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Chapter 10: Pityriasis lichenoides et varioliformis acuta (PLEVA)

Manasmon Chairatchaneeboon; Ellen J. Kim

PITYRIASIS LICHENOIDES ET VARIOLIFORMIS ACUTA

Therapeutic Approach

Pityriasis lichenoides et varioliformis acuta (PLEVA) is an uncommon, idiopathic, inflammatory skin disease characterized by recurrent crops of erythematous, scaly papules, vesicles or hemorrhagic crusted ulcers, most commonly on the trunk and extremities. Lesions typically regress within a few weeks, resulting in a postinflammatory hypopigmentation, hyperpigmentation, and varioliform scar. Patients with PLEVA are often asymptomatic but can be pruritic. Systemic symptoms may be seen in the rare, more severe variant, febrile ulceronecrotic Mucha–Habermann disease (FUMHD).

There are no standard treatment guidelines for PLEVA. Treatment consideration is generally based on the severity of the disease and the treatment adverse effect profiles. In the authors experience, initial treatment includes mid- to high-potency topical steroids combined with either oral tetracycline class antibiotics or narrowband UVB phototherapy, with refractory cases treated with low dose oral weekly methotrexate. Combination treatment regimens are acceptable. Relapse is not uncommon. See Table 10-1.

Table 10-1PLEVA Treatment Table
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Table 10-1 PLEVA Treatment Table
MEDICATION NAME INDICATION** MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Corticosteroids (if BSA > 5%, mid-potency such as triamcinolone cream or ointment 0.1% BID × 2-4 wk, if BSA < 5% high-potency such as clobetasol cream or ointment 0.05% BID × 2-4 wk Localized or generalized PLEVA Anti-inflammatory Apply QD-BID Skin atrophy, telangiectasia, striae, acneiform reaction, infection, hypertrichosis, systemic absorption None III1-4
Calcineurin inhibitors Localized PLEVA Immunomodulating, anti-inflammatory Tacrolimus ointment 0.03%-0.1% apply BID Skin irritation None IV5,6
Ultraviolet B (UVB) phototherapy—broadband or narrowband Generalized PLEVA Immunomodulatory, immunosuppressive, T-cell apoptosis 2-3 times a week Erythema, blistering, pruritus, pigmentation, photoaging, skin cancer None III2,4,7-16
PUVA (psoralen plus UVA) phototherapy Generalized PLEVA Immunomodulatory, immunosuppressive, T-cell apoptosis 2-3 times a week Nausea (30%), erythema, blistering, pruritus, pigmentation, photoaging, skin cancer, cataracts None III1,4,10,11, 17,18
UVA1 phototherapy Generalized PLEVA Immunomodulatory, immunosuppressive, T-cell apoptosis 5 times a week Erythema, pruritus, marked pigmentation, photoaging, skin cancer None IV19
Sunlight/heliotherapy Generalized PLEVA Immunomodulatory, immunosuppressive, T-cell apoptosis QD (no standard exposure time) Erythema, pruritus, marked pigmentation, photoaging, skin cancer None IV4
Systemic Therapy
Erythromycin Generalized PLEVA Anti-inflammatory, antimicrobial Children: 15-50 mg/kg/d Adult: 500 mg BID-QID GI irritation None III2,3,7,18,20-23
Azithromycin Generalized PLEVA Anti-inflammatory, antimicrobial 500 mg on d 1 and 250 mg on d 2-5 bimonthly GI irritation None IV24-26
Tetracycline Generalized PLEVA Anti-inflammatory, antimicrobial 500 mg BID-QID Contraindicated in nursing, pregnancy, children < 8 yo; dental hyperpigmentation, GI irritation None III1,27
Minocycline Generalized PLEVA Anti-inflammatory, antimicrobial 100 mg BID Contraindicated in nursing, pregnancy, children < 8 yo; headache, lupus, skin/nails/dental pigmentation, GI irritation None IV28
Methotrexate Recalcitrant or FUMHD Immunosuppressive 7.5–25 mg weekly Contraindicated in ...

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