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
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Dowling-Degos disease.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Dowling-Degos disease.
Community
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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.