Pityriasis Lichenoides 

  • Author: Peter A Klein, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 12, 2010
 

Background

Pityriasis lichenoides is a rare cutaneous disorder of unknown etiology. Pityriasis lichenoides encompasses a spectrum of clinical presentations ranging from acute papular lesions that rapidly evolve into pseudovesicles and central necrosis (pityriasis lichenoides et varioliformis acuta or PLEVA) to small, scaling, benign-appearing papules (pityriasis lichenoides chronica or PLC).[1, 2] Although historically, the term Mucha-Habermann disease has referred only to PLEVA, the term applies broadly to the entire spectrum of disease including PLC. A rare febrile ulceronecrotic variant has been reported, which is a severe form of PLEVA with high fever and marked constitutional symptoms. Lesions may self-involute and resolve completely over weeks, or new lesions occasionally may appear in crops, waxing and waning spontaneously for months to years thereafter.

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Pathophysiology

Mucha-Habermann disease is not a vasculitic process despite reports of immunoglobulin and complement deposition in vessels. Fibrin is not present in the walls of vessels, and thrombi are not found in the lumen. A cell-mediated mechanism has been proposed based on a T-lymphocytic infiltrate with a cytotoxic/suppressor phenotype, diminished epidermal Langerhans cells, and a reduction of the CD4/CD8 ratio. CD30 (Ki-1) cells, which are associated with large cell lymphoma, have been identified in the infiltrate of both lymphomatoid papulosis and Mucha-Habermann disease, leading some authors to view this as a self-limited self-healing lymphoproliferative disease.[3, 4] One study suggests that pityriasis lichenoides is a form of a T-cell dyscrasia, based on the presence of intraepithelial atypical lymphocytes, phenotypic abnormalities, and TCR-gamma rearrangements.[5]

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Epidemiology

Frequency

United States

The incidence of Mucha-Habermann disease in the United States has not been reported.

International

In approximately 44,000 patients seen over 10 years in 3 catchment areas in Great Britain, 17 cases of PLEVA were diagnosed.

Mortality/Morbidity

A case series of 22 children revealed a mean duration in PLEVA of 1.6 months to complete resolution and a mean duration in PLC of 7.5 months. The natural tendency of the disease is to remit spontaneously, but some cases may wax and wane over years. Disease duration may be longer in adults. A rare severe variant of PLEVA presents with a sudden eruption of diffuse coalescent necrotic ulcerations associated with high fever.[6] Patients may develop complications such as interstitial pneumonitis, abdominal pain, malabsorption, central nervous system involvement, bacteremia, sepsis, and rheumatic manifestations. T-cell receptor clonal rearrangements of lymphocytic infiltrates have been detected in patients with PLEVA. Occasional cases (< 2%) have been reported to evolve into cutaneous lymphoma, although some reports may have represented misdiagnosis of lymphomatoid papulosis.[7]

Race

All races are affected. A racial predisposition has not been reported.

Sex

A male predominance has been reported in the pediatric population and in patients presenting with febrile ulceronecrotic Mucha-Habermann disease.

Age

Most patients present during the first 3 decades of life. Studies of children have shown a variable age of onset from 3-15 years, with a mean age of 9.3 years.

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Contributor Information and Disclosures
Author

Peter A Klein, MD  Residency Program Director, Department of Dermatology, University Hospital, State University of New York at Stony Brook

Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety monitoring committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring committee

Specialty Editor Board

Gregory J Raugi, MD, PhD  Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Daniel S Loo, MD  Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center

Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology and Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. Oct 2006;55(4):557-72; quiz 573-6. [Medline].

  2. Fernandes NF, Rozdeba PJ, Schwartz RA, Kihiczak G, Lambert WC. Pityriasis lichenoides et varioliformis acuta: a disease spectrum. Int J Dermatol. Mar 2010;49(3):257-61. [Medline].

  3. Panhans A, Bodemer C, Macinthyre E, Fraitag S, Paul C, de Prost Y. Pityriasis lichenoides of childhood with atypical CD30-positive cells and clonal T-cell receptor gene rearrangements. J Am Acad Dermatol. Sep 1996;35(3 Pt 1):489-90. [Medline].

  4. Weiss LM, Wood GS, Ellisen LW, Reynolds TC, Sklar J. Clonal T-cell populations in pityriasis lichenoides et varioliformis acuta (Mucha-Habermann disease). Am J Pathol. Mar 1987;126(3):417-21. [Medline].

  5. Magro C, Crowson AN, Kovatich A, Burns F. Pityriasis lichenoides: a clonal T-cell lymphoproliferative disorder. Hum Pathol. Aug 2002;33(8):788-95. [Medline].

  6. Smith JJ, Oliver GF. Febrile ulceronecrotic Mucha-Habermann disease associated with herpes simplex virus type 2. J Am Acad Dermatol. Jan 2009;60(1):149-52. [Medline].

  7. 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(1):65-9. [Medline].

  8. Clayton R, Warin A. Pityriasis lichenoides chronica presenting as hypopigmentation. Br J Dermatol. Mar 1979;100(3):297-302. [Medline].

  9. 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. Mar 1978;3(1):51-6. [Medline].

  10. Edwards BL, Bonagura VR, Valacer DJ, Ilowite NT. Mucha-Habermann's disease and arthritis: possible association with reactivated Epstein-Barr virus infection. J Rheumatol. Mar 1989;16(3):387-9. [Medline].

  11. Andreev VC, Angelov N, Zlatkov NB. Skin manifestations in toxoplasmosis. Arch Dermatol. Aug 1969;100(2):196-9. [Medline].

  12. Zlatkov NB, Andreev VC. Toxoplasmosis and pityriasis lichenoides. Br J Dermatol. Aug 1972;87(2):114-6. [Medline].

  13. Rongioletti F, Rivara G, Rebora A. Pityriasis lichenoides et varioliformis acuta and acquired toxoplasmosis. Dermatologica. 1987;175(1):41-4. [Medline].

  14. Nassef NE, Hammam MA. The relation between toxoplasmosis and pityriasis lichenoides chronica. J Egypt Soc Parasitol. Apr 1997;27(1):93-9. [Medline].

  15. Ostlere LS, Langtry JA, Branfoot AC, Staughton RC. HIV seropositivity in association with pityriasis lichenoides et varioliformis acuta. Clin Exp Dermatol. Jan 1992;17(1):36-7. [Medline].

  16. Smith KJ, Nelson A, Skelton H, Yeager J, Wagner KF. Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. The Military Medical Consortium for the Advancement of Retroviral Research (MMCARR). Int J Dermatol. Feb 1997;36(2):104-9. [Medline].

  17. Griffiths JK. Successful long-term use of cyclosporin A in HIV-induced pityriasis lichenoides chronica. J Acquir Immune Defic Syndr Hum Retrovirol. Aug 1 1998;18(4):396-7. [Medline].

  18. Piamphongsant T. Tetracycline for the treatment of pityriasis lichenoides. Br J Dermatol. Sep 1974;91(3):319-22. [Medline].

  19. Ackerman AB, Chongchitnant N, Sanchez J, et al. Histologic diagnosis of inflammatory skin diseases: an algorithmic method based on pattern analysis. 1997. Baltimore, Md: Lippincott Williams & Wilkins; 553-60.

  20. Aydogan K, Saricaoglu H, Turan H. Narrowband UVB (311 nm, TL01) phototherapy for pityriasis lichenoides. Photodermatol Photoimmunol Photomed. Jun 2008;24(3):128-33. [Medline].

  21. Pugashetti R, Koo J. Phototherapy in pediatric patients: choosing the appropriate treatment option. Semin Cutan Med Surg. Jun 2010;29(2):115-20. [Medline].

  22. LeVine MJ. Phototherapy of pityriasis lichenoides. Arch Dermatol. May 1983;119(5):378-80. [Medline].

  23. Panse I, Bourrat E, Rybojad M, Morel P. Photochemotherapy for pityriasis lichenoides: 3 cases. Ann Dermatol Venereol. 2004;131(2):201-3. [Medline].

  24. Pavlotsky F, Baum S, Barzilai A, Shpiro D, Trau H. UVB therapy of pityriasis lichenoides--our experience with 29 patients. J Eur Acad Dermatol Venereol. May 2006;20(5):542-7. [Medline].

  25. Powell FC, Muller SA. Psoralens and ultraviolet A therapy of pityriasis lichenoides. J Am Acad Dermatol. Jan 1984;10(1):59-64. [Medline].

  26. 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. Feb 2010;49(2):215-7. [Medline].

 
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Typical hemorrhagic crusted papules of pityriasis lichenoides et varioliformis acuta.
Close-up view of typical lesions of pityriasis lichenoides et varioliformis acuta.
Scaling papules of pityriasis lichenoides chronica.
Close-up view of typical pityriasis lichenoides chronica lesions. Note papules in different stages of evolution and the scale with frosted-glass appearance in the lower right-hand corner.
 
 
 
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