Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Pityriasis lichenoides et varioliformis acuta (PLEVA)

James R Treat, MD
Section Editors
Moise L Levy, MD
John A Zic, MD
Deputy Editor
Abena O Ofori, MD


Pityriasis lichenoides et varioliformis acuta (PLEVA), otherwise known as Mucha-Habermann disease, is an uncommon cutaneous inflammatory disorder that most frequently affects young adults and children. PLEVA usually presents as an acute eruption of inflammatory papules and papulovesicles that rapidly develop hemorrhagic or necrotic crusts (picture 1A-C). Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a potentially life-threatening, severe presentation of PLEVA.

Pityriasis lichenoides chronica (PLC), which is characterized by the appearance of multiple scaly, red-brown papules on the skin, is often considered to be on a disease continuum with PLEVA (picture 2) [1]. The term "pityriasis lichenoides" is frequently used to refer to the spectrum of these disorders.

The clinical features, diagnosis, and management of PLEVA will be discussed here. PLC is reviewed separately. (See "Pityriasis lichenoides chronica".)


PLEVA is a rare disorder. The precise incidence and prevalence are not known. PLEVA may occur at any age (including infants) [2], but most frequently occurs in children and young adults.

Data on PLEVA and PLC are often combined in the literature under pityriasis lichenoides, complicating the assessment of the epidemiology of PLEVA. A slight male predominance has been detected among children with pityriasis lichenoides [1], and in one of the largest series of children with pityriasis lichenoides, 40 of 71 children with PLEVA (56 percent) were male [3]. A male predominance is less consistently observed in the general population of patients with pityriasis lichenoides [1].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Aug 08, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol 2006; 55:557.
  2. Hoshina D, Akiyama M, Hamasaka K, Shimizu H. An infantile case of pityriasis lichenoides et varioliformis acuta. Br J Dermatol 2007; 157:194.
  3. Ersoy-Evans S, Greco MF, Mancini AJ, et al. Pityriasis lichenoides in childhood: a retrospective review of 124 patients. J Am Acad Dermatol 2007; 56:205.
  4. 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. 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.
  6. 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. 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.
  8. Weiss LM, Wood GS, Ellisen LW, et al. Clonal T-cell populations in pityriasis lichenoides et varioliformis acuta (Mucha-Habermann disease). Am J Pathol 1987; 126:417.
  9. Rongioletti F, Rivara G, Rebora A. Pityriasis lichenoides et varioliformis acuta and acquired toxoplasmosis. Dermatologica 1987; 175:41.
  10. Boss JM, Boxley JD, Summerly R, Sutton RN. The detection of Epstein Barr virus antibody in 'exanthematic' dermatoses with special reference to pityriasis lichenoides. A preliminary survey. Clin Exp Dermatol 1978; 3:51.
  11. Tomasini D, Tomasini CF, Cerri A, et al. Pityriasis lichenoides: a cytotoxic T-cell-mediated skin disorder. Evidence of human parvovirus B19 DNA in nine cases. J Cutan Pathol 2004; 31:531.
  12. English JC 3rd, Collins M, Bryant-Bruce C. Pityriasis lichenoides et varioliformis acuta and group-A beta hemolytic streptococcal infection. Int J Dermatol 1995; 34:642.
  13. Ostlere LS, Langtry JA, Branfoot AC, Staughton RC. HIV seropositivity in association with pityriasis lichenoides et varioliformis acuta. Clin Exp Dermatol 1992; 17:36.
  14. Smith JJ, Oliver GF. Febrile ulceronecrotic Mucha-Habermann disease associated with herpes simplex virus type 2. J Am Acad Dermatol 2009; 60:149.
  15. Longley J, Demar L, Feinstein RP, et al. Clinical and histologic features of pityriasis lichenoides et varioliformis acuta in children. Arch Dermatol 1987; 123:1335.
  16. Gunatheesan S, Ferguson J, Moosa Y. Pityriasis lichenoides et varioliformis acuta: a rare association with the measles, mumps and rubella vaccine. Australas J Dermatol 2012; 53:e76.
  17. Torinuki W. Mucha-Habermann disease in a child: possible association with measles vaccination. J Dermatol 1992; 19:253.
  18. Baykal L, Arıca DA, Yaylı S, et al. Pityriasis lichenoides et varioliformis acuta; Association with tetanus vaccination. J Clin Case Rep 2015; 5:518.
  19. Castro BA, Pereira JM, Meyer RL, et al. Pityriasis lichenoides et varioliformis acuta after influenza vaccine. An Bras Dermatol 2015; 90:181.
  20. Herron MD, Bohnsack JF, Vanderhooft SL. Septic, CD-30 positive febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta. Pediatr Dermatol 2005; 22:360.
  21. 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.
  22. 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.
  23. Martínez-Peinado C, Galán-Gutiérrez M, Ruiz-Villaverde R, Solorzano-Mariscal R. Adalimumab-Induced Pityriasis Lichenoides Chronica That Responded Well to Methotrexate in a Patient With Psoriasis. Actas Dermosifiliogr 2016; 107:167.
  24. Echeverri AF, Vidal A, Cañas CA, et al. Etanercept-induced pityriasis lichenoides chronica in a patient with rheumatoid arthritis. Case Rep Dermatol Med 2015; 2015:168063.
  25. Kawamura K, Tsuji T, Kuwabara Y. Mucha-Habermann disease-like eruptions due to Tegafur. J Dermatol 1999; 26:164.
  26. Stosiek N, Peters KP, von den Driesch P. [Pityriasis-lichenoides-et-varioliformis-acuta-like drug exanthema caused by astemizole]. Hautarzt 1993; 44:235.
  27. Jowkar F, Namazi MR, Bahmani M, Monabati A. Triggering of pityriasis lichenoides et varioliformis acuta by radiocontrast iodide. J Dermatolog Treat 2008; 19:249.
  28. Hollander A, Grots IA. Mucha-Habermann disease following estrogen-progesterone therapy. Arch Dermatol 1973; 107:465.
  29. Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol 2007; 8:29.
  30. Wood GS, Hu CH, Liu R. Parapsoriasis and pityriasis lichenoides. In: Fitzpatrick's Dermatology in General Medicine, 8th ed, Goldsmith LA, Katz SI, Gilchrest BA, et al. (Eds), McGraw Hill, New York 2012. Vol 1, p.285.
  31. Wahie S, Hiscutt E, Natarajan S, Taylor A. Pityriasis lichenoides: the differences between children and adults. Br J Dermatol 2007; 157:941.
  32. Rosman IS, Liang LC, Patil S, et al. Febrile ulceronecrotic Mucha-Habermann disease with central nervous system vasculitis. Pediatr Dermatol 2013; 30:90.
  33. Auster BI, Santa Cruz DJ, Eisen AZ. Febrile ulceronecrotic Mucha-Habermann's disease with interstitial pneumonitis. J Cutan Pathol 1979; 6:66.
  34. 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.
  35. 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.
  36. Romaní J, Puig L, Fernández-Figueras MT, de Moragas JM. Pityriasis lichenoides in children: clinicopathologic review of 22 patients. Pediatr Dermatol 1998; 15:1.
  37. Fernandes NF, Rozdeba PJ, Schwartz RA, et al. Pityriasis lichenoides et varioliformis acuta: a disease spectrum. Int J Dermatol 2010; 49:257.
  38. Hood AF, Mark EJ. Histopathologic diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation. Arch Dermatol 1982; 118:478.
  39. 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.
  40. Piamphongsant T. Tetracycline for the treatment of pityriasis lichenoides. Br J Dermatol 1974; 91:319.
  41. Shelley WB, Griffith RF. Pityriasis lichennoides et varioliformis acuta. A report of a case controlled by a high dose of tetracycline. Arch Dermatol 1969; 100:596.
  42. Truhan AP, Hebert AA, Esterly NB. Pityriasis lichenoides in children: therapeutic response to erythromycin. J Am Acad Dermatol 1986; 15:66.
  43. Hapa A, Ersoy-Evans S, Karaduman A. Childhood pityriasis lichenoides and oral erythromycin. Pediatr Dermatol 2012; 29:719.
  44. Rasmussen JE. Mucha-Habermann's disease. Arch Dermatol 1979; 115:676.
  45. Skinner RB, Levy AL. Rapid resolution of pityriasis lichenoides et varioliformis acuta with azithromycin. J Am Acad Dermatol 2008; 58:524.
  46. Di Costanzo L, Balato N, La Bella S, Balato A. Successful association in the treatment of pityriasis lichenoides et varioliformis acuta. J Eur Acad Dermatol Venereol 2009; 23:971.
  47. Aydogan K, Saricaoglu H, Turan H. Narrowband UVB (311 nm, TL01) phototherapy for pityriasis lichenoides. Photodermatol Photoimmunol Photomed 2008; 24:128.
  48. Pavlotsky F, Baum S, Barzilai A, et al. UVB therapy of pityriasis lichenoides--our experience with 29 patients. J Eur Acad Dermatol Venereol 2006; 20:542.
  49. Pinton PC, Capezzera R, Zane C, De Panfilis G. Medium-dose ultraviolet A1 therapy for pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica. J Am Acad Dermatol 2002; 47:410.
  50. Simon D, Boudny C, Nievergelt H, et al. Successful treatment of pityriasis lichenoides with topical tacrolimus. Br J Dermatol 2004; 150:1033.
  51. Lynch PJ, Saied NK. Methotrexate treatment of pityriasis lichenoides and lymphomatoid papulosis. Cutis 1979; 23:634.
  52. Cornelison RL Jr, Knox JM, Everett MA. Methotrexate for the treatment of Mucha-Habermann disease. Arch Dermatol 1972; 106:507.
  53. Lazaridou E, Fotiadou C, Tsorova C, et al. Resistant pityriasis lichenoides et varioliformis acuta in a 3-year-old boy: successful treatment with methotrexate. Int J Dermatol 2010; 49:215.
  54. Pansé I, Bourrat E, Rybojad M, Morel P. [Photochemotherapy for pityriasis lichenoides: 3 cases]. Ann Dermatol Venereol 2004; 131:201.
  55. Fortson JS, Schroeter AL, Esterly NB. Cutaneous T-cell lymphoma (parapsoriasis en plaque). An association with pityriasis lichenoides et varioliformis acuta in young children. Arch Dermatol 1990; 126:1449.
  56. Thomson KF, Whittaker SJ, Russell-Jones R, Charles-Holmes R. Childhood cutaneous T-cell lymphoma in association with pityriasis lichenoides chronica. Br J Dermatol 1999; 141:1146.
  57. Panizzon RG, Speich R, Dazzi H. Atypical manifestations of pityriasis lichenoides chronica: development into paraneoplasia and non-Hodgkin lymphomas of the skin. Dermatology 1992; 184:65.