Erosive pustular dermatosis
See also in: Hair and ScalpAlerts and Notices
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.
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
L98.8 – Other specified disorders of the skin and subcutaneous tissue
SNOMEDCT:
403530003 – Erosive pustular dermatosis
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
The differential diagnosis for EPD of the scalp is:
- Bacterial folliculitis
- Impetigo
- Ecthyma
- Folliculitis decalvans – Staphylococcus 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.
- Kerion
- Amicrobial pustulosis of the folds
- Pyoderma gangrenosum
- Pyoderma vegetans
- Pemphigus vulgaris
- Pemphigus foliaceus
- Mucous membrane pemphigoid
- Squamous cell carcinoma
- Pustular psoriasis
- Subcorneal pustular dermatosis
- Darier disease
- Langerhans cell histiocytosis
- Bacterial folliculitis
- Impetigo
- Ecthyma
- Majocchi granuloma
- Pustular psoriasis
- Bowel-associated dermatosis-arthritis syndrome
- Localized bullous pemphigoid
- Amyloidosis-related bullous dermatosis
- Venous ulcers
- Cellulitis
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
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.
Subscription Required
References
Subscription Required
Last Reviewed:03/21/2021
Last Updated:03/21/2021
Last Updated:03/21/2021
Erosive pustular dermatosis
See also in: Hair and Scalp