Pityriasis lichenoides chronica


Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Pityriasis lichenoides chronica (PLC) typically consists of small, erythematous papules, which may be purpuric. These develop a characteristic, shiny, mica-like scale attached to the center. They occur predominantly over the trunk and proximal limbs. As the name implies, PLC may persist for many years, though spontaneous resolution does occur. Patients should be warned that relapse is common and that recurrent courses of therapy may be required.

Anecdotally, PLC was said to run a more benign, self-limiting course in children, but more recently it has been shown that in children it is more likely to run an unremitting course, with greater lesional distribution, more dyspigmentation, and a poorer response to treatment. Some authors argue that there is an overlap with cutaneous lymphoma.

Pityriasis lichenoides chronica: stratification by molecular and phenotypic profile

Crowson AN, Morrison C, Li J. Hum Pathol 2007; 38: 479–90.

A prospective study of 46 patients concluded that PLC is an indolent, cutaneous T-cell dyscrasia with a limited propensity for progression to mycosis fungoides.

Management Strategy

There are few therapeutic trials for this condition, and case series are only small. In many therapeutic trials PLC has been grouped together with pityriasis lichenoides et varioliformis acuta (PLEVA), and management strategies are therefore often similar or interchangeable.

Topical corticosteroids are generally reported as effective anecdotally in textbooks rather than in studies. They are often used with antihistamines to reduce pruritus, but they are not reported to affect the course of the disease.

The majority of reports describe benefits with ultraviolet ( UV) therapy , and therefore either UV alone or psoralen plus UVA (PUVA) therapy is recommended for all patients. The response appears to be unpredictable, however, and the total dose required is extremely variable.

Antibiotics appear to be more helpful in children, sometimes used in combination therapy.

For severe or refractory cases, methotrexate , acitretin combined with PUVA , and etanercept have all been described as effective in small numbers of patients.

For most treatment modalities, patients who have been described as improved have usually had fewer new lesions developing, a shortened disease course, and a greater time to relapse than untreated patients.

Specific Investigation

  • Consider skin biopsy

Although a skin biopsy is usually unnecessary in clinically obvious cases, it may be useful before commencing systemic therapy with more potential adverse effects.

An infective etiology is often suggested, but no pathogen has yet been implicated, though associations with toxoplasmosis, human herpesvirus-8 (HHV-8), and parvovirus B19 have been described. These reports tend to come from endemic areas, and so investigation for a triggering infection is unnecessary in cases without evidence of specific infection.

The relationship between toxoplasmosis and pityriasis lichenoides chronica

Nassef NE, Hamman MA. J Egypt Soc Parasitol 1997; 27: 93–9.

Twenty-two patients with PLC and 20 healthy controls were examined clinically and serologically for toxoplasmosis. Three (15%) of the controls had toxoplasmosis, compared with 8 (36%) of the patients with PLC. Five of the latter had subsidence of skin lesions after pyrimethamine and sulfapyrimidine treatment.

Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica: comparison of lesional T-cell subsets and investigation of viral associations

Kim JE, Yun WJ, Mun SK, et al. J Cutan Pathol 2011; 38: 649–56.

Fifty-one patients with pityriasis lichenoides (not subdivided into PLEVA and PLC) were analyzed. HHV-8 was found in 11 (21%) of affected patients but in none of the 25 controls.

Pityriasis lichenoides: a cytotoxic T-cell-mediated skin disorder. Evidence of human parvovirus B19 DNA in nine cases

Tomasini D, Tomasini CF, Cerri A, et al. J Cutan Pathol 2004; 31: 531–8.

Thirty tissue samples from cases of pityriasis lichenoides (both PLEVA and PLC) underwent molecular investigation for parvovirus B19 DNA, which was present in nine cases (30%).

First-Line Therapies

  • Narrowband ultraviolet B (NB-UVB)

  • B

  • Combined UVA and UVB

  • D

  • PUVA

  • D

Is narrowband ultraviolet B monotherapy effective in the treatment of pityriasis lichenoides?

Park JM, Jwa SW, Song M, et al. Int J Dermatol 2013; 52: 1013–8.

This retrospective study of 70 patients did not separate PLEVA from PLC but reported a 91.9% complete response rate with narrowband ultraviolet B (NB-UVB). This was not significantly different from the response rate to systemic immunosuppressive therapy or a combination of systemic therapy and NB-UVB.

The effectiveness of narrow band UVB (NB-UVB) in the treatment of pityriasis lichenoides chronica (PLC) in Vietnam

Huu DL, Minh TN, Van TN, et al. Open Access Maced J Med Sci 2019; 7: 221–3.

In this prospective study, PLC completely responded to NB-UVB in 24 out of 29 patients (82.8%), after a mean number of 13.8 treatments.

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