History

In generalized pustular psoriasis, the skin initially becomes fiery red and tender. Constitutional signs and symptoms include headache, fever, chills, arthralgia, malaise, anorexia, and nausea. Within hours, clusters of nonfollicular, superficial, 2- to 3-mm pustules may appear in a diffuse pattern.

Flexural and anogenital areas are most commonly involved in pustular psoriasis. Less often, facial lesions occur. Pustules can appear on the tongue and develop subungually, resulting in dysphagia and nail shedding, respectively.^[26]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 recur.

Episodes of pustulation occur for days to weeks, causing the patient severe discomfort and exhaustion. A telogen effluvium type of hair loss may develop in 2-3 months.

Upon remission of pustules, most systemic symptoms disappear. However, patients can experience an erythrodermic state or residual lesions of psoriasis vulgaris. Patients with a history of psoriasis may show a predilection for particular pustular psoriasis subtypes, particularly generalized pustular psoriasis and acrodermatitis of Hallopeau^[4]; however, a preceding history of psoriasis is not a requirement.^[14]

Circinate or annular-type pustular psoriasis predominates in childhood and runs a more subacute course with less severe manifestations. Often, recurrent episodes of annular or circinate erythematous plaques are seen, with pustules and scaling along the periphery.^[3]These lesions appear primarily on the trunk and undergo peripheral expansion with central healing over hours to days. Other systemic signs and symptoms are either mild or absent.

The juvenile/infantile type of pustular psoriasis typically has a benign course. Systemic involvement is not common, and spontaneous remissions frequently occur.

Physical Examination

Patients appear distressed, often tachypneic, tachycardic, and febrile. The oropharyngeal mucosa may be hyperemic, and a geographic tongue or fissured tongue may be appreciated. Skin findings include a generalized or patchy erythema studded with interfollicular pustules that may have an annular or generalized/nonspecific configuration.

Lesions appear on the trunk, extremities, and, rarely, on the face. Flexural and anogenital accentuation may be present. Pustulation may also involve the nail beds, resulting in onychodystrophy, onycholysis, and defluvium unguium.

Peripheral scaling may be observed, especially in areas that have undergone pustulation. The rest of the physical examination depends on systemic complications.

Complications

Occasionally, acute respiratory distress syndrome may complicate generalized pustular psoriasis.

Other possible complications in pustular psoriasis include the following:

Secondary bacterial skin infections, hair loss ( telogen effluvium), and nail loss
Hypoalbuminemia secondary to loss of plasma protein into tissues
Hypocalcemia
Renal tubular necrosis as a result of oligemia
Osteoarthritis
Uveitis
Neutrophilic cholangitis
Liver damage as a result of oligemia, neutrophilic cholangitis, ^[27]and general toxicity
Malabsorption and malnutrition

Differential Diagnoses

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Media Gallery

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.
Palmoplantar pustular psoriasis, a type of pustular psoriasis that appears on the palms of the hands or the soles of the feet. Courtesy of Hon Pak, MD.

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Author

Clay J Cockerell, MD President and Owner, Cockerell Dermatopathology; Adjunct Clinical Professor, Department of Internal Medicine (Dermatology), Division of Dermatopathology, University of North Texas Health Science Center at Forth Worth

Clay J Cockerell, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of HIV Medicine, American College of Osteopathic Family Physicians, American College of Physician Executives, American Contact Dermatitis Society, American Dermatological Association, American Medical Association, American Society for Clinical Pathology, American Society for Dermatologic Surgery, American Society of Dermatology, American Society of Dermatopathology, California Society of Dermatology and Dermatologic Surgery, College of American Pathologists, Dallas County Medical Society, Dermatology Foundation, International Academy of Pathology, International AIDS Society, International Society of Dermatology, Louisiana Dermatological Society, Noah Worcester Dermatological Society, North American Clinical Dermatologic Society, Pacific Dermatologic Association, Physicians Association for AIDS Care, Society for Investigative Dermatology, Southern Medical Association, Texas Dermatological Society, Texas Medical Association, Texas Society of Pathologists, United States and Canadian Academy of Pathology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Christen M Mowad, MD Professor, Department of Dermatology, Geisinger Medical Center

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, Noah Worcester Dermatological Society, Pennsylvania Academy of Dermatology, American Academy of Dermatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Mark G Lebwohl, MD Chairman, Department of Dermatology, Mount Sinai School of Medicine

Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Received none from Amgen for consultant & investigator; Received none from Novartis for consultant & investigator; Received none from Pfizer for consultant & investigator; Received none from Celgene Corporation for consultant & investigator; Received none from Clinuvel for consultant & investigator; Received none from Eli Lilly & Co. for consultant & investigator; Received none from Janssen Ortho Biotech for consultant & investigator; Received none from LEO Pharmaceuticals for consultant & inves.

Carlos Ricotti, MD Fellow, Department of Dermatology, Division of Dermatopathology, University of Texas Southwestern School of Medicine

Carlos Ricotti, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, International Society of Dermatopathology

Disclosure: Nothing to disclose.

Annie O Morrison, MD Fellow in Dermatopathology, Cockerell Dermatopathology

Annie O Morrison, MD is a member of the following medical societies: Academy of Clinical Laboratory Physicians and Scientists, American Society for Clinical Pathology, American Society of Dermatopathology, College of American Pathologists, Digital Pathology Association, International Society of Dermatopathology, Phi Beta Kappa, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Christie A Riemer, MD, (NRM) Resident Physician, Department of Dermatology, Mayo Clinic School of Graduate Medical Education

Christie A Riemer, MD, (NRM) is a member of the following medical societies: American Academy of Dermatology, American Medical Student Association/Foundation

Disclosure: Nothing to disclose.

Cooper Benjamin Tye University of Texas Health Science Center at San Antonio School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous authors Elma Baron, MD; Charles R Taylor, MD; John D Wilkinson, MD, MBBS, MRCS, FRCP; John Reed, MBBS, MRCP; Sarah E Dick, MD; and Abby S Van Voorhees, MD, to the development and writing of the source articles.

Pustular Psoriasis Clinical Presentation

History

Physical Examination

Complications

References

Tables

Contributor Information and Disclosures

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