Pityriasis lichenoides chronica
- Amy Musiek, MD
Amy Musiek, MD
- Assistant Professor of Medicine
- Washington University, St. Louis
- Section Editors
- John A Zic, MD
John A Zic, MD
- Section Editor — Cutaneous Lymphoma
- Associate Professor of Medicine/Dermatology
- Vanderbilt University School of Medicine
- Moise L Levy, MD
Moise L Levy, MD
- Section Editor — Pediatric Dermatology
- Clinical Professor of Dermatology and Pediatrics
- Baylor College of Medicine
- Clinical Professor of Dermatology, UTSW Medical School
- Dell Children's Medical Center
Pityriasis lichenoides is a term used to refer to a group of rare acquired inflammatory skin disorders that includes pityriasis lichenoides chronica (PLC), pityriasis lichenoides et varioliformis acuta (PLEVA), and the febrile ulceronecrotic Mucha-Habermann disease (FUMHD) variant of PLEVA. The use of the term pityriasis lichenoides to refer to all three disorders is representative of the theory that PLC, PLEVA, and FUMHD may represent a clinical spectrum of a single disease.
PLC is clinically characterized by the development of multiple, scaly, erythematous to brown papules on the trunk and extremities. The condition usually has a relapsing and remitting course that persists for months or years.
The clinical manifestations, diagnosis, and management of PLC will be reviewed here. PLEVA is discussed separately. (See "Pityriasis lichenoides et varioliformis acuta (PLEVA)".)
Definitive conclusions on the epidemiology of pityriasis lichenoides chronica (PLC) are hindered by the frequent lack of distinction between the subtypes of pityriasis lichenoides in the published literature and limited data. In general, PLC is considered a rare disorder that is most likely to occur in young adults and children . However, PLC may develop at any age.
The existence of sex, ethnic, or racial predilections in PLC is uncertain . Based upon data from a few retrospective studies of children with pityriasis lichenoides, there may be a slight male predilection for pityriasis lichenoides in the pediatric population [3-7]. In one of the largest series of children with PLC (n = 46), 59 percent of the children were male .
- Fernandes NF, Rozdeba PJ, Schwartz RA, et al. Pityriasis lichenoides et varioliformis acuta: a disease spectrum. Int J Dermatol 2010; 49:257.
- Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol 2006; 55:557.
- Wahie S, Hiscutt E, Natarajan S, Taylor A. Pityriasis lichenoides: the differences between children and adults. Br J Dermatol 2007; 157:941.
- 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.
- Romaní J, Puig L, Fernández-Figueras MT, de Moragas JM. Pityriasis lichenoides in children: clinicopathologic review of 22 patients. Pediatr Dermatol 1998; 15:1.
- Gelmetti C, Rigoni C, Alessi E, et al. Pityriasis lichenoides in children: a long-term follow-up of eighty-nine cases. J Am Acad Dermatol 1990; 23:473.
- Truhan AP, Hebert AA, Esterly NB. Pityriasis lichenoides in children: therapeutic response to erythromycin. J Am Acad Dermatol 1986; 15:66.
- Almagro M, Del Pozo J, Martínez W, et al. Pityriasis lichenoides-like exanthem and primary infection by Epstein-Barr virus. Int J Dermatol 2000; 39:156.
- Rongioletti F, Delmonte S, Rebora A. Pityriasis lichenoides and acquired toxoplasmosis. Int J Dermatol 1999; 38:372.
- 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.
- Kim JE, Yun WJ, Mun SK, et al. Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica: comparison of lesional T-cell subsets and investigation of viral associations. J Cutan Pathol 2011; 38:649.
- Dupont C. Pityriasis lichenoides in a family. Br J Dermatol 1995; 133:338.
- Magro CM, Crowson AN, Morrison C, Li J. Pityriasis lichenoides chronica: stratification by molecular and phenotypic profile. Hum Pathol 2007; 38:479.
- Shieh S, Mikkola DL, Wood GS. Differentiation and clonality of lesional lymphocytes in pityriasis lichenoides chronica. Arch Dermatol 2001; 137:305.
- Weinberg JM, Kristal L, Chooback L, et al. The clonal nature of pityriasis lichenoides. Arch Dermatol 2002; 138:1063.
- López-Ferrer A, Puig L, Moreno G, et al. Pityriasis lichenoides chronica induced by infliximab, with response to methotrexate. Eur J Dermatol 2010; 20:511.
- Massay RJ, Maynard AA. Pityriasis lichenoides chronica associated with use of HMG-CoA reductase inhibitors. West Indian Med J 2012; 61:743.
- Newell EL, Jain S, Stephens C, Martland G. Infliximab-induced pityriasis lichenoides chronica in a patient with psoriasis. J Eur Acad Dermatol Venereol 2009; 23:230.
- Lane TN, Parker SS. Pityriasis lichenoides chronica in black patients. Cutis 2010; 85:125.
- Clayton R, Warin A. Pityriasis lichenoides chronica presenting as hypopigmentation. Br J Dermatol 1979; 100:297.
- Mobini N, Toussaint S, Kamino H. Noninfectious erythematous, papular, and squamous diseases. In: Lever's Histopathology of the Skin, 10th ed, Elder DE (Ed), Lippincott Williams & Wilkins, Philadelphia 2009. p.169.
- Heng YK, Koh MJ, Giam YC, et al. Pediatric mycosis fungoides in Singapore: a series of 46 children. Pediatr Dermatol 2014; 31:477.
- Boulos S, Vaid R, Aladily TN, et al. Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: a case series of 34 patients. J Am Acad Dermatol 2014; 71:1117.
- Piamphongsant T. Tetracycline for the treatment of pityriasis lichenoides. Br J Dermatol 1974; 91:319.
- Hapa A, Ersoy-Evans S, Karaduman A. Childhood pityriasis lichenoides and oral erythromycin. Pediatr Dermatol 2012; 29:719.
- Skinner RB, Levy AL. Rapid resolution of pityriasis lichenoides et varioliformis acuta with azithromycin. J Am Acad Dermatol 2008; 58:524.
- 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.
- Aydogan K, Saricaoglu H, Turan H. Narrowband UVB (311 nm, TL01) phototherapy for pityriasis lichenoides. Photodermatol Photoimmunol Photomed 2008; 24:128.
- Ersoy-Evans S, Hapa AA, Boztepe G, et al. Narrowband ultraviolet-B phototherapy in pityriasis lichenoides chronica. J Dermatolog Treat 2009; 20:109.
- Siew NT. UV-B phototherapy for pityriasis lichenoides. Australas J Dermatol 1985; 26:9.
- 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.
- LeVine MJ. Phototherapy of pityriasis lichenoides. Arch Dermatol 1983; 119:378.
- Pasić A, Ceović R, Lipozencić J, et al. Phototherapy in pediatric patients. Pediatr Dermatol 2003; 20:71.
- Farnaghi F, Seirafi H, Ehsani AH, et al. Comparison of the therapeutic effects of narrow band UVB vs. PUVA in patients with pityriasis lichenoides. J Eur Acad Dermatol Venereol 2011; 25:913.
- 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.
- Stern RS, PUVA Follow-Up Study. The risk of squamous cell and basal cell cancer associated with psoralen and ultraviolet A therapy: a 30-year prospective study. J Am Acad Dermatol 2012; 66:553.
- Mallipeddi R, Evans AV. Refractory pityriasis lichenoides chronica successfully treated with topical tacrolimus. Clin Exp Dermatol 2003; 28:456.
- Simon D, Boudny C, Nievergelt H, et al. Successful treatment of pityriasis lichenoides with topical tacrolimus. Br J Dermatol 2004; 150:1033.
- Said BB, Kanitakis J, Graber I, et al. Pityriasis lichenoides chronica induced by adalimumab therapy for Crohn's disease: report of 2 cases successfully treated with methotrexate. Inflamm Bowel Dis 2010; 16:912.
- Lynch PJ, Saied NK. Methotrexate treatment of pityriasis lichenoides and lymphomatoid papulosis. Cutis 1979; 23:634.
- 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.
- Pansé I, Bourrat E, Rybojad M, Morel P. [Photochemotherapy for pityriasis lichenoides: 3 cases]. Ann Dermatol Venereol 2004; 131:201.
- 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.
- Khachemoune A, Blyumin ML. Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol 2007; 8:29.
- Fernández-Guarino M, Harto A, Reguero-Callejas ME, et al. Pityriasis lichenoides chronica: good response to photodynamic therapy. Br J Dermatol 2008; 158:198.
- Boccara O, Blanche S, de Prost Y, et al. Cutaneous hematologic disorders in children. Pediatr Blood Cancer 2012; 58:226.
- Pileri A, Neri I, Raone B, et al. Mycosis fungoides following pityriasis lichenoides: an exceptional event or a potential evolution. Pediatr Blood Cancer 2012; 58:306.
- 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.
- 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.
- Tomasini D, Zampatti C, Palmedo G, et al. Cytotoxic mycosis fungoides evolving from pityriasis lichenoides chronica in a seventeen-year-old girl. Report of a case. Dermatology 2002; 205:176.
- Lazarov A, Lalkin A, Cordoba M, Lishner M. Paraneoplastic pityriasis lichenoides chronica. J Eur Acad Dermatol Venereol 1999; 12:189.
- CLINICAL FEATURES
- DIFFERENTIAL DIAGNOSIS
- Approach to treatment
- First-line therapy
- - Topical corticosteroids
- - Oral antibiotics
- Second-line therapy
- - Phototherapy
- Additional therapies
- - Topical tacrolimus
- - Methotrexate
- - Other
- ASSOCIATION WITH MALIGNANCY
- SUMMARY AND RECOMMENDATIONS