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Pustular psoriasis in Adult
See also in: Nail and Distal Digit
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Pustular psoriasis in Adult

See also in: Nail and Distal Digit
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Contributors: 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, sterile pustules on clinical examination and a predominantly neutrophilic infiltrate with intraepidermal and subcorneal pustules histopathologically. 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.

The etiology of pustular psoriasis is incompletely understood, but cases have been associated with hypocalcemia, infection (Trichophyton rubrum, cytomegalovirus, Streptococcus spp, varicella-zoster, and Epstein-Barr virus), a rapid withdrawal of corticosteroids, pregnancy, medications (salicylates, 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 dysregulation in the interleukin-36 pathway. Some patients with a history of plaque psoriasis can develop pustular psoriasis, but the two conditions appear to have distinct genetic and pathophysiologic mechanisms.

There are 4 subtypes of pustular psoriasis:
  • von Zumbusch type: 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.
  • 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. There is no racial predilection. Pustular psoriasis may occur in children but is more commonly seen in middle-aged adults. It may be precipitated by use and withdrawal from systemic corticosteroids.

Extracutaneous manifestations of pustular psoriasis may be severe and are associated with a great deal of morbidity and mortality. 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. This is because the use of systemic steroids will lead to severe psoriasis rebound after steroid discontinuation.

For more information on generalized pustular psoriasis, see OMIM.

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 and is 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, restricted to hands and feet, look for deep-seated vesicles that look like tapioca pudding.
  • Erythema annulare centrifugum – To be considered when annular-type psoriasis is observed. No associated systemic findings. Individual lesions can last for months.
  • Disseminated herpes simplex

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: 06/29/2018
Last Updated: 06/29/2018
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Pustular psoriasis in Adult
See also in: Nail and Distal Digit
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Pustular psoriasis : Erythroderma, Painful skin lesions, Widespread, Pustules
Clinical image of Pustular psoriasis
A close-up of well-demarcated, erythematous plaques with peripherally accentuated scale.
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