Recurrent aphthous stomatits (RAS) is also known as recurrent oral ulcers, recurrent aphthous ulcers, or simple or complex aphthosis. RAS is the most common inflammatory ulcerative condition of the oral mucosa in North American patients. RAS has been the subject of active investigation along multiple lines of research including epidemiology, immunology, clinical correlations and therapy. Clinical evaluation of the patient requires correct diagnosis of RAS and classification of the disease based on morphology (MIAU, MJAU, HU) and severity (simple versus complex). In order to properly diagnose and treat a patient with lesions of RAS, the clinician must exclude other causes of acute oral ulcers. Complex aphthosis and complex aphthosis variants associated with systemic disorders should be considered. The aphthous-like oral ulcerations of patients with HIV disease represent a challenging differential diagnosis. The association of lesions of RAS with hematinic deficiencies and gastrointestinal diseases provides an opportunity to identify a "correctable cause" which, with appropriate treatment, can result in a remission or substantial lessening of disease activity. Finally, when all of these factors are considered, the evaluation of the patient for Behcet's disease can be continued on firm grounds that one of the major criteria for the diagnosis of Behcet's disease has been met.
Behcet's Syndrome/diagnosis* ; Diagnosis, Differential ; Human ; Recurrence ; Stomatitis, Aphthous/etiology ; Stomatitis, Aphthous/diagnosis* ; Stomatitis, Aphthous/classification

Humans ; Stomatitis, Aphthous ; diagnosis ; etiology ; therapy

Consensus ; Diagnosis, Differential ; Glucocorticoids ; therapeutic use ; Humans ; Practice Guidelines as Topic ; Recurrence ; Stomatitis, Aphthous ; diagnosis ; therapy

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is one of the causes of periodic fever in pediatrics with unknown etiology. It is characterized by abrupt onset of fever, malaise, aphthous stomatitis, pharyngitis and cervical adenitis without long-term sequelae. Laboratory findings of this sporadic and nonhereditary syndrome are so non-specific that the diagnosis is based on clinical findings. Oral prednisolone is quite effective in controlling the symptoms. We report a case of a 6-year-old girl who was diagnosed as having PFAPA syndrome after 2 years of episodes, by excluding other disease entities with similar clinical features. The patient was treated with oral prednisolone and her symptoms improved dramatically.
Child ; Diagnosis ; Female ; Fever* ; Humans ; Lymphadenitis ; Pediatrics ; Pharyngitis* ; Prednisolone ; Stomatitis, Aphthous* ; Yemen

Behcet's disease is a systemic vasculitis that is characterized mainly by recurrent oral and genital aphthous ulcers, uveitis, and skin findings. Its neurological manifestations are well recognized. Recurrent meningitis in Behcet's disease is exceptional. We describe herein the case of a 31-year-old man who presented with recurrent aseptic meningitis without any specific cause. A few years later he developed oral and genital ulcers, and uveitis. Behcet's disease should always be considered in a differential diagnosis of recurrent aseptic meningitis without viral infection, particularly in the context of multisystem manifestations.
Adult ; Diagnosis, Differential ; Humans ; Meningitis ; Meningitis, Aseptic ; Neurologic Manifestations ; Skin ; Stomatitis, Aphthous ; Systemic Vasculitis ; Ulcer ; Uveitis

OBJECTIVE: To study the clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, a polygenic and multifactorial autoinflammatory disease with unknown pathogenesis. METHODS: A retrospective analysis was performed on the medical data of 13 children with PFAPA syndrome. RESULTS: All 13 children had disease onset within the age of 3 years, with a mean age of onset of (14±10) months. They all had periodic fever, with 8-18 attacks each year. The mean interictal period of fever was (30±5) days. Pharyngitis, cervical adenitis, and aphthous stomatitis were the three cardinal symptoms, with incidence rates of 100% (13/13), 85% (11/13), and 38% (5/13) respectively. There were increases in white blood cells, C-reactive protein, and erythrocyte sedimentation rate during fever. Of all the 13 children, 6 underwent whole exome sequencing and 7 underwent panel gene detection for autoinflammatory disease, and the results showed single heterozygous mutations in the CONCLUSIONS For children with unexplained periodic fever with early onset accompanied by pharyngitis, cervical adenitis, aphthous stomatitis, elevated inflammatory indices, and good response to glucocorticoids, PFAPA syndrome should be considered. This disorder has good prognosis, and early diagnosis can avoid the long-term repeated use of antibiotics.
Child ; Child, Preschool ; Fever/etiology* ; Humans ; Infant ; Lymphadenitis/diagnosis* ; Pharyngitis/drug therapy* ; Pyrin ; Retrospective Studies ; Stomatitis, Aphthous/genetics*

Behcet's disease is an inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. A few cases of hematologic disease in patients with Behcet's disease have been reported in the literature. However, acute precursor T cell lymphoblastic leukemia has never been described in association with Behcet's disease. We recently encountered a case of acute precursor T cell lymphoblastic leukemia in a 62-year-old man with a prior diagnosis of Behcet's disease. The patient presented with febrile neutropenia and his bone marrow biopsy revealed acute precursor T cell lymphoblastic leukemia. He was scheduled to undergo therapeutic chemotherapy, but unfortunately he died from pneumonia prior to treatment.
Biopsy ; Bone Marrow ; Diagnosis ; Drug Therapy ; Febrile Neutropenia ; Hematologic Diseases ; Humans ; Middle Aged ; Pneumonia ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* ; Skin ; Stomatitis, Aphthous ; Ulcer ; Uveitis

Behcet's disease is classically described as featuring recurrent aphthous ulcers in the mouth and genital organs and as having relapsing iritis. Now it is being recognized as a mul tisystem disorder that involves of the skin, gastrointestinal system, cardiovascular system, lung and the central nervous system as well as the joints, blood vessels and urologic systems. Large vessel diseases are unusual but aneurysm may occur in which the pulmonary circulation may give rise to the massive and often fatal hemoptysis. A 29 year-old man who complained of having dyspnea and hemoptysis during six months visited our hospital. He received right bilobectomy for a mass located in the right lower lobe. He underwent right bilobectomy. The final pathologic diagnosis was a pulmonary artery aneurysm which origina ted from the pulmonary artery.
Adult ; Aneurysm* ; Blood Vessels ; Cardiovascular System ; Central Nervous System ; Diagnosis ; Dyspnea ; Genitalia ; Hemoptysis ; Humans ; Iritis ; Joints ; Lung ; Mouth ; Pulmonary Artery* ; Pulmonary Circulation ; Skin ; Stomatitis, Aphthous

Since its introduction, fluorescein angiography has been widely used in diagnosing and evaluating treatment of diseases of the retina, uveal tract, and optic nerve. A case with Behcet's disease was studied by means of fluorescein fundus angiography. This, pre summed a retinal angiopathy, has been diagnosed Behcet's disease after developing symptoms of hypopyon iritis, intermittent fever, aphthous stomatitis, and genital ulceration. The fluorescein angiography in Behcet's disease is typically shown a specific finding. Extravasation of the dye waS most conspicuous in capillaries over-lying the optic nerve head, the radial peripapillary capillaries, retinal capillaries proper and retinal veins. Retinal arteriols generally lacked extravasation phenomenon. Edematous swelling of the disc, diffuse turbidity of the posterior fundus and macular cysts are regarded as consquences of the fluorographically confirmed increased permeability of the retinal vessels with resultant edema of the neuropithelium. Pathogenesis of the retinal angiopathy in Behcet's disease and diseases with retinal edema and hyperpermeability were discussed with references. The fluorographic findings thus indicate that the pathological involvement of retinal vessels play a major factor in the diagnosis and prognosis of ocular manifestation of Behcet's disease.
Angiography* ; Capillaries ; Diagnosis ; Edema ; Fever ; Fluorescein Angiography ; Fluorescein* ; Iritis ; Optic Disk ; Optic Nerve ; Papilledema ; Permeability ; Prognosis ; Retina ; Retinal Vein ; Retinal Vessels ; Retinaldehyde ; Stomatitis, Aphthous ; Ulcer

Behcet's disease (BD) is a multisystemic chronic inflammatory disease. It is characterized by recurrent oral and genital ulcers, uveitis, skin lesions and other manifestations, including neurologic, vascular, joint, and gastrointestinal ulcers of variable severity. Recurrent aphthous ulcer (RAU) represents a very common, but poorly understood, mucosal disorder. If a patient of RAU without any other typical symptoms of BD has gastrointestinal symptoms, it is difficult to distinguish this RAU from true BD with gastrointestinal involvement. Because pathognomonic clinical features and tools are absent, the differential diagnosis of these two diseases relies on the characteristic clinical features and the judgement of an experienced physician. Sixty-five out of a total 960 RAU patients and forty-four of 556 BD patients with gastrointestinal symptoms between January 1996 and December 2003 participated in this study. All were evaluated with esophagogastroduodenoscopy and colonoscopy. Clinical, endoscopic and histopathologic findings were analyzed and ELISA tests were conducted to detect serum levels of ASCA and pANCA. No significant difference was found between the two groups. Differential diagnosis between RAU with gastrointestinal symptoms and BD with gastrointestinal involvement requires further prospective, large-scale study.
Adolescent ; Adult ; Aged ; Antibodies, Antineutrophil Cytoplasmic/blood ; Antibodies, Fungal/blood ; Behcet Syndrome/*diagnosis/immunology/pathology ; Comparative Study ; Diagnosis, Differential ; Endoscopy ; Female ; Gastrointestinal Diseases/*diagnosis/immunology/pathology ; Humans ; Male ; Middle Aged ; Saccharomyces cerevisiae/immunology ; Serologic Tests ; Stomatitis, Aphthous/*diagnosis/immunology/pathology