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Pustular psoriasis in Adult
See also in: Nail and Distal Digit
Other Resources UpToDate PubMed

Pustular psoriasis in Adult

See also in: Nail and Distal Digit
Contributors: Haya Raef MD, Jeffrey M. Cohen MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Pustular psoriasis is an uncommon variant of psoriasis that is characterized by the presence of widespread, erythematous, and sterile neutrophilic pustules, accompanied by constitutional symptoms. Pustular psoriasis, particularly in the acute setting, can be a severe inflammatory disease that requires hospitalization and aggressive therapy. Untreated disease can also progress to erythroderma.

Pustular psoriasis may occur in children but is more commonly seen in middle-aged adults. It is also found more frequently in Asian and Hispanic individuals than in White individuals. Some patients with a history of plaque psoriasis can develop pustular psoriasis, but the two conditions appear to have distinct genetic and pathophysiologic mechanisms.

The etiology of pustular psoriasis is incompletely understood, but cases have been associated with hypocalcemia, infection (Trichophyton rubrum, cytomegalovirus, Streptococcus spp, varicella-zoster virus, and Epstein-Barr virus), a rapid withdrawal of corticosteroids, pregnancy, medications (NSAIDs, lithium, potassium iodine, trazodone, penicillin, interferon, and hydroxychloroquine), and topical irritants such as tar and anthralin. While tumor necrosis factor (TNF)-alpha antagonists such as infliximab and adalimumab are used to treat pustular psoriasis, they have also been reported paradoxically to induce it.

Additionally, there is emerging evidence that pustular psoriasis is associated with mutations in the IL36RN gene that encodes the interleukin-36 receptor antagonist (IL-36RA). Mutations in this gene have been detected in different variants of pustular psoriasis, including generalized pustular psoriasis, impetigo herpetiformis, palmoplantar pustulosis, and the exanthematic type of pustular psoriasis. Deficiency of interleukin-36 (IL-36) receptor antagonist (DITRA) syndrome is an autoimmune inflammatory disorder caused by loss of function mutations in the IL36RN gene that manifests in early childhood with generalized pustular psoriasis, fever, leukocytosis, and elevated C-reactive protein (CRP) levels.

There are 4 subtypes of pustular psoriasis:
  • von Zumbusch type (generalized pustular psoriasis) – Acute onset of generalized erythema and pustules with systemic manifestations including fever, skin tenderness, malaise, arthralgias, headache, and nausea. After several days, the pustules resolve to become confluent, scaling plaques.
  • Exanthematic type – Acute onset of small pustules that are triggered by an infection or a drug. This subtype usually lacks systemic symptoms.
  • Annular subtype – Erythematous, annular lesions that have pustules at the advancing edge of a lesion and is associated with fever, malaise, and other systemic manifestations. The annular variant is the most common form of pustular psoriasis in children.
  • Localized pattern – Pustules appear in existing psoriatic plaques. This can be seen in active plaques. Palmoplantar psoriasis is the most common form of localized pustular psoriasis.
Approximately 50% of pregnant patients with psoriasis report improvement of disease burden. However, there are many reports that show the development of pustular psoriasis in pregnant patients who are hypocalcemic. Pustular psoriasis that occurs during pregnancy is termed impetigo herpetiformis. This represents a risk to both maternal and fetal health (including risk of stillbirth) and should be treated aggressively.

Patients may experience relapses and remissions over a period of years. It may be precipitated by use and withdrawal from systemic corticosteroids.

Extracutaneous manifestations of pustular psoriasis may be severe. The most common extracutaneous manifestations of pustular psoriasis include cholestasis, cholangitis, arthritis, intestinal pneumonitis, oral lesions, and acute renal failure. Electrolyte disturbances such as hypocalcemia may occur and can be life-threatening. Lesions may also become superinfected.

Note: Only in rare cases, such as severe pustular psoriasis or pustular psoriasis of pregnancy, should oral steroids be considered. The use of systemic steroids will lead to severe psoriasis rebound after steroid discontinuation.

Codes

ICD10CM:
L40.1 – Generalized pustular psoriasis

SNOMEDCT:
200973000 – Pustular psoriasis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Acute generalized exanthematous pustulosis (AGEP) – Clinically indistinguishable from pustular psoriasis. Time of onset and a drug history may help differentiate AGEP from pustular psoriasis. Antibiotics are the likely causative agents in AGEP. Histology can also help differentiate between the two. Also look for high fever, edema of the face, pustular eruption that occurs shortly after drug administration (fewer than 2 days), marked serum leukocytosis with neutrophilia, and associated petechiae, purpura, and vesicles in AGEP.
  • Drug reaction with eosinophilia and systemic symptoms (DRESS) – Look for marked eosinophilia, visceral involvement (most commonly hepatitis), less acute onset, facial edema, and atypical lymphocytosis.
  • Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) – Associated with IgA paraproteinemia. Very responsive to dapsone. Pustular psoriasis is not responsive to dapsone and does not have an IgA paraproteinemia.
  • Keratoderma blennorrhagicum seen in reactive arthritis disease – Look for characteristic associated findings including urethritis, arthritis, and ocular findings.
  • Dyshidrotic eczema – Extremely pruritic, and restricted to hands and feet. Look for deep-seated vesicles that look like tapioca pudding.
  • Erythema annulare centrifugum – Consider this when annular-type psoriasis is observed. No associated systemic findings. Individual lesions can last for months.
  • Disseminated herpes simplex
  • Eczema with secondary infection

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:01/19/2022
Last Updated:01/23/2022
Copyright © 2022 VisualDx®. All rights reserved.
Pustular psoriasis in Adult
See also in: Nail and Distal Digit
Pustular psoriasis (von Zumbusch type) : Fever, Headache, Nausea, Erythema, Flexural distribution, Malaise, Arthralgia, WBC increased
Clinical image of Pustular psoriasis
A close-up of well-demarcated, erythematous plaques with peripherally accentuated scale.
Copyright © 2022 VisualDx®. All rights reserved.