisone and mycophenolic acid were tapered and then discontinued. The patient completed 12 weeks of sofosbuvir/velpatasvir treatment, which led to a complete remission of the oral, facial, and acral lesions (Figure, B) and of arthralgia. At this point, the viral load was undetectable. The patient has remained asymptomatic without treatment and has maintained an undetectable viral load after 18 months of follow-up.
Viruses have been accepted as etiologic or triggering agents in the etiopathogenesis of autoimmune disorders. Hepatitis C virus has been linked to immunopathologic manifestations ranging from serologic autoantibody positivity and immune complex–mediated disorders (eg, vasculitis, cryoglobulinemia, glomerulonephritis) to overt autoimmune diseases, including thyroiditis, hepatitis, and Sjögren syndrome. The connection between systemic LE and HCV is still uncertain. Case-control studies have shown a higher proportion of HCV infection among patients with systemic LE.1,2 Some authors suggest that HCV infection may mimic systemic LE, whereas others accept it as a triggering factor for the latter disease.1,2
The introduction of DAA agents has revolutionized the management of HCV infection, with a sustained virologic response in more than 95% of cases. Current guidelines recommend that all patients with HCV infection be considered for DAA therapy.3 Improvement or total remission of some HCV-associated dermatoses, including lichen planus, porphyria cutanea tarda, and symptomatic cryoglobulinemia, has been observed after successful DAA treatment.4,5 To our knowledge, no cases of systemic or cutaneous LE with complete remission after DAA therapy have been reported to date, although a case of subacute cutaneous LE triggered by DAA treatment for HCV infection was described in 2019.6
This patient fulfilled the criteria for systemic LE together with the characteristic clinical and histologic features of hypertrophic discoid cutaneous LE. We believe that HCV infection may have acted as a trigger, disrupting immunity and aggravating cutaneous involvement. Screening for HCV infection could be considered in patients with refractory cutaneous LE, and in the case of positive findings, DAA therapy is recommended.
Corresponding Author: José M. Mascaró Jr, MD, Department of Dermatology, Hospital Clínic de Barcelona, IDIBAPS, Carrer de Villarroel 170, 08036 Barcelona, Spain (email@example.com).
Published Online: March 4, 2020. doi:10.1001/jamadermatol.2020.0013
Conflict of Interest Disclosures: Dr Forns reports receiving personal fees from Gilead Sciences and AbbVie. No other disclosures were reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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Ertekin SS, Morgado-Carrasco D, Forns X, Mascaró JM. Complete Remission of Hypertrophic Discoid Cutaneous Lupus Erythematosus After Treatment of Chronic Hepatitis C With Direct-Acting Antivirals. JAMA Dermatol. Published online March 04, 2020. doi:10.1001/jamadermatol.2020.0013
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