[Sneddon-Wilkinson disease. Four cases report].
BACKGROUND
We report four cases of subcorneal pustular dermatosis or Sneddon-Wilkinson disease. Clinical and histological lesions and immunofluorescence data were presented. This disease is classified among neutrophilic dermatitis.


METHODS
All of four patients presented with clinical and histological lesions compatible with the diagnosis of Sneddon-Wilkinson disease. Indeed, direct and indirect immuno-testing were negative. We noted an association with a benign IgA monoclonal gammapathy in one case and with a seronegative polyarthritis in one other case. Three patients correctly responded to dapsone. One of them after transient improvement was resistant to dapsone and then dramatically responded to etretinate.


CONCLUSIONS
Subcorneal pustular dermatosis is a chronic disease, rarely described in literature. It's a pustular eruption, involving the trunck, axillae and inguinal holds. It's often associated with monoclonal gammapathy, particulary IgA. Its nosological situation is still contested, especially with IgA pemphigus sharing with it the association with IgA monoclonal gammapathy and the same efficacy of dapsone. We discuss relationships between both diseases.
Launay
F
F
Service de dermatologie, CHU Purpan, Toulouse, France.
Albès
B
B
Bayle
P
P
Carrière
M
M
Lamant
L
L
Bazex
J
J
D000368:Aged; D000369:Aged, 80 and over; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D005050:Etretinate / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007641:Keratolytic Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D007070:Immunoglobulin A; D007641:Keratolytic Agents; D005050:Etretinate; D003622:Dapsone