Published on 18/03/2015 by admin
Filed under Dermatology
Last modified 22/04/2025
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Alex Milligan and Graham A. Johnston
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 is 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.
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.
There are no controlled 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 only 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 UV therapy, and therefore either UV alone or with 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, cyclosporine, and acitretin 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.
Consider skin biopsy
Although a skin biopsy is usually unnecessary in clinically obvious cases, it may be useful before commencing aggressive systemic therapy.
An infective etiology is often suggested, but no pathogen has yet been implicated, though an association with toxoplasmosis has 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.
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 to eight (36%) of the patients with PLC. Five of the latter had subsidence of skin lesions after pyrimethamine and sulfapyrimidine treatment.
Viral infection has been found in association.
Kim JE, Yun WJ, Mun SK, Yoon GS, Huh J,Choi JH, Chang S. J Cutan Pathol 2011; 38: 649–56.
Fifty-one patients with pityriasis lichenoides (not subdivided into PLEVA and PLC) were analyzed. Human herpes virus-8 (HHV-8) was found in 11 (21%) of affected patients but in none of the 25 controls.
Gritiyarangsan P, Pruenglampoo S, Ruangratanarote P. J Dermatol 1987; 14: 258–61.
In this open study 30 patients with pityriasis lichenoides were recruited, although the authors did not specify how many had PLEVA and how many PLC. The first group of eight were given topical corticosteroid and half had a partial or complete response. The second group were also given oral tetracycline and the majority had a partial response. The third group of eight chronic refractory cases were given oral methoxsalen 0.6 mg/kg with UVA three times per week for an average of 2 months; five were cleared and two had a partial response.
Pavlotsky F, Baum S, Barzilai A, Shapiro D, Trau H. J Eur Acad Dermatol Venereol 2006; 20: 542–7.
This retrospective study of 29 patients again failed to separate PLC and PLEVA but reported a complete response in 93% of patients treated with UVB; 73% remained disease-free after 3 years.
Aydogen K, Saricaoglu H, Turan H. Photodermatol Photoimmunol Photomed 2008; 24: 128–33.
TL-01 phototherapy led to clearance in seven out of eight PLC patients (87.5%) with a mean cumulative dose of 18.4 J/cm2 after a mean of 45.8 exposures. Relapses occurred in four patients within a mean period of 6 months.
Tham SN. Australas J Dermatol 1985; 26: 9–13.
Seventeen patients with PLC were treated with UVB three to five times per week with a starting dose of 80–90% of the minimal erythema dose. They received an average of 33 treatments: nine completely cleared and five had 90% clearance. Only half had relapsed at 3-year follow-up.
LeVine MJ. Arch Dermatol 1983; 119: 378–80.
PLC in 12 patients cleared completely after an average of 30 treatments of minimally erythemogenic doses of UVA/UVB from fluorescent sunlamps. The average UV dose required was 388 mJ/cm2.
Han HK, Kim JK, Kook HL. Korean J Dermatol 1982; 20: 413–17.
Nine patients with PLC of relatively short duration were treated with oral methoxsalen and UVA and were cleared completely after between eight and 45 treatments.
Farnaghi F, Seirafi H, Ehsani AH, Agdari ME, Noormohammadpour P. J Eur Acad Dermatol Venereol 2011; 25: 913–16.
This was a study of 15 patients (not stated how many were PLC or PLEVA) who were randomized to receive UVB (complete response seven of eight, partial one of eight) or PUVA (complete five of seven, partial two of seven). The authors state that the difference in response is insignificant and that both options are acceptable.
Wahie S, Hiscutt E, Natarajan S, Taylor A. Br J Dermatol 2007; 157: 941–5.
In this retrospective study only two of eight children cleared with erythromycin, whereas three out of four adults cleared without relapse. Phototherapy was more effective in both groups.
Ersoy-Evans S, Greco MF, Mancini AJ, Subasi N, Paller AS. J Am Acad Dermatol 2007; 56: 205–10.
This was a retrospective study of 124 children, of whom 46 had PLC. The median age of onset was 60 months and median duration was 20 months (range 3–132 months). Two-thirds of children had at least a partial response to erythromycin.
Hapa A, Ersoy-Evans S, Karaduman A. Pediatr Dermatol 2012; [Epub ahead of print].
The records of 24 children (age range 2–14), 15 with PLC, six with PLEVA and three overlap, started on erythromycin (30–50 mg/kg/day for 1 to 4 months) were reviewed. Sixty-four percent showed a good response at 1 month rising to 83% at 3 months. Of 16 patients followed up, three relapsed (time not given).
Piamphongsant T. Br J Dermatol 1974; 91: 319.
Twelve patients in Bangkok were given tetracycline 2 mg daily. All responded within 4 weeks. Seven required maintenance therapy of 1 mg daily for 6 months.
Lynch PJ, Saied NK. Cutis 1979; 23: 635–6.
Three patients received methotrexate 25 mg/week intramuscularly or orally. All responded within weeks, but two relapsed on cessation of therapy.
Hay IC, Omerod AD. J Dermatol Treat 1988; 9: 53–4.
A further report of a patient with PLC who, having failed to respond to prednisolone, oxytetracycline, dapsone, methotrexate, azathioprine, UVB, and PUVA, responded to 50 mg acitretin daily.
Panse I, Bourrat E, Rybojad M, Morel P. Ann Dermatol Venereol 2004; 131: 201–3.
One patient with pityriasis lichenoides and two patients with PLEVA unresponsive to other therapies, including topical corticosteroids, antibiotics, and UVB, responded within weeks to acitretin plus PUVA.
Gupta AK, Brown MD, Ellis CN, Rocher LL, Fisher GJ, Baadsgaard O, et al. J Am Acad Dermatol 1989; 21: 1245–56.
In a review of cyclosporine in a wide variety of dermatoses the authors report successful clearance within 8 weeks in a man with a 24-year history of PLC. The dose was 6 mg/kg daily, and an improvement in scaling and erythema was noticed after the first week.
Pinton PC, Capezzera R, Zane C, De Panfilis G. J Am Acad Dermatol 2002; 47: 410–14.
Eight patients (five with PLC and three with PLEVA) were treated. Three patients with PLC showed complete clinical and histological recovery. Two showed partial improvement.
Massimiliano R, Pietro R, Paolo S, Sara P, Michele F. J Dermatol Treat 2007; 18: 219–22.
Eight patients with PLC were treated for 3 months with oral bromelain, a crude aqueous extract of the stems and immature fruit of pineapple. The authors claim that all cleared completely and only two relapsed over 12 months.
Mallipeddi R, Evans AV. Clin Exp Dermatol 2003; 28: 456–8.
A 41-year-old woman with an 8-year history of PLC unresponsive to erythromycin, UVB, and PUVA showed almost complete clearance after 4 weeks of treatment with tacrolimus ointment. Subsequent relapses responded to further treatment.
Simon D, Boudny C, Nievergelt H, Simon HU, Braathen LR. Br J Dermatol 2004; 150: 1033–5.
Two children with long-lasting refractory PLC were cleared of skin lesions after 14 and 18 weeks of treatment, respectively.
Fernandez-Guarino M, Harto A, Reguero-Callerjas ME, Urrutia S, Jaen P. Br J Dermatol 2008; 158: 198–200.
A woman with 15 lesions of PLC had each occluded with methyl aminolevulinic acid for 3 hours followed by a 595 nm pulsed dye laser as a light source (one pulse for each lesion). Lesions cleared after only one treatment.
Nikkels AF, Gillard P, Pierard GE. J Drugs Dermatol 2008; 7: 990–2.
A 65-year-old woman had a 5-year history of pityriasis lichenoides unhelped by UVB, PUVA, methotrexate, dapsone, and cyclosporine. She was commenced on etanercept with marked improvement in pruritus and inflammation after 2 months and no new lesions after 4 months when treatment was stopped. However, she relapsed after 1 month.
There are reports of infliximab and adalimumab causing pityriasis lichenoides.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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