A RARE CASE OF GENERALIZED PUSTULAR PSORIASIS

Keywords: Generalized pustular psoriasis, multisystem disease

Introduction:
Generalized pustular psoriasis (GPP) is a severe inflammatory dermatosis characterized by am eruption of sterile pustules, in the setting of severe inflammation [1]. The condition is rare and can be triggered by infections; viral or bacterial, drug use; particularly corticosteroids, psychological stress, pregnancy, and sunlight exposure or phototherapy [2]. At present, no standardized criteria exist for the diagnosis of GPP [3]. Described below, is a case of this rare phenomenon.

Case Summary:
A 31-year-old man presented to the dermatologist with a scaly, erythematous plaque seen on the hands, chest, back for 2 days [Figure 1]. He reported severe aggravation after working in direct sunlight. Some follicular scaly lesions on the scalp were seen as well. Differential diagnosis included psoriasis with erythroderma. On histopathological examination, lymphocytic infiltrate and intense papillary and epidermal edema causing spongiosis can be seen. The neutrophillic infiltrate causes spongiform pustule formation known as pustules of Kogoj. Acanthosis with rete ridge elongation can also seen [Figure 2].

Figure 1

Figure 2

Discussion:
Germline mutations due to deletions in IL36RN have been reported previously in literature [1]. Those with such mutations are at higher risk of developing early-onset GPP and subsequent systemic inflammatory response [3]. Studies have shown that the disease most commonly occurs in patients of east Asian backgrounds, at approximately 40 years of age. Severe flares can be potentially fatal, especially in those over 50 years [1]. By understanding the signaling pathway of interleukin (IL)-36, ‘Spesolimab’, a monoclonal antibody against IL-36 receptors, has been shown to improve and clear pustules in the acute stage [4].

Conclusion:
In GPP, the pathological aspects of the disease are amplified. GPP can be life threatening and critical, prompting the need for quick treatment, especially when a trigger is known.

References:
[1] Mirza HA, Badri Talel, Kwan, E (2022) StatPearls,
[2] Choon SE, Navarini AA, Pinter A (2022) Am J Clin Dermatol. 23, 21-29
[3] Teshima R, Saito-Sasaki N, Sawada Y (2024), Int J Mol Sci, 25
[4] Gwillim EC, Nichols AJ (2024) Front Immunol, 15

from Gulf Medical University and completed her internship under the Dubai Academic Health Corporation (Dubai Health). She has since been involved in clinical teaching of undergraduate medical students at Gulf Medical University, whilst actively preparing for Residency training in the UK. She possesses a keen interest in clinical research and has published numerous case reports and original studies in peer-reviewed, pubMed-indexed journals.

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