• Constantin E. OrfanosEmail author


There are no accurate data on the prevalence and incidence of the clinically multifaceted group of parapsoriasis. The clinical variables in this group have a chronic course with only few clinical symptoms, if any; they may remain underreported although patients with parapsoriasis have an increased risk of subsequent cancers and increased mortality [1]. The group of parapsoriasis comprises several inhomogeneous, chronic-intermittently developing skin diseases which may clinically mimic psoriasis but are unrelated to each other with respect to their pathogenesis, histopathology, and response to treatment. Their etiology is unknown and the pathogenetic mechanisms are not fully understood. A part of these entities present inflammatory features as a reactive T-cell skin disease with vasculitis, often arising following infections, and another part is considered as precursor or early lymphoproliferative disorder with long-term progression into cutaneous T-cell lymphoma (CTCL). The majority of the entities included are associated with male predominance.


  1. 1.
    Lindahl LM, Fenger-Grøn M, Iversen L. Subsequent cancers, mortality, and causes of death in patients with mycosis fungoides and parapsoriasis: a Danish nationwide, population-based cohort study. J Am Acad Dermatol. 2014;71:529–35.CrossRefPubMedGoogle Scholar
  2. 2.
    Weinberg JM, Kristal L, Chooback L, et al. The clonal nature of pityriasis lichenoides. Arch Dermatol. 2002;138:1063–7.CrossRefPubMedGoogle Scholar
  3. 3.
    Lane TN, Parker SS. Pityriasis lichenoides chronica in black patients. Cutis. 2010;85:125–9.PubMedGoogle Scholar
  4. 4.
    Maranda EL, Smith M, Nguyen AH, et al. Phototherapy for pityriasis lichenoides in the pediatric population: a review of the published literature. Am J Clin Dermatol. 2016;17:583–91.CrossRefPubMedGoogle Scholar
  5. 5.
    Fernández-Guarino M, Aboin-Gonzalez S, Ciudad Blanco C, et al. Treatment of adult diffuse pityriasis lichenoides chronica with narrowband ultraviolet B: experience and literature review. Clin Exp Dermatol. 2017;42:303–5.CrossRefPubMedGoogle Scholar
  6. 6.
    Koh WL, Koh MJ, Tay YK. Pityriasis lichenoides in an Asian population. Int J Dermatol. 2013;52:1495–9.CrossRefPubMedGoogle Scholar
  7. 7.
    Ankad BS, Beergouder SL. Pityriasis lichenoides et varioliformis acuta in skin of color: new observations by dermoscopy. Dermatol Pract Concept. 2017;7:27–34.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    English JC, Collins M, Bryant-Bruce C. Pityriasis lichenoides varioliformis acuta and group-a beta hemolytic streptococcal infection. Int J Dermatol. 1995;34:642–4.CrossRefPubMedGoogle Scholar
  9. 9.
    Edwards BL, Bonagura VR, Valacer DJ, et al. Mucha Habermann’s disease and arthritis: possible association with reactivated Epstein-Barr virus infection. J Rheumatol. 1989;16:387–9.PubMedGoogle Scholar
  10. 10.
    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.CrossRefPubMedGoogle Scholar
  11. 11.
    Fölster-Holst R, Zawar VP, Chuh A. Paraviral exanthems. Expert Rev Anti-Infect Ther. 2016;14:601–11.CrossRefPubMedGoogle Scholar
  12. 12.
    Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. 2007;8:29–36.CrossRefPubMedGoogle Scholar
  13. 13.
    Romaní J, Puig L, Fernández-Figueras MT, et al. Pityriasis lichenoides in children: clinicopathologic review of 22 patients. Pediatr Dermatol. 1998;15:1–6.CrossRefPubMedGoogle Scholar
  14. 14.
    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.PubMedGoogle Scholar
  15. 15.
    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.PubMedGoogle Scholar
  16. 16.
    Suárez J, López B, Villalba R, et al. Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. Dermatology. 1996;192:277–9.CrossRefPubMedGoogle Scholar
  17. 17.
    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.CrossRefPubMedGoogle Scholar
  18. 18.
    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.CrossRefPubMedGoogle Scholar
  19. 19.
    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.PubMedGoogle Scholar
  20. 20.
    Kikuchi A, Naka W, Harada T, et al. Parapsoriasis en plaques: its potential for progression to malignant lymphoma. J Am Acad Dermatol. 1993;29:419–22.CrossRefPubMedGoogle Scholar
  21. 21.
    Väkevä L, Sarna S, Vaalasti A, et al. A retrospective study of the probability of the evolution of parapsoriasis en plaques into mycosis fungoides. Acta Derm Venereol. 2005;85:318–23.CrossRefPubMedGoogle Scholar
  22. 22.
    Bloom B, Marchbein S, Fischer M, et al. Poikilodermatous mycosis fungoides. Dermatol Online J. 2012;18(12):4.Google Scholar
  23. 23.
    Shiomi T, Monobe Y, Kuwabara C, et al. Poikilodermatous mycosis fungoides with a CD8+ CD56+ immunophenotype: a case report and literature review. J Cutan Pathol. 2013;40:317–20.CrossRefPubMedGoogle Scholar
  24. 24.
    Wain EM, Orchard GE, Mayou S, et al. Mycosis fungoides with a CD56+ immunophenotype. J Am Acad Dermatol. 2005;53:158–63.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.The Free University of Berlin, Medical School CharitéBerlinGermany

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