1/91. Intercellular IgA dermatosis.We report three cases of intercellular IgA dermatosis (IAD) and review the literature. IAD is a spectrum of vesiculobullous or vesiculopustular diseases mediated by intercellular IgA deposition. The clinical picture may vary from a vesiculopustular eruption with centrifugal evolution mainly involving the trunk and extremities, to the typical picture of classic pemphigus variants (foliaceus, vegetans). Histologically, infiltrating polymorphonuclear cells (mainly neutrophils) are observed in the epidermis with formation of pustules and bullae at various levels. However cases with typical histological features of pemphigus (variants) are described. Direct immunofluorescence on perilesonal skin typically displays intercellular IgA deposition at different levels or throughout the epidermis and indirect immunofluorescence often detects low levels of circulating antibodies. The disease has been repeatedly reported in association with monoclonal IgA gammopathy. Most cases respond to dapsone. In some cases IgA is directed against known pemphigus antigens whereas their targets in other cases are newly discovered antigens (105 kD, 115 kD, 120 kD). We observe a heterogeneity within the clinical, histological and immunological characteristics of the disease. Many reported cases feature various combinations of these characteristics. We therefore consider IAD as a disease spectrum with IgA pemphigus (clinical and histological pemphigus) at one end and intercellular IgA vesiculopustular dermatosis at the other end.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/91. Scrape cytology of oral pemphigus. Report of a case with immunocytochemistry and light, scanning electron and transmission electron microscopy.BACKGROUND: pemphigus vulgaris is a disseminated disease of the skin and mucous membranes characterized by recurrent vesicular and bullous lesions due to the autoantigen belonging to the cadherin type of cell adhesion molecules. The presence of acantholysis associated with immunoglobulins in the intercellular spaces and on the cell membrane are diagnostic features. However, the appearance of smears from the oral cavity by scanning (SEM) and transmission electron microscopic (TEM) study as well as immunocytochemistry of cadherin does not appear to have been previously reported. CASE: A 67-year-old female developed erosion on her gingiva with severe pain. On oral examination, there were ulcerations on the palate, and the Nikolsky sign was positive. The characteristic cytologic findings from oral scrapes were high cellularity, a bloody background and a predominant cell population consisting of polygonal basal and parabasal cells with pronounced nucleoli. Also present were degenerative cell changes: e.g., cytoplasmic vacuoles and a homogeneous nuclear appearance. Immunocytochemical staining for IgG and cadherin gave a positive reaction in the intercellular spaces and on the cell membranes. The surface of cells in pemphigus vulgaris by SEM showed somewhat irregularly distributed microridges, and TEM revealed desmosomal attachments, degenerated tonofilaments with pronounced nucleoli and heterochromatin. As a result of cytodiagnosis, additional appropriate specimens were obtained at the time of the scraping for confirmatory immunocytochemistry for cadherin, SEM and TEM studies. CONCLUSION: The results demonstrate that a precise diagnosis of pemphigus vulgaris can be rendered on cellular material and cadherin immunocytochemistry obtained by scrape from the oral mucosa.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
3/91. Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour.A 42-year-old woman presented with oral and labial erosions, conjunctivitis, facial rash and lichenoid erythematous papules on the trunk. Paraneoplastic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an intra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Castleman tumour, the skin and mucosal inflammation gradually subsided over the next 12 months. However, due to irreversible pulmonary involvement the patient died of intractable respiratory distress 2 years after the onset of the disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high-dose intravenous immunoglobulins and thalidomide were ineffective. The diagnosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
4/91. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). Antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
5/91. Intercellular IgA dermatosis (IgA pemphigus)--two cases illustrating the clinical heterogeneity of this disorder.IgA pemphigus is rare but may be underdiagnosed. We describe two cases, a 50-year-old female with a pustular eruption resembling subcorneal pustular dermatosis and a 55-year-old male with a pruritic vesiculopustular eruption simulating dermatitis herpetiformis. They illustrate the clinical heterogeneity of IgA pemphigus which is likely to reflect differences in autoantigens, analogous to pemphigus vulgaris and pemphigus foliaceus. There is now evidence that IgA pemphigus encompasses at least two subgroups: a subcorneal pustular dermatosis (SPD)-type, (see case 1) characterized by subcorneal pustules and autoantibodies to desmocollin 1; and intra-epidermal neutrophilic dermatosis (IEN)-type cases (see case 2) which show intra-epidermal pustules and in whom the autoantigen may be desmoglein 3, the pemphigus vulgaris antigen.- - - - - - - - - - ranking = 1.5keywords = antigen (Clic here for more details about this article) |
6/91. Paraneoplastic pemphigus associated with bronchiolitis obliterans.Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease associated with underlying neoplasms. autoantibodies of PNP bind to the plakin family of cytoplasmic proteins and desmogleins of cell-surface target antigens. We describe a 36-year-old female patient with PNP who had non-Hodgkin's lymphoma, and who developed bronchiolitis obliterans and died of respiratory failure. autopsy findings confirmed luminal narrowing of bronchioles by scarring, which is a histopathologic features of bronchiolitis obliterans. After the onset of respiratory failure, the reaction of autoantibodies against the plakins detected by immunoprecipitation at the onset of PNP disappeared with negative immunofluorescence within the bronchial epithelium. It is thought that autoantibodies against some of these antigens play a role in causing acute inflammation of the respiratory epithelium. In treating PNP, the possibility of the patient developing the lethal complication bronchiolitis obliterans should be kept in mind. Furthermore, prevention of the initial autoantibody-mediated injury to the respiratory epithelium should be an important treatment goal.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/91. pemphigus vulgaris in adolescence: case report.pemphigus vulgaris is an uncommon mucocutaneous disease caused by autoantibodies against desmosomal antigens. It affects mainly middle-aged adults, and juvenile cases are rare. The authors present a case of pemphigus vulgaris in adolescence and review the literature. A 16-year-old girl showed oral and cutaneous lesions suggestive of pemphigus vulgaris. Histopathology and direct immunofluorescence of the oral mucosa confirmed the diagnosis and systemic steroid therapy was efficient in controlling the disease. The recognition of the oral lesions of pemphigus by the clinician, its early diagnosis, and prompt therapy are essential for a favorable prognosis.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
8/91. Lichenoid dermatitis in paraneoplastic pemphigus: a pathogenic trigger of epitope spreading?BACKGROUND: In select cases, lichen planus has been observed to be a paraneoplastic condition sometimes associated with paraneoplastic pemphigus, a disease featuring autoantibodies directed against plakin proteins, desmogleins 3 and 1, and a still uncharacterized 170-kd antigen. Epitope spreading describes the phenomenon where underlying chronic inflammation leads to the sequential recognition of new epitopes on self-proteins over time. OBSERVATIONS: Five of 6 patients diagnosed as having paraneoplastic pemphigus had concomitant clinical and histological features of lichen planus. In 1 patient, results of the initial indirect immunofluorescence on rat bladder were negative and only 2 of the 5 antigens were identified by immunoprecipitation. After 1 year of worsening disease, repeated testing confirmed the presence of antibodies directed against all 6 of the implicated antigens, supportive of our hypothesis that epitope spreading may occur in paraneoplastic pemphigus. CONCLUSIONS: lichenoid eruptions may predispose to an early evolutionary stage of paraneoplastic pemphigus. Cell-mediated autoimmunity at the dermoepidermal junction may promote the exposure of self-antigens and the development of subsequent and progressive humoral autoimmunity. As such, paraneoplastic pemphigus may demonstrate epitope spreading in a human, humoral-mediated autoimmune disease.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/91. Identification of desmoglein 1 as autoantigen in a patient with intraepidermal neutrophilic IgA dermatosis type of IgA pemphigus.In a 51-year-old female patient with intraepidermal neutrophilic IgA dermatosis (IEN) type of IgA pemphigus, circulating IgA, but not IgG, autoantibodies were detected to bind to the cell surface of the whole epidermis, being much stronger in the upper epidermis. In the patient's skin a heavy intraepidermal IgA staining was observed throughout the whole epidermis, accompanied by a weak IgG and a more prominent C3 staining. IgA from the patient's serum showed no reactivity either with epidermal proteins by immunoblot analysis, or with COS 7 cells transiently transfected with mammalian cell expression constructs containing full length human Dsc1, Dsc2 and Dsc3. Our patient's IgA specifically reacted with conformational epitopes of human desmoglein (Dsg) 1 but not Dsg 3, when studied in a previously established, here for IgA antibody detection modified enzyme-linked immunoabsorbent assay (ELISA) of baculovirus expression system. The immunoreactivity against keratinocyte cell surface was completely removed from the serum of the patient by pre-incubation with recombinant Dsg1 baculoprotein. This finding indicates that the sera possess only IgA antibodies against the extracellular domain of Dsg1 baculoprotein, but no antibodies against components of keratinocyte cell surface other than Dsg1. This is the first case of IgA pemphigus where Dsg1 has been identified as the autoantigen.- - - - - - - - - - ranking = 2.5keywords = antigen (Clic here for more details about this article) |
10/91. Paraneoplastic pemphigus associated with Castleman's tumour.We present a case of paraneoplastic pemphigus associated with Castleman's disease. Our patient had stomatitis and vulvar erosions followed by a cutaneous polymorphous eruption. Investigations for neoplasia disclosed a Castleman's tumour suggesting the diagnosis of paraneoplastic pemphigus. The diagnosis was supported by immunoblotting using an extract of cow tongue although keratinocytes extracts did not identify relevant target antigens. One year after surgical excision of the tumour the patient remained unwell with persistent buccal erosions and lymphopenia. This case is unusual because of the length of its evolution before the discovery of the Castleman's tumour. It is only the second occasion that the association between paraneoplastic pemphigus and Castleman's tumour has been reported. Our case emphasizes the usefulness of immunoblotting on cow tongue in suspected cases of paraneoplastic pemphigus.- - - - - - - - - - ranking = 0.5keywords = antigen (Clic here for more details about this article) |
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