Skip to main content
U.S. Department of Health & Human Services
National Institutes of Health
NCATS

COVID-19 is an emerging, rapidly evolving situation.

Get the latest public health information from CDC: https://www.coronavirus.gov (link is external)
Get the latest research information from NIH: https://www.nih.gov/coronavirus (link is external)

National Center for Advancing and Translational Sciences National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences
1-888-205-2311
Menu Search
1-888-205-2311
Menu
  • Home
  • Diseases expand submenu for Diseases
    • Browse A-Z
    • Find Diseases By Category expand submenu for Find Diseases By Category
      • Autoimmune / Autoinflammatory diseases
      • Bacterial infections
      • Behavioral and mental disorders
      • Blood Diseases
      • Chromosome Disorders
      • Congenital and Genetic Diseases
      • Connective tissue diseases
      • Digestive Diseases
      • Ear, Nose, and Throat Diseases
      • Endocrine Diseases
      • Environmental Diseases
      • Eye diseases
      • Female Reproductive Diseases
      • Fungal infections
      • Heart Diseases
      • Hereditary Cancer Syndromes
      • Immune System Diseases
      • Kidney and Urinary Diseases
      • Lung Diseases
      • Male Reproductive Diseases
      • Metabolic disorders
      • Mouth Diseases
      • Musculoskeletal Diseases
      • Myelodysplastic Syndromes
      • Nervous System Diseases
      • Newborn Screening
      • Nutritional diseases
      • Parasitic diseases
      • Rare Cancers
      • RDCRN
      • Skin Diseases
      • Viral infections
    • List of FDA Orphan Drugs
    • GARD Information Navigator
    • FAQs About Rare Diseases
  • Guides expand submenu for Guides
    • Patients, Families and Friends expand submenu for Patients, Families and Friends
      • How to Find a Disease Specialist
      • Tips for the Undiagnosed
      • Support for Patients and Families
      • Tips for Finding Financial Aid
      • Help with Travel Costs
      • How to Get Involved in Research
      • FAQs About Chromosome Disorders
      • Medical and Science Glossaries
    • Healthcare Professionals expand submenu for Healthcare Professionals
      • Caring for Your Patient with a Rare Disease
      • ICD Coding for Rare Diseases
      • FindZebra Diagnosis Assist Tool
    • Researchers expand submenu for Researchers
      • Finding Funding Opportunities
    • Teachers and Students expand submenu for Teachers and Students
      • Teaching Resources
  • News expand submenu for News
    • In The Spotlight
    • News Archive
    • Connect with GARD
    • Media Requests
  • About GARD expand submenu for About GARD
    • Contact GARD
    • About GARD
    • Videos
    • Brochures and Publications
  • En Español expand submenu for En Español
    • Enfermedades
    • Contacte GARD
    • Guías de Información expand submenu for Guías de Información
      • Preguntas Más Frecuentes Sobre Enfermedades Raras
      • Como Encontrar un Especialista en su Enfermedad
      • Consejos Para una Condición no Diagnosticada
      • Consejos Para Pacientes y Familias
      • Consejos Para Obtener Ayuda Financiera Para Una Enfermedad
      • Participe en Estudios de Investigación
      • Preguntas Más Frecuentes Sobre los Trastornos Cromosómicos
    • Sobre GARD
  • Home
© Positive Exposure
banner showing people with disabilities and/or their relatives
  1. Home
  2. Diseases
  3. Subcorneal pustular dermatosis
print
  • Table of Contents expand submenu for Table of Contents
    • Summary
    • Symptoms
    • Cause
    • Diagnosis
    • Treatment
    • Prognosis
    • Statistics
    • Related Diseases
    • Research
    • Organizations
    • Learn More
    • GARD Answers
    • References
  • Browse A-Z
  • Find Diseases By Category expand submenu for Find Diseases By Category
    • Autoimmune / Autoinflammatory diseases
    • Bacterial infections
    • Behavioral and mental disorders
    • Blood Diseases
    • Chromosome Disorders
    • Congenital and Genetic Diseases
    • Connective tissue diseases
    • Digestive Diseases
    • Ear, Nose, and Throat Diseases
    • Endocrine Diseases
    • Environmental Diseases
    • Eye diseases
    • Female Reproductive Diseases
    • Fungal infections
    • Heart Diseases
    • Hereditary Cancer Syndromes
    • Immune System Diseases
    • Kidney and Urinary Diseases
    • Lung Diseases
    • Male Reproductive Diseases
    • Metabolic disorders
    • Mouth Diseases
    • Musculoskeletal Diseases
    • Myelodysplastic Syndromes
    • Nervous System Diseases
    • Newborn Screening
    • Nutritional diseases
    • Parasitic diseases
    • Rare Cancers
    • RDCRN
    • Skin Diseases
    • Viral infections
  • List of FDA Orphan Drugs
  • GARD Information Navigator
  • FAQs About Rare Diseases
You can help advance
rare disease research!
You can help advance rare disease research!
toolbox
Find out how with the NCATS Toolkit.

Subcorneal pustular dermatosis


Title


How can we make GARD better? We want to hear from you.
Provide Feedback

Other Names:
Sneddon-Wilkinson disease; Sneddon Wilkinson disease; Pustulosis subcornealis; Sneddon-Wilkinson disease; Sneddon Wilkinson disease; Pustulosis subcornealis; Subcorneal pustular dermatitis See More
Categories:
Skin Diseases

Summary Summary


Listen
Subcorneal pustular dermatosis (SPD) is a rare skin disease in which pus-filled pimples or blisters (pustules) form under the top (subcorneal) layer of the skin.[1][2][3][4][5] It is most common in middle-aged adults (particularly women) but can develop in children.[2][5] Pustules usually appear over a few hours and grow together to form round or wavy patterns.[2][3] They most often form in areas where the skin may touch or rub together, such as the groin area, underarms, inside the elbows, and behind the knees.[2][3] The pustules may be mildly itchy or painful, but despite being pus filled, are not infected.[2] The diagnosis of SPD is made based on the appearance of the pustules and the  results of a skin biopsy (histologic findings).[2][3] The cause of SPD is not known.[2] There is currently no evidence it is inherited (no familial cases have been reported) and it is not contagious.[1][2] SPD may be associated with other diseases or health problems including several autoimmune diseases, blood (hematologic) diseases, infections, and cancers. Rarely, it has been associated with taking certain medications (drug-induced SPD).[2] SPD may be treated with is oral dapsone, which often improves symptoms within one month. However, the pustules may return when treatment is stopped.[2][4] Other therapies have been tried with mixed results.[2] While SPD may cause discomfort and cosmetic concerns, it typically does not affect overall health.[3]
Last updated: 9/17/2018

Symptoms Symptoms


Listen
The main symptom of subcorneal pustular dermatosis (SPD) is an outbreak of round bumps on the skin that may resemble pus-filled pimples or blisters (pustules).[1][2] The pustules appear over a short period of time (hours), usually on healthy skin, but sometimes on skin that is irritated or inflamed.[2][4] Most commonly the outbreak appears in areas of the body where skin may touch or rub together through movement, including the groin area, under women's breasts, underarms, inside the elbows and behind the knees. Other areas of the trunk, the upper arms and upper legs may be affected. [2][3][4] Pustules rarely form on the palms of the hands or soles of the feet, and the face is almost never affected.[2][4] The pustules initially appear separated from each other (isolated) or be grouped together in small clusters, but may spread and join together within one or two days, forming circular, ring-shaped (faded in the center), or wavy patterns on the skin.[1][2][3][5] The pustules have an unusual appearance because the pus tends to settle in the lower half of the blister leaving the upper half of the blister clear (known as hypopyon pustules).[1][2] 

The pustules sometimes cause irritation, pain, or mild itching.[2][3] They break open easily and crust over before healing, sometimes leaving behind darkened spots on the skin (hyperpigmentation). The pustules typically do not cause scarring.[2][4] SPD usually affects the skin only, but some people may have generalized (systemic) symptoms such as a general feeling of being sick (malaise), fever, or joint pain.[2] Additional symptoms may suggest other associated diseases or health problems may be present. [2][3][4]

Last updated: 9/17/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 10 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Erythema 0010783
Hyperpigmentation of the skin
Patchy darkened skin
0000953
Pustule
Pimple
0200039
30%-79% of people have these symptoms
Pruritus
Itching
Itchy skin
Skin itching
[ more ]
0000989
5%-29% of people have these symptoms
Hyperthyroidism
Overactive thyroid
0000836
Hypothyroidism
Underactive thyroid
0000821
Increased circulating antibody level 0010702
Multiple myeloma 0006775
Rheumatoid arthritis 0001370
Systemic lupus erythematosus 0002725
Showing of 10 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 1/1/2021

Cause Cause


Listen
The reason that a person develops subcorneal pustular dermatosis (SPD) is not known.[2] The pustules are thought to result from a signal pulling (recruiting) neutrophils to the affected areas of skin. Neutrophils are a type of white blood cell (WBC). Skin disorders that involve an abnormal recruitment of neutrophils are called neutrophilic dermatoses (NDs). While there are known triggers of neutrophil recruitment for some NDS, no specific triggers have been found to cause the pustule formation in SPD. An exception is rare cases associated with the use of certain medications.[2][5]

SPD has been associated in case reports with a number of diseases that affect other parts or systems of the body, including but not limited to:[2][3][4]

• Autoimmune diseases such as rheumatoid arthritis, lupus, and Sjogren syndrome.
• Hematologic diseases or cancers such as monoclonal gammopathies and multiple myeloma.
• Infections such as urinary tract infections and respiratory tract infections.

Rarely, SPD has developed in association with taking certain medications (drug-induced SPD).[2]

Last updated: 9/17/2018

Diagnosis Diagnosis


Listen
There are currently no official diagnostic criteria for subcorneal pustular dermatosis (SPD). The disease is diagnosed based on the appearance of the pustules,  a skin biopsy which identifies certain characteristics when examined under the microscope (histologic findings), and ruling out other diseases that cause similar pustules.[3] There are no blood tests that can confirm a diagnosis of SPD.[3] Pustular psoriasis can be difficult to distinguish from SPD, and some people initially diagnosed with SPD have later been found to have pustular psoriasis.[3] Identifying a family history of psoriasis may influence whether one or the other is diagnosed.[5] Some cases of SPD have been considered a variant of pustular psoriasis.[5] At this time, the exact relationship between SPD and pustular psoriasis is not understood.[2][3][4][5]
Last updated: 9/17/2018

Treatment Treatment


Listen
The main goals of treatment for subcorneal pustular dermatosis (SPD) are to minimize discomfort and improve quality of life.[3] Treatment usually begins with oral dapsone, which often clears the pustules within one month.[2][3] Although there have not been randomized clinical trials studying its use for SPD, clinical experience has suggested oral dapsone is effective and well-tolerated.[3] When treatment with dapsone is stopped, the pustules may return, so continued therapy at a lower dose may be recommended to prevent recurrence.[2] Other treatments have been tried with variable success, and data on their use are limited to reports of individual cases. These treatments are generally only tried when dapsone is not effective or not well-tolerated.[3] Possible treatment options include topical or oral corticosteroids, oral retinoids, phototherapy, immunosuppressive oral therapies, and biologic agents.[2][3] In some cases, a combination of therapies may be used. Since SPD does not cause serious health issues, it is important to consider the possible side effects of treatment options.[3]
Last updated: 9/17/2018

Prognosis Prognosis


Listen
Subcorneal pustular dermatosis (SPD) generally is a long-lasting (chronic) disease where the pustules go away for a while and then come back. In medical terms, this is called a relapsing and remitting course.[3] Some people do have long-term or permanent remission of SPD. The course of the disease in each person cannot be predicted.[3] While SPD can cause discomfort, itching, and concerns about appearance, overall health is typically not affected. In people with SPD who have associated diseases, the long-term outlook may depend upon the other disease present.[3][5]
Last updated: 9/17/2018

Statistics Statistics


Listen
The number of people who have subcorneal pustular dermatosis (SPD) is not known. Based on the limited number of cases that have been reported, it is considered a rare disease.[1][2][3][4][5] In the United States, a rare disease is defined as one that affects fewer than 200,000 people.[6] SPD most commonly develops in people between 40 and 70 years of age. It is four times more common in women than in men and is extremely rare in children.[2] There are no ethnic or geographical factors known to increase a person’s chance of having SPD.[3]
Last updated: 9/17/2018
Do you have updated information on this disease? We want to hear from you.

Related Diseases Related Diseases


Listen

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis includes the subcorneal-type of IgA pemphigus, pemphigus foliaceus, dermatitis herpetiformis, pustular psoriasis, generalized pustulosis, generalized exanthematous pustulosis and bacterial impetigo (see these terms).
Visit the Orphanet disease page for more information.

Research Research


Listen

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Subcorneal pustular dermatosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations


Listen

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Social Networking Websites

  • Visit the following Facebook groups related to Subcorneal pustular dermatosis:
    Sneddon Wilkinson's Disease

Organizations Providing General Support

  • Skin Support
    Website: http://www.skinsupport.org.uk/
Do you know of an organization? We want to hear from you.

Learn More Learn More


Listen

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The British Association of Dermatologists has a patient information leaflet about Subcorneal pustular dermatosis.
  • DermNet New Zealand is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

GARD Answers GARD Answers


Listen

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease). British Association of Dermatologists. January, 2017; http://www.bad.org.uk/shared/get-file.ashx?id=236&itemtype=document.
  2. Watts PJ, Khachemoune A. Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress. Am J Clin Dermatol. December, 2016; 17(6):653-671. https://www.ncbi.nlm.nih.gov/pubmed/27349653.
  3. Kourosh AS. Subcorneal pustular dermatosis. UpToDate. August 21, 2018; https://www.uptodate.com/contents/subcorneal-pustular-dermatosis.
  4. Cheng S, Edmonds E, Ben-Gashir M, Yu RC. Subcorneal pustular dermatosis: 50 years on. Clin Exp Dermatol. May 2008; 33(3):229-233. https://www.ncbi.nlm.nih.gov/pubmed/18355359.
  5. Sekulovic LK. Subcorneal Pustular Dermatosis. Medscape Reference. Sept 22, 2016; https://emedicine.medscape.com/article/1124252-overview.
  6. H.R. 4013 - 107th Congress: Rare Diseases Act of 2002. GovTrack.us. https://www.govtrack.us/congress/bills/107/hr4013. Accessed 9/12/2018.
Do you know of a review article? We want to hear from you.
You can help advance
rare disease research!
You can help advance rare disease research!
toolbox
Find out how with the NCATS Toolkit.

Share this content:

Share this content:

twitter-icon facebook-icon contact-icon link-icons

Copy Link

Link copied to your clipboard.

printer-icon

You May Be Interested In

How to Find a Disease Specialist

How to Find a Disease Specialist

Tips for the Undiagnosed

Tips for the Undiagnosed

Support for Patients and Families

Support for Patients and Families

Tips for Finding Financial Aid

Tips for Finding Financial Aid

Help with Travel Costs

Help with Travel Costs

How to Get Involved in Research

How to Get Involved in Research

FAQs About Chromosome Disorders

FAQs About Chromosome Disorders

Medical and Science Glossaries

Medical and Science Glossaries

Caring for Your Patient with a Rare Disease

Caring for Your Patient with a Rare Disease

ICD Coding for Rare Diseases

ICD Coding for Rare Diseases

FindZebra Diagnosis Assist Tool

FindZebra Diagnosis Assist Tool

Finding Funding Opportunities

Finding Funding Opportunities

Teaching Resources

Teaching Resources

placeholder for the horizontal scroll slider Back to top
GARD Home NCATS Home Site Map Browse Glossary A-Z Privacy Notice Disclaimer Accessibility FOIA OIG

If you have problems viewing PDF files, download the latest version of Adobe Reader

For language access assistance, contact the NCATS Public Information Officer

Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311

contact gard Office of Rare Disease Research Facebook Page Office of Rare Disease Research on Twitter
Listen