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 etiopathogenesis of this chronic pustulosis is unknown and its nosological classification remains controversial. No evidence of pathogens in the pustules. The migration of neutrophils through the epidermis indicates the presence of chemotactic factors in the upper epidermis. Tumor necrosis factor (TNF)-alpha, interleukin-8, C5a complement and IgA were detected in the subcorneal vesicles. An increase in interleukin-1b was detected in the eruption phase of the pustule. Interleukin-1b is an activator of TNF-alpha, suggesting that it may play a role in the pathogenesis of SPD. The role of TNF-alpha in the pathogenesis of the disease is supported by the response of the disease to TNF-alpha blockers such as adalimumab, and it has been suggested that neutrophil priming is a consequence of excessive production of TNF-alpha by keratinocytes. Serum levels of CCL17 (C-C motif chemokine ligand 17) are elevated, suggesting a Th2 response (Bhargava S et al. 2020).
There are associations with connective tissue diseases, thyroid diseases, medications and infections. The disease has been associated with neutrophilic dermatoses, including pyoderma gangrenosum and inflammatory bowel disease (i.e. Crohn's disease and ulcerative colitis).
Hematologic disorders: Numerous hematologic disorders, including aplastic anemia, lymphoma, multiple myeloma, chronic lymphocytic leukemia, IgG cryoglobulinemia, and monoclonal gammopathies, particularly IgA myeloma, have been associated with subcorneal pustulosis and may occur years after its onset. Therefore, screening for paraproteinemia is required to rule out myeloma. Regular screening is also recommended for patients who initially test negative. Malignancies of solid organs that have been reported include metastatic thymoma, apudoma and epidermoid carcinoma of the lung (Bhargava S et al. 2020). Associations with Sjögren's syndrome and systemic lupus erythematosus have also been reported.
Drugs: There have been reports of drugs that can cause reactions with PS-like features. This concerns multikinase inhibitors sorafenib and gefitinib. Furthermore, paclitaxel, isoniazid, amoxicillin, cefazolin sodium. The occurrence of subcorneal pustules due to dapsone and adalimumab has been reported as a therapeutic paradox.
Infections: Subcorneal pustulosis can be aggravated by various infections. Subcorneal pustulosis can be exacerbated by various infections, especially Mycoplasma pnemoniae infection, primary pulmonary coccidioidomycosis and urinary tract infections. Remarkably, the occurrence of pustules after echocardiography has been reported to develop 10 to 12 hours after the procedure (Ingber et al., 1983).
ManifestationThis section has been translated automatically.
Occurs more frequently from the age of 50.
Women are affected 4 times more frequently than men (?).
Less common in children.
LocalizationThis section has been translated automatically.
Mainly trunk and extremities, especially intertriginous areas are affected. The soles of the feet and palms, head and mucous membranes remain free.
Clinical featuresThis section has been translated automatically.
Symmetrically arranged, grouped, also anularly arranged, initially firm, as they grow larger, flaccid, "hypopyon-like" pustules, which are surrounded by a narrow inflammatory border. As the surface of the pustules is very easily damaged, they burst prematurely.
Confluence and transformation lead to the formation of circinate or polycyclic, weeping and crusty areas with collerette-like blister cover remnants.
The healing pattern with extensive erythema surrounded by a raised scaly ridge is also characteristic. The exanthema eruptions often have a craniocaudal course.
In fresh pustular eruptions, considerable feeling of illness, accompanied by a steep rise in temperature. Neutrophilic leukocytosis is always detectable.
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.
Dermatitis herpetiformis: Immunohistologic differentiation possible by detection of granular IgA deposits.
Impetigo contagiosa: Bacteriologic examination
Bullous pemphigoid: Immunohistologic differentiation possible by detection of immunoglobulin deposits on the dermo-epidermal basement membrane.
Pemphigus vulgaris: Immunohistologic differentiation by detection of immunoglobulin deposits
Pemphigus foliaceus: (minus) variant of pemphigus vulgaris with high intraepidermal (subcorneal) continuity separation and thus very thin, volatile, easily tearing blister cover. In contrast to pemphigus vulgaris, mucosal changes are always absent in pemphigus foliaceus.
Pemphigus erythematosus: Detection of IgG antibodies in the intercellular substance and on the basement membrane, up to 60% positive lupus band test.
IgA pemphigus: IgA-AK: Positive immunofluorescence on the keratinocyte surface.
Pustular psoriasis: Some authors suggest that subcorneal pustulosis is closely related to pustular psoriasis. However, typical histopathologic features of pustular psoriasis, such as psoriasiform hyperplasia, parakeratosis and spongiform pustules, are not found in subcorneal pustulosis.
AGEP: Acute generalized exanthematous pustulosis: AGEP is a sudden, self-limited eruption typically triggered by a drug or infection. The presence of systemic symptoms, eosinophilia, marked edema of the papillary dermis, keratinocyte necrosis, mixed neutrophil-rich interstitial and mid-dermal infiltrates with eosinophils in the pustules or dermis distinguishes AGEP from Sneddon-Wilkinson type subcorneal pustulosis.
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.
Note(s)This section has been translated automatically.
The entity of this clinical picture is increasingly being questioned.
LiteratureThis section has been translated automatically.
Bhargava S et al. (2020) Subcorneal pustular dermatosis: Comprehensive review and report of a case presenting during pregnancy. Int J Womens Dermatol 20:131-136
- 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
Ingber et al.(1983) Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) after a diagnostic echogram. Report of two cases. J Am Acad Dermatol 9: 393-396
- Kocak M et al. (2003) Juvenile subcorneal pustular dermatosis: a case report. Pediatr Dermatol 20: 57-59
Kreyberg I et al. (2023) Successful treatment of subcorneal pustular dermatosis targeting an underlying monoclonal IgA gammopathy. JAAD Case Rep 41:33-36.
Kundak S et al. (2017) A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease). Reumatologia 55: 323-327.
- 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
Incoming links (17)Acitretin; Betamethasone valerate emulsion hydrophilic 0,025/0,05 or 0,1 % (nrf 11.47.); CCL17; Clioquinol lotio 0.5-5%; Collerette; Deficiency of IL-1 Receptor Antagonist; Dermatitis-arthritis syndromes; Erythema necrolyticum migrans; Etanercept; Pemphigus iga pemphigus; ... Show all
Outgoing links (30)Acitretin; Acute generalized exanthematous pustulosis; 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; ... Show all
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