Acute Generalised Pustular Psoriasis of Von Zumbusch: A

Conflict in the Flare of Psoriatic Arthritis

Sabrina Ab Wahab

, Tarita Taib

, N. A. Ahmad

, M. Kuppusamy

, W. S. A. Wan Ahmad Kamal

L. D. Aminuddin

, R. Ridzwan

Dermatology Unit, Faculty of Medicine, Sungai Buloh Campus, Universiti Teknologi MARA, Sungai Buloh, Selangor,

Malaysia

Department of Dermatology, Selayang Hospital, Selayang, Malaysia

Keywords: Generalized pustular psoriasis, psoriatic arthritis, methotrexate, prednisolone, withdrawal.

Abstract: Psoriasis is a chronic autoimmune disease with characteristic inflammation of the skin and joints.

Immunosuppressant drugs, especially methotrexate has emerged since the past decade in psoriasis

treatment, mainly for psoriatic arthritis. Withdrawal of immunosuppressant drugs may trigger a more severe

condition; acute generalized pustular psoriasis, which is a rare form of psoriasis. We report a 46 years old

Indian woman, who had chronic plaque psoriasis with psoriatic arthropathy for the past 3 years and was

stable on oral methotrexate 10mg weekly and oral prednisolone 5mg daily. She presented with generalized

pustular eruptions for 1 week associated with fever for 3 weeks and bilateral knee pain. There was

widespread erythema and scaling, painful and studded with pustules appearing on all limbs, trunk and face

with body surface area of more than 90% involved. She had swelling of both knees which was warm and

tender. Further history revealed that she had stopped taking methotrexate for 2 weeks prior. Her condition

was complicated with Klebsiella pneumoniae bacteremia and flare of psoriatic arthropathy. She was started

on oral prednisolone 30mg daily, followed by tapering doses. She was monitored in the ward in view of

high risk of worsening pustular psoriasis while tapering the dose of systemic corticosteroids. Her condition

improved with a short course of oral acitretin 25mg daily. We discussed this case to highlight the dilemma

faced by both dermatologist and rheumatologist when immunosuppressants are withdrawn in psoriatic

patients and the difficulties of managing flares of psoriatic arthropathy in the same settings.

1 INTRODUCTION

Generalized pustular psoriasis (GPP) is a rare and

severe variant of psoriasis characterized by

generalized inflammatory plaques with multiple

sterile pustules (Griffiths & Baker, 2007). The

commonest precipitating factor include abrupt

withdrawal of systemic or ultrapotent topical

corticosteroids and infection (Kamarashev et al.,

2002). Withdrawal of systemic immunosuppressant

therapy is also associated with GPP and risks of

rebound of the disease is of concern. The

complication of GPP is multi-system organ failure

and early recognition and treatment is necessary

(Pomahac et al., 2008).

2 CASE

We report a case of a 46 years old Indian women

who presented with generalized pustular eruptions

for 1 week associated with fever for 3 weeks with

body aches and bilateral knee pain for 1 week. The

rash was described as worsening widespread

erythema and scaling, painful and non-itchy, studded

with pustules appearing on all limbs, trunk and face.

She had swelling of both lower limbs and exfoliated

skin areas over all limbs. She was diagnosed with

plaque psoriasis 4 years prior to this, which

progressed to psoriatic arthritis Her latest dosage

oral methorexate 10mg weekly, however she

stopped the medication herself for 2 months without

advice from doctors. She continued taking oral

prednisolone 5mg daily and was adherent with no

change in dosage. On examination, 90% of body

surface areas were involved. The rash was

Wahab, S., Taib, T., Ahmad, N., Kuppusamy, M., Kamal, W., Aminuddin, L. and Ridzwan, R.

Acute Generalised Pustular Psoriasis of Von Zumbusch: A Conﬂict in the Flare of Psoriatic Arthritis.

DOI: 10.5220/0008160104770480

In Proceedings of the 23rd Regional Conference of Dermatology (RCD 2018), pages 477-480

ISBN: 978-989-758-494-7

477

characterized as multiple pustules about 1–2 mm in

size and located on the anterior chest (figure 1), both

arms, abdomen and both lower limbs (figure 2).

There were few pustular lesions over the chin and

perioral. The pustules were studded on erythematous

plaques with scales and desquamation. Multiple

erythematous plaque with scales were seen. Her face

and scalp had faint erythematous plaque with thick

scales. There was onycholysis of both finger nails

and toes. Bilateral knee was swollen and tender upon

palpation, warm to touch and erythematous changes

over the overlying skin area.

Blood investigations showed white cell count of

21.32 x 10

/L, with neutrophil predominance 82% ,

haemoglobin of 10.9 /dl and platelet 442 x 10

/L .

Inflammatory markers were raised, erythrocyte

sedimentary rate was 107mm/hr and C-reactive

protein was 28.7 mg/L . She had liver impairment ,

with raised alanine aminotransferase (ALT) 96 IU/L

and alkaline phosphate (ALP) was 211 IU/L . Her

renal profile was normal and corrected calcium was

2.28 mmol/l. Anti streptolysin O titer was negative.

Pus cultures from the pustules showed no growth.

She was diagnoses with acute generalized pustular

psoriasis. The identified triggering factor was the

withdrawal of the immunosuppressant drug,

methotrexate. She required skin nursing with

potassium permanganate dressing and emollients.

Topical corticosteroids applied included

hydrocortisone 1% ointment and bethamethasone

valearate 1:4 cream twice daily. Blood cultures taken

grew Klebsiella Pneumoniae sensitive to ampicillin

and she was on intravenous piperacillin tazobactam

4.5 g TDS for 1 week.

She was maintained on oral prednisolone 30mg

od and dosage was tapered slowly. She was

monitored in the ward in view of high risk of

worsening pustular psoriasis during the period of

withdrawing the systemic corticosteroids. Oral

acitretin 25mg od was started, however it was given

only for a short duration for as patient could not

tolerate the medication. Side effects of actitretin was

observed as she had dry lips, mouth and eyes and

skin peeling. The pustular lesions cleared with the

short course acitretin

3 DISCUSSION

Psoriasis is a chronic autoimmune disease with

characteristic inflammation of the skin and joints. At

present, guidelines have recommended that

methotrexate is preferred in psoriatic arthritis with

skin involvement (Gossec et al., 2015).

Dermatologists usually avoids systemic

corticosteroids when treating psoriasis because of

the potential risk of pustular psoriasis when systemic

corticosteroids are discontinued. In contrast,

rheumatologists often use systemic corticosteroids,

in smaller dosages of 5-10 mg/d for the treatment of

psoriatic arthritis and combined with methotrexate.

Generalized pustular psoriasis (GPP) was

described in in 1968 by Baker and Ryan and

categorized into four clinical variants: acute GPP of

von Zumbusch; subacute annular pustular psoriasis

(APP); exanthematic; and localized GPP (Baker &

Ryan, 1968). Systemic features are commonly

involved, and patients appear ill, febrile, malaise

with leukocytosis. Derangement in liver enzymes

and elevation of acute phase reactants are associated

with this disease. Complications reported are sepsis

and renal, hepatic, respiratory, and cardiac failure.

Case reports have described in severe psoriasis

with pronounced arthritis presenting with pustular

exacerbation after withdrawal of an

immunosuppressant drug (Kamarashev et al., 2002;

Benner et al., 2009). In this present case, withdrawal

of systemic immunosuppressants therapy triggered

the pustular psoriasis. After restarted on systemic

Figure 1: Multiple pustules seen on

erythematous plaques on anterior chest.

Figure 2: Multiple pustules on the scaly

erythematous plaque seen on right leg.

RCD 2018 - The 23rd Regional Conference of Dermatology 2018

478

corticosteroids, the patient was closely monitored for

any rebound of her condition. A case review by

Choon et al. described the commonest triggering

factor was withdrawal of systemic therapy and was

associated with higher risks of recurrent flares of

GPP apart from other factors such as pregnancy and

upper respiratory tract infection (Choon et al., 2014).

Despite early identification of the factors

mentioned, GPP may still lead to unstable disease

and frequently occurring pustular flares. Majority of

corticosteroid induced GPP had mild psoriasis

requiring systemic steroid indicated for both

psoriatic lesions or arthritis (Choon et al., 2014).

Corticosteroid has been well known as a trigger or

aggravating factor for GPP ,and well reported in

various case series , with strong association

withdrawal of steroids (Brennet et al., 2009; Borges-

Costa et al., 2011) . Patients are more commonly on

these drugs when there is association with psoriatic

arthritis.

Management GPP variant of psoriasis is still

based on evidence from case reports and no

universal guidelines are available at present. In this

case, patient was given acitretin, however she

developed side effects such as dry lips, mouth and

eyes and skin peeling which are very common and

not tolerable to some patients and was therefore

withheld. Concerns on risk recurrence of pustular

eruption and the need to maintain steroids for her

arthritis arise in our case. The initial preference of

methotrexate usage is due to its known therapeutic

efficacy in improving both chronic plaque psoriasis

and arthropathy.

Biologics alone or in combination with acitretin

should be considered as have been describes in

recent case reports in managing GPP. Evidence of

rapid resolution of GPP with biologics especially

with infliximab and also Etanercept was observed

(Chandran & Chong, 2005). Combination therapy

has also reported efficacy, acitretin combined with a

biologic agent, adalimumab resulted in clearance of

pustular lesions over 10 months (Gallo et al., 2013).

However further studies are needed to address the

efficacy of the biologics especially which agent

would be more beneficial in both pustular psoriasis

and psoriatic arthropathies.

4 CONCLUSION

Withdrawal of immunosuppressant drugs may

trigger generalized pustular psoriasis and infection

may worsened the pustular psoriasis flare. Acitretin

has been shown to improve the pustular psoriasis in

our case. The associated psoriatic arthropathy in

patients with generalized pustular psoriasis need to

be managed with caution when tapering the

corticosteroid due to the risk of rebound of pustular

psoriasis.

ACKNOWLEDGEMENT

We would like to thank our Head of Department, Dr

Mohd Arif bin Mohd Zim for his support in

reporting this case.

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