Generalized pustular psoriasis 1_1213 Derm Dx
Generalized pustular psoriasis 2_1213 Derm Dx
Generalized pustular psoriasis 3_1213 Derm Dx
Generalized pustular psoriasis 4_1213 Derm Dx
Generalized pustular psoriasis 5_1213 Derm Dx
Generalized pustular psoriasis 6_1213 Derm Dx
Generalized pustular psoriasis 7_1213 Derm Dx
A 48-year-old patient presents complaining of rash. She had initially presented to urgent care with a milder rash several weeks ago and was prescribed a prednisone taper. The prednisone cleared the initial rash, but two days after stopping the prednisone she developed a more severe rash (pictured). She complains of malaise. On exam she is shivering and tachycardic.
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Generalized pustular psoriasis (GPP) is a rare form of psoriasis that has a wide array of clinical presentations. Unlike chronic plaque psoriasis, it can be a life-threatening emergency associated with fever, malaise, diarrhea, hypocalcemia and leukocytosis.
In severe cases, it can be confused with sepsis. Hypoalbuminemia can result from a loss of plasma proteins into tissues with subsequent renal tubular necrosis and transaminitis. Patients can also suffer from malabsorption and malnutrition. There are case reports of death from GPP, and mortality is usually a result of cardiorespiratory failure.
Triggering factors for disease include pregnancy, rapid withdraw of corticosteroids, drugs, hypocalcemia and infection. In pregnancy, the disease is called impetigo herpetiformis. Most GPP cases present in patients with a prior history of psoriasis.
Patients complain of flaming red and tender skin. The most common sites of involvement are the flexural and anogenital regions, though all areas of the body can be affected. Some patients experience headache, fevers, chills, malaise, nausea, vomiting and diarrhea.
Superficial 2mm to 4mm pustules appear over the affected area. Typically the pustules converge within the same day of onset and form lakes of pus that dry and desquamate in sheets. The entire process frequently repeats itself and new pustules may appear. These episodes may occur for days or weeks, causing severe discomfort and pain to the patient. Upon remission of the pustular component, most systemic symptoms disappear.
The most acute form of the disease is called “acute von Zumbusch” and was originally described by German dermatologist Leo Ritter von Zumbusch. Patients experience severe pain and systemic signs are typically noted. After several days, the pustules resolve and extensive scaling is observed.
The diagnosis of GPP is made by history and skin examination. On physical exam, patients may present with tachypnea, tachycardia and may be febrile. The skin shows a generalized or patchy erythema with widespread interfollicular pustules with an annular or nonspecific configuration on an erythematous base. Pustulation can occur on the nail beds, resulting in onchodystrophy.
Laboratory findings include an elevated ESR, CRP and leukocytosis. If the patient is oligemic, the BUN/creatinine ratio will be increased. Blood cultures are negative, which can be useful in differentiating GPP from sepsis.
The diagnosis is frequently confirmed from a biopsy. As in chronic plaque psoriasis, parakeratosis and elongation of the rete ridges is seen within the epidermis. An infiltrate of neutrophils is present within the lesions, migrating from papillary capillaries to the epidermis. Macropustules within the epidermis are seen and are termed the “Spongiform Pustule of Kogoj.”
Patients are often admitted to the hospital for close observation and to ensure adequate hydration, thermodynamic regulation and hemodynamic stabilization.
Treatment of disease varies and should be managed by a dermatologist. In mild cases, topical agents such as corticosteroids are most commonly used. Oral retinoids, methotrexate and cyclosporine, as well as biological agents such as adalimumab, etanercept and infliximab are used in severe cases.
Christopher Downing, MD, is a dermatology research fellow at the Center for Clinical Studies in Houston, Texas.
Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine also in Houston.
- Bolognia J, Jorizzo J, Shaffer J. “Chapter 8 – Psoriasis.” Dermatology 3rd Edition. St. Louis, Mo.: Saunders, 2012.
- Viguier M, Allez M, Zagdanski AM et al. Hepatology. 2004; 40: 452.
- Steffen C. Am J Dermatopathol. 2002; 24(4): 364-368.
- Choon SE, Lai NM, Mohammad NA et al. Int J Dermatol. 2013; doi:10.1111/ijd.12070.