Friday, November 14, 2008
Pityriasis Lichenoides et Variolaformis Acuta / Pityriasis
This condition is a benign and often self-limited lymphocytic
vasculitis. Pityriasis lichenoides chronica (PLC) is treated like
pityriasis lichenoides et varioliformis acuta (PLEVA). A rare
variant of PLEVA, the febrile ulceronecrotic form can be very
aggressive and even fatal in adults.
- Oral antibiotics, usually tetracycline or erythromycin 250-500
mg 4 times daily.
- Mild PLC and PLEVA may be treated with superpotent topical
steroids if few lesions are present.
- Topical tacrolimus 0.03-0.1% twice daily may be effective.
Narrowband UVB, UVA1, or PUVA may be used and are indicated for
symptomatic generalized disease. Lesions not in the irradiated
fields may not respond.
Most patients are managed with the above modalities. Exceptional
patients with progressive or incapacitating disease may be
considered for the following therapies:
- Phototherapy may be combined with retinoid treatment (acitretin
25-50 mg/day) for refractory cases.
- Low-dose oral methotrexate 5.0-20 mg/week is usually
efficacious. Recurrences may occur. For severe febrile
ulceronecrotic PLEVA, methotrexate in doses up to 75 mg per week
may be used.
- Pulse intravenous systemic steroids may be used in combination
with methotrexate to control severe febrile ulceronecrotic
- Tetracycline is contraindicated in pregnancy and in children
under 8 years of age.
- Because PLEVA and PLC are benign disorders, methotrexate should
be reserved for severe cases, and informed consent is
- Lymphomatoid papulosis and PLEVA may appear clinically similar.
A biopsy of one or more lesions is necessary to establish the
- PLEVA and PLC may be associated with a monoclonal T-cell
infiltrate. This does not imply the evolution into a malignancy and
the presence of a monoclonal population does not alter the
management of the patient.