Erosive pustular dermatosis of the scalp

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Overview

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory skin condition that mainly affects the scalp. It is sometimes called 'erosive pustular dermatosis' for short. The condition causes the skin on the scalp to develop pustules (small pus-filled bumps), crusts, and open sores (erosions) that can be painful and uncomfortable. Over time, if left untreated, these sores can lead to permanent scarring and hair loss in the affected areas, a condition called scarring alopecia. EPDS most often appears in older adults, particularly those over 60, and tends to develop on areas of the scalp that have already been damaged — for example, by sun exposure, injury, surgery, or skin treatments like radiotherapy. The exact cause is not fully understood, but it is thought to involve a combination of local skin damage and an overactive immune response in the skin. Treatment usually focuses on calming the inflammation and protecting the skin. Doctors most commonly prescribe strong topical steroid creams or ointments applied directly to the scalp. Tacrolimus ointment (an immune-modulating cream) is another option. In some cases, oral medications may be needed. With early and consistent treatment, many people can control the condition and prevent further hair loss, though complete regrowth of hair in scarred areas is generally not possible.

Key symptoms:

Pus-filled bumps (pustules) on the scalpCrusting and scabbing on the scalpOpen sores or raw patches on the scalpPermanent hair loss in affected areasRedness and inflammation of the scalp skinItching or burning sensation on the scalpScarring of the scalp skin over timeOozing or weeping from sores on the scalpThickened or discolored skin patches on the scalp

Clinical phenotype terms (5)— hover any for plain English
Scarring alopecia of scalpHP:0004552
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Late onset

Begins later in life, typically after age 50

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Erosive pustular dermatosis of the scalp.

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No actively recruiting trials found for Erosive pustular dermatosis of the scalp at this time.

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No specialists are currently listed for Erosive pustular dermatosis of the scalp.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Erosive pustular dermatosis of the scalp.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.How confident are you in this diagnosis, and what other conditions have been ruled out?,What treatment do you recommend first, and how long before I should expect to see improvement?,Are there any side effects I should watch for with the prescribed treatment?,How often do I need to come back for check-ups?,Is there anything I can do to prevent flares or stop the condition from getting worse?,Will any of my hair grow back, or is the hair loss permanent?,Should I see any other specialists, such as a dermatopathologist, for a second opinion?

Common questions about Erosive pustular dermatosis of the scalp

What is Erosive pustular dermatosis of the scalp?

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory skin condition that mainly affects the scalp. It is sometimes called 'erosive pustular dermatosis' for short. The condition causes the skin on the scalp to develop pustules (small pus-filled bumps), crusts, and open sores (erosions) that can be painful and uncomfortable. Over time, if left untreated, these sores can lead to permanent scarring and hair loss in the affected areas, a condition called scarring alopecia. EPDS most often appears in older adults, particularly those over 60, and tends to develop on areas of the sc

How is Erosive pustular dermatosis of the scalp inherited?

Erosive pustular dermatosis of the scalp follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Erosive pustular dermatosis of the scalp typically begin?

Typical onset of Erosive pustular dermatosis of the scalp is late onset. Age of onset can vary across affected individuals.