Febrile Ulceronecrotic Mucha-Habermann Disease: A Rare Case Report and Review of Cases Treated With Oral Cyclosporine

Authors

  • Apasee Sooksamran Department of Medical Services, Institute of Dermatology, Phayathai, Bangkok 10400, Thailand.
  • Poonnawis Sudtikoonaseth Department of Medical Services, Institute of Dermatology, Phayathai, Bangkok 10400, Thailand.
  • Praneet Sajjachareonpong Skin Clinic, Phayathai 2 International Hospital, Phayathai, Bangkok 10400, Thailand.
  • Tanongkiet Tienthavorn Department of Medical Services, Institute of Dermatology, Phayathai, Bangkok 10400, Thailand.

DOI:

https://doi.org/10.31584/jhsmr.2021811

Keywords:

cyclosporine, cytomegalovirus, febrile ulceronecrotic Mucha-Habermann disease, Pityriasis lichenoides with ulceronecrosis and hyperthermia

Abstract

We present a case of febrile ulceronecrotic Mucha-Habermann disease who presented with widespread erythematous crusted papules, which rapidly progressed to ulceronecrotic lesions accompanied by fever. The serologies showed high titers of cytomegalovirus IgG (>1:3,200) and IgM (1:800). The histopathological study showed epidermal necrosis. The treatment was begun with systemic steroids and roxithromycin to which the patient did not respond. After switching the treatment to methotrexate, the patient was further complicated by getting hepatitis. Low dose cyclosporine resolved the situation within 2 weeks. In patients for whom methotrexate is contra-indicated or ineffective, cyclosporine can suppress T-lymphocyte hyperresponsiveness and resolve this disease.

References

Nofal A, Assaf M, Alakad R, Amer H, Nofal E, Yosef A. Febrile ulceronecrotic Mucha-Habermann disease: proposed diagnostic criteria and therapeutic evaluation. Int J Dermatol 2016;55: 729-38.

Perrin BS, Yan AC, Treat JR. Febrile ulceronecrotic Mucha- Habermann disease in a 34-month-old boy: a case report and review of the literature. Pediatr Dermatol 2012;29:53-8.

Yamada K, Motegi S, Matsushima Y. Febrile ulceronecrotic Mucha-Habermann disease in a young boy: a case report and review of the literature. Acta Derm Venereol 2014;94:603-4.

Dupin A, Bosset D, Atger L, Chevallier B, Saiag P, Benoist G. Febrile ulceronecrotic Mucha-Habermann disease. Arch Pediatr 2016;23:82-5.

Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha- Habermann disease with extensive skin necrosis in intertriginous areas. European J Dermatol 2003;13:493-6.

Nofal A, Alakad R, Assaf M, Nofal E. A fatal case of febrile ulceronecrotic Mucha-Habermann disease in a child. JAAD Case Rep 2016;2:181-5.

Tsai KS, Hsieh HJ, Chow KC, Lin TY, Chiang SF, Huang HH. Detection of cytomegalovirus infection in a patient with febrile ulceronecrotic Mucha-Habermann's disease. Int J Dermatol 2001;40:694-8.

De Paschale M, Agrappi C, Manco MT, Clerici P. Positive predictive value of anti-HCMV IgM as an index of primary infection. J Virol Methods 2010;168:121-5.

Marchetti S, Santangelo R, Manzara S, D'Onghia S, Fadda G, Cattani P. Comparison of real-time PCR and pp65 antigen assays for monitoring the development of Cytomegalovirus disease in recipients of solid organ and bone marrow transplants. New Microbiol 2011;34:157-64.

Ljungman P, Boeckh M, Hirsch HH, Josephson F, Lundgren J, Nichols G, et al. Definitions of Cytomegalovirus Infection and Disease in Transplant Patients for Use in Clinical Trials. Clin Infect Dis 2017;64:87-91.

Amarapal P, Tantivanich S, Balachandra K. Prevalence of cytomegalovirus in Thai blood donors by monoclonal staining of blood leukocytes. Southeast Asian J Trop Med Public Health 2001;32:148-53.

Herron MD, Bohnsack JF, Vanderhooft SL. Septic, CD-30 positive febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. Pediatr Dermatol 2005;22:360-5.

Kim HS, Yu DS, Kim JW. A case of febrile ulceronecrotic Mucha-Habermann's disease successfully treated with oral cyclosporin. J Eur Acad Dermatol Venereol 2007;21:272-3.

Uzoma MA, Wilkerson MG, Carr VL, Westhoven GS, Raimer SA. Pentoxifylline and cyclosporine in the treatment of febrile ulceronecrotic Mucha-Habermann disease. Pediatr Dermatol 2014;31:525-7.

Lode HN, Doring P, Lauenstein P, Hoeger P, Dombrowski F, Bruns R. Febrile ulceronecrotic Mucha-Habermann disease following suspected hemorrhagic chickenpox infection in a 20-month-old boy. Infection 2015;43:583-8.

Downloads

Published

2021-05-17

How to Cite

1.
Sooksamran A, Sudtikoonaseth P, Sajjachareonpong P, Tienthavorn T. Febrile Ulceronecrotic Mucha-Habermann Disease: A Rare Case Report and Review of Cases Treated With Oral Cyclosporine. J Health Sci Med Res [Internet]. 2021 May 17 [cited 2024 Mar. 29];40(1):89-94. Available from: https://he01.tci-thaijo.org/index.php/jhsmr/article/view/255354

Issue

Section

Case Report