ABSTRACT
Pemphigus vulgaris (PV) is an acquired autoimmune disease in which the disease characteristic antibodies are directed against the desmosomal transmembrane glycoprotein, desmoglein 3 (Dsg 3), resulting in flaccid blisters and erosions of skin and mucous membrane. Among various affected sites, ocular involvement may often persist or relapse even after remission of other mucocutaneous lesions, and also represent a higher morbidity. We describe such an example case of mucosal PV, whose oral and ocular manifestations were responded specifically to oral cyclosporine and mizoribine, respectively. To our knowledge, this is the first case of the site-specific efficacy of mizoribine in PV.
Subject(s)
Cyclosporine/administration & dosage , Immunosuppressive Agents/administration & dosage , Pemphigoid, Benign Mucous Membrane/drug therapy , Pemphigus/drug therapy , Ribonucleosides/administration & dosage , Administration, Oral , Biopsy , Fluorescent Antibody Technique , Humans , Male , Middle Aged , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/immunology , Pemphigus/diagnosis , Pemphigus/immunology , Remission Induction , Treatment OutcomeABSTRACT
Behcet's disease (BD) is a mysterious multisystemic disorder characterized by recurrent involvement of mucocutaneous (including recurrent aphthous stomatitis; RAS), ocular, intestinal, vascular, and/or nervous system organs. Previously, the positivity of "pathergy test", which is one of the diagnostic examinations, was reported to be related to the possession of HLA-B51 gene in BD patients, even though the positivity is low and different from the countries. Here, instead of the ordinal pathergy test, we would like to propose the prick with self-saliva as a new diagnostic way for patients with RAS of BD based on the genetic intrinsic factors including HLA-B51 and extrinsic triggering factors. BD patients are considered to acquire the hypersensitivity against oral streptococci through the innate immune mechanism in the oral cavity. Bes-1 gene and 65 kD of heat shock protein (HSP-65) derived from oral S. sanguinis are supposed to play important roles as extrinsic factors in BD pathogenesis. Although the prick positivity was not related to the possession of HLA-B51 gene, the method is suggested to be a significant way for BD diagnosis. The results also suggest that BD symptoms are due to the vascular immune responses by monocytes expressed oral streptococcal agents of the patients.
Subject(s)
Dermis/pathology , Hair Follicle/pathology , Neoplasms/pathology , Pilomatrixoma/pathology , Skin Neoplasms/pathology , Adolescent , Arm , Child , Female , Humans , Male , Young AdultABSTRACT
Benign fibrous histiocytomas of the skin sometimes extend into the deeper dermis with higher cellularity and show more aggressive clinical courses in comparison with typical dermatofibromas. They may occur either as a true neoplasm or in a reactive process. We describe a case of fibrous histiocytoma which was triggered by tuberculin skin test.
