Scleromyxedema (Arndt - Gottron Syndrome) Developing Under Tenofovir Treatment for Hepatitis B: Unique Presentation in a Bulgarian Patient!
DOI:
https://doi.org/10.3889/oamjms.2019.181Keywords:
Scleromyxedema, Arndt - Gottron syndrome, Tenofovir, Hepatitis B, Diabetes mellitus, Survival benefit, Pathogenetic relationship, TreatmentAbstract
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.
Downloads
Metrics
Plum Analytics Artifact Widget Block
References
Rongioletti F, Rebora A. Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema. J Am Acad Dermatol. 2001; 44(2):273-81. https://doi.org/10.1067/mjd.2001.111630 PMid:11174386
Sala A, Cunha P, Pinto C, Alves C, Paiva I, Araujo A. Scleromyxedema: clinical diagnosis and autopsy findings. A Bras Dermatol. 2016; 91(5 Suppl 1):48–50. https://doi.org/10.1590/abd1806-4841.20164527 PMid:28300892 PMCid:PMC5324991
Saniee S, Davarnia G. Scleromyxedema without Paraproteinemia: Treatment with Thalidomide and Prednisolone. Case Rep Dermatol. 2016; 8(3):327–332. https://doi.org/10.1159/000452319 PMid:27990110 PMCid:PMC5156886
Rongioletti F, Merlo G, Cinotti E, Fausti V, Cozzani E, Cribier B, Metze D, Calonje E, Kanitakis J, Kempf W, Stefanato C, Marinho E, Parodi A. Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol. 2013; 69(1):66-72. https://doi.org/10.1016/j.jaad.2013.01.007 PMid:23453242
Smith J, Kalimullah F, Erickson C, Peng L. Scleromyxedema secondary to hepatitis C virus and successfully treated with antiviral therapy. Dermatol Online J. 2015; 21(9).
Azfar N, Khan A, Zaman T, Jahangir M. Scleromyxedema in an HCV-positive male. J Pak Assoc Dermatol. 2008; 18:116-118.
Rongioletti F, Rebora A. Worsening of lichen myxedematosus during interferon alfa-2a therapy for chronic active hepatitis C. J Am Acad Dermatol. 1998; 38(5 Pt 1):760-1. https://doi.org/10.1016/S0190-9622(98)70205-1
Dolenc-VoljÄ M, JurÄić V, HoÄevar A, TomÅ¡iÄ M. Scleromyxedema with subcutaneous nodules: successful treatment with thalidomide and intravenous immunoglobulin. Case Rep Dermatol. 2013; 5(3):309-15. https://doi.org/10.1159/000356469 PMid:24348379 PMCid:PMC3843934
Fleming K, Virmani D, Sutton E, Langley R, Corbin J, Pasternak S, Walsh N. Scleromyxedema and the dermato-neuro syndrome: case report and review of the literature. J Cutan Pathol. 2012; 39(5):508-17. https://doi.org/10.1111/j.1600-0560.2012.01882.x PMid:22515222
Kumar N, Rodriguez M. Scleromyxedema in a patient with multiple sclerosis and monoclonal gammopathy on interferon beta-1a. Mult Scler. 2004; 10(1):85-6. https://doi.org/10.1191/1352458504ms987cr PMid:14760958
Abbott R, Calonje E, Almaani N, Kulasegram R, McGibbon D. Widespread papules in a patient with human immunodeficiency virus. Papular mucinosis (PM) in association with HIV infection. Clin Exp Dermatol. 2010; 35(7):801-2. PMid:20831608
Feig J, Mediero A, Corciulo C, Liu H, Zhang J, Perez-Aso M, Picard L, Wilder T, Cronstein B. The antiviral drug tenofovir, an inhibitor of Pannexin-1-mediated ATP release, prevents liver and skin fibrosis by downregulating adenosine levels in the liver and skin. PLoS One. 2017; 12(11):e0188135. https://doi.org/10.1371/journal.pone.0188135 PMid:29145453 PMCid:PMC5690602
Girón J, Dean M. Resolution of papular mucinosis in a person with HIV infection. AIDS Read. 2007; 17(8):418-20. PMid:17717886
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, Cutolo M, Rongioletti F, Denton C, Rudnicka L, Frasin L, Smith V, Gabrielli A, Aberer E, Bagot M, Bali G, Bouaziz J, Braae Olesen A, Foeldvari I, Frances C, Jalili A, Just U, Kähäri V, Kárpáti S, Kofoed K, Krasowska D, Olszewska M, Orteu C, Panelius J, Parodi A, Petit A, Quaglino P, Ranki A, Sanchez Schmidt J, Seneschal J, Skrok A, Sticherling M, Sunderkötter C, Taieb A, Tanew A, Wolf P, Worm M, Wutte N, Krieg T. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis. J Eur Acad Dermatol Venereol. 2017; 31(10):1581-1594. https://doi.org/10.1111/jdv.14466 PMid:28786499
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2019 Ivanka Temelkova, James Patterson, Georgi Tchernev
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
http://creativecommons.org/licenses/by-nc/4.0
