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CLINICAL REPORT
Methotrexate Treatment for Pityriasis Rubra Pilaris: A Case Series
and Literature Review
Lukas KOCH 1 , Clemens SCHÖFFL 1 , Werner ABERER 1 and Cesare MASSONE 2
Department of Dermatology, Medical University of Graz, Austria, and 2 Dermatology Unit, Galliera Hospital, Genova, Italy
1
Treatment recommendations for pityriasis rubra pila-
ris (PRP) are based solely on case reports and small
case series, as to-date no randomized controlled trials
are available. We present here a case series of 3 pa-
tients and a literature review of 28 studies treating a
total of 116 patients, with the aim of providing data
regarding efficacy and safety of methotrexate in the
treatment of PRP. Methotrexate was effective in our
patients; the review showed an overall response rate
of 65.5% with complete clearing in 23.3% and excel-
lent improvement in 17.2%, respectively. After exclu-
ding studies with other concurrent systemic therapies
or low reliability, the overall response rate increased
to 90.9%, with complete clearing in 40.9% and excel-
lent improvement in 31.8%, respectively. Sixteen ad-
verse reactions, of which 11 were mild, were observed
in 15 patients (12.9%). In conclusion, the available
literature supports good response rates and safety of
methotrexate in PRP.
Key words: pityriasis rubra pilaris; methotrexate; treatment;
response; review.
Accepted Jan 15, 2018; Epub ahead of print Jan 16, 2018
Acta Derm Venereol 2018; 98: 501–505.
Corr: Cesare Massone, Dermatology Unit–Ospedali Galliera, Via Volta 6,
IT-16128, Genova, Italy. E-mail: [email protected]
T
reatment recommendations for pityriasis rubra pila-
ris (PRP) are based solely on case reports or small
case series, as to-date no randomized controlled trials
assessing different treatment efficacies are available. We
report here our experience with methotrexate (MTX) in
the treatment of PRP and provide a systematic review
of the literature regarding the efficacy and tolerability of
treatment with MTX.
CASE REPORTS
Case 1
A 55-year-old man consulted our outpatient clinic in
August 2014 with a 2-month history of skin changes,
which had started on the scalp and spread consecutively
to his shoulders and trunk. Prior treatment with systemic
corticosteroids had not improved the disease. Apart from
an umbilical hernia the patient was otherwise healthy and
did not take any medication regularly.
Skin examination revealed scaling erythematous to
orange confluent plaques with islands of unaffected skin
on his trunk, buttocks, neck, face and scalp (Fig. 1A, B).
Furthermore, the patient had palmoplantar keratoderma
with fissures; the affected body surface area (BSA) was
approximately 50%. Histopathological examination of
a skin biopsy was compatible with a clinical diagnosis
of PRP and after unremarkable pre-treatment screening
(Table SI 1 ), MTX treatment was initiated, 20 mg subcu-
taneously once weekly, followed by 5 mg oral folic acid
substitution every following day.
At 1-month follow-up there was considerable impro-
vement in the erythema on the trunk, but scaling and
erythema on the arms and palmar keratoderma persisted.
The dosage of MTX was increased to 25 mg once weekly.
At 4-month follow-up the patient showed further
improvement on the trunk, but only little improvement
of palmoplantar keratoderma, which later completely
disappeared after 9 months of MTX treatment, when only
a few erythematous non-scaling patches persisted on the
trunk. MTX was then consecutively reduced each month,
down to 7.5 mg/week in February 2016, when the skin
had almost completely cleared. MTX was ceased in June
2016: the total MTX dosage applied was 817.5 mg, with
no adverse drug reactions during the whole treatment
period. A 26-month follow-up in October 2016 revealed
an almost complete remission of PRP with minimal non-
scaling tender plaques on the ventral trunk affecting less
than 1% of the BSA (Fig. 1C, D).
Case 2
A 50-year-old man with no comorbidities or long-term
medication had developed facial erythema with scaling
in June 2015, which then had spread to the trunk and
arms after 2 weeks. He was hospitalized in our inpatient
clinic in July, with a diffuse-scaling erythema covering
approximately 50% of the BSA with multiple islands
of sparing. The palms showed orange-red discoloration
with mild keratoderma. A skin biopsy was compatible
with the diagnosis of PRP and after unremarkable pre-
treatment screening MTX was initiated, 10 mg/week
subcutaneously, followed by 5 mg oral folic acid every
following day. The MTX dose was increased by 2.5 mg/
https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-2885
1
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2018 Acta Dermato-Venereologica.
doi: 10.2340/00015555-2885
Acta Derm Venereol 2018; 98: 501–505