Behcet's disease is particularly prevalent in "Silk Route" populations, but it has a global distribution. The diagnosis of the disease is based on clinical criteria as there is as yet no pathognomonic test, and mucocutaneous lesions, which figure prominently in the presentation and diagnosis, may be considered the diagnostic hallmarks. Among the internationally accepted criteria, painful oral and genital ulcers, cutaneous vasculitic lesions and reactivity of the skin to needle prick or injection (the pathergy reaction) are considered hallmarks of Behcet's disease, and often precede other manifestations. Their recognition may permit earlier diagnosis and treatment, with salutary results. This paper describes the various lesions that constitute the syndrome and focuses on those that may be considered characteristic.
Behcet Syndrome/drug therapy/*pathology ; Female ; Humans ; Male ; Oral Ulcer/drug therapy/pathology ; Skin Ulcer/drug therapy/pathology ; Thrombophlebitis/drug therapy/pathology

Administration, Topical ; Humans ; Immunosuppressive Agents ; administration & dosage ; adverse effects ; therapeutic use ; Lichen Planus, Oral ; complications ; drug therapy ; pathology ; Oral Ulcer ; drug therapy ; etiology ; Tacrolimus ; administration & dosage ; adverse effects ; therapeutic use ; Treatment Outcome

Administration, Oral ; Adolescent ; Female ; Histiocytosis, Langerhans-Cell ; complications ; drug therapy ; pathology ; Humans ; Lymph Nodes ; pathology ; Prednisone ; administration & dosage ; therapeutic use ; Retrospective Studies ; Skin Ulcer ; drug therapy ; etiology ; pathology ; Thalidomide ; administration & dosage ; therapeutic use ; Treatment Outcome

Pyoderma gangrenosum (PG) is a rare, noninfectious neutrophilic dermatosis. Clinically, it begins with sterile pustules that rapidly progress into painful ulcers of variable depth and size with undermined violaceous borders. The diagnosis of PG is based on the history of an underlying disease, a typical clinical presentation, histopathology, and exclusion of other diseases. The peak incidence occurs between the ages of 20 to 50 years with women being more often affected than men. There have been very few reports of pyoderma gangrenosum with oral mucosal involvement. Oral lesions in previously reported cases have included ulcers of varying sizes from a few mm to several cm and have been reported to have been found on the tongue, soft and hard palate, buccal mucosa, and gingiva. Some of these oral lesions have been associated with ulcerative colitis, inflammatory bowel disease, and polycythemia rubra vera. A few cases were reported with biopsy findings, the histological picture being nonspecific, showing ulceration, and necrosis with inflammatory cell infiltrate. A peculiar case of pyoderma gangrenosum with an oral lesion is presented here, and the differential diagnosis is discussed.
Adult ; Anti-Infective Agents ; therapeutic use ; Chlorhexidine ; therapeutic use ; Dapsone ; therapeutic use ; Drug Therapy, Combination ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Metronidazole ; therapeutic use ; Oral Ulcer ; drug therapy ; etiology ; pathology ; Prednisolone ; therapeutic use ; Pyoderma Gangrenosum ; complications ; Tooth Mobility ; etiology

Crohn's disease is a chronic inflammatory bowel disease that can involve the whole gastrointestinal tract. The orofacial manifestation of Crohn's disease, which is rare, can develop irrespective of intestinal involvement. These orofacial lesions are often misdiagnosed as simple oral ulcers. Corticosteroids are the mainstay of therapy for orofacial Crohn's disease. However, infliximab, the chimeric monoclonal antibody to tumor necrosis factor-alpha, is now considered as a primary treatment because of the disease's relatively high rate of steroid resistance. We present a case of deep oral ulcer and periorbital swelling in a 65-year-old woman. She was diagnosed with intestinal Crohn's disease 7 years ago, which was in remission after treatment with an immunosuppressive agent (azathioprine). The patient was given the diagnosed with orofacial Crohn's disease and successfully treated with infliximab.
6-Mercaptopurine/analogs & derivatives/therapeutic use ; Aged ; Anti-Inflammatory Agents/*therapeutic use ; Antibodies, Monoclonal/*therapeutic use ; Crohn Disease/diagnosis/*drug therapy ; Female ; Gastrointestinal Diseases/pathology ; Humans ; Immunosuppressive Agents/therapeutic use ; Oral Ulcer/diagnosis

Severe congenital neutropenia is a rare hematological disease characterized by a selective decrease in circulating neutrophils, maturation arrest of granulocytic precursors at the promyelocyte stage, and recurrence of infections. A 2-month-old male infant (patient A) and a 14-month-old female child (patient B) were referred to our hospital due to severe neutropenia. Sequencing analysis of ELA2 and HAX1 genes was performed. Two single nucleotide polymorphisms of HAX1 gene were found. They were 5,104T-->G point mutation of exon 1 and 5,474A-->G point mutation of intron 1 in HAX1 gene. The mutation of ELA2 gene was not found. The patient A showed a good response to granulocyte colony-stimulating factor (G-CSF) treatment and the absolute neutrophil count recovered to 1,195/microliter. But the patient B showed a partial response to G-CSF treatment and experienced several episodes of herpetic gingivostomatitis, oral ulcer, acute pharyngotonsillitis and otitis media during follow-up.
Adaptor Proteins, Signal Transducing/genetics ; Bone Marrow/pathology ; Female ; Granulocyte Colony Stimulating Factor, Recombinant/adverse effects/therapeutic use ; Humans ; Infant ; Male ; Neutropenia/congenital/drug therapy/*genetics ; Neutrophils/cytology/pathology ; Oral Ulcer/etiology ; Otitis Media/etiology ; Polymorphism, Single Nucleotide ; Serine Endopeptidases/genetics ; Stomatitis, Herpetic/etiology