Acute generalized pustular psoriasis (Zumbusch type): successful treatment without biologics

Vladimirova E.V.

Academy of postgraduate education under Federal Research and Clinical Center of Specialized Medical Care and Medical Technologies FMBA of Russia

ORCID: 0000-0002-4123-4204

Murakov S.V.

Academy of Postgraduate Education under Federal Scientific and Clinical Center of Federal Medical-Biological Agency of Russia

ORCID: 0000-0003-4330-2570

Markova Yu.A.

Department of skin and venereal diseases with cosmetology course Federal state budgetary educational institution of additional professional education Institute of advanced training of Federal medical and biological Agency

ORCID: 0000-0002-6271-9403

Matushevskaya E.V.

Federal Research and Clinical Center of Specialized Medical Care and Medical Technologies FMBA

ORCID: 0000-0003-4583-0617

Vladimirov A.A.

I.M. Sechenov First Moscow State Medical University

ORCID: 0000-0002-3943-8099

Kapuler O.M.

Center of Cosmetology, Plastic and Reconstructive Surgery

ORCID: 0000-0001-5714-5495

Bezugly A.P.

Academy of Postgraduate Education under Federal Scientific and Clinical Center of Federal Medical-Biological Agency of Russia

Scopus AuthorID: 26646232100
ORCID: 0000-0001-5192-4252

Sirmays N.S.

Federal Research and Clinical Center of Specialized Medical Care and Medical Technologies of the Federal Medical Agency;
Centre of Modern paediatrics

ORCID: 0000-0003-0162-132X

Petinati Ya.A.

Academy of Postgraduate Education under Federal Scientific and Clinical Center of Federal Medical-Biological Agency of Russia

ORCID: 0000-0002-3994-4346

Acute generalized pustular psoriasis (Zumbusch type): successful treatment without biologics

Authors:

Vladimirova E.V., Murakov S.V., Markova Yu.A., Matushevskaya E.V., Vladimirov A.A., Kapuler O.M., Bezugly A.P., Sirmays N.S., Petinati Ya.A.

More about the authors

Journal: Russian Journal of Clinical Dermatology and Venereology. 2023;22(1): 26‑32

DOI: 10.17116/klinderma20232201126

Downloaded: 297

To cite this article:

Vladimirova EV, Murakov SV, Markova YuA, et al. . Acute generalized pustular psoriasis (Zumbusch type): successful treatment without biologics. Russian Journal of Clinical Dermatology and Venereology. 2023;22(1):26‑32. (In Russ., In Engl.)
https://doi.org/10.17116/klinderma20232201126

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BACKGROUND

Psoriasis is a chronic inflammatory disease predominantly affecting the skin. Acute generalized pustular psoriasis (GPP) is one of the rarest and most severe types of psoriasis. GPP manifests as spontaneously occurring specific skin lesions and is accompanied by a worsening of the general condition (fever, chills, malaise, nausea, severe pain, joint pain). Among the factors provoking pustular psoriasis are abrupt withdrawal of systemic steroid drugs, pregnancy, hypocalcemia, upper respiratory tract infections, and mental disorders. The main pathophysiological mechanism of the occurrence and progression of GPP is the migration of polymorphonuclear leukocytes from the vascular wall into the tissues and the activation of interleukins and nonspecific cytokines. Acitretin, cyclosporine, and methotrexate are used as first-line therapy. In addition, retinoids are also used in the basic therapy of various types of psoriasis. Second-line therapy includes adalimumab, etanercept, PUVA therapy, topical corticosteroids, and topical calcipotriol. The UK National Institute for Health and Clinical Excellence (NICE) has issued guidelines on non-biological therapies.

OBJECTIVE

A clinical case of Zumbusch-type GPP in a 25-year-old patient with a history of the disease since 3 years old is described.

MATERIAL AND METHODS

Treatment included cyclosporine A in the first phase and the combination of retinoids with total PUVA therapy in the second phase.

RESULTS

This treatment method made it possible to achieve 100% regression of the rash and ensure the absence of relapses without expensive biologicals.

CONCLUSION

The results suggest that effective treatment of acute GPP (Zumbusch type) is possible without biological therapy.

Keywords:

pustular psoriasis

Zumbusch psoriasis

cyclosporine A

acitretin

PUVA therapy

Authors:

Vladimirova E.V.

Academy of postgraduate education under Federal Research and Clinical Center of Specialized Medical Care and Medical Technologies FMBA of Russia

ORCID: 0000-0002-4123-4204

Murakov S.V.

Academy of Postgraduate Education under Federal Scientific and Clinical Center of Federal Medical-Biological Agency of Russia

ORCID: 0000-0003-4330-2570

Markova Yu.A.

ORCID: 0000-0002-6271-9403

Matushevskaya E.V.

Federal Research and Clinical Center of Specialized Medical Care and Medical Technologies FMBA

ORCID: 0000-0003-4583-0617

Vladimirov A.A.

I.M. Sechenov First Moscow State Medical University

ORCID: 0000-0002-3943-8099

Kapuler O.M.

Center of Cosmetology, Plastic and Reconstructive Surgery

ORCID: 0000-0001-5714-5495

Bezugly A.P.

Academy of Postgraduate Education under Federal Scientific and Clinical Center of Federal Medical-Biological Agency of Russia

Scopus AuthorID: 26646232100
ORCID: 0000-0001-5192-4252

Sirmays N.S.

Federal Research and Clinical Center of Specialized Medical Care and Medical Technologies of the Federal Medical Agency;
Centre of Modern paediatrics

ORCID: 0000-0003-0162-132X

Petinati Ya.A.

Academy of Postgraduate Education under Federal Scientific and Clinical Center of Federal Medical-Biological Agency of Russia

ORCID: 0000-0002-3994-4346

Received:

03.08.2022

Accepted:

14.11.2022

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