Pustular psoriasis of von Zumbusch
| Generalized pustular psoriasis | |
|---|---|
| Classification and external resources | |
| Specialty | dermatology |
| ICD-10 | L40.1 (ILDS L40.100) |
Generalized pustular psoriasis (GPP) is an extremely rare type of psoriasis that can present in a variety of forms. Unlike the most general and common forms of psoriasis, GPP usually covers the entire body and with pus-filled blisters rather than plaques. GPP can present at any age, but is rarer in young children. It can appear with or without previous psoriasis conditions or history, and can reoccur in periodic episodes.
GPP presents as pustules and plaques over a wide area of the body. It differs from the localized form of pustular psoriasis in that patients are often febrile and systemically ill.
However, the most prominent symptom, as described in the Archives of Dermatology, is “sheeted, pinhead-sized, sterile, sub-corneal pustules”. The IPC roundtable adds that these pustules often occur either at the edges “of expanding, intensely inflammatory plaques” or “within erythrodermic skin.”
GPP is a rare and severe type of psoriasis. It in rare cases it is said to be fatal and in some cases has driven patients to intensive burn units. An article published in Pediatric Dermatology said, “The GPP pattern is as an acute, episodic, and potentially life-threatening form of psoriasis.”
There is no cure-all treatment for GPP, and as such, the mortality rate is high. Ryan and Baker observed 155 patients with GPP, 106 of which were followed up with. 26 of those 106 died as a result of the psoriasis or the treatment. Their data gives a 25% mortality rate.
There are many cases where GPP has been treated or reduced to a manageable level. The treatments vary depending on the specific factors of each case.
It is important to note that while there are different forms of GPP, they are not exclusive of each other. One can morph into another, or multiple forms can occur simultaneously.
Von Zumbusch psoriasis is named after the German dermatologist Leo Ritter von Zumbusch (1874–1940), son of Kaspar von Zumbusch who described the first documented case of generalized pustular psoriasis in the early 1900s. See Case Report #1. Sometimes all or any of GPP is referred to as von Zumbusch psoriasis, but in the literature it is often distinguished as one specific form of GPP.
Eugene M. Farber, MD and colleagues provide a description of von Zumbusch psoriasis in "Pustular Psoriasis", published in Cutis. They describe the pattern as having “waves of widespread or universally fiery redness”. The affected areas are “painful and tender”. Small sub-corneal pustules form, with sizes originally between 1 and 10 mm in diameter. These pustules may merge to form “yellow-green lakes of pus”. The pustules dry out, and “Waves of scarlatiniform [resembling scarlet fever] peeling follow, removing the desiccating pustules”. In regards to the onset, the von Zumbusch form may “supervene on any previous pattern of psoriasis”. It also may or may not recur periodically.
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