Generalized pustular psoriasis following withdrawal of short-term cyclosporin therapy for psoriatic arthritis.
S-B Hong, N-I Kim*
Department of Dermatology, College of Medicine, Kyung Hee University,
1 Hoeki-Dong, Dongdaemun-Ku, Seoul 130-702, Korea. *Corresponding
author, tel. +82 2 958 8511; fax +82 2 969 6538; E-mail: nikim@khmc.or.kr
Generalized pustular psoriasis following
withdrawal of short-term cyclosporin
therapy for psoriatic arthritis
To the Editor
Generalized pustular psoriasis (GPP) is characterized by widespread
pustulation with systemic symptoms such as high fever
and generalized arthralgia and is related to some drugs in its
development.1 Cyclosporin A (CyA) is an established treatment
in the management of psoriatic arthritis (PsA) and has also been
used for GPP.2 We report the case of patient who developed GPP
on the withdrawal of oral CyA treatment for PsA.
Our patient, an 27-year-old male with a 8-year history of
plaque psoriasis and a 12-year history of PsA, presented with a
widespread cutaneous pustular eruption and aggravation of the
joint condition. In the previous 5 years he had been treated in
another arthritic clinic with nonsteroidal anti-inflammatory
drugs (NSAIDs) and methotrexate at a dose of 7.5 ?-15 mg weekly
and the disease activity had been successfully controlled. However,
his PsA and chronic plaque psoriasis had recently gradually
worsened and he was advised to start a short-term course of
CyA. CyA was started at 3 mg/kg/day and continued at the same
dosage for 10 weeks. This led to an alleviation of the joint pains
but failed to improve the condition of his skin. Therefore, in an
attempt to substitute oral methotrexate, CyA had been withdrawn
at 4 weeks prior to presentation in our clinic. However,
within 3 weeks of withdrawing CyA the patient had developed
GPP with an erythrodermic skin and was admitted to our clinic
(fig 1). On admission he looked unwell and was pyrexial with a
temperature of 39°C and complained of malaise, muscle weakness
and generalized myalgia. A laboratory work-up disclosed a
white blood cell count of 22.3 cells/mm3 (85% neutrophils), normochromic anaemia (Hb 8.5 g/dL), reactive thrombocythaemia
(platelets 617 × 103/L), an erythrocyte sedimentation
rate (ESR) of 56 mm/h, and a C-reactive protein (CRP) level of
12.3 mg/dL. His blood chemistry results were within the normal
range. No factors were found that could have triggered exacerbation
of the psoriasis. The patient was started on oral methotrexate
15 mg/week with acitretin 40 mg/day. Two weeks later, all pustules
were cleared and the patient’'s joint pains were markedly
improved and he remained afebrile. The acitretin dose was gradually
reduced to 20 mg/day and finally discontinued 3 months
after treatment. He remains on methotrexate 15 mg/week and
has so far had no relapse of GPP.
PsA is an inflammatory arthritis associated with psoriasis.1 It
is recognized in about 10% of individuals with well-established
psoriasis. In parallel with the skin disease, PsA is exacerbated by
physical stress, nonspecific inflammation or certain infections.
The treatment of PsA includes control of both skin and joint
manifestations. Initially NSAIDs are used, particularly when
both joint and skin manifestations are mild. For the treatment
of severe destructive arthritis, NSAIDs are supplemented with a
more potent anti-inflammatory agent with immunomodulatory
properties, such as methotrexate, CyA or tumour necrosis
factor (TNF) blockers.3
CyA has been shown to be effective in a prospective, nonrandomized
study in the treatment of PsA.4 In particular, shortterm
therapy with CyA (12?-24 weeks) at a daily dose of 3 ?-5 mg/kg
body weight, frequently used nowadays in the treatment of severe
psoriasis, has been shown to be effective and safer than continuous
therapy.5 The present case suggests that small reductions in the
CyA dose may lead to GPP during short-term therapy with
CyA. In our review of the literature we were able to find only
seven cases of GPP upon CyA withdrawal (Table 1).6?-10 As in
previously reported cases, our case had severe psoriasis with
pronounced arthritis and the pustular exacerbation followed
shortly after the withdrawal of CyA. This rebound would seem
to be similar to that which is seen on withdrawal of potent topical
or oral steroid therapy in patients with psoriasis.
As the use of short-term therapy with CyA is becoming very
popular in the treatment of psoriasis, the possibility of a
rebound phenomenon occurring on reduction of CyA during
therapy must be considered.
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