1/9. pityriasis rubra pilaris with acantholysis and lichenoid histology.Acantholytic foci have been reported several times in pityriasis rubra pilaris (PRP). Lichenoid tissue reactions were also mentioned in the literature regarding PRP. We report a 58-year-old patient who, after having colon cancer, had PRP with biopsies showing acantholytic lesions and a heavy lichenoid lymphocytic infiltration. Investigation by serial sectioning of the acantholytic lesion suggested an involvement of the intraepidermal eccrine duct and further investigation with carcinoembryonic antigen (CEA) staining demonstrated a CEA-positive eccrine duct in the acantholytic foci. We suggest that acantholysis in PRP is induced by proteolytic enzymes, urea, and other substances in eccrine sweat in keratin-plugged acrosyringia. This patient had a combination of three relatively rare features of PRP-acantholysis, lichenoid reaction, and a cancer background.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/9. A localized variant of paraneoplastic pemphigus: acantholysis associated with malignant melanoma.We report a 72-year-old male patient with a nodular malignant melanoma that was associated with focal suprabasal acantholysis (FSA). This phenomenon, which is regarded as an incidental finding by dermatopathologists, may be associated with inflammatory and also neoplastic skin diseases. Haematoxylin and eosin stained sections from an erythematous plaque surrounding the patient's tumour showed FSA, direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) on normal human skin, monkey oesophagus and rat urinary bladder were negative. On electron microscopy few desmosomes could be detected in the basal cell layer of the acantholytic areas and there was a nearly complete loss of these structures in the spinous cell layer. Only remnants of cytoplasmic plaques and keratin filaments could be observed in those areas. In contrast, adherens junctions appeared to be well preserved. An enzyme-linked immunosorbent assay (ELISA) using recombinant fusion proteins as antigens did not show circulating autoantibodies against desmoglein 1 (Dsg1) or desmoglein 3 (Dsg3). In contrast, immunoblotting revealed autoantibodies directed against keratinocyte antigens with a molecular weight of 85 kDa and 250 kDa, the first band corresponding to the molecular weight of comigrating plakoglobin. immunoprecipitation with patient serum also revealed a 85-kDa band. We conclude that these autoantibodies, probably in conjunction with cofactors produced by the tumour, could play a part in the pathogenesis of this variant of FSA, for which we propose the term 'localized paraneoplastic pemphigus.'- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/9. IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis.A patient with clinical features of herpetiform pemphigus of the foliaceus type had histologic findings consistent with pemphigus. Intercellular IgA deposits in a pattern like that of IgG in pemphigus were present. Circulating pemphigus-type IgA-class antibodies reacted first only with guinea pig and later in the disease also with monkey esophagus sections. IgG-class pemphigus antibodies were blocked by the IgA-class antibodies of this patient. In addition, the IgA-class pemphigus antibodies in this patient were blocked by the IgG-class pemphigus antibodies in tests on guinea pig and monkey esophagus. This indicates that the IgA-class antibodies in this patient were directed either to the same antigen as the IgG-class pemphigus foliaceus antibodies or to one that is close enough to it to give steric hindrance. The skin lesions responded poorly to systemic corticosteroid therapy. dapsone therapy initially produced dramatic improvement, but the condition flared to the point that plasmapheresis, in addition to high doses of corticosteroids and cyclophosphamide, had to be used to control it.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/9. Can pemphigus vulgaris become pemphigus foliaceus?Among 31 patients with pemphigus, we observed two women with clinical and histologic features characteristic of pemphigus vulgaris that later became those of pemphigus foliaceus. Western blot study indicated that serum from one patient, when she had the cutaneous manifestations of pemphigus foliaceus, contained antibodies reactive with a desmosomal antigen, suggestive of desmoglein 1, the same as recognized by pemphigus foliaceus serum. The present study suggests that two distinct types of pemphigus can occur in the same person simultaneously or separately.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/9. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia.BACKGROUND and methods. We describe five patients with underlying neoplasms in whom painful mucosal ulcerations and polymorphous skin lesions developed, usually with progression to blistering eruptions on the trunk and extremities. Histologic examination showed vacuolization of epidermal basal cells, keratinocyte necrosis, and acantholysis. Immunofluorescence testing revealed atypical pemphigus-like autoantibodies in perilesional epithelium and serum from all five patients. We studied the antigenic specificities of the autoantibodies by indirect immunofluorescence and immunoprecipitation, using extracts of 14C-labeled human keratinocytes. IgG purified from the serum of one patient was passively transferred to four neonatal mice to test for pathogenicity. RESULTS. Immunofluorescence testing showed that the autoantibodies bound to the surface of tissues containing desmosomes, including complex and simple epithelia, and myocardium. An identical and unique complex of four polypeptides with molecular weights of 250, 230, 210, and 190 was immunoprecipitated by all serum samples. The 250-kd polypeptide comigrated with desmoplakin I (a protein found in the desmosomes of all epithelia), and the 230-kd antigen comigrated with the antigen of bullous pemphigoid. Cutaneous blisters, a positive Nikolsky's sign, and epidermal and esophageal acantholysis developed in all mice into which the autoantibody was injected. Electron microscopy showed epidermal acantholysis similar to lesions of experimentally induced pemphigus vulgaris. CONCLUSION. These five patients with cancer had a novel acantholytic mucocutaneous disease characterized by autoantibodies that were pathogenic after passive transfer. The autoantibodies from these patients reacted with an antigen complex composed of desmoplakin I and the 230-kd antigen of bullous pemphigoid and two as yet unidentified epithelial antigens. We suggest the term "paraneoplastic pemphigus" for this disease.- - - - - - - - - - ranking = 6keywords = antigen (Clic here for more details about this article) |
6/9. Paget's disease of the nipple resembling an acantholytic disease on microscopic examination.Two biopsies of an erosive lesion of the nipple had an appearance of an acantholytic disease without showing malignant cells. Only a third biopsy through the nipple with removal of a larger portion revealed some nests of atypical, large cells with clear cytoplasm, typical of Paget's disease. Immunohistochemical findings with carcinoembryonic antigen confirmed the diagnosis of Paget's disease of the nipple. This is the first case of Paget's disease which shows extensive acantholysis on microscopic examination and which resembles pemphigus vulgaris histologically. Acantholytic diseases are easily distinguished from Paget's disease and have never been mentioned in the differential diagnosis of this disease. A large biopsy through the nipple with the removal of a liberal portion of the nipple is suggested in every case of a suspected unilateral lesion of the nipple in order to avoid the overlooking of small nests of Paget's cells, as in our first biopsies, showing a histological picture of an acantholytic disease.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/9. Bullous transient acantholytic dermatosis.We have been three cases of a bullous variant of transient acantholytic dermatosis. Skin biopsy specimens showed histopathologic changes identical to pemphigus foliaceus, but immunofluorescent studies showed no tissue-fixed or circulating antibodies to intercellular antigens. The bullous eruption lasted a few weeks to several months and healed without scarring or recurrence. The lesions healed with topical and/or systemic corticosteroid therapy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/9. Paraneoplastic pemphigus triggered by radiotherapy.Paraneoplastic pemphigus is a recently described autoimmune disease characterized by painful mucosal ulceration and polymorphous skin lesions in association with an underlying neoplasm. Distinct autoantibodies bind desmoplakin I, desmoplakin II, bullous pemphigoid antigen and an uncharacterized 190 kDa antigen. A case is presented of paraneoplastic pemphigus that developed after radiotherapy for non-Hodgkin's lymphoma in a 53 year old man. Multiple skin biopsies showed a lichenoid reaction without acantholysis. Immunofluorescence and mucosal biopsies were required to establish the correct diagnosis. Corneal opacities resembling lichenoid graft-versus-host disease and retinal haemorrhages, which developed in the patient, have not been previously documented. Despite high doses of immunosuppressive agents and plasmaphoresis, the patient eventually died from respiratory failure.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/9. Sudoriferous acrosyringeal acantholytic disease. A subset of Grover's disease.Three selected cases of transient acantholytic dermatosis were studied because of their definitive correlation with sweating due to fever and/ or bed-ridden situations. biopsy specimens were serially sectioned and acantholysis was found in the acrosyringium or traced to connect to the acrosyringium in all biopsy specimens. carcinoembryonic antigen (CEA) and eccrine gland-specific monoclonal antibody, IKH-4, were positive in acantholytic cells. Electron microscopy revealed electron dense material filling the lumen of intraepidermal eccrine ducts. This material leaked into lateral intercellular spaces of the luminal cells, passing tight junctions. Marked edema and numerous lysosomes were reminiscent of those found when eccrine acrosyringium is formed in the embryo; this suggested that an occluded and damaged eccrine intraepidermal duct was being rebuilt via lysosomal digestion.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |