Overview
Acquired methemoglobinemia is a condition where an abnormally high amount of methemoglobin builds up in the blood. Methemoglobin is a form of hemoglobin that cannot carry oxygen effectively. Unlike inherited forms of methemoglobinemia, the acquired type is not caused by a genetic defect you are born with. Instead, it is triggered by exposure to certain drugs, chemicals, or toxins. Common culprits include certain local anesthetics (like benzocaine and lidocaine), nitrates, nitrites, dapsone, and some industrial chemicals. When methemoglobin levels rise too high, the body's tissues do not get enough oxygen, which can become a medical emergency. The hallmark symptom is a bluish or grayish discoloration of the skin, lips, and nail beds, known as cyanosis, that does not improve with supplemental oxygen. Other symptoms include headache, fatigue, dizziness, shortness of breath, confusion, and in severe cases, seizures, coma, or even death. The severity of symptoms depends on how high the methemoglobin level climbs. Mild cases (methemoglobin levels around 10-20%) may cause only slight skin color changes and mild symptoms, while levels above 50% can be life-threatening. The good news is that acquired methemoglobinemia is usually treatable and often reversible once the offending agent is identified and removed. The primary treatment is methylene blue, given intravenously, which works quickly to convert methemoglobin back to normal hemoglobin. In most cases, patients recover fully with prompt treatment. Supportive care, including supplemental oxygen, is also provided. For patients who cannot receive methylene blue (such as those with G6PD deficiency), alternative treatments like ascorbic acid or exchange transfusion may be considered.
Also known as:
Key symptoms:
Bluish or grayish skin color (cyanosis)Blue or gray lips and nail bedsHeadacheFatigue and weaknessDizziness or lightheadednessShortness of breathRapid heart rateConfusion or altered mental statusNauseaChocolate-brown colored bloodAnxiety or irritabilityLoss of consciousness in severe casesSeizures in severe cases
Clinical phenotype terms (21)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
1 eventProvayBlue: FDA approved
For treatment of pediatric and adult patients with acquired methemoglobinemia.
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableProvayBlue
For treatment of pediatric and adult patients with acquired methemoglobinemia.
Clinical Trials
View all trials with filters →No actively recruiting trials found for Acquired methemoglobinemia at this time.
New trials open frequently. Follow this disease to get notified.
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 Acquired methemoglobinemia.
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.What specific drug or chemical caused my methemoglobinemia?,Are there other medications or substances I need to avoid in the future?,Should I be tested for G6PD deficiency or any underlying conditions that could make me more vulnerable?,What should I do if I notice symptoms returning?,Should I wear a medical alert bracelet or carry an information card?,Are my family members at any increased risk for this condition?,How can I make sure my other doctors and dentists know about this condition?
Common questions about Acquired methemoglobinemia
What is Acquired methemoglobinemia?
Acquired methemoglobinemia is a condition where an abnormally high amount of methemoglobin builds up in the blood. Methemoglobin is a form of hemoglobin that cannot carry oxygen effectively. Unlike inherited forms of methemoglobinemia, the acquired type is not caused by a genetic defect you are born with. Instead, it is triggered by exposure to certain drugs, chemicals, or toxins. Common culprits include certain local anesthetics (like benzocaine and lidocaine), nitrates, nitrites, dapsone, and some industrial chemicals. When methemoglobin levels rise too high, the body's tissues do not get en
How is Acquired methemoglobinemia inherited?
Acquired methemoglobinemia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Acquired methemoglobinemia?
21 specialists and care centers treating Acquired methemoglobinemia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Acquired methemoglobinemia?
1 patient support program are currently tracked on UniteRare for Acquired methemoglobinemia. See the treatments and support programs sections for copay assistance, eligibility, and contact details.