Mortality risk factors in febrile ulceronecrotic Mucha- Habermann disease: a systematic review of therapeutic outcomes and complications
https://doi.org/10.4081/dr.2022.9492
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Febrile ulceronecrotic Mucha-Habermann Disease (FUMHD) is a variant of Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA). Although rare, the condition may progress to involve serious complications and even lead to fatal outcomes if diagnosis and appropriate treatment is delayed. A PubMed search following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRIMSA) guidelines was performed to find cases of FUMHD from the earliest records to October 2021. Treatments, complications, and patient outcomes were extracted from the literature and summarized, while a review of quality was also performed. A total of 63 publications with 68 patients were found. Successful treatment modalities for FUMHD included antibiotics, antivirals, systemic steroids, methotrexate (MTX), cyclophosphamide, cyclosporine (CYA), intravenous immunoglobulins (IVIG), pentoxifylline, and ultraviolet B phototherapy. Out of 68 patients, 55 patients had their condition fully resolved and 13 cases were fatal. Increased age, systemic involvement, and monoclonal T-cell receptor rearrangement were associated with worst prognosis, but mucosal involvement did not affect mortality risk. Overall, the publications had low risk of bias, but most lacked adequate follow-up periods. FUMHD is a diagnostic and therapeutic challenge due to the lack of clearly defined diagnostic criteria and optimum treatment. Further studies with larger patient populations and longer follow-up periods may lead to refinement of diagnostic criteria, establish an optimum treatment regimen, and better estimate the likelihood of recurrence.
Degos R, Duperrat B, Daniel F. [Hyperthermic ulcero-necrotic parapsoriasis. Subacute form of parapsoriasis guttata]. Ann Dermatol Syphiligr (Paris) 1966;93:481-96.
Burke DP, Adams RM, Arundell FD. Febrile ulceronecrotic mucha Habermann's disease. Arch Dermatol 1969;100:200-6. DOI: https://doi.org/10.1001/archderm.100.2.200
Luo DQ. Febrile ulceronecrotic Mucha-Habermann disease. Cutis 2013;92:E9-E12.
Bellinato F, Maurelli M, Gisondi P, et al. A systematic review of treatments for pityriasis lichenoides. J Eur Acad Dermatol Venereol 2019;33:2039-49. DOI: https://doi.org/10.1111/jdv.15813
De Cuyper C, Hindryckx P, Deroo N. Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. Dermatology 1994;189:50-3. DOI: https://doi.org/10.1159/000246991
Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol 2006;55:557-72; quiz 73-6. DOI: https://doi.org/10.1016/j.jaad.2005.07.058
Nofal A, Assaf M, Alakad R, et al. Febrile ulceronecrotic Mucha-Habermann disease: proposed diagnostic criteria and therapeutic evaluation. Int J Dermatol 2016;55:729-38. DOI: https://doi.org/10.1111/ijd.13195
Kaufman WS, McNamara EK, Curtis AR, et al. Febrile ulceronecrotic Mucha-Habermann disease (pityriasis lichenoides et varioliformis acuta fulminans) presenting as Stevens-Johnson syndrome. Pediatr Dermatol 2012;29:135-40. DOI: https://doi.org/10.1111/j.1525-1470.2011.01608.x
Suarez J, Lopez B, Villalba R, et al. Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. Dermatology 1996;192:277-9. DOI: https://doi.org/10.1159/000246385
Sotiriou E, Patsatsi A, Tsorova C, et al. Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. Acta Derm Venereol 2008;88:350-5.
Blohm ME, Ebenebe CU, Rau C, et al. Mucha-Habermann disease: a pediatric case report and proposal of a risk score. Int J Dermatol 2022;61:401-9. DOI: https://doi.org/10.1111/ijd.15770
Cozzio A, Hafner J, Kempf W, et al. Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity? J Am Acad Dermatol 2004;51:1014-7. DOI: https://doi.org/10.1016/j.jaad.2004.06.009
Aytekin S, Balci G, Duzgun OY. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. Dermatol Online J 2005;11:31. DOI: https://doi.org/10.5070/D36KP6F1J2
Bhide DS, Tulpule MS, Pethe SV. A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities. Indian J Dermatol Venereol Leprol 2019;85:660-3. DOI: https://doi.org/10.4103/ijdvl.IJDVL_552_17
Gungor E, Alli N, Artuz F, et al. Febrile ulceronecrotic Mucha-Habermann's disease. Int J Dermatol 1996;35:895-6. DOI: https://doi.org/10.1111/j.1365-4362.1996.tb05064.x
Hoghton MA, Ellis JP, Hayes MJ. Febrile ulceronecrotic Mucha Habermann disease: a fatality. J R Soc Med 1989;82:500-1. DOI: https://doi.org/10.1177/014107688908200820
Malnar T, Milavec-Puretic V, Rados J, et al. Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta with fatal outcome. J Eur Acad Dermatol Venereol 2006;20:303-7. DOI: https://doi.org/10.1111/j.1468-3083.2006.01389.x
Meziane L, Caudron A, Dhaille F, et al. Febrile ulceronecrotic Mucha-Habermann disease: treatment with infliximab and intravenous immunoglobulins and review of the literature. Dermatology 2012;225:344-8. DOI: https://doi.org/10.1159/000346245
Miyamoto T, Takayama N, Kitada S, et al. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. J Clin Pathol 2003;56:795-7. DOI: https://doi.org/10.1136/jcp.56.10.795
Nofal A, Alakad R, Assaf M, et al. A fatal case of febrile ulceronecrotic Mucha-Habermann disease in a child. JAAD Case Rep 2016;2:181-5. DOI: https://doi.org/10.1016/j.jdcr.2016.03.002
Puddu P, Cianchini G, Colonna L, et al. Febrile ulceronecrotic Mucha-Habermann's disease with fatal outcome. Int J Dermatol 1997;36:691-4.
Xing C, Shen H, Xu J, et al. A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease which Presenting as Toxic Epidermal Necrolysis. Indian J Dermatol 2017;62:675. DOI: https://doi.org/10.4103/ijd.IJD_631_16
Alratrout J, Alshammasi F, Ansari N. Febrile ulceronecrotic Mucha-Habermann disease in an 8-year-old boy responding to methotrexate. Int J Dermatol 2016;55:1205-9. DOI: https://doi.org/10.1111/ijd.13323
Auster BI, Santa Cruz DJ, Eisen AZ. Febrile ulceronecrotic Mucha-Habermann's disease with interstitial pneumonitis. J Cutan Pathol 1979;6:66-76. DOI: https://doi.org/10.1111/j.1600-0560.1979.tb00307.x
Aydingoz IE, Kocaayan N, Mansur AT, et al. A case of ulceronecrotic Mucha-Habermann disease with pulmonary involvement. Dermatology 2006;212:388-90. DOI: https://doi.org/10.1159/000092294
Bulur I, Kaya Erdoğan H, Nurhan Saracoglu Z, et al. Methotrexate Treatment in Children with Febrile Ulceronecrotic Mucha-Habermann Disease: Case Report and Literature Review. Case Rep Dermatol Med 2015;2015:357973. DOI: https://doi.org/10.1155/2015/357973
Fink-Puches R, Soyer HP, Kerl H. Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. J Am Acad Dermatol 1994;30:261-3. DOI: https://doi.org/10.1016/S0190-9622(08)81921-4
Griffith-Bauer K, Leitenberger SL, Krol A. Febrile Ulceronecrotic Mucha-Habermann Disease: Two Cases with Excellent Response to Methotrexate. Pediatr Dermatol 2015;32:e307-8. DOI: https://doi.org/10.1111/pde.12687
Harenberg PS, Hrabowski M, Ryssel H, et al. CASE REPORT Febrile Ulceronecrotic Mucha-Habermann Disease. Eplasty 2010;10:e53.
Helbling I, Chalmers RJ, Yates VM. Febrile ulceronecrotic Mucha-Habermann disease: a rare dermatological emergency. Clin Exp Dermatol 2009;34:e1006-7. DOI: https://doi.org/10.1111/j.1365-2230.2009.03666.x
Helmbold P, Gaisbauer G, Fiedler E, et al. Self-limited variant of febrile ulceronecrotic Mucha-Habermann disease with polyclonal T-cell receptor rearrangement. J Am Acad Dermatol 2006;54:1113-5. DOI: https://doi.org/10.1016/j.jaad.2006.02.043
Herron MD, Bohnsack JF, Vanderhooft SL. Septic, CD-30 positive febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. Pediatr Dermatol 2005;22:360-5. DOI: https://doi.org/10.1111/j.1525-1470.2005.22418.x
Hervas JA, Martin-Santiago A, Hervas D, et al. Varicella precipitating febrile ulceronecrotic Mucha-Habermann disease. Pediatr Dermatol 2013;30:e216-7. DOI: https://doi.org/10.1111/pde.12008
Ito N, Ohshima A, Hashizume H, et al. Febrile ulceronecrotic Mucha-Habermann's disease managed with methylprednisolone semipulse and subsequent methotrexate therapies. J Am Acad Dermatol 2003;49:1142-8. DOI: https://doi.org/10.1016/S0190-9622(03)00461-4
Kilgallon K, Urs J, Fernandez Faith E. Visual Diagnosis: Severe Ulceronecrotic Eruption with Systemic Symptoms. Pediatr Rev 2018;39:e54-e6. DOI: https://doi.org/10.1542/pir.2017-0039
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. DOI: https://doi.org/10.1111/j.1468-3083.2006.01843.x
Kreuter A, Knispel S, Wieland U, et al. Complete resolution of febrile ulceronecrotic Mucha-Habermann disease following infliximab therapy. J Dtsch Dermatol Ges 2016;14:184-6. DOI: https://doi.org/10.1111/ddg.12742
Lejuste FX, Michaux C, Lehners C, et al. Febrile ulceronecrotic Mucha-Habermann disease. BMJ Case Rep 2013;2013:bcr2013009739. DOI: https://doi.org/10.1136/bcr-2013-009739
Lin CY, Cook J, Purvis D. Febrile ulceronecrotic Mucha-Habermann disease: a case with systemic symptoms managed with subcutaneous methotrexate. Australas J Dermatol 2012;53:e83-6. DOI: https://doi.org/10.1111/j.1440-0960.2011.00812.x
Lode HN, Döring P, Lauenstein P, et al. Febrile ulceronecrotic Mucha-Habermann disease following suspected hemorrhagic chickenpox infection in a 20-month-old boy. Infection 2015;43:583-8. DOI: https://doi.org/10.1007/s15010-015-0726-5
Lopez-Estebaranz JL, Vanaclocha F, Gil R, et al. Febrile ulceronecrotic Mucha-Habermann disease. J Am Acad Dermatol 1993;29:903-6. DOI: https://doi.org/10.1016/0190-9622(93)70267-W
Luberti AA, Rabinowitz LG, Ververeli KO. Severe febrile Mucha-Habermann's disease in children: case report and review of the literature. Pediatr Dermatol 1991;8:51-7. DOI: https://doi.org/10.1111/j.1525-1470.1991.tb00841.x
Marenco F, Fava P, Fierro MT, et al. High-dose immunoglobulines and extracorporeal photochemotherapy in the treatment of febrile ulceronecrotic Mucha-Habermann disease. Dermatol Ther 2010;23:419-22. DOI: https://doi.org/10.1111/j.1529-8019.2010.01343.x
Nakamura S, Nishihara K, Nakayama K, et al. Febrile ulceronecrotic Mucha-Habermann's disease and its successful therapy with DDS. J Dermatol 1986;13:381-4. DOI: https://doi.org/10.1111/j.1346-8138.1986.tb02959.x
Nanda A, Alshalfan F, Al-Otaibi M, et al. Febrile ulceronecrotic Mucha-Habermann disease (pityriasis lichenoides et varioliformis acuta fulminans) associated with parvovirus infection. Am J Dermatopathol 2013;35:503-6. DOI: https://doi.org/10.1097/DAD.0b013e3182770626
Nassif PW, Godoy DA, Nakandakari S, et al. Febrile ulceronecrotic Mucha-Habermann disease in adult patient successfully treated with systemic corticosteroid. An Bras Dermatol 2010;85:891-4. DOI: https://doi.org/10.1590/S0365-05962010000600018
Ngo T, Hossain C, Cohen J, et al. A diagnostically challenging case of febrile ulceronecrotic mucha-habermann disease in an adult female successfully treated with methotrexate and cyclosporine. Case Rep Dermatol 2021;13:12-7. DOI: https://doi.org/10.1159/000511537
Oliveira LM, de Seixas Rocha M, Patriota GS, et al. Febrile ulceronecrotic mucha habermann disease: case report of a dark-skinned patient. Case Rep Dermatol 2013;5:4-10. DOI: https://doi.org/10.1159/000346742
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. DOI: https://doi.org/10.1111/j.1525-1470.2011.01531.x
Pyrpasopoulou A, Athyros VG, Karagiannis A, et al. Intravenous immunoglobulins: a valuable asset in the treatment of a case of septic febrile ulceronecrotic Mucha-Habermann disease. Dermatology 2007;215:164-5. DOI: https://doi.org/10.1159/000104271
Ricci G, Patrizi A, Misciali D, et al. Pathological case of the month. Febrile Mucha-Haberman disease. Arch Pediatr Adolesc Med 2001;155:195-6. DOI: https://doi.org/10.1001/archpedi.155.2.195
Rivera R, Ortiz P, Rodriguez-Peralto JL, et al. Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta with atypical cells. Int J Dermatol 2003;42:26-8. DOI: https://doi.org/10.1046/j.1365-4362.2003.16981.x
Rosman IS, Liang LC, Patil S, et al. Febrile ulceronecrotic Mucha-Habermann disease with central nervous system vasculitis. Pediatr Dermatol 2013;30:90-3. DOI: https://doi.org/10.1111/j.1525-1470.2011.01669.x
Schaan de Souza M, Perinazzo Pauvels LS, Martins Costa Jappur D, et al. Combination therapy with cyclosporine and intravenous immunoglobulin for febrile ulcerative Mucha-Habermann disease. Dermatol Ther 2021;34:e14655. DOI: https://doi.org/10.1111/dth.14655
Shah DJ, Dhir R, Shah H, et al. Steroid unresponsive case of ulcerative mucha-habermann disease (febrile ulcernecrotic mucha-habermann disease) treated with methotrexate. Indian J Dermatol 2014;59:631. DOI: https://doi.org/10.4103/0019-5154.143572
Smith JJ, Oliver GF. Febrile ulceronecrotic Mucha-Habermann disease associated with herpes simplex virus type 2. J Am Acad Dermatol 2009;60:149-52. DOI: https://doi.org/10.1016/j.jaad.2008.07.036
Tsai KS, Hsieh HJ, Chow KC, et al. Detection of cytomegalovirus infection in a patient with febrile ulceronecrotic Mucha-Habermann's disease. Int J Dermatol 2001;40:694-8. DOI: https://doi.org/10.1046/j.1365-4362.2001.01301.x
Tsianakas A, Hoeger PH. Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour necrosis factor-alpha. Br J Dermatol 2005;152:794-9. DOI: https://doi.org/10.1111/j.1365-2133.2005.06485.x
Uzoma MA, Wilkerson MG, Carr VL, et al. Pentoxifylline and cyclosporine in the treatment of febrile ulceronecrotic Mucha-Habermann disease. Pediatr Dermatol 2014;31:525-7. DOI: https://doi.org/10.1111/j.1525-1470.2012.01862.x
Wang B, Li J, Xie HF, et al. Striking case of Febrile ulceronecrotic Mucha-Habermann disease responding to lymphoplasmapheresis and methotrexate. J Dermatol 2020;47:e430-e1. DOI: https://doi.org/10.1111/1346-8138.15598
Warshauer BL, Maloney ME, Dimond RL. Febrile ulceronecrotic Mucha-Habermann's disease. Arch Dermatol 1983;119:597-601. DOI: https://doi.org/10.1001/archderm.119.7.597
Weins AB, Theiler M, Bogatu B, et al. Febrile ulceronecrotic Mucha-Habermann disease mimicking Kawasaki disease. J Dtsch Dermatol Ges 2020;18:140-2. DOI: https://doi.org/10.1111/ddg.13989
Wu R, DiLorenzo A, Lotke M, et al. Evaluation and treatment of febrile ulceronecrotic Mucha-Habermann disease with ruxolitinib and tocilizumab as guided by cytokine profile. JAMA Dermatol 2021;157:1381-3. DOI: https://doi.org/10.1001/jamadermatol.2021.4047
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. DOI: https://doi.org/10.2340/00015555-1791
Yanaba K, Ito M, Sasaki H, et al. A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft. Br J Dermatol 2002;147:1249-53. DOI: https://doi.org/10.1046/j.1365-2133.2002.05039.x
Yang CC, Lee JY, Chen W. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol 2003;13:493-6.
Zhang LX, Liang Y, Liu Y, et al. Febrile ulceronecrotic Mucha-Habermann's disease with pulmonary involvement. Pediatr Dermatol 2010;27:290-3. DOI: https://doi.org/10.1111/j.1525-1470.2010.01141.x
Dereure O, Levi E, Kadin ME. T-Cell clonality in pityriasis lichenoides et varioliformis acuta: a heteroduplex analysis of 20 cases. Arch Dermatol 2000;136:1483-6. DOI: https://doi.org/10.1001/archderm.136.12.1483
Johnston A, Gudjonsson JE, Sigmundsdottir H, et al. The anti-inflammatory action of methotrexate is not mediated by lymphocyte apoptosis, but by the suppression of activation and adhesion molecules. Clin Immunol 2005;114:154-63. DOI: https://doi.org/10.1016/j.clim.2004.09.001
Weinberg JM. The anti-inflammatory effects of tetracyclines. Cutis 2005;75:6-11.
Debol SM, Herron MJ, Nelson RD. Anti-inflammatory action of dapsone: inhibition of neutrophil adherence is associated with inhibition of chemoattractant-induced signal transduction. J Leukoc Biol 1997;62:827-36. DOI: https://doi.org/10.1002/jlb.62.6.827
Inoue K, Takano H, Yanagisawa R, et al. Anti-inflammatory effect of pentoxifylline. Chest 2004;126:321. DOI: https://doi.org/10.1378/chest.126.1.321
Liu D, Ahmet A, Ward L, et al. A practical guide to the monitoring and management of the complications of systemic corticosteroid therapy. Allergy Asthma Clin Immunol 2013;9:30. DOI: https://doi.org/10.1186/1710-1492-9-30
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