1/19. Coexistence of psoriasis and linear iga bullous dermatosis.linear iga bullous dermatosis (LABD) is characterized by IgA autoantibodies against components of the basement membrane zone (BMZ). A 97-kDa protein is one of the major autoantigens associated with this disease. We report a 68-year-old man who developed LABD after a 3-year history of psoriasis and in the context of active hepatitis c virus infection. He had been treated with cyclosporin for psoriasis for about 9 months. Histologically, there was a subepidermal blister containing neutrophils and eosinophils with lymphocytes infiltrating predominantly in the dermis. Direct immunofluorescent staining showed linear IgA deposition at the BMZ. The patient's IgA autoantibodies bound exclusively to the epidermal side of 1 mol/L salt-split normal human skin. Immunoblot analysis identified a 97-kDa autoantigen in epidermal extracts. This appears to be the first case of LABD with IgA autoantibodies against a 97-kDa autoantigen, associated with psoriasis and hepatitis c virus infection.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/19. Pemphigoid: clinical, histologic, immunopathologic, and therapeutic considerations.Autoimmune blistering diseases are generally distinct entities characterized by relatively consistent clinical, histologic, and immunopathologic findings. These disorders may cause impaired adhesion of epidermis to epidermal basement membrane (eg, the pemphigoid group of disorders [bullous, gestational, and mucous membrane]) or impaired adhesion of epidermal cells to each other (eg, the pemphigus group of disorders). Recent studies have shown that these disorders are characterized by autoantibodies that often display pathogenic (ie, blister-forming) activity in passive transfer models. Interestingly, the autoantigens targeted by these patients' autoantibodies represent important structural proteins that promote cell matrix (eg, pemphigoid) or cell-to-cell (eg, pemphigus) adhesion in skin. Autoimmune blistering diseases are characterized by substantial morbidity (pruritus, pain, disfigurement), and in some instances, mortality (secondary to loss of epidermal barrier function). Treatment with systemic immunosuppressives has reduced morbidity and mortality in patients with these diseases. JAMA. 2000;284:350-356- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
3/19. pemphigus with pemphigoid-like presentation, associated with squamous cell carcinoma of the tongue.A 53-year-old woman presented with an inoperable squamous cell carcinoma of the tongue associated with tense large bullae consistent with bullous pemphigoid, preceded by a prodrome of urticarial plaques. The histological findings showed a regenerating subepidermal blister with eosinophils and no acantholysis. Direct immunofluorescence study, however, showed positive staining for IgG and C3 throughout the epidermis consistent with pemphigus. The blistering eruption had no mucosal involvement and responded to low dose corticosteroids. Our patient may represent another presentation of a 'paraneoplastic pemphigus spectrum'.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
4/19. Pemphigoid nodularis associated with autoantibodies to the NC16A domain of BP180 and a hyperproliferative integrin profile.Pemphigoid nodularis, a rare variant of bullous pemphigoid, has clinical features resembling prurigo nodularis, with blisters arising from normal-appearing or nodular skin. The fine antigenic epitope of the autoantibodies and the mechanism accounting for the nodular phenotype has not been delineated. We describe a patient with pemphigoid nodularis that fulfilled the criteria of bullous pemphigoid by histopathologic examination and direct and indirect immunofluorescence studies. Immunopathologic examination also revealed in situ deposition and circulating autoantibodies of all IgG subclasses, except IgG3, and both light chains to the patient's skin basement membrane. By immunoblotting, the patient's IgG autoantibodies labeled BP180, BP230, and an unidentified 150-kd epidermal protein and mapped the BP180 epitope to the MCW-1, region 2 of the NC16A domain. The nodular plaque skin showed expression of alpha-6 and beta-1 integrin subunits, mediators of matrix-cell signaling and proliferation, at the basal and the suprabasal epidermis, a pattern found in psoriasis, which is the prototype of hyperproliferative dermatoses.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/19. A case of anti-p200 pemphigoid with autoantibodies against both a novel 200-kD dermal antigen and the 290-kD epidermolysis bullosa acquisita antigen.BACKGROUND: Anti-p200 pemphigoid with autoantibodies against the 200-kD dermal antigen has recently been identified. OBJECTIVES: Our patient showed small and tense blisters on her face and trunk. methods: immunoblotting (IB), using extracts of normal human epidermis and dermal skin, and immunoelectron microscopy (IEM), using normal human skin, were performed using the patient's serum. RESULTS: IB analysis showed that the patient's serum did not react with 180-kD bullous pemphigoid (BP180) or BP230 antigens; however, IgG autoantibodies in the patient's serum reacted with a 200-kD dermal antigen as well as the 290-kD epidermolysis bullosa acquisita (EBA) antigen. IEM showed that IgG antibodies in the patient's serum bound to the lamina lucida, as well as both the lamina densa and sublamina densa. After the treatment with prednisolone, the 290-kD protein reactivity decreased rapidly; however, the 200-kD protein band was still observed. CONCLUSION: We describe a rare case with immunoreactive autoantibodies against both a novel dermal 200-kD autoantigen and the 290-kD EBA antigen.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
6/19. Antibasement membrane zone antibodies in localized pretibial pemphigoid.To clarify the nosologic position of localized pretibial pemphigoid, Western immunoblotting analysis was carried out using the sodium dodecyl sulfate extracts of normal human epidermis. Sera from patients with localized pretibial pemphigoid and generalized bullous pemphigoid reacted with 220- to 240-kd polypeptide, which is a critical point for a definite diagnosis of bullous pemphigoid. This result suggests that localized pretibial pemphigoid belongs to the same nosologic position as bullous pemphigoid.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
7/19. Coexistence of pemphigus foliaceus and bullous pemphigoid. Demonstration of autoantibodies that bind to both the pemphigus foliaceus antigen complex and the bullous pemphigoid antigen.pemphigus and bullous pemphigoid are autoimmune blistering diseases of the skin characterized by circulating autoantibodies directed against the keratinocyte cell surface and the epidermal basement membrane zone, respectively. The coexistence of pemphigus and bullous pemphigoid is very uncommon. We describe a patient with pemphigus foliaceus who later developed bullous pemphigoid and show, by means of immunoprecipitation studies utilizing both cultured keratinocytes and suction blister epidermis, that our patient had circulating autoantibodies directed against both the pemphigus foliaceus antigen complex and the bullous pemphigoid antigen. This report is the first to demonstrate the coexistence of pemphigus foliaceus and bullous pemphigoid at the molecular level.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
8/19. Vesicular pemphigoid--ultrastructural and immunoelectron microscopic study.We report a patient with vesicular pemphigoid who showed clinically numerous small vesicles and figurate erythema over the trunk and extremities. Although large blisters were absent, immunofluorescent study revealed IgG and C3 deposition at the basement membrane zone of the lesional skin. The patient responded well to the combined use of corticosteroid and dapsone. Immunoelectron microscopy showed IgG and C3 deposition at the undersurface membrane of the basal cells and the lamina lucida. Electron microscopy of the nonvesiculated early erythematous skin showed small vacuoles and lacunae along the uppermost portion of the dermis. Although the clinical features of this patient and the good therapeutic response to dapsone were atypical, the results of immunopathology and immunoelectron microscopy were consistent with typical pemphigoid.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
9/19. Localized chronic pemphigoid of Brunsting-Perry. Ultrastructural localization of IgG and complement components.Immune electron microscopic data are presented of a typical case of localized chronic pemphigoid (LCP) of Brunsting-Perry. The peroxidase-antiperoxidase multistep method for the electron microscopic localization of immunoglobulins and complement components showed that IgG, C3, and C4 were distributed in the lamina lucida and on the undersurface of the basal keratinocytes. These findings support the concept that LCP, bullous pemphigoid, and benign mucosal pemphigoid are closely related diseases. Unlike in bullous pemphigoid, however, complement was occasionally found in this case of LCP beyond the basal lamina in the uppermost portion of the dermis. This finding may explain the occurrence of scar formation in LCP.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
10/19. epidermolysis bullosa acquisita: a clinical disorder of varied etiologies. Two cases and a review of immunologic and other reported findings.epidermolysis bullosa acquisita (EBA) is an acquired mechanobullous disorder characterized by traumatically induced bullae in skin subjected to frictional injury, i.e., acral extensor surfaces or any point of sufficient mechanical distress. Two new patients with EBA are reported. Histopathologic findings were subepidermal ballae with sparse superficial perivascular mononuclear cell inflammatory infiltrates. The periodic acid-Schiff (PAS)-positive basement membrane zone (BMZ) was split between the floor and roof of the blisters with most attached to the blister roof. Electron microscopy of normal skin from one patient revealed a bandlike zone of granular material within the dermis immediately beneath the basal lamina. Nonbranching filaments were embedded in the granular material. Electron microscopy of normal skin from the second patient revealed a markedly diminished density of anchoring fibrils but no granular material. Immunologic findings in skin biopsies were C'3, IgG, and other immunoglobulins in varying combinations in both cases and in all reported cases. Comparisons of direct immunofluorescent (IF) findings in nine reported cases with 223 cases of bullous pemphigoid (BP) suggest that BMZ deposits of IgG plus IgA and/or IgM occur more frequently in EBA than in BP. Indirect IF studies of patients' sera revealed antibodies to the BMZ in two samples of one of our two cases and in one of seven cases reported by others. While both EBA and BP characteristically yield positive IF findings in the BMZ, clinical, electron microscopic, and some immunopathologic findings indicate that the two diseases are distinct.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
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