Be Cautious When Making a Diagnosis of Behçet's Disease

DESTIN, FLA. – A conservative approach is best when it comes to making a diagnosis of Behçet’s disease, Dr. Kenneth Calamia said at the Congress of Clinical Rheumatology.

Although oral and genital ulcers are common in the disease, they also are a common manifestation of many other conditions, and it is important to consider the other possible causes first.

The importance of a Behçet’s diagnosis doesn’t have anything to do with ulcers – it has to do with the risk or presence of serious manifestations, including vascular disease, central nervous system manifestations, and uveitis, said Dr. Calamia of the department of medicine at the Mayo Clinic in Jacksonville, Fla.

"You don’t want to [needlessly] give a patient the baggage of that diagnosis," he said, noting that in patients diagnosed with Behçet’s, everything will be attributed to the disease for the rest of their lives.

In the United States and Europe, true Behçet’s is quite rare (about 0.3-7.5 cases/100,000 population), compared with places like Turkey and other "Silk Road" areas, which have a very high prevalence (100-370 cases/100,000 population). In those areas, more severe forms are much more prevalent, and the benign mucocutaneous symptoms that comprise most of the cases in America are referred to as American Behçet’s disease, Dr. Calamia said.

The term "Behçet’s syndrome" also can be used to describe the types of cases typically seen in the United States, but in many cases, the diagnosis is actually "complex aphthosis," he said, adding that Behçet’s treatment principles can nonetheless be used to help patients with this condition.

Complex aphthosis is a term used by oral dermatologists to help classify types of recurrent aphthous stomatitis. As opposed to simple aphthosis, which is characterized by episodic, short-lived lesions that are few in number, recur three to six times per year, and tend to affect nonkeratinized mucosa, complex aphthosis lesions can be continuous, numerous, large, slow-healing, and debilitating.

Keep in mind that both simple and complex aphthosis can be associated with menstruation, sprue, inflammatory bowel disease, HIV, hematologic disorders (such as cyclic neutropenia, IgA deficiency, myelodysplasia/myeloproliferation), various deficiencies (B vitamins, folate, iron, and zinc), and smoking cessation, Dr. Calamia said, explaining that smoking tends to increase keratinization, which protects against ulcers, and that protection is lost when a patient quits.

In a study conducted by an oral dermatologist several years ago, only 9% of 269 patients with severe complex aphthosis – 16% of whom also had genital ulcers – had a Behçet’s diagnosis, he noted.

Some other diagnoses in the cohort included anemia in 25%, gastrointestinal disease in 16%, hematologic disorders in 5%, mucosal disease in 6%, smoking discontinuation in 4%, and drug-related ulcers in 3%.

"[Complex aphthosis] is the diagnosis I prefer in those who have mouth and genital ulcers, but nothing else to support a diagnosis of Behçet’s," he said.

Consider the other possible causes of the ulcers, and also consider the differential diagnoses for recurrent aphthous stomatitis, which include recurrent intraoral herpes simplex virus, Wegener’s granulomatosis, oral Crohn’s disease, pyostomatitis vegetans, erythema multiforme, lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris, he said.

A diffuse, widespread, and chronic presentation, which is not characteristic of recurrent aphthous stomatitis or Behçet’s, can help differentiate between those conditions and these differential diagnoses, he said.

Dr. Calamia disclosed that he has received research support from Genentech and Celgene, and has served on an advisory board for Centocor.

DESTIN, FLA. – A conservative approach is best when it comes to making a diagnosis of Behçet’s disease, Dr. Kenneth Calamia said at the Congress of Clinical Rheumatology.

Although oral and genital ulcers are common in the disease, they also are a common manifestation of many other conditions, and it is important to consider the other possible causes first.

The importance of a Behçet’s diagnosis doesn’t have anything to do with ulcers – it has to do with the risk or presence of serious manifestations, including vascular disease, central nervous system manifestations, and uveitis, said Dr. Calamia of the department of medicine at the Mayo Clinic in Jacksonville, Fla.

"You don’t want to [needlessly] give a patient the baggage of that diagnosis," he said, noting that in patients diagnosed with Behçet’s, everything will be attributed to the disease for the rest of their lives.

In the United States and Europe, true Behçet’s is quite rare (about 0.3-7.5 cases/100,000 population), compared with places like Turkey and other "Silk Road" areas, which have a very high prevalence (100-370 cases/100,000 population). In those areas, more severe forms are much more prevalent, and the benign mucocutaneous symptoms that comprise most of the cases in America are referred to as American Behçet’s disease, Dr. Calamia said.

The term "Behçet’s syndrome" also can be used to describe the types of cases typically seen in the United States, but in many cases, the diagnosis is actually "complex aphthosis," he said, adding that Behçet’s treatment principles can nonetheless be used to help patients with this condition.

Complex aphthosis is a term used by oral dermatologists to help classify types of recurrent aphthous stomatitis. As opposed to simple aphthosis, which is characterized by episodic, short-lived lesions that are few in number, recur three to six times per year, and tend to affect nonkeratinized mucosa, complex aphthosis lesions can be continuous, numerous, large, slow-healing, and debilitating.

Keep in mind that both simple and complex aphthosis can be associated with menstruation, sprue, inflammatory bowel disease, HIV, hematologic disorders (such as cyclic neutropenia, IgA deficiency, myelodysplasia/myeloproliferation), various deficiencies (B vitamins, folate, iron, and zinc), and smoking cessation, Dr. Calamia said, explaining that smoking tends to increase keratinization, which protects against ulcers, and that protection is lost when a patient quits.

In a study conducted by an oral dermatologist several years ago, only 9% of 269 patients with severe complex aphthosis – 16% of whom also had genital ulcers – had a Behçet’s diagnosis, he noted.

Some other diagnoses in the cohort included anemia in 25%, gastrointestinal disease in 16%, hematologic disorders in 5%, mucosal disease in 6%, smoking discontinuation in 4%, and drug-related ulcers in 3%.

"[Complex aphthosis] is the diagnosis I prefer in those who have mouth and genital ulcers, but nothing else to support a diagnosis of Behçet’s," he said.

Consider the other possible causes of the ulcers, and also consider the differential diagnoses for recurrent aphthous stomatitis, which include recurrent intraoral herpes simplex virus, Wegener’s granulomatosis, oral Crohn’s disease, pyostomatitis vegetans, erythema multiforme, lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris, he said.

A diffuse, widespread, and chronic presentation, which is not characteristic of recurrent aphthous stomatitis or Behçet’s, can help differentiate between those conditions and these differential diagnoses, he said.

Dr. Calamia disclosed that he has received research support from Genentech and Celgene, and has served on an advisory board for Centocor.

DESTIN, FLA. – A conservative approach is best when it comes to making a diagnosis of Behçet’s disease, Dr. Kenneth Calamia said at the Congress of Clinical Rheumatology.

Although oral and genital ulcers are common in the disease, they also are a common manifestation of many other conditions, and it is important to consider the other possible causes first.

The importance of a Behçet’s diagnosis doesn’t have anything to do with ulcers – it has to do with the risk or presence of serious manifestations, including vascular disease, central nervous system manifestations, and uveitis, said Dr. Calamia of the department of medicine at the Mayo Clinic in Jacksonville, Fla.

"You don’t want to [needlessly] give a patient the baggage of that diagnosis," he said, noting that in patients diagnosed with Behçet’s, everything will be attributed to the disease for the rest of their lives.

In the United States and Europe, true Behçet’s is quite rare (about 0.3-7.5 cases/100,000 population), compared with places like Turkey and other "Silk Road" areas, which have a very high prevalence (100-370 cases/100,000 population). In those areas, more severe forms are much more prevalent, and the benign mucocutaneous symptoms that comprise most of the cases in America are referred to as American Behçet’s disease, Dr. Calamia said.

The term "Behçet’s syndrome" also can be used to describe the types of cases typically seen in the United States, but in many cases, the diagnosis is actually "complex aphthosis," he said, adding that Behçet’s treatment principles can nonetheless be used to help patients with this condition.

Complex aphthosis is a term used by oral dermatologists to help classify types of recurrent aphthous stomatitis. As opposed to simple aphthosis, which is characterized by episodic, short-lived lesions that are few in number, recur three to six times per year, and tend to affect nonkeratinized mucosa, complex aphthosis lesions can be continuous, numerous, large, slow-healing, and debilitating.

Keep in mind that both simple and complex aphthosis can be associated with menstruation, sprue, inflammatory bowel disease, HIV, hematologic disorders (such as cyclic neutropenia, IgA deficiency, myelodysplasia/myeloproliferation), various deficiencies (B vitamins, folate, iron, and zinc), and smoking cessation, Dr. Calamia said, explaining that smoking tends to increase keratinization, which protects against ulcers, and that protection is lost when a patient quits.

In a study conducted by an oral dermatologist several years ago, only 9% of 269 patients with severe complex aphthosis – 16% of whom also had genital ulcers – had a Behçet’s diagnosis, he noted.

Some other diagnoses in the cohort included anemia in 25%, gastrointestinal disease in 16%, hematologic disorders in 5%, mucosal disease in 6%, smoking discontinuation in 4%, and drug-related ulcers in 3%.

"[Complex aphthosis] is the diagnosis I prefer in those who have mouth and genital ulcers, but nothing else to support a diagnosis of Behçet’s," he said.

Consider the other possible causes of the ulcers, and also consider the differential diagnoses for recurrent aphthous stomatitis, which include recurrent intraoral herpes simplex virus, Wegener’s granulomatosis, oral Crohn’s disease, pyostomatitis vegetans, erythema multiforme, lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris, he said.

A diffuse, widespread, and chronic presentation, which is not characteristic of recurrent aphthous stomatitis or Behçet’s, can help differentiate between those conditions and these differential diagnoses, he said.

Dr. Calamia disclosed that he has received research support from Genentech and Celgene, and has served on an advisory board for Centocor.