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Sneddon-Wilkinson subcorneal pustulosis - Skin
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Sneddon-Wilkinson subcorneal pustulosis - Skin

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Contributors: Alexis Perkins MD, Nikki Levin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Sneddon-Wilkinson disease, or subcorneal pustular dermatosis (SPD), is a rare, recurrent dermatosis characterized by crops of sterile subcorneal vesicles or pustules occurring in an annular configuration on normal to slightly erythematous flexural skin. In some cases, it is associated with an immunoglobulin A (IgA) paraproteinemia and intraepidermal deposits of IgA directed against desmocollin 1, and therefore considered to be a variant of IgA pemphigus. It is distinguished from pustular psoriasis by the presence of subcorneal pustules in the absence of spongiform pustules and epidermal changes of psoriasis, and by the notable response to dapsone.

The etiology is unknown. However, SPD sometimes occurs in association with IgA paraproteinemia, IgA myeloma, pyoderma gangrenosum, ulcerative colitis, and Crohn's disease.

There is no racial or ethnic predilection, but the disease is more common in women and in those over the age of 40.

Lesions tend to be asymptomatic, but patients may report itching or burning. Although the disorder's presentation may look severe, it is a benign condition.

SPD usually presents as crops of small, flaccid pustulo-vesicles that coalesce into annular or serpiginous configurations on previously normal skin. The lesions most commonly occur symmetrically on the flexural extremities, as well as the axillae, groin, abdomen, and inframammary skin. The pustules dry up after a few days, leaving scaling and crusts that may resemble impetigo. This is followed by new crops of vesicles or pustules developing at the periphery of older lesions, leaving annular or polycyclic lesions. The lesions resolve without scarring but may leave post-inflammatory hyperpigmentation. This cycle may repeat itself a few days or weeks later if the patient is not treated.

Codes

ICD10CM:
L13.1 – Subcorneal pustular dermatitis

SNOMEDCT:
25147002 – Subcorneal pustular dermatosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 01/23/2015
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Sneddon-Wilkinson subcorneal pustulosis - Skin
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Sneddon-Wilkinson subcorneal pustulosis : Bullae, Primarily truncal, Serpiginous configuration, Widespread, Vesicles
Clinical image of Sneddon-Wilkinson subcorneal pustulosis
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