Generalized Granuloma Annulare in a Cirrhotic Patient Treated with Narrowband Ultraviolet B Therapy.

Sir

Generalized granuloma annulare (GGA) is an uncommon variant of granuloma annulare (GA) presenting with multiple asymptomatic or slightly pruritic, skin-colored, or erythematous papules, which may coalesce into annular plaques, occurring on trunk and extremities. Typically, GGA shows a protracted course and poor response to therapy.[12] Hepatitis C virus (HCV) infection may be associated with such disease.[3] We describe the first case of GGA in a HCV antibody-positive cirrhotic patient successfully treated with narrowband ultraviolet B (Nb-UVB) therapy.

A 70-year-old woman with a 8-year history of Child-Pugh class A HCV-related cirrhosis (previously treated unsuccessfully with two cycles of interferon plus ribavirin, the last of which two years earlier) presented with numerous mildly pruritic, partially confluent, pink-reddish papules on the trunk and limbs of 4 months duration [Figures 1a and b]. The patient denied drug intake or other significant health problems. Routine laboratory examinations showed no alteration except for hypoalbuminemia (2.8 mg/dl) and a slightly prolonged prothrombin time (INR value of 1.7) due to chronic liver failure. Antinuclear antibodies and HIV serology were negative. Histopathology from a papule showed interstitial infiltration of histiocytes and lymphocytes in upper and medium dermis [Figure 2a] Colloidal iron stain displayed mucin deposition in reticular dermis [Figure 2b]. Therefore, a diagnosis of GGA was made. The patient was treated with Nb-UVB therapy (three times weekly) with a starting dose of 0.35 J/cm2 and subsequent increments of 10% every session. After 8 weeks the lesions regressed [Figures 3a and b], and there was no recurrence after 3 months of follow-up. No side-effects were observed.

Figure 1

Numerous partially confluent, pink-reddish papules localized on the abdomen (inset shows a particular of the lesions) (a) and thighs (b)

Figure 2

Interstitial infiltration of histiocytes and lymphocytes in the upper and medium dermis (H and E ×100) (a); mucin deposition in reticular dermis (Colloidal iron stain ×50) (b)

Figure 3

Regression of the lesions on the abdomen (a) and thighs (b) after 8 weeks of treatment with Nb-UVB therapy (three weekly) with a starting dose of 0.35 J/cm2 and subsequent increments of 10% every session

Although many anecdotal therapies have been used with varying degrees of success in the treatment of GGA,[1] the literature supports the use of isotretinoin, dapsone and phototherapy with oral psoralen and ultraviolet A (PUVA) as first-line options.[2] However, these therapies may be associated with significant side-effects and are notoriously contraindicated in patients with liver impairment, as in our case.

Bath-PUVA with sun exposure (PUVASOL)[2] and Nb-UVB therapy[345] are reported to be efficacious in GGA and could be considered as ultraviolet-based alternatives to PUVA therapy for patients with hepatic failure since they don’t require oral psoralen administration and present a good safety profile.[2345] Regarding Nb-UVB therapy, Inui et al. reported a case of GGA (previously resistant to topical steroids and tacrolimus) resulting in resolution after 24 sessions,[3] while Yashar et al. described another instance with “minimal to mild” response (treatment regimen, duration, patient compliance and satisfaction were not indicated).[4] Moreover, Nb-UVB has also been successfully used as adjuvant therapy with insulin and oral tranilast in a case of eruptive GGA.[3]

Albeit the mechanism by which NB-UVB acts upon GA is not completely clear, it is thought that it is able to reduce the lymphoproliferation and cytokine production, thus counteracting granuloma formation.[4]

In conclusion, our case confirms the efficacy of Nb-UVB therapy in GGA and emphasizes its utility in patients with chronic liver failure, in which first-line treatments are contraindicated. Anyhow, further studies are needed to confirm these assumptions.