Pustulose subcorneal L13.1

Sneddon and Wilkinson, 1956

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

The pathogenesis is unknown. No evidence of pathogens in the pustules. Associations with IgA or IgG gammopathy were observed. Further coincidences with ulcerative colitis, pyoderma gangraenosum, marginal cell lymphoma and systemic lupus erythematosus have been reported.

Occurs more frequently after the age of 50.

Women are 4 times more frequently affected than men.

Rarely in children.

Mainly stem, especially intertriginous areas and head are affected. The soles of the feet and palms of the hands and mucous membranes remain free.

Symmetrically arranged, grouped, also anularly arranged, initially tight pustules which become flabby as they grow larger, surrounded by a narrow inflammatory border. As the cover of the pustules is very vulnerable, they burst prematurely.

Confluence and transformation lead to the formation of circulatory or polycyclic, weeping and crust-covered areas with collerette-like bladder cover remnants. Also typical is the healing pattern with extensive erythema surrounded by a marginal scarlatinous scaling border. Often craniocaudal sequence of the exanthema eruption.

In case of fresh pustule, considerable feeling of illness, accompanied by a steep rise in fever. Always detectable neutrophil leukocytosis.

• Increase of SPA and CRP.
• Mostly pronounced neutrophilic leukocytosis.
• Pustular smear: Numerous neutrophils, rarely eosinophils.
• Serum protein electrophoresis: paraproteinemia in up to 40% of cases. Often monoclonal IgA gammopathy or IgG gammopathy.

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.

Dermatitis herpetiformis; impetigo contagiosa; psoriasis pustulosa generalisata; bullous pemphigoid; pemphigus vulgaris; pemphigus foliaceus; pemphigus erythematosus; vulgar eczema.

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.

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

• DADPS (e.g. Dapson-Fatol) 100-150 mg/day, but only a morbostatic effect can be achieved Experimentally aromatic retinoids such as acitretin (neotigason) 0.5-1.0 mg/kg bw/day or isotretinoin (e.g. isotretinoin-ratiopharm; acne normin) 20-40 mg/day.
• In therapy-resistant cases, methotrexate therapy(e.g. MTX) at 5-15 mg/week should be considered.
• Alternatively, an experiment with fumaric acid esters (Fumaderm) can be undertaken.
• Alternative: In individual cases the TNF-alpha inhibitors Infliximab and Etanercept were successful.
• Alternative: In further individual cases, therapy successes with Ciclosporin A, Mycophenolatmofetil, Adalimumab were reported.

The entity of this clinical picture is increasingly being questioned.

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. Pediatric 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-Wilkinsondisease
   ) occurring in association with nodal marginal zone lymphoma: a casereport
   . Dermatol Online J 15:6.
   
   
8. Reed J, Wilkinson J (2000) Subcorneal pustular dermatosis. Clin Dermatol 18: 301-313

9. Sandhu K et al (2003) Inverse subcorneal pustular dermatosis. J Eur Acad Dermatol Venereol 17: 348-349

10. 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.
11. Sneddon I, Wilkinson D (1956) Subcorneal pustular dermatosis. Br J Dermatol 68: 385-394