einstein (São Paulo). 13/ago/2024;22(Suppl 2):MV005.

Generalized pustular psoriasis (von Zumbusch)

DOI: 10.31744/einstein_journal/2024ABS_EISIC_MV005

Category: Infection

Introduction:

Von Zumbusch syndrome, also known as Leiner-Moussous ichthyosiform erythroderma, is a rare and severe form of generalized pustular psoriasis. Characterized by the sudden appearance of sterile pustules (filled with liquid) on the skin, which then turn red and peel intensely, it initially appears in areas of flexure. It may be accompanied by fever, general malaise, muscle weakness and other systemic symptoms. It can be dangerous due to potential complications such as dehydration, secondary infection and organ failure. It requires urgent medical attention and aggressive treatment with medications, which may include corticosteroids, retinoids, immunosuppressants and biological therapies.^(–)

Objective:

This report aims to illustrate an individual with a characteristic clinical presentation of Von Zumbusch syndrome.

Case description:

JDD, 66 years old, female, with chronic obstructive pulmonary disease GOLD IV D, systemic arterial hypertension, heart failure, type 2 diabetes mellitus, hypothyroidism and grade 3 obesity. Patient was admitted to the Hospital Regional José Alencar Gomes da Silva in Uberaba – Minas Gerais, on 06/09/2023 due to acute respiratory failure due to an exacerbation of the underlying disease due to pneumonia. During treatment, the patient suddenly developed the presence of widespread pustular papules, with erythema, which progressed to desquamation during the use of systemic corticosteroids and not after their discontinuation, as is commonly observed (). There was already a history of similar previous injuries in another hospitalization. The diagnostic hypothesis was raised and a biopsy of the lesions was performed, resulting in skin with foci of parakeratosis, agranulosis and an intense neutrophilic inflammatory process with intraepidermal and subepidermal vesico-bullous formations. The use of corticosteroids was suspended due to the possibility of their use being a trigger for the development of the disease. A significant improvement in the lesions was observed in addition to the cessation of the appearance of other lesions.

Comments:

In this case, the skin changes were extremely suggestive of generalized pustular psoriasis, but due to the scarcity of data on the disease it is difficult to think about the diagnostic hypothesis. In this sense, more studies and evidence are needed to help.