Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis
- Robert E Kalb, MD
Robert E Kalb, MD
- Clinical Professor of Dermatology
- State University of New York at Buffalo
Psoriasis is a common skin disorder characterized by the development of erythematous, scaling plaques on the skin and a wide spectrum of clinical presentations. The most common presentation of psoriasis is chronic plaque psoriasis, which manifests as well-defined inflammatory, red, scaling plaques on the skin. Pustular psoriasis is a less common subtype of psoriasis that presents as an acute, subacute, or chronic pustular eruption. Pustular psoriasis primarily affects adults, but can also occur in children.
Pustular psoriasis develops independently or in association with preexisting psoriasis and may occur in a generalized or localized distribution. Generalized pustular psoriasis (GPP) presents as an acute or subacute, widely distributed eruption of pustules arising on inflamed, erythematous skin (picture 1A-C). Some cases of generalized pustular psoriasis may actually represent a new genetic autoinflammatory disease based on mutations in the IL-36 receptor antagonist. Localized forms of pustular psoriasis primarily affect the palms, soles, or extremity digits.
The epidemiology, pathogenesis, and clinical manifestations of pustular psoriasis will be reviewed here. Other forms of psoriasis and the management of pustular psoriasis are reviewed separately. (See "Epidemiology, clinical manifestations, and diagnosis of psoriasis" and "Guttate psoriasis" and "Pustular psoriasis: Management".)
The clinical presentations of pustular psoriasis traditionally have been classified into generalized and localized forms. The major clinical variants of generalized pustular psoriasis (GPP) include acute GPP (also known as generalized pustular psoriasis of von Zumbusch) and generalized annular pustular psoriasis (also known as subacute GPP) . Acute GPP is characterized by the abrupt onset of numerous pustules, widespread erythema, and systemic symptoms. The terms "pustular psoriasis of pregnancy" and "impetigo herpetiformis" are used to refer to acute GPP that occurs during pregnancy. Generalized annular pustular psoriasis is a less acute presentation of GPP in which patients develop widespread annular or figurate erythematous plaques studded by pustules. (See "Dermatoses of pregnancy", section on 'Pustular psoriasis of pregnancy'.)
Descriptions of localized forms of pustular psoriasis include acrodermatitis continua of Hallopeau, a chronic pustular condition that primarily involves the digits, and palmoplantar pustulosis, a chronic condition in which the pustular eruption is primarily limited to the palms and soles. It is controversial whether palmoplantar pustulosis is a variant of psoriasis or an independent entity [2,3]. Alternative terms used to refer to palmoplantar pustulosis include palmoplantar pustular psoriasis, pustulosis palmoplantaris, and pustulosis palmaris et plantaris. (See 'Localized pustular psoriasis' below and "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis".)
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- GENERALIZED PUSTULAR PSORIASIS
- - Genetics
- - Precipitating factors
- Clinical manifestations
- Clinical course
- Laboratory abnormalities
- - History and physical examination
- - Skin biopsy
- - Laboratory tests
- Differential diagnosis
- LOCALIZED PUSTULAR PSORIASIS
- Acrodermatitis continua of Hallopeau
- Palmoplantar pustulosis
- SUMMARY AND RECOMMENDATIONS