Skip to main content

Pityriasis Lichenoides

Pathophysiology, Classification, and Treatment

Abstract

Pityriasis lichenoides (PL) is an uncommon, acquired spectrum of skin conditions that poses various challenges to patients as well as clinicians. It is a difficult and debatable disorder to diagnose, categorize, and treat. Besides these inherent obstacles, PL merits awareness because of its potential to progress to cutaneous lymphoma or an ulceronecrotic presentation, both of which carry a significant risk of mortality.

The scope of PL presentations is delineated along a continuum of multiple variants including pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC), and febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Classification of these presentations as separate subsets is debatable in view of their overlapping clinical, histopathologic, and etiologic features. PLEVA generally presents as an acute-to-subacute skin eruption of multiple, small, red papules that develops into polymorphic lesions and vacillates with periods of varying remissions as well as possible sequelae of hyper/hypopigmentation and varicella-like scars. PLC has a more gradual manifestation of very small red-to-brown flat maculopapules with mica-like scale; it also follows a relapsing course but with long periods of remission. FUMHD is an acute and severe generalized eruption of purpuric and ulceronecrotic plaques with associated systemic involvement and a mortality rate of up to 25%; hence, it should be approached as a dermatologic emergency.

Histopathological evaluation of PL usually reveals dermal, wedge-shaped, lymphocytic infiltrate, epidermal spongiosis, parakeratosis, and variable necrosis of keratinocytes. PLC demonstrates more subtle histology whereas, at the other end of the spectrum, febrile ulceronecrotic FUMHD exhibits the most exaggerated histological features.

The pathogenic mechanism behind PL is unclear although infectious or drug-related hypersensitivity reactions versus premycotic lymphoproliferative disorder are the mainstay theories.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Table I

References

  1. Cozzio A, Hafner J, Kempf W.: Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity? J Am Acad Dermatol 2004; 51: 1014–7

    PubMed  Article  Google Scholar 

  2. Daoud MS, Pittelkow MR.: Pityriasis lichenoides. In. Freedberg IM, Eisen AZ, Wolff Keditors. Fitzpatrick’s dermatology in general medicine. 6th edition. New York: McGraw-Hill, Inc., 2003: 456–62

    Google Scholar 

  3. Wood GS, Reizner G.: Other papulosquamous disorders.In: Bolognia JL, Jorizzo J, Rapini R, editors. Dermatology. London: CV Mosby, 2003: 151–63

    Google Scholar 

  4. Ricci G, Patrizi A, Misciali D.: Pathological case of the month: febrile Mucha-Habermann disease. Arch Pediatr Adolesc Med 2001; 155: 195–6

    PubMed  CAS  Google Scholar 

  5. Lichenoides and interface dermatoses. In:McKee PH, Calonje E, Granter SR, editors. Pathology of the skin with clinical correlations. 3rd edition. London: Mosby Ltd, 2005: 217–60

  6. Horn TD.: Inflammatory reactions in the skin. In: aiBarnhill RL. Textbook of dermatopathology. New York: McGraw-Hill, Inc, 1998: 31–54

    Google Scholar 

  7. Gelmetti C, Rigoni C, Alessi E.: Pityriasis lichenoides in children: a long term follow-up of eighty-nine cases. J Am Acad Dermatol 1990; 23: 473–8

    PubMed  Article  CAS  Google Scholar 

  8. Tsianakas A, Hoeger PH.: Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumor necrosis factor-alpha. Br J Dermatol 2005; 152: 794–9

    PubMed  Article  CAS  Google Scholar 

  9. Tomasini D, Zampatti C, Palmedo G.: Cytotoxic mycosis fungoides evolving from pityriasis lichenoides chronica in a seventeen-year-old girl. Dermatology 2002; 205: 176–9

    PubMed  Article  Google Scholar 

  10. Rivera R, Ortiz J, Rodriguez-Peralto L.: Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta with atypical cells. Int J Dermatol 2003; 42: 26–8

    PubMed  Article  CAS  Google Scholar 

  11. Degos R, Duperrat B, Daniel F.: Le parapsoriasis ulceronecrotique hyperthermique. Ann Dermatol Venereol 1966; 93: 481–96

    CAS  Google Scholar 

  12. Yang C, Lee J, Chen W.: Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas. Eur J Dermatol 2003; 13: 493–6

    PubMed  Google Scholar 

  13. Yanaba K, Ito M, Sasaki H.: A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft. Br J Dermatol 2002; 147: 1249–53

    PubMed  Article  CAS  Google Scholar 

  14. Tsuji T, Kasamatsu M, Yokota M.: Mucha-Habermann disease and its febrile ulceronecrotic variant. Cutis 1996; 58: 123–31

    PubMed  CAS  Google Scholar 

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

    PubMed  Article  Google Scholar 

  16. Malnar T, Milaver-Puretic V, Rados J.: Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta with fatal outcome. J Eur Acad Dermatol Venereol 2006; 20: 303–7

    PubMed  Article  CAS  Google Scholar 

  17. Miyamoto T, Takayama N, Kitada S.: Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. J Clin Pathol 2003; 56: 795–7

    PubMed  Article  CAS  Google Scholar 

  18. Patel DG, Kihiczak G, Schwartz RA.: Pityriasis lichenoides. Cutis 2000; 65: 17–20, 23

    PubMed  CAS  Google Scholar 

  19. Henning JS.: Pityriasis lichenoides chronica. Dermatol Online J 2004; 10: 8

    PubMed  Google Scholar 

  20. Ko J, Seong J, Suh K.: Pityriasis lichenoides-like mycosis fungoides in children. Br J Dermatol 2000; 142: 347–52

    PubMed  Article  CAS  Google Scholar 

  21. Slater DN.: The new World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphoma: a practical marriage of two giants. Br J Dermatol 2005; 153: 874–80

    PubMed  Article  CAS  Google Scholar 

  22. Varga FJ, Vonderheid EC, Olbricht SM.: Immunohistochemical distinction of lymphomatoid papulosis and pityriasis lichenoides et varioliformis acuta. Am J Pathol 1990; 136: 979–87

    PubMed  CAS  Google Scholar 

  23. Panhans A, Bodemer C, Macinthyre E.: Pityriasis lichenoides of childhood with atypical CD30-positive cells and clonal T-cell receptor gene rearrangements. J Am Acad Dermatol 1996; 35: 489–90

    PubMed  Article  CAS  Google Scholar 

  24. Zlatkov NB, Adreev VC.: Toxoplasmosis and pityriasis lichenoides. Br J Dermatol 1972; 87: 114–6

    PubMed  Article  CAS  Google Scholar 

  25. Tomasini D, Tomasini CF, Cerri A.: Pityriasis lichenoides: a cytotoxic T-cell-mediated skin disorder. Evidence of human parvovirus B19 DNA in nine cases. J Cutan Pathol 2004; 31: 531–8

    PubMed  Article  Google Scholar 

  26. Boralevi F, Cotto E, Baysse L.: Is varicella zoster virus involved in the etiopathogeny of pityriasis lichenoides? J Invest Dermatol 2003; 121: 647–8

    PubMed  Article  Google Scholar 

  27. Klein PA, Jones EC, Nelson JL.: Infectious causes of pityriasis lichenoides: a case of fulminant infectious mononucleosis. J Am Acad Dermatol 2003; 49: 151–3

    Article  Google Scholar 

  28. Tsai KS, Hsieh HJ, Chow KC.: Detection of cytomegalovirus infection in a patient with febrile ulceronecrotic Mucha-Habermann’s disease. Int J Dermatol 2001; 40: 694–8

    PubMed  Article  CAS  Google Scholar 

  29. Rongioletti F, Delmonte S, Rebora A.: Pityriasis lichenoides and acquired toxoplasmosis. Int J Dermatol 1999; 38: 372–4

    PubMed  Article  CAS  Google Scholar 

  30. Smith KJ, Nelson A, Skelton H.: Pityriasis lichenoides et varioliformis acuta in HIV-1+ patient: a marker of early stage disease. Int J Dermatol 1997; 36: 104–9

    PubMed  Article  CAS  Google Scholar 

  31. Kawamura K, Tsuji T, Kuwabara Y.: Mucha-Habermann disease-like eruptions due to tegafur. J Dermatol 1999; 26: 164–7

    PubMed  CAS  Google Scholar 

  32. Hollander A, Grots IA.: Mucha-Habermann disease following estrogen-progesterone therapy. Arch Dermatol 1973; 107: 465

    PubMed  Article  CAS  Google Scholar 

  33. Stosiek N, Peters KP, Von den DrieschP.: Pityriasis-lichenoides-et-varioliformis-acuta-like drug exanthema caused by astemizole. Hautarzt 1993; 44: 235–7

    PubMed  CAS  Google Scholar 

  34. Tanaka M.: Drug-induced pityriasis lichenoides et varioliformis acuta. Rinsho Hifuka 1990; 44: 307–10

    Google Scholar 

  35. Nishimura M, Matsuda T, Hori Y.: Mucha-Habermann disease resolves after tonsillectomy. Int J Dermatol 1991; 30: 896–7

    PubMed  Article  CAS  Google Scholar 

  36. Zechini B, Teggi A, Antonelli M.: A case report of pityriasis lichenoides in a patient with chronic hepatitis C. J Infect 2005; 51: e23–5

    Article  Google Scholar 

  37. Clayton R, Haffenden G.: An immunofluorescence study of pityriasis lichenoides. Br J Dermatol 1978; 99: 491–3

    PubMed  Article  CAS  Google Scholar 

  38. Clayton R, Haffenden G, Du Vivier A.: Pityriasis lichenoides: an immune complex disease. Br J Dermatol 1977; 97: 629–34

    PubMed  Article  CAS  Google Scholar 

  39. Muhlbauer JE, Bhan AK, Harrist TJ.: Immunopathology of pityriasis lichenoides acuta. J Am Acad Dermatol 1984; 10: 783–95

    PubMed  Article  CAS  Google Scholar 

  40. Khosrotehrani K, Guegan S, Fraitag S.: Presence of chimeric maternally derived keratinocytes in cutaneous inflammatory diseases of children: the example of pityriasis lichenoides. J Invest Dermatol 2006; 126: 345–8

    PubMed  Article  CAS  Google Scholar 

  41. 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

    PubMed  Article  CAS  Google Scholar 

  42. Margo C, Crowson NA, Kovatich A.: Pityriasis lichenoides: a clonal T-cell lymphoproliferative disorder. Hum Pathol 2002; 33: 788–95

    Article  Google Scholar 

  43. Weinberg JM, Kristal L, Chooback L.: The clonal nature of pityriasis lichenoides. Arch Dermatol 2002; 138: 1063–7

    PubMed  Article  Google Scholar 

  44. Black MM.: Lymphomatoid papulosis and pityriasis lichenoides: are they related? Br J Dermatol 1982; 106: 717–21

    PubMed  CAS  Google Scholar 

  45. Niemczyk UM, Zollner TM, Wolter M.: The transformation of pityriasis lichenoides chronica into parakeratosis variegata in an 11-year-old girl. Br J Dermatol 1997; 137: 983–7

    PubMed  Article  CAS  Google Scholar 

  46. Panizzon RG, Speich R, Dazzi H.: Atypical manifestations of pityriasis lichenoides chronica: development into paraneoplasia and non-Hodgkin lymphoma of the skin. Dermatology 1992; 184: 65–9

    PubMed  Article  CAS  Google Scholar 

  47. 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: 1449–53

    PubMed  Article  CAS  Google Scholar 

  48. Wenzel J, Gutgemann I, Distelmaier M.: The role of cytotoxic skin-homing CD8+ lymphocytes in cutaneous cytotoxic T-cell lymphoma and pityriasis lichenoides. J Am Acad Dermatol 2005; 53: 422–7

    PubMed  Article  Google Scholar 

  49. Dupont C.: Pityriasis lichenoides in a family. Br J Dermatol 1995; 133: 338–9

    PubMed  Article  CAS  Google Scholar 

  50. Garcia B, Connelly EA, Newbury R.: Pityriasis lichenoides and idiopathic thrombocytopenia purpura in a young girl. Pediatr Dermatol 2006; 23: 21–3

    PubMed  Article  Google Scholar 

  51. Kempf W, Kutzner H, Kettelhack G.: Paraneoplastic pityriasis lichenoides in cutaneous lymphoma: a case report and review of the literature on paraneoplastic reactions of skin in lymphoma and leukemia. Br J Dermatol 2005; 152: 1327–31

    PubMed  Article  CAS  Google Scholar 

  52. Brazzini B, Ghersetich I, Urso C.: Pityriasis lichenoides et varioliformis acuta during pregnancy. J Eur Acad Dermatol Venereol 2001; 15: 458–60

    PubMed  Article  CAS  Google Scholar 

  53. Eskandar MA.: Pityriasis lichenoides et varioliformis acuta in pregnancy. Saudi Med J 2001; 22: 1127–9

    PubMed  CAS  Google Scholar 

  54. Milligan A, Johnston G.: Pityriasis lichenoides chronica and pityriasis lichenoides et varioliformis acuta. In: Lebwohl M, Berth-Jones J, Heymann WR, editors. Treatment of skin disease. London: CV Mosby, 2002: 472–7

    Google Scholar 

  55. Pinton PC, Capezzera R, Zane C.: Medium-dose ultraviolet A1 therapy for pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica. J Am Acad Dermatol 2002; 47: 410–4

    PubMed  Article  Google Scholar 

  56. Tay YK, Morelli JG, Weston WL.: Experience with UVB phototherapy in children. Pediatr Dermatol 1996; 13: 406–9

    PubMed  Article  CAS  Google Scholar 

  57. Pavlotsky F, Baum S, Barzilai A.: UVB therapy of pityriasis lichenoides: our experience with 29 patients. J Eur Acad Dermatol Venereol 2006; 20: 642–7

    Google Scholar 

  58. 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

    PubMed  Article  CAS  Google Scholar 

  59. Maekawa Y, Nakamura T, Nogami R.: Febrile ulceronecrotic Mucha-Habermann’s disease. J Dermatol 1994; 21: 46–9

    PubMed  CAS  Google Scholar 

  60. Griffiths JK.: Successful long-term use of cyclosporin A in HIV-induced pityriasis lichenoides chronica. J Acquir Immune Defic Syndr Hum Retrovirol 1998; 18: 396–7

    PubMed  Article  CAS  Google Scholar 

  61. Simon D, Boudny C, Nievergelt H.: Successful treatment of pityriasis lichenoides with topical tacrolimus. Br J Dermatol 2004; 150: 1033–5

    PubMed  Article  CAS  Google Scholar 

  62. Ito N, Ohshima A, Hashizume H.: Febrile ulceronecrotic Mucha-Habermann’s disease managed with methylprednisolone semipulse and subsequent methotrexate therapies. J Am Acad Dermatol 2003; 49: 1142–8

    PubMed  Article  Google Scholar 

  63. Nakamura S, Nishihara K, Nakajima K.: Febrile ulceronecrotic Mucha-Habermann’s disease and its successful therapy with DDS. J Dermatol 1989; 13: 381–4

    Google Scholar 

  64. Suarez J, Lopez B, Villalba R.: Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. Dermatology 1996; 192: 277–9

    PubMed  Article  CAS  Google Scholar 

Download references

Acknowledgments

No sources of funding were used to assist in the preparation of this review. The authors have no conflicts of interest that are directly relevant to the content of this review.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Amor Khachemoune.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Khachemoune, A., Blyumin, M.L. Pityriasis Lichenoides. Am J Clin Dermatol 8, 29–36 (2007). https://doi.org/10.2165/00128071-200708010-00004

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.2165/00128071-200708010-00004

Keywords

  • Mycosis Fungoides
  • Foreign Agent
  • Cutaneous Lymphoma
  • Erythematous Papule
  • Secondary Syphilis