Friday, May 11, 2018

Generalized Pustular Eruption in a 27 yo woman


The patient is a 27 yo woman with a 2 week history of an evolving, wide-spread eruption.  The initial lesions were on the popliteal fossae.  These were described as erythematous areas studded with pustules.  Over a week or two these generalized.  She has a history of mild psoriasis (scalp and elbows) for over a decade.  About three months ago she was started on bupropion 75 mg a day for anxiety and restlessness.  This was increased to 150 mg per day ~ 2 week before the onset of the dermatitis.  The patient has moderate cognitive impairment and Type 2 diabetes.  Here other medications include thyroid supplementation, metformin and insulin.  In the days since her initial office visit, the eruption has become more extensive and is taking on an erythrodermic appearance.  She was admitted to hospital two days after her office appointment.               

O/E:  When seen on May 7, 2018, she had a widespread eruption on arms, legs and torso,  The lesions were large arcuate patches with pustules at the periphery.  She was experiencing considerable pain.

Clinical Images:


 Pathology: (courtesy of DR. Erin Tababa, Fellow in Dermatology, Boston University)  

The biopsy shows prominent, relatively large, subcorneal pustules that are filled with a dense exudate of acute inflammatory. The neutrophils extended into the underlying epidermis, which has evidence of mild spongiosis. The papillary dermis is slightly oedematous, and there is a moderately dense perivascular inflammatory infiltrate with a predominance of neutrophils, although no eosinophils are noted.


Lab:
WBC: 29,000
Differential:  Shift to left
Eosinophils: normal
G6PD Normal

Chemistries normal.

Wound Culture: Pending

Dx:  We are initially considering subcorneal pustulr dermatosis, but with more history, especially considering the recent prescription of bupropion a drug-induced annular pustular psoriasis evolving into pustular and exanthematous psoriasis seemed more accurate.  Histopathology supported that.  Similar reactions have been reported to bupropion.  Our patient had been on the bupropion for over a month when this began which is longer than the patients in the case report below.  Reference 2 is a similar patient with a long latent period between initiation of drug and development of GPP.

Follow-up in hospital.  The eruption continued to evolve. It became more exanthematous and desquamative. These pictures were sent us by her mother.


Plan: 
Cyclosporin 3 – 4 mg per kg per day in divided doses
Wet dressings followed by triamcinolone 0.1% ointment bid – tid
Adjunctive secukinumab has been reported to be effective.

Follow-up:
Patient is doing very well.  These photos were taken 5 days after starting cyclosporine 100 gm q.i.d.  Her dose was dropped to 100 mg t.i.d.  She also was treated with wet dressings and triamcinalone ointment 0.1% (although the hospital only gave her 15 mg tubes, so she could not cover most ot the lesions. 
References:
1. Generalized pustular and erythrodermic psoriasis associated with bupropion treatment. Cox NH, Gordon PM, Dodd H. Br J Dermatol. 2002 Jun;146(6):1061-3.
Abstract: Severe drug eruptions may cause diagnostic and therapeutic difficulty when they mimic or provoke endogenous patterns of dermatosis. We report three patients with known psoriasis in whom use of bupropion (Zyban), prescribed to assist with cessation of smoking, led to severe pustular or erythrodermic exacerbation of psoriasis within 3-5 weeks. All patients were systemically unwell and required hospitalization to control the disease flare.

2. A diagnostic challenge: acute generalized exanthematous
pustulosis or pustular psoriasis due to terbinafine
L. Duckworth et.al. Clin Exp Dermatol. 2012 Jan;37(1):24-7
Abstract:  A 72-year-old man developed a generalized erythematous pustular eruption 11 weeks after commencing terbinafine. Clinically and histologically, the appearance was that of acute generalized exanthematous pustulosis (AGEP), and the disease was managed with topical preparations. Initial improvement was marred by relapse of acute pustulosis, now more in keeping with terbinafine-induced pustular psoriasis (PP),which was successfully treated with acitretin. This case highlights the difficulty of differentiating between AGEP and PP.

3. Acute generalized exanthematous pustulosis mimicking toxic epidermal necrolysis in patients with psoriasis: a coincidence?
Worsnop F, et. a. Clin Exp Dermatol. 2015 Aug;40(6):688-9

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5 comments:

  1. Comments of Dr. Richard Sontheimer: “Interesting case. I have nothing to add to the assessment and treatment plan. A better mechanistic understanding of why certain systemic drug classes trigger clinical expression of certain autoimmune inflammatory dermatoses in certain individuals at certain points in time might lead to a better understanding and improved treatment of the “non-drug-induced” forms of these dermatoses.”

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  2. Professor Sharquie from Baghdad writes: This is a typical picture of acute pustular psoriasis induced by drug In genetically susceptible individual.I believe that there is no so called AGEP and this entity does not exist.This is according to a study carried out in my department where we could not distinguish between agep and acute pustular psoriasis

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  3. Comments of Dr. Cheng Leng, Malaysia: She has history of psoriasis, so we must think of psoriasis first. Her type of palmar lesions are taught by examiners as a matter of fact, one of the three diagnoses:-
    1. Psoriasis
    2. Reiter's syndrome
    3. Secondary syphilis, which can easily be ruled out by blood test.

    Clinically she has typical annular psoriasis plaques with flexural psoriasis. Pustules are one of the primary lesions of psoriasis. She may be at one end of spectrum, bearing more pustules.

    She is not toxic looking, and there was no lakes of pus to suggest von zambush type of generalised pustular psoriasis. At most, she may progress to non- von zambush generalised pustular psoriasis.

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  4. The upper back is is conspicuous by it's lack of involvement. Hmmm. Need to pay more attention to whether this is only incidental or a significant point. I know this is a bizarre thought - but wondering if pressure prevent the eruption. If so that would be helpful in limiting the area of involvement by preventive compressive dressing in a bad Von Zumbusch.

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  5. Prof. Bhushan KumarMay 15, 2018

    Classical case of pustular psoriasis. The best response is seen with methotrexate because the drug acts directly to suppress the leucocyte activity.

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