Wednesday, June 24, 2020

A Case for Diagnosis

Presented by  Dr. Henry Foong, Ipoh, Malaysia

The patient, a Chinese woman in her 30s, presented with sudden flare of her skin eruptions on the legs past 2 weeks.  She had been having multiple erythematous plaques on the abdominal wall and legs since March 2020 and had tried various topical creams and oral supplements.  As it happened during lockdown she had tried online medications and topical creams.  The lesions on the abdominal wall improved but suddenly those on the legs flared up with painful pustules.  She denied any fever.  Apparently she was on oral methotrexate previously for psoriasis but according to her, MTX did not help her much.  

Clinical:
Her vital signs are stable. No signs of dehydration. 
Extensive erythematous plaques on the lower limbs.  Edges well demarcated. scaly surface. some of the larger lesions had a central smooth glazed appearance surrounded by crops of tiny superficial pustules.  Post inflammatory hyperpigmentation were prominent on the anterior abdominal wall.
No nail changes were noted.  Joints were normal.
Images:

Diagnosis:
Generalised pustular psoriasis of von Zumbusch

Note:  We presented a similar patient two years ago.

I suspect she may have taken oral corticosteroids and when she stopped the oral medications, the lesions flare up.  A skin biopsy was done.  This may be useful to exclude AGEP.  Blood counts/biochem/swab culture including serum calcium were done and pending.   
In the meantime, she is on IV fluids, IV antibiotics, topical moisturisers, KMNO4 soaks and topical corticosteroids.

Q1.  Would IV hydrocortisone be useful in this patient or avoided at all costs?
Q2.   Is it worthwhile to try MTX weekly dose again?  Cyclosporin? Oral acetretin would not be practical for a woman of child bearing age group. 
Q3.  Biologics may have a role.  Anti IL-36 recently was found to be useful in pustular psoriasis but this drug is not available yet.  Which other biologics would be useful? Risankizumab?

Thank you for your thoughts on this patient.

5 comments:

  1. Krystal Jones, Wyoming: "Biopsy for dif would be helpful too to consider IgA pemphigus and sneddon Wilkinson. The IL-17 inhibitors are best for pustular psoriasis and would provide the most rapid relief. If bad insurance, I would start with cyclosporine and bridge to Cosentyx or Taltz as you get them covered w insurance.

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  2. Khalifa Sharquie, Baghdad, Iraq: This is secondary pustular psoriasis on the top of psoriasis vulgaris but not the other types of pustulosis.As a therapy I suggest oral methotrexate combined with a acitrecin
    plus topical steroids.Other types of therapy like biologic might be given if no response to other treatment.

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  3. Richard Sontheimer, Utah: "Agree with pustular psoriasis flare possibly resulting from discontinuing self-treatment with systemic steroids during the Covid pandemic lockdown. Also agree with the treatment recommendations in Comments section."

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  4. Prof. Bhushan KumarJune 25, 2020

    Pustular psoriasis of Zumbusch. Methotrexate alone is the ideal treatment if there are no risk factors including altered renal or liver functions.

    ReplyDelete
  5. Comments of Dato’ Dr ONG Cheng Leng: Patient is not having Von Zambush generalised postural psoriasis for the following reasons:-
    1. She is too well, no fever too.
    2. There is no physical sign of “ lakes of pus”
    In fact pustules are acceptable primary lesions in psoriasis. The lesions of the legs are just aggravation of psoriatic plaques already existing.
    The fact that abdominal lesions are clearing up is a good sign, we know psoriasis is one of the skin disease that show cephalo-caudal spread, either for better or for worse. So her lesions over the legs may be next to improve, except for the other fact that shin lesions are notorious for being stubborn!

    We have no proof that she took systemic steroid during the Covid lockdown, just guessing. The pustules may be just a flare-up due to stress we all face during lockdown. Psoriasis is a neutrophilic skin condition, the neutrophils are overcrowded and are just taking some ‘social distancing ‘ at the borders of the plaques. But I suggest no to test our luck, so no iv. hydrocortisone.

    If she agrees, we can have another good go with oral methotrexate or even acitretin ( which be more effective for pustules, provided she is not planning to be pregnant again, same restriction actually applies to methotrexate.)

    Topically I suggest Xamiol ( calcipotriol/betamethasone ) plus lots of topical medical device to moisturise, to reduce inflammation and itch without steroids in them).
    All the other measures and precautions apply.

    ReplyDelete

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