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Erosive pustular dermatosis of the scalp - Hair and Scalp
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Erosive pustular dermatosis of the scalp - Hair and Scalp

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Contributors: Deren Özcan MD, Deniz Seçkin MD, Mary Gail Mercurio MD, Jeffrey D. Bernhard MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Erosive pustular dermatosis (EPD) is a rare inflammatory disease of unknown etiology that usually occurs in the elderly with a slight female predominance. Two types of EPD are described, namely, EPD of the scalp and EPD of the legs. Clinical appearance, histology, and response to therapy of the lesions in those two sites are essentially identical, but the settings in which they occur differ remarkably. On the scalp, EPD invariably develops in areas of long-standing solar damage, whereas leg involvement occurs on non-sun-damaged skin of patients with chronic venous stasis.

The etiology of EPD remains unknown. However, it is claimed that the major predisposing condition is cutaneous atrophy, which is primarily the result of actinic damage on the scalp and stretching of the skin secondary to the edema of venous insufficiency on the legs. Given this predisposing condition, EPD then develops from one or more triggering factors like physical, medical, or surgical trauma. The inflammation induced by this trauma probably plays an important role as well. Moreover, association of EPD with autoimmune disorders like rheumatoid arthritis, Hashimoto's thyroiditis, autoimmune hepatitis, and Takayasu's aortitis suggests that the physical trauma to the skin might trigger production of auto-antibodies against skin structures, resulting in a secondary inflammatory reaction.

Erosive pustular dermatosis is clinically characterized by sterile pustules and chronic crusted erosions. Crusts resolve leaving atrophic skin, and new areas of pustulation develop within a few days. Often, the presence of crusted erosions or ulcers predominates, and pustules are not seen.

EPD has a chronic, recurring, and slow but progressive course. Scarring alopecia and cutaneous malignancies such as basal cell carcinoma and squamous cell carcinoma may eventuate in the scar tissue.

Codes

ICD10CM:
L98.8 – Other specified disorders of the skin and subcutaneous tissue

SNOMEDCT:
238733003 – Erosive pustular dermatosis of the scalp

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The clinical differential diagnoses for EPD of the scalp are:
  • Chronic bacterial or fungal infection – Response to antibacterial or antimycotic medications; bacterial or fungal cultures are positive for the etiologic organisms.
  • Folliculitis decalvansStaphylococcus aureus usually contributes to the pathology. Emergence of several hairs from a single hair follicle (so-called "tufting") is characteristic. Histopathologically, together with suppurative folliculitis, interstitial and perifollicular infiltrate with neutrophil predominance is noted.
  • Dissecting cellulitis – Look for draining sinus tracts, scalp nodules, cysts, and abscesses. Also, it may be associated with other diseases characterized by follicular occlusion and secondary infection such as acne conglobata and hidradenitis suppurativa.
Typical histopathological patterns differentiate other diseases such as:
A direct immunofluorescence test can complete the histopathological examination to distinguish diseases such as:
Note: The differential diagnoses for EPD of the legs are:
  • Bacterial or fungal infection – Response to antibacterial or antimycotic medications; bacterial or fungal cultures are positive for the etiologic organisms.
  • Arterial or venous vascular ulceration.

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: 06/10/2016
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Erosive pustular dermatosis of the scalp - Hair and Scalp
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Erosive pustular dermatosis of the scalp : Crust, Exudative, weeping, Pustule, Scalp, Skin erosion, Skin ulcer
Clinical image of Erosive pustular dermatosis of the scalp
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