Disease directory
Subcorneal pustular dermatosis (Sneddon-Wilkinson)
Published: September 18, 2026Updated: September 18, 2026
Important: Informational content intended for healthcare professionals. It does not replace clinical evaluation or establish a doctor-patient relationship.
Overview
- Definition and synonyms
- Subcorneal pustular dermatosis (SPD) = Sneddon-Wilkinson disease .
- Chronic and relapsing neutrophilic dermatosis, with sterile, flaccid, subcorneal pustules , affecting mainly the trunk and flexural areas, in patients generally in good general condition.
- It is considered a sterile neutrophilic dermatosis, distinct from but overlapping with pustular psoriasis and IgA-pemphigus type DPS. Cheng 2008 , Watts 2016 , Bhargava 2020 .
- Synonyms:
- Sneddon-Wilkinson disease
- Subcorneal pustular dermatosis
- neutrophilic subcorneal pustulosis
- History: First described as a clinical entity by Sneddon and Wilkinson in 1956 , based on a series of patients with sterile subcorneal pustules. Cheng 2008 , Watts 2016 .
- Epidemiology
- Very rare disease ; the estimated prevalence is <1/1,000,000 according to rare disease databases and reviews; most publications are case reports and small series. Watts 2016 , Bhargava 2020 .
- It is more prevalent in women (female:male ratio ≈ 3–4:1). Cheng 2008 , Watts 2016 .
- Peak onset in middle age (4th–6th decade), although there are exceptional cases in the elderly and pediatric cases . Bhargava 2020 , Alhafi 2021 .
- It can be associated with:
- IgA monoclonal gammopathy / IgA multiple myeloma . Kasha 1988 , Wallach 1982 , von dem Borne 2017 , Cheng 2008 , Watts 2016 .
- Other autoimmune/inflammatory diseases (rheumatoid arthritis, inflammatory bowel disease, pyoderma gangrenosum) described in series and case reports. Cheng 2008 , Watts 2016 , Bhargava 2020 .
- ICD Codes
- ICD-10: L13.1 – “Subcorneal pustular dermatitis (includes Sneddon-Wilkinson disease)” (WHO classification).
- ICD-11: The disease is listed as a specific entity within the group of neutrophilic dermatoses / sterile pustular dermatoses , with its own code of “Sneddon-Wilkinson disease” in the WHO/Orphanet rare disease database (EB2Y).
- Diagnostic checklist (quick)
- Essential clinic
- Superficial flaccid pustules (hypopyon) on trunk and folds.
- Pattern in rings/polycyclic figures, chronic-relapsing course.
- Patient in good general condition, without marked fever or systemic toxicity.
- Histology required
- Biopsy of recent pustule with large neutrophilic subcorneal pustule.
- Absence of typical psoriasiform hyperplasia and frank vasculitis.
- Special stains negative for significant fungi and bacteria.
- Immunology / Differentials
- IFD performed if there is doubt about IgA pemphigus / other bullous pemphigus:
- Intercellular IgA → suggests DPS-type IgA pemphigus.
- Negative or non-specific → favors classic DPS.
- IFD performed if there is doubt about IgA pemphigus / other bullous pemphigus:
- Minimal laboratory
- Blood count, liver and kidney function tests.
- Serum protein electrophoresis + immunofixation in all adults (rule out monoclonal IgA).
- Before dapsone: G6PD , blood count + reticulocytes, liver and kidney tests.
- Biopsy / Logistics
- Punch 4–5 mm of new pustule for H&E.
- Additional perilesional punch for IFD (if autoimmune bullous disease is suspected).
- Essential clinic
Doctors who treat Subcorneal pustular dermatosis (Sneddon-Wilkinson)
Directory specialists related to Subcorneal pustular dermatosis (Sneddon-Wilkinson).
There are no specialists associated with this record yet
You can browse the full directory and filter by the condition you're interested in.
Go to directory