Synonym(s)
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Rare, chronic, recurrent disease with formation of strictly subcorneal, sterile pustules.
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EtiopathogenesisThis section has been translated automatically.
The etopathogenesis of this chronic pustulosis is unknown. No evidence of pathogens in the pustules.
Associations with IgA or IgG gammopathy have been observed.
Other coincidences with ulcerative colitis, pyoderma gangraenosum, marginal cell lymphoma, and systemic lupus erythematosus have been reported.
ManifestationThis section has been translated automatically.
Occurs more frequently after the age of 50.
Women are affected 4 times more often than men (?).
Rare in children.
LocalizationThis section has been translated automatically.
Mainly trunk, especially intertriginous areas are affected. Soles and palms, head and mucous membranes remain free.
Clinical featuresThis section has been translated automatically.
Symmetrically arranged, grouped, also anularly arranged, initially tight, as they grow larger, flaccid, hypopyon-like pustules surrounded by a narrow inflammatory fringe. Since the cover of the pustules is very vulnerable, they burst early.
Confluence and transformation result in the formation of circular or even polycyclic, weeping and crust-covered areas with collerette-like blister cover remnants. The healing pattern is also typical, with planar erythema surrounded by a marginal fringe of scales. Frequent craniocaudal course of exanthema eruptions.
In fresh pustular eruptions, considerable feeling of illness accompanied by a steep rise in fever. Neutrophilic leukocytosis is always present.
LaboratoryThis section has been translated automatically.
Elevation of ESR and CRP.
Mostly pronounced neutrophilic leukocytosis.
Pustular smear: Numerous neutrophils, rarely eosinophils.
Serum protein electrophoresis: Paraproteinemia in up to 40% of cases. Frequent monoclonal IgA gammopathy or IgG gammopathy.
HistologyThis section has been translated automatically.
Differential diagnosisThis section has been translated automatically.
External therapyThis section has been translated automatically.
Radiation therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
DADPS (e.g., dapsone-fatol) 100-150 mg/day, but only morbostatic effect can be achieved. Trial aromatic retinoids such as acitretin (Neotigasone) 0.5-1.0 mg/kg bw/day or isotretinoin (e.g., isotretinoin-ratiopharm; Acnenormin) 20-40 mg/day.
For refractory cases, consider methotrexate therapy(e.g., MTX) 5-15 mg/week.
Alternatively, a trial of fumaric acid esters (Fumaderm) may be undertaken.
Alternative: In single cases, the TNF-alpha inhibitors infliximab and etanercept have been successful.
Alternative: In other individual cases, therapeutic success with ciclosporin A, mycophenolate mofetil, adalimumab has been reported.
Note(s)This section has been translated automatically.
The entity of this clinical picture is increasingly being questioned.
LiteratureThis section has been translated automatically.
- Canpolat F et al (2010) A case of subcorneal pustular dermatosis in association with monoclonal IgA gammopathy successfully treated with acitretin. J Dermatolog Treat 21:114-116
- Dallot A et al (1988) Subcorneal pustular dermatosis (Sneddon-Wilkonson disease) with amicrobial lymph node suppuration and aseptic spleen abscesses. Brit J Dermatol 119: 803-807
- Kocak M et al (2003) Juvenile subcorneal pustular dermatosis: a case report. Pediatr Dermatol 20: 57-59
- Nagai H, Harada S. (2002) Subcorneal pustular dermatosis accompanied by seronegative arthritis. Acta Derm Venereol 82: 318-319
- Naretto C et al (2009) The case of SLE associated Sneddon-Wilkinson pustular disease successfully and safely treated with infliximab. Lupus 18:856-857
- Rasch A et al (2009) Subcorneal pustulosis with combined lack of IgG/IgM and monoclonal gammopathy type IgA/Kappa. J Dtsch Dermatol Ges 7:693-696.
- Ratnarathorn M et al. (2008) Subcorneal pustular dermatosis (Sneddon-Wilkinson
- disease) occurring in association with nodal marginal zone lymphoma: a case
- report. Dermatol Online J 15:6.
- Reed J, Wilkinson J (2000) Subcorneal pustular dermatosis. Clin Dermatol 18: 301-313
- Sandhu K et al (2003) Inverse subcorneal pustular dermatosis. J Eur Acad Dermatol Venereol 17: 348-349.
- Scalvenzi M et al (2013) Subcorneal pustular dermatosis in childhood: a case report and review of the literature. Case Rep Dermatol Med doi: 10.1155/2013/424797.
- Sneddon I, Wilkinson D (1956) Subcorneal pustular dermatosis. Br J Dermatol 68: 385-394.
Young PA et al (2021) Subcorneal pustular dermatosis associated with IgG monoclonal gammopathy of undetermined significance. Dermatol Online J 27:13030/qt71k801d3
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Incoming links (16)
Acitretin; Betamethasone valerate emulsion hydrophilic 0,025/0,05 or 0,1 % (nrf 11.47.); Clioquinol lotio 0.5-5%; Collerette; Deficiency of IL-1 Receptor Antagonist; DIRA; Dermatitis-arthritis syndromes; Erythema necrolyticum migrans; Etanercept; Pemphigus iga pemphigus; Pustular psoriasis; ... Show allOutgoing links (32)
Acitretin; Adalimumab; Betamethasone; Betamethasone valerate emulsion hydrophilic 0,025/0,05 or 0,1 % (nrf 11.47.); Ciclosporin a; Clioquinol; Clioquinol lotio 0.5-5%; Contagious impetigo; Dadps; Dermatitis herpetiformis; ... Show allDisclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer
Dr. med. Lucian Cajacob
