Scleromyxedema (Arndt - Gottron Syndrome) Developing Under Tenofovir Treatment for Hepatitis B: Unique Presentation in a Bulgarian Patient!

Abstract

Background: Scleromyxedema, also referred to as the Arndt-Gottron (S-AG) syndrome or the systemic form of Lichen myxedematosus (LM), is a cutaneous mucinosis with a chronic course and high lethality from systemic involvement of other organs and systems. Interesting in several aspects is the association between scleromyxedema and viral hepatitis about: 1) hepatitis virus infection as a possible etiological factor for the development of scleromyxedema, 2) antiretroviral therapy for the treatment of hepatitis as a method of reversing scleromyxedema and 3) antiviral drugs as inducers of scleromyxedema.

Case report: We present a 53-year old patient who for nine months had been on tenofovir disoproxil 245 mg (0-0-1) therapy for chronic hepatitis B. Three months after the start of antiviral therapy (i.e. for a period of 6 months), the patient observed swelling, itching and hardening of the skin on the face, ears and hands, which subsequently spread throughout the trunk. Subsequent histological study of a skin biopsy revealed changes of scleromyxedema at an advanced stage, though immunoelectrophoresis of serum and urine excluded the presence of paraproteinaemia or para proteinuria. Systemic antihistamine and topical corticosteroid therapy were instituted. Bone involvement with possible plasmacytoma was excluded, and a myelogram showed evidence of an erythroblastic reaction of bone marrow.

Conclusion: We believe that drug-induced scleromyxedema is a rare but possible phenomenon. We describe the first case of tenofovir-induced scleromyxedema within the framework of chronic hepatitis B treatment.

Keywords: Arndt - Gottron syndrome; Diabetes mellitus; Hepatitis B; Pathogenetic relationship; Scleromyxedema; Survival benefit; Tenofovir; Treatment.

Figure 1

a) Hardening of the face skin; b) Skin-colored small papules on the ear skin; c) Hardening of the skin on the back and neck; d) Multiple disseminated papules on the skin of the hands and arthropathy

Figure 2

a) This skin biopsy shows a combination of numerous fibroblasts, mucin, and irregularly arranged collagen bundles; b) At higher magnification, there are irregularly arranged collagen and scattered spindled cells, representing fibroblasts, within a mucinous background; c) This image shows details of fibroblasts around the cross-sectional profile of an eccrine sweat duct