Subcorneal pustular dermatitis
Subcorneal Pustular Dermatitis Subcorneal pustular dermatitis (SPD), also known as Sneddon-Wilkinson disease, is a rare, chronic skin condition characterized by the presence of pustules just beneath the stratum corneum, the outermost layer of the skin. This condition is considered a type of neutrophilic dermatosis and is often associated with other systemic diseases.
Clinical Presentation[edit | edit source]
Patients with subcorneal pustular dermatitis typically present with flaccid pustules that are superficial and easily ruptured. These pustules are often grouped and can coalesce to form larger areas of pus-filled lesions. The condition primarily affects the trunk and intertriginous areas, but it can also appear on the face and extremities.
Symptoms[edit | edit source]
- Flaccid pustules
- Erythematous base
- Pruritus (itching)
- Possible systemic symptoms such as fever
Pathophysiology[edit | edit source]
The exact pathogenesis of SPD is not fully understood, but it is believed to involve an abnormal immune response. The condition is characterized by the accumulation of neutrophils in the subcorneal layer of the epidermis, leading to pustule formation.
Diagnosis[edit | edit source]
Diagnosis of subcorneal pustular dermatitis is primarily clinical, supported by histopathological examination. A skin biopsy typically shows subcorneal pustules filled with neutrophils. Direct immunofluorescence is usually negative, helping to differentiate SPD from other blistering diseases such as pemphigus.
Differential Diagnosis[edit | edit source]
SPD must be differentiated from other pustular dermatoses, including:
Treatment[edit | edit source]
The mainstay of treatment for SPD is dapsone, which is effective due to its anti-inflammatory and neutrophil-modulating properties. Other treatments may include:
- Topical corticosteroids
- Systemic corticosteroids
- Retinoids
Prognosis[edit | edit source]
The prognosis for SPD is generally good with appropriate treatment. However, the condition can be chronic and relapsing, requiring long-term management.
Associations[edit | edit source]
SPD has been associated with several systemic conditions, including:
Epidemiology[edit | edit source]
Subcorneal pustular dermatitis is a rare condition, with no specific predilection for age or gender. It is considered a rare disease, with an incidence that is not well-documented due to its rarity.
See Also[edit | edit source]
External Links[edit | edit source]
- [Link to a reputable dermatology resource]
NIH genetic and rare disease info[edit source]
Subcorneal pustular dermatitis is a rare disease.
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Contributors: Prab R. Tumpati, MD
