Facial Flushing Caused by Mastocytosis and Other Conditions

Differential Diagnosis of Facial Flushing and Mastocytosis

middle aged woman with facial flushing
Learn about facial flushing with mastocytosis. Istockphoto.com/Stock Photo©IpekMorel

If you're coping with episodic facial flushing you may be frustrated at how hard it is to get a diagnosis. What is facial flushing related to mastocytosis like and what are some of the conditions which could instead be the cause of your symptoms?

What is Mastocytosis - Definition

Mast cells are cells of the immune system which are found around blood vessels in the skin, gastrointestinal tract, respiratory tract, and genitourinary tract.

They contain granules of several substances, the most common of which is histamine. These granules are released in response to contact with certain foreign substances. Histamine, in turn, causes many of the symptoms we equate with an allergic reaction, such as itchy, watery eyes and noses and tightening of the airways in our lungs.

Mastocytosis is a condition in which there are a greater than normal number of mast cells in the tissue. These mast cells cause a typical skin response called urticaria pigmentosa (hives) immediately after stroking the skin with a blunt object.

Facial Flushing Symptoms of Mastocytosis

Facial flushing with mastocytosis usually comes on rapidly. It is most prominent on the face and upper trunk and may have reddish-brown bumps. The face usually becomes red and hot and can be very itchy (pruritic.) It may also feel like it is burning or on fire.

changes in the blood flow in the face

Other Symptoms With Mastocytosis

In addition to facial flushing, people with mastocytosis often experience symptoms throughout their body caused by the release of large amounts of histamine or other chemicals. Some symptoms include:

  • Palpitations
  • Low blood pressure
  • Chest pain
  • Explosive diarrhea
  • Nausea and vomiting
  • Abdominal pain
  • Fatigue

Mastocytosis Triggers

Many people with mastocytosis cannot identify the triggers which result in flushing, but some people have found that exercise, heat, or anxiety are a possible trigger.

Medications such as opioid narcotics like morphine and codeine, and aspirin or other non-steroidal anti-inflammatory drugs like ibuprofen or naproxen can also start a flushing attack.

Diagnosis of Facial Flushing Caused by Mastocytosis

Mastocytosis can be difficult to diagnose and it can a very frustrating process. Correctly identifying the clinical symptoms aids in the diagnosis. Sometimes skin lesions and bone marrow are biopsied and the urine checked for increased levels of the chemicals contained in mast cells.

The diagnosis is made a bit easier in those who have the classic urticaria pigmentosa, but can still be a challenge. It may be suspected when people have what appears to be a severe allergic reaction without exposure to any obvious allergens or if a person develops a very severe insect related anaphylactic reaction.

Other Possible Causes of Facial Flushing - Differential Diagnosis

As noted, mastocytosis can be very challenging to diagnose both due to the wide range of symptoms (which can be caused by a wide range of conditions) and due to other possible causes of the facial flushing. These other causes may be a distinct clinical entity (a disease all on their own) or instead part of another condition or disease. Some other conditions which may cause facial flushing that are part of the differential diagnosis include:

  • Contact dermatitis
  • Photodermatitis - Rash from sun exposure
  • Rosacea
  • Menopausal hot flashes
  • Drug allergies
  • Heart disease
  • Carcinoid syndrome - Facial flushing in carcinoid syndrome can appear very similar to that with mastocytosis and is caused by chemicals released by the tumor
  • Pheochromocytoma
  • Cushing syndrome
  • Anaphylaxis
  • Other tumors such as medullary carcinoma of the thyroid, pancreatic islet tumor, and renal carcinoma (kidney cancer.)

Treatment of Facial Flushing Caused by Mastocytosis

There are numerous treatment options for mastocytosis depending on the main symptoms. Treatments may include:

  • Antihistamines
  • Aspirin and non-steroidal anti-inflammatory drugs such as Advil (ibuprofen) - Although in some people these medications can actually trigger the flushing
  • Cromolyn sodium - Cromolyn is a mast cell stabilizer sometimes used in the treatment of asthma
  • Topical steroid creams
  • PUVA.(photochemotherapy)

Bottom Line on Facial Flushing and Mastocytosis

Whether you've been diagnosed with mastocytosis as the cause of your facial flushing or your doctor mentioned that mastocytosis is just one of the possible causes of your symptoms, you're probably feeling very frustrated on top of being frightened. Keep asking questions and be your own advocate in your care. Finding a diagnosis and eventually a treatment can take time. If you're not getting answers, consider getting a second opinion.

Disorders such as mastocytosis (and other conditions in the differential diagnosis) are uncommon, and not every physician has seen the classic flushing reaction. That said, we have learned a lot about these conditions in recent years as our understanding of the immune and endocrine systems advance. Since these conditions are rare it's not likely that you have a support group in your neighborhood, but online support communities allow people to connect with others facing similar challenges around the world.

Sources:

Gulen, T., Hagglund, H., Dahlen, B., and G. Nilsson. Mastocytosis: The Puzzling Clinical Spectrum and Challenging Diagnostic Aspects of an Enigmatic Disease. Journal of Internal Medicine. 2016. 279(3):211-28.

Hannah-Shmouni, F., Stratakis, C., and C. Koch. Flushing in Neuro(Endocrinology). Reviews in Endocrine and Metabolic Disorders. 2016. 17(3):373-380.

Ikizoglu, G. Red Face Revisited: Flushing. Clinical Dermatology. 2014. 32(6):800-8.

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