Pustulose subcorneal L13.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 08.08.2022

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Synonym(s)

Sneddon-Wilkinson disease; Sneddon-Wilkinson Syndrome; subcorneal pustular dermatosis; subcorneal pustulose; subcorneal pustulosis

History
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Sneddon and Wilkinson, 1956

Definition
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Rare, chronic, recurrent disease with formation of strictly subcorneal, sterile pustules.

Etiopathogenesis
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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.

Manifestation
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Occurs more frequently after the age of 50.

Women are affected 4 times more often than men (?).

Rare in children.

Localization
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Mainly trunk, especially intertriginous areas are affected. Soles and palms, head and mucous membranes remain free.

Clinical features
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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.

Laboratory
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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.

Histology
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In clinically fully-developed pustular lesions there is a large, usually single-chambered, intraepithelial macropustule, the upper covering of which is formed in sections or exclusively by the stratum corneum (subcorneal pustule). The content of the pustule consists almost exclusively of neutrophilic granulocytes mixed with a few apoptotic keratinocytes. The epithelial pustular wall is only formed by a thin epidermal seam, which is spongiform and interspersed with neutrophil granulocytes. Dense inflammatory, predominantly neutrophilic infiltration of the underlying dermis.

External therapy
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Drying measures with lotio alba and addition of 3-5% clioquinol R050, if necessary with glucocorticoids such as betamethasone emulsion/cream R030 or triamcinolone cream R259.

Radiation therapy
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Therapeutic successes with local PUVA therapy, also in combination with acitretin (RePUVA) are described.

Internal therapy
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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)
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The entity of this clinical picture is increasingly being questioned.

Literature
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  1. 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
  2. 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
  3. Kocak M et al (2003) Juvenile subcorneal pustular dermatosis: a case report. Pediatr Dermatol 20: 57-59
  4. Nagai H, Harada S. (2002) Subcorneal pustular dermatosis accompanied by seronegative arthritis. Acta Derm Venereol 82: 318-319
  5. Naretto C et al (2009) The case of SLE associated Sneddon-Wilkinson pustular disease successfully and safely treated with infliximab. Lupus 18:856-857
  6. 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.
  7. Ratnarathorn M et al. (2008) Subcorneal pustular dermatosis (Sneddon-Wilkinson
  8. disease) occurring in association with nodal marginal zone lymphoma: a case
  9. report. Dermatol Online J 15:6.
  10. Reed J, Wilkinson J (2000) Subcorneal pustular dermatosis. Clin Dermatol 18: 301-313
  11. Sandhu K et al (2003) Inverse subcorneal pustular dermatosis. J Eur Acad Dermatol Venereol 17: 348-349.
  12. 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.
  13. Sneddon I, Wilkinson D (1956) Subcorneal pustular dermatosis. Br J Dermatol 68: 385-394.
  14. 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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Last updated on: 08.08.2022