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Erosive pustular dermatosis
See also in: Hair and Scalp
Other Resources UpToDate PubMed

Erosive pustular dermatosis

See also in: Hair and Scalp
Contributors: Deren Özcan MD, Deniz Seçkin MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Erosive pustular dermatosis (EPD) is a rare inflammatory disease of unknown etiology that usually occurs in elderly patients with lighter skin phototypes. There is a slight male predominance. Two types are described: EPD of the scalp and EPD of the legs. Clinical appearance, histology, and response to therapy of the lesions in those 2 sites are essentially identical, but the settings in which they occur differ remarkably. On the scalp, EPD invariably develops in areas of long-standing sun 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. EPD then develops from one or more triggering factors, eg, physical, medical, or surgical trauma. Treatment of actinic keratosis on the scalp (including liquid nitrogen and topical chemotherapeutic agents), surgical procedures (such as neurosurgery, skin grafting, hair transplant, laser therapy, and radiation therapy), physical injury, and sunburn have all been reported as provoking factors in the development of EPD of the scalp. Similarly, compression therapy has been found to be an inciting factor for EPD of the legs.

EPD is clinically characterized by sterile pustules and chronic crusted erosions. Crusts resolve leaving atrophy and scarring alopecia, and new areas of pustulation develop within a few days. Often, the presence of crusted erosions or ulcers predominates, and pustules are not seen. Itch or mild to moderate pain may be noted.

EPD has rarely been seen in patients with autoimmune disorders such as rheumatoid arthritis, Hashimoto thyroiditis, autoimmune hepatitis, and Takayasu arteritis. The significance of this is unclear.

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:
403530003 – Erosive pustular dermatosis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The differential diagnosis for EPD of the scalp is:
  • Bacterial Folliculitis
  • Bullous impetigo
  • Ecthyma
  • 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 of scalp – 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.
  • Kerion
  • Amicrobial pustulosis of the folds
  • Pyoderma gangrenosum
  • Pyodermatitis vegetans
  • Pemphigus vulgaris
  • Pemphigus foliaceus
  • Mucous membrane pemphigoid
  • Cutaneous squamous cell carcinoma
  • Pustular psoriasis
  • Subcorneal pustular dermatosis
  • Darier disease
  • Langerhans cell histiocytosis
    The differential diagnosis for EPD of the legs is:
    • Bacterial Folliculitis
    • Bullous impetigo
    • Ecthyma
    • Majocchi granuloma
    • Pustular psoriasis
    • Bowel-associated dermatosis-arthritis syndrome
    • Localized Bullous pemphigoid
    • AL amyloidosis-related bullous dermatosis
    • Stasis ulcer
    • Cellulitis

    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 Reviewed:03/21/2021
    Last Updated:03/21/2021
    Copyright © 2024 VisualDx®. All rights reserved.
    Erosive pustular dermatosis
    See also in: Hair and Scalp
    A medical illustration showing key findings of Erosive pustular dermatosis (Scalp) : Crust, Exudative, weeping, Pustule, Scalp, Skin erosion, Skin ulcer
    Clinical image of Erosive pustular dermatosis - imageId=4056502. Click to open in gallery.  caption: 'A crusted erosion with a surrounding faint pink scar on the central scalp. Note also the single pustule on the anterior scalp.'
    A crusted erosion with a surrounding faint pink scar on the central scalp. Note also the single pustule on the anterior scalp.
    Copyright © 2024 VisualDx®. All rights reserved.