Dowling-Degos disease

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ORPHA:79145OMIM:179850L81.8
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1Specialists8Treatment centers

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Overview

Dowling-Degos disease (DDD) is a rare inherited skin condition that causes dark, patchy discoloration of the skin, especially in the body's natural folds and creases. It is also sometimes called reticulate pigmented anomaly of the flexures or Kitamura's reticulate acropigmentation in related forms. The condition develops because certain skin cells called melanocytes produce too much pigment in specific areas, leading to a net-like or spotted pattern of darkening. The skin changes most often appear in the armpits, groin, neck, and under the breasts — areas where skin rubs together. Over time, small dark pits or comedone-like spots may also appear on the face and back. Some people develop small, pitted scars around the mouth. The condition is not dangerous to overall health and does not affect internal organs, but it can cause significant emotional distress and affect self-confidence because of its visible nature. There is currently no cure for Dowling-Degos disease. Treatment focuses on managing the appearance of the skin. Options include topical retinoids, chemical peels, and laser therapy, though results vary from person to person. The condition tends to slowly worsen over time, but it does not shorten life expectancy. Working with a dermatologist experienced in pigmentation disorders is the most important step after diagnosis.

Also known as:

Key symptoms:

Dark, net-like or spotted patches of skin in body folds such as armpits and groinSkin darkening in the neck creasesSmall dark spots or pits on the face, especially around the mouthComedone-like (blackhead-like) bumps on the back and chestPitted or depressed scars near the mouthDarkening under the breastsSlowly spreading pigmentation over timeItching or mild irritation in affected areas in some peopleSkin changes that worsen with age

Clinical phenotype terms (25)— hover any for plain English
Progressive reticulate hyperpigmentationHP:0007456Inguinal frecklingHP:0030052Heat intoleranceHP:0002046Scrotal hyperpigmentationHP:0012855Hyperpigmented papuleHP:0025473Digital pitting scarHP:0031293Penile frecklingHP:0031447Acne inversaHP:0040154Hyperkeratotic papuleHP:0045059Mixed hypo- and hyperpigmentation of the skinHP:0009123Palmar pitsHP:0010610
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Dowling-Degos disease.

View clinical trials →

No actively recruiting trials found for Dowling-Degos disease at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Dowling-Degos disease community →

Specialists

1 foundView all specialists →

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 Dowling-Degos disease.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Dowling-Degos disease

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene is most likely causing my condition, and should I have genetic testing?,Should my family members be tested or examined for this condition?,What treatment options are available to improve the appearance of my skin?,How quickly is this condition likely to spread, and what can I do to slow it down?,Are there any clinical trials or new treatments I should know about?,Can you refer me to a mental health professional or support group for people with visible skin conditions?,How often should I have follow-up appointments, and what changes in my skin should prompt me to come in sooner?

Common questions about Dowling-Degos disease

What is Dowling-Degos disease?

Dowling-Degos disease (DDD) is a rare inherited skin condition that causes dark, patchy discoloration of the skin, especially in the body's natural folds and creases. It is also sometimes called reticulate pigmented anomaly of the flexures or Kitamura's reticulate acropigmentation in related forms. The condition develops because certain skin cells called melanocytes produce too much pigment in specific areas, leading to a net-like or spotted pattern of darkening. The skin changes most often appear in the armpits, groin, neck, and under the breasts — areas where skin rubs together. Over time,

How is Dowling-Degos disease inherited?

Dowling-Degos disease follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Dowling-Degos disease typically begin?

Typical onset of Dowling-Degos disease is adult. Age of onset can vary across affected individuals.

Which specialists treat Dowling-Degos disease?

1 specialists and care centers treating Dowling-Degos disease are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.