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a dozen entities is increasingly important since more diverse treatment options are employed. Exact diagnosis is also pivotal for the prognosis, since some autoimmune bullous diseases may indicate an underlying tumor. Association with a malignancy has been shown in paraneoplasticpemphigus (in 100%) and anti-laminin 332 mucous pemphigoid (in 25%) In pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, autoantibodies to desmoglein 3, desmoglein 1, and BP180, respectively, have been shown
immunofluorescence (IIF), and enzyme-linked immunosorbent assay (ELISA) quantification of anti-BP180-NC16a and anti-BP230, or antidesmoglein (Dsg) 1 and 3 antibodies. There were 56 cases of bullous pemphigoid (BP), 22 cases of mucous membrane pemphigoid (MMP), eight cases of epidermolysis bullosa acquisita (EBA), two cases of bullous systemic lupus erythematosus (BSLE), 17 cases of pemphigus vulgaris (PV), and four cases each of pemphigus foliaceus (PF) and paraneoplasticpemphigus (PNP).In BP, the three methods (...) had similar sensitivity (84-89%) for both anti-BP180-NC16a and anti-BP230 antibody detection. In MMP, autoantibodies (mainly directed against BP180 or laminin 332 subunits) were detected in 77% of patients by IB, compared with only 9% by IIF on rat and monkey oesophagus and 36% on salt-split skin, and 14% by anti-BP180-NC16a and anti-BP230 ELISA. In patients with pemphigus, ELISA had 92% sensitivity for anti-Dsg1 and 3, but IB and rat bladder IIF were necessary to confirm PNP by revealing specific
. . Bullous phototoxic reactions. Chemical or thermal burns. . . Maculopapular drug rashes. Paraneoplasticpemphigus acantholysis. . . . Investigations Serum electrolytes, glucose and bicarbonate are essential to assess clinical severity and level of dehydration. [ ] Diagnosis is based on clinical classification - as in the table, above - and on histopathology. Skin biopsy: demonstrates that the bullae are subepidermal. Epidermal cell necrolysis may be seen and perivascular areas are infiltrated
bullae are subepidermal. Three major variants of the disorder have been described, each with characteristic clinical and immunological features: Pemphigus vulgaris (PV) is the most common subset or variant and accounts for 70% of cases of pemphigus. Pemphigus foliaceus (PF) is characterised by lesions which occur only in the skin and associated with antibodies to desmoglein 1 (DSG1). Pemphigus herpetiformis, IgA pemphigus, paraneoplasticpemphigus and IgG/IgA pemphigus are rarer forms (...) lesions. Pemphigus foliaceus (PF): Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. Paraneoplasticpemphigus (PNP): It may present in patients who are already known to have an underlying neoplasm such as the B-cell lymphoproliferative disorders (eg, non-Hodgkin's lymphoma and chronic lymphocytic leukaemia). It may present with mucocutaneous lesions before any underlying tumour has been diagnosed. Patients present with painful ulcers on their mucous
pemphigoid where bullae are subepidermal. Three major variants of the disorder have been described, each with characteristic clinical and immunological features: Pemphigus vulgaris (PV) is the most common subset or variant and accounts for 70% of cases of pemphigus. Pemphigus foliaceus (PF) is characterised by lesions which occur only in the skin and associated with antibodies to desmoglein 1 (DSG1). Pemphigus herpetiformis, IgA pemphigus, paraneoplasticpemphigus and IgG/IgA pemphigus are rarer forms (...) lesions. Pemphigus foliaceus (PF): Presents with lesions on the skin only, and these patients will not go on to develop oral blisters. Paraneoplasticpemphigus (PNP): It may present in patients who are already known to have an underlying neoplasm such as the B-cell lymphoproliferative disorders (eg, non-Hodgkin's lymphoma and chronic lymphocytic leukaemia). It may present with mucocutaneous lesions before any underlying tumour has been diagnosed. Patients present with painful ulcers on their mucous
Retroperitoneal liposarcoma associated with small plaque parapsoriasis Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplasticpemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis (...) syndrome was done.Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma.
to tolerate protocol therapy History of mucocutaneous reactions (paraneoplasticpemphigus, Stevens-Johnson syndrome, lichenoid dermatitis, vesiculobullous dermatitis, and toxic epidermal necrolysis) Known anaphylaxis or IgE mediated hypersensitivity to murine proteins or to any component of this product Inability to self inject the study medication or to have it administered by a third person Inability to understand the investigational nature of the study ability to provide informed consent Contacts
, organomegally, endocrinopathy, monoclonal gammopathy, and skin changes), paraneoplasticpemphigus, Hodgkin's disease, and follicular dendritic cell sarcoma. We present a case of Castleman's disease associated with myasthenia gravis, the third reported case in the literature. We discuss Castleman's disease and review the literature.
therapy, high-potency topical corticosteroids are very useful as initial treatment and, in the elderly, should be used instead of oral prednisolone wherever possible. Pemphigus is a chronic blistering disease of which there are two main subtypes: vulgaris and foliaceous. Paraneoplasticpemphigus is a unique clinical, histological and immunologically distinct autoimmune mucocutaneous disease which tends to be relentlessly progressive. Lichen sclerosus presents specific complications and a small
in 26 treatment-resistant patients with the vulgaris, foliaceus, and paraneoplastic variants of pemphigus as well as in bullous pemphigoid and epidermolysis bullosa acquisita. All but a single patient showed clinical improvement with reduction of lesion formation. In about a third, a clinical remission requiring further immunsuppressive medication was achieved, and in about a quarter, complete remission was induced. In addition, the mode of action and adverse events of rituximab as well as adjuvant
PNP with Waldenström's macroglobulinaemia. We describe a 65-year-old Caucasian man with Waldenstrom's macroglobulinaemia who developed paraneoplasticpemphigus (PNP) 3 years after his haematological diagnosis. This is a very rare malignancy that is associated with PNP. The evolution of PNP in this patient appears to exhibit the postulated immunological phenomenon of epitope spreading.
Sera from patients with toxic epidermal necrolysis contain autoantibodies to periplakin. The pathophysiological mechanism of toxic epidermal necrolysis (TEN) with extensive bullae that is induced suddenly by drugs is not well understood. The individual patterns and distribution of the widespread mucocutaneous reactions of TEN often show striking similarities with those of paraneoplasticpemphigus (PNP), which is known to involve autoantibodies (aAbs) to members of the plakin family.To
Rituximab: applications in dermatology. Rituximab is a chimeric anti-CD20 monoclonal antibody which has been used extensively for B-lymphocytic malignancies. In addition, applications for autoimmune diseases have emerged in recent years. Case reports support the use of rituximab in certain dermatologic conditions, including paraneoplasticpemphigus, pemphigus vulgaris, graft versus host disease, and cutaneous B-cell malignancies. Clinical trials are lacking and would be an appropriate next step.
to desmosomal and BMZ proteins, and with sera from 76 patients with AIBDs including pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid (BP), pemphigoid gestationis, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, paraneoplasticpemphigus and mucous membrane pemphigoid.The desmosomes and BMZ of the amnion tissue were ultrastructurally similar to those in skin. Antigen mapping confirmed that amnion contains all the proteins that were recognized by a panel of monoclonal and polyclonal
pemphigus. It shares some heterogeneous immunoreactivity with paraneoplasticpemphigus but is not associated with malignant tumors. The disease resembles Senear-Usher syndrome (pemphigus and lupus) but occurs endemically either with a localized stable clinical course or in a systemic form. This systemic form may affect organs other than skin and is characterized by episodic relapses and poor prognosis in comparison with the localized form.We have confirmed endemic pemphigus foliaceus in El Bagre (...) as an autoimmune disease that shares features with Senear-Usher syndrome but occurs in an endemic fashion. Heterogeneous antigenic reactivity is observed as in paraneoplasticpemphigus but with no evidence of association with neoplasia. In addition, constant exogenous antigenic stimulation and a genetic predisposition may be required in the pathogenesis of this disease.
proteins with molecular weights of 250 kd, 210 kd, and 190 kd, which appear to correspond to desmoplakin I, envoplakin, and periplakin, respectively.This endemic pemphigus disease in El Bagre showed immunologic features similar to pemphigus foliaceus or erythematosus. In addition, paraneoplasticpemphigus-like reactivity with various epidermal antigens was detected. (...) Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. We previously described a new focus of endemic pemphigus foliaceus in rural areas of El Bagre, Colombia, with clinical and direct immunofluorescence characteristics of pemphigus erythematosus.The aim of this study was to characterize autoantigen profiles for 34 serum samples obtained from patients with this condition.Immunofluorescence, various immunoblot analyses with different antigen sources and detection
Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies. Anti-desmoplakin (DP) antibodies are present in paraneoplasticpemphigus (PNP) as a component of a complex humoral autoimmune reaction characterized by antibodies against proteins of the plakin family, desmogleins, and an unidentified 170 kd protein. Anti-DP antibodies have also been rarely identified in other blistering diseases. The significance of anti-DP antibodies in the pathogenesis of bullous diseases is unclear.We (...) studied 3 patients with severe and chronic mucosal dominant pemphigus vulgaris (PV). In addition to anti-desmoglein 3 antibodies, these patients had anti-DP autoantibodies, demonstrable by immunofluorescence (IF), immunoprecipitation (IP), and indirect immunoelectromicroscopy (IIEM). This finding suggested these patients may have had PNP and not PV. However, antibodies against periplakin, envoplakin, bullous pemphigoid antigen 1 (BPAG 1), plectin, and 170 kd PNP antigen could not be detected using IP
Antidesmoplakin antibodies in pemphigus vulgaris. Besides being present in paraneoplasticpemphigus (PNP), circulating antidesmoplakin (DP) antibodies have been found anecdotally in other bullous diseases, including pemphigus foliaceus and pemphigus vulgaris.To verify how frequent anti-DP antibodies are in pemphigus vulgaris.We studied 48 sera from patients with proven pemphigus vulgaris (29 mucosal dominant pemphigus and 19 mucocutaneous pemphigus) by indirect immunofluorescence (IIF) with rat (...) , which, in patients with pemphigus vulgaris, are probably caused by an epitope-spreading phenomenon.
pemphigus.Patients with active, refractory pemphigus were treated with MMF. Our series included 12 cases of pemphigus vulgaris, four cases of pemphigus foliaceous and one case of paraneoplasticpemphigus. All patients were monitored to assess disease control and mycophenolate toxicity.Of the 17 cases, MMF has been of benefit to 12. MMF was well tolerated and there were no treatment withdrawals because of safety concerns.We found that MMF permitted a reduction in prednisolone dosage without disease relapse. (...) An evaluation of the usefulness of mycophenolate mofetil in pemphigus. Pemphigus is a group of autoimmune blistering diseases of the skin and/or mucous membranes requiring management with immunosuppressive therapy. The optimal therapeutic regimen would rapidly induce remission and maintain effectiveness with minimal adverse effects in the long term.The present study describes our experience of the addition of mycophenolate mofetil (MMF) to prednisolone in the management of severe, refractory
myeloma. From the results of immunoblotting using normal human epidermal extracts and indirect immunofluorescence using rat bladder sections, and her clinical manifestations, our case does not seem to be one of paraneoplasticpemphigus. (...) A case of pemphigus vulgaris accompanied by multiple myeloma. Pemphigus is a mucocutaneous intraepithelial blistering disease caused by autoantibodies to epithelial cell adhesion molecules (desmoglein). The association between pemphigus and malignant neoplasm is well recognized. We present the case of a 62-year-old woman with pemphigus vulgaris accompanied by multiple myeloma. To the best of our knowledge, this is the first report of a case of pemphigus vulgaris concomitant with multiple