Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with absence of desmoglein 1 and 3 antibodies: case report and literature review.
Subcorneal pustular dermatosis (SPD) [Sneddon-Wilkinson disease] is a benign and uncommon disorder characterized by a chronic, relapsing vesiculopustular eruption of unknown etiology. We present a case of SPD in a young Black woman in whom ELISA was performed to test for desmoglein 1 and 3 antigens (the first reported case of evaluation for these antigens in a patient with SPD). The test revealed the absence of both antibodies. The patient was successfully treated with topical corticosteroids and narrow-band UVB phototherapy. In this report, we review both the pathophysiology of SPD, which has yet to be clarified, and its treatment. Data obtained from our case report add further support to the hypothesis that a non-antibody-mediated mechanism is operative in SPD. The treatment of choice for SPD is dapsone. However, the combination of corticosteroids and UVB phototherapy should be considered a valid therapeutic option in patients who are not appropriate candidates for dapsone therapy.
Bordignon
Matteo
M
Unit of Dermatology, University of Padua, Padua, Italy.
Zattra
Edoardo
E
Montesco
Maria Cristina
MC
Alaibac
Mauro
M
D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D003622:Dapsone / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D051183:Desmoglein 1 / Q000276:immunology*; D051184:Desmoglein 3 / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D010789:Phototherapy; D012867:Skin / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology
D001323:Autoantibodies; C071894:DSG3 protein, human; D003879:Dermatologic Agents; D051183:Desmoglein 1; D051184:Desmoglein 3; D005938:Glucocorticoids; D003622:Dapsone