eMedicine Specialties > Dermatology > Papulosquamous Diseases
Psoriasis, Pustular
Updated: Dec 29, 2009
Introduction
Background
Pustular psoriasis is an uncommon form of psoriasis consisting of widespread pustules on an erythematous background, as shown in the image below.
Note the clearly defined, raised bumps on the skin that are filled with pus (pustules). The skin under and around these bumps is reddish. Courtesy of Hon Pak, MD.
Pustular psoriasis may result in an erythroderma. Cutaneous lesions characteristic of psoriasis vulgaris may be present before, during, or after an acute pustular episode. Generally, pustular psoriasis may be classified into several types depending on the clinical course, which may be acute, subacute, or chronic. The acute generalized type accompanied by fever and toxicity also is termed the von Zumbusch variant. An annular or circinate type that tends to run a subacute or chronic course with less systemic manifestations also has been described. In addition, a juvenile or infantile type is seen that is the least common.
Pathophysiology
Enhanced polymorphonuclear leukocyte (PMNL) chemotaxis is much more pronounced in pustular psoriasis than in psoriasis vulgaris.1 This observation has been attributed to either an intrinsic PMNL defect or to the presence of chemoattractants in the psoriatic epidermis. Although the principal stimulus that triggers the phenomenon of massive PMNL migration from the vasculature to the epidermis is unknown, cytokines elaborated by keratinocytes are believed to aid the process.
Electron microscopic studies have shown the presence of basal keratinocyte herniations. These are cytoplasmic processes from basal keratinocytes that protrude into the dermis through gaps in the basal lamina in lesions of pustular psoriasis. These herniations mostly are clustered over collections of neutrophils in the dermis. This finding suggests an increased production of neutrophilic proteolytic enzymes in the dermis of these patients.
Immunohistochemical methods have determined the involvement of some of these proteases and their inhibitors in the development of pustulation. Elastase is a proteolytic enzyme released by PMNLs during the process of extravasation and migration through the dermoepidermal junction. An epidermal elastase inhibitor, termed skin-derived antileukoproteinase, was found expressed in psoriatic skin prior to influx of PMNLs and to disappear when the composition of the infiltrate changed. This finding was not confirmed by other studies. Additional studies investigating other potential mechanisms have shown decreased natural killer cell activity in generalized pustular psoriasis. An increased incidence of HLA-B27 also has been found among patients with pustular psoriasis. This haplotype is seen in psoriasis patients with peripheral arthritis, as well as in patients with ankylosing spondylitis and reactive arthritis.
Frequency
United States
Pustular psoriasis is uncommon in the United States.
International
The prevalence of pustular psoriasis in Japan is 7.46 cases per 1 million people.
Mortality/Morbidity
The generalized pustular psoriasis of von Zumbusch may be lethal if proper supportive measures are not taken during the acute phase.
Race
Pustular psoriasis affects all races.
Sex
- Male-to-female ratio for pustular psoriasis is 1:1 in adults.
- Male-to-female ratio for pustular psoriasis is 3:2 in children.
Age
- The average age among adult pustular psoriasis patients is 50 years.
- Children aged 6 weeks to 10 years can be affected, although rarely. One case report describes generalized pustular psoriasis in a 6-week-old infant.2
Clinical
History
In the generalized type, skin initially becomes fiery red and tender. The patient experiences constitutional signs and symptoms, such as headache, fever, chills, arthralgia, malaise, anorexia, and nausea. Within hours, clusters of nonfollicular, superficial 2- to 3-mm pustules may appear in a generalized pattern.
The most common sites of involvement for pustular psoriasis are the flexural and anogenital areas. Less often, facial lesions occur. Pustules may occur on the tongue and subungually, resulting in dysphagia and nail shedding, respectively.3 These pustules coalesce within 1 day to form lakes of pus that dry and desquamate in sheets, leaving behind a smooth erythematous surface on which new crops of pustules may appear. These episodes of pustulation may occur for days to weeks, thereby causing the patient severe discomfort and exhaustion. A telogen effluvium type of hair loss may develop in 2-3 months.
Upon remission of the pustular component, most systemic symptoms disappear; however, the patient may be in an erythrodermic state or may have residual lesions of psoriasis vulgaris.
The circinate or annular type predominates in infancy. This subtype tends to run a more subacute or chronic course, with less severe manifestations. Often, recurrent episodes of annular or circinate erythematous plaques are seen, with pustules on the periphery. These lesions primarily appear over the trunk but also involve the extremities. They undergo peripheral expansion and central healing. Other systemic signs and symptoms are either absent or mild. The juvenile/infantile type of pustular psoriasis typically has a benign course. Systemic involvement is not common, and spontaneous remissions occur frequently.
Physical
The patient appears frightened, often tachypneic, tachycardic, and febrile. The oropharyngeal mucosa may be hyperemic, and a geographic tongue or fissured tongue may be appreciated. The skin shows a generalized or patchy erythema studded with interfollicular pustules that may have an annular or nonspecific configuration. Flexural and anogenital accentuation may be present. The lesions may appear on the trunk, extremities, and rarely, on the face. Pustulation also occurs on the nailbeds, resulting in onychodystrophy, onycholysis, and defluvium unguium. Scaling may be observed, especially in areas that already have undergone pustulation. The rest of the physical examination depends on complications in other organ systems, such as the cardiovascular system.
Causes
The following have reportedly triggered an eruption of pustular psoriasis:
- Withdrawal of systemic steroids4
- Drugs, including salicylates, iodine, lithium, phenylbutazone, oxyphenbutazone, trazodone, penicillin, hydroxychloroquine, calcipotriol, interferon-alpha, and recombinant interferon-beta injection5
- Strong, irritating topicals, including tar, anthralin, steroids under occlusion, and zinc pyrithione in shampoo
- Infections6
- Sunlight or phototherapy
- Cholestatic jaundice
- Hypocalcemia
- Idiopathic in many patients
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References
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Further Reading
Keywords
psoriasis, pustular psoriasis, von Zumbusch psoriasis
