June 2001

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3.

DIAGNOSIS: Subcorneal Pustular Dermatosis (Sneddon-Wilkinson Disease)

HISTOPATHOLOGY: A skin biopsy showed subcorneal pustules filled with neutrophils. A mononuclear cell infiltrate was present in the papillary dermis. Direct and indirect immunofluorescence were negative.

DISCUSSION: The cause of subcorneal pustular dermatosis is unknown. Associations have been made between subcorneal pustular dermatosis and several other conditions, including multiple myeloma, pyoderma gangrenosum, and inflammatory bowel disease. The eruption tends to occur symmetrically involving mostly intertriginous areas and flexor surfaces of the extremities. The main differential diagnoses include pemphigus foliaceous, glucagonoma syndrome, dermatitis herpetiformis and impetigo. The drug of choice is dapsone at a dose between 50mg and 150mg per day. Our patient cleared with 100mg per day of dapsone. She is now able to control infrequent flares with 25 mg of dapsone daily for 1-2 weeks.

REFERENCES:

I. Hönigsmann H, Trautinger F, Wolff K: Fitzpatrick's Dermatology in General Medicine, Fifth Edition. p. 719-722

2. Niimi Y, Kawana S, Kusuncki T. : IgA Pemphigus: a case report and its characteristic clinical features compared with subcorneal pustular dermatosis." J Am Acad Dermatol, 2000, 43, 546-9.

3. Stone MS, Lyckholm LJ: Pyoderma Gangrenosum and Subcorneal Pustular Dermatosis: clues to underlying immunoglobulin A myeloma. Am J Med,1996,100, 663-4.

My thanks to Dr. Jessica Mehta for assisting in the presentation of this case.

My thanks to Dr. Elsa Gutierrez for assisting in the presentation of this case.