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subcorneal pustular dermatosis (Sneddon-Wilkinson disease)
Etiology:
- may represent a form of pustular psoriasis
- no etiologic pathogen has been identified
Epidemiology:
- rare
- most commonly affects women > 40 years of age
Pathology:
- benign but chronic disorder
- direct & indirect immunofluorescence staining of skin biopsy is commonly negative
- a subgroup may have IgA deposition against desmocollin 1
- accumulation of neutrophils in the subcorneal layer suggests the presence of chemoattractants in the uppermost epidermis
Clinical manifestations:
- chronic relapsing sterile pustular eruption
- primary lesions are flaccid pustules, several millimeters in diameter, on normal or mildly erythematous skin
- classic lesion is a '1/2-1/2' blister, in which purulent fluid accumulates in the lower 1/2 of the blister
- pustules may be grouped or isolated; they tend to coalesce
- pustules are superficial & rupture easily, resulting in a superficial crust
- mild hyperpigmentation often remains after pustular lesions have resolved
- most commonly affected areas include the axillae, groin, neck, & submammary region
- proximal flexural aspects of the extremities may be affected
- palmar, plantar, face & mucous membrane involvement is unusual
Laboratory:
- serum protein electrophoresis: monoclonal gammopathy in 40%
- skin culture for bacteria, fungi
- skin scraping, KOH mount
Differential diagnosis:
- dermatitis herpetiformis
- pemphigus foliaceus
- dermatophytosis
- pemphigus, IgA
- pustular psoriasis
- eosinophilic pustular folliculitis
- impetigo
Management:
- dapsone is treatment of choice
- acitretin has been used successfully
- PUVA may be useful
- systemic or topical glucocorticoids generally not effective
- empiric antibiotics if cellulitis suspected
General
skin disease (dermatologic disorder, dermatopathy, dermatosis)
References
- Groysman V and Sami N
eMedicine: Subcorneal Pustular Dermatosis
http://emedicine.medscape.com/article/1124252-overview