1/61. 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 = epidermis (Clic here for more details about this article) |
2/61. Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature.Pyodermatitis-pyostomatitis vegetans is a benign, rare disorder characterized by a pustular eruption in the oral mucosa and vegetating plaques involving the groin and axillary folds. Its association with inflammatory bowel disease is well established. We report the case of a 49-year-old-white man with ulcerative colitis who manifested a vegetating, annular plaque in the left inguinal region of 2 months' duration. Oral examination disclosed an erythematous mucosa with multiple painful pustules involving the labial and gingival mucosa. Histopathologic study demonstrated epidermal hyperplasia and an inflammatory infiltrate composed mostly of neutrophils and eosinophils, grouped into microabscesses within the epidermis and with a bandlike configuration in the upper dermis. Results of direct and indirect immunofluorescence studies were negative. We discuss the differential diagnosis between pyodermatitis-pyostomatitis vegetans and pemphigus vegetans.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
3/61. pemphigus vulgaris preceded by herpetiform-like skin lesions with negative immunofluorescence findings.We report a case of pemphigus vulgaris in a 63-year-old woman. At the first onset, she developed herpetiform-like skin lesions. Their histology showed eosinophilic spongiosis and intraepidermal blister formation with an infiltrate of eosinophils. Both direct and indirect immunofluorescence studies were negative. She remained in remission for 6 years. At the second onset, she developed bullous skin lesions; histological examination disclosed suprabasal acantholysis and infiltration of eosinophils. Direct immunofluorescence revealed IgG and C3 deposits at the cell surface, predominantly in the lower epidermis. Indirect immunofluorescence using normal human skin as a substrate demonstrated anti-cell surface IgG antibodies at a titer of 1:160. Immunoblot analysis showed that the patient's serum reacted only with a 130 kD protein. This case indicates that pemphigus vulgaris can follow herpetiform-like skin lesions with negative immunofluorescence findings after a long remission time.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
4/61. pemphigus vulgaris localized to the vagina presenting as chronic vaginal discharge.Involvement in pemphigus vulgaris of the female genital tract including the vulva, vagina and cervix has previously been described. In all these cases other cutaneous and mucosal sites have also been affected at some time. We describe a case of pemphigus vulgaris which only involved the vaginal mucosa. The patient presented with a persistent vaginal discharge and examination showed extensive vaginal erosions. histology of vaginal biopsies was non-diagnostic. The recognition that the vaginal changes may represent an immunobullous disease led to further vaginal biopsies on which direct immunofluorescence studies were performed. These biopsies showed IgG and C3 in the intercellular epidermis, suggesting a diagnosis of pemphigus vulgaris. During the 3-year period that the patient has been under review there have never been any other cutaneous or mucosal lesions. To our knowledge, this is the first case of pemphigus vulgaris localized exclusively to the vaginal mucosa. There was considerable delay in diagnosis and this case highlights how important it is to recognize that chronic mucosal lesions at genital sites may be caused by immunobullous diseases such as cicatricial pemphigoid and pemphigus, and to institute appropriate investigations.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
5/61. A child with localized vulval pemphigoid and IgG autoantibodies targeting the C-terminus of collagen XVII/BP180.Localized vulval pemphigoid of childhood (LVPC) has previously been reported in six girls. Clinical features and immunopathological data have suggested it to be a morphological variant of bullous pemphigoid. Epitope targets of the autoantibodies of these patients have not been defined in detail. We describe a 9-year-old girl with possible cicatricial LVPC and circulating IgG antibodies directed against native collagen XVII/BP180, its 120-kDa soluble ectodomain and against the C-terminus of collagen XVII/BP180. No reactivity was detected towards the NC16A domain of collagen XVII/BP180. Linear IgG and C3 deposits were found along the cutaneous basement membrane zone. On 1 mol/L salt-split skin, IgG autoantibodies were shown to bind to the epidermis, and the HLA type II allele DQB1*0301, a marker with significantly increased occurrence in patients with ocular and oral cicatricial pemphigoid, was identified in this patient. Our data suggest that LVPC is a variant of bullous pemphigoid in which direct immunofluorescence microscopy combined with immunoblot analysis can deliver valuable diagnostic information for differential diagnosis. However, differentiation between the scarring and non-scarring course of the disease cannot be made with the present diagnostic markers and therefore careful follow-up of patients with LVPC is required.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
6/61. Induction of keratinocyte IL-8 expression and secretion by IgG autoantibodies as a novel mechanism of epidermal neutrophil recruitment in a pemphigus variant.A subset of pemphigus herpetiformis, a rare pemphigus variant, is characterized histopathologically by subcorneal acantholysis and neutrophilic infiltration. The mechanism of neutrophil infiltration is unknown, but chemokines such as IL-8 may play a role. We investigated the possible role of IL-8 in two such cases. Direct and indirect immunofluorescence studies demonstrated in vivo-bound and circulating IgG epithelial cell surface-binding autoantibodies, both predominated by IgG4 subclass. ELISA and immunoblotting studies revealed that the patients' IgG autoantibodies recognized recombinant desmoglein 1 but not desmoglein 3. Preadsorption of the patients' sera with recombinant desmoglein 1 completely removed the epidermal cell surface immunostaining. Significantly, immunohistochemistry demonstrated intense expression of IL-8, co-localized with in vivo-bound IgG, in the upper epidermis, where the acantholysis took place. Affinity-purified sera IgG from these two patients, a normal individual, and a pemphigus vulgaris patient containing desmoglein 1 autoantibodies, were incubated with normal human keratinocytes in vitro. cells treated with these patients' IgG secreted a seven-to-nine-fold increase of IL-8 (30-37 pg/ml) compared with the controls (2-4 pg/ml) and expressed a higher intensity of cytoplasmic IL-8 staining. These data demonstrate a novel functional role for IL-8 in the pathogenesis of the neutrophil-dominant subset of pemphigus herpetiformis. The autoantibody-induced epidermal cell IL-8 expression may represent a novel mechanism of epidermal neutrophil recruitment.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
7/61. Oral acantholytic itching disease responding to dapsone. dermatitis herpetiformis, pemphigus, or a new disease?A patient had a blistering and severely itching disease confined solely to the oral cavity. The histopathologic findings had the features of both pemphigus and dermatitis herpetiformis. There were no gastrointestinal symptoms, no IgA could be found in uninvolved skin, and no intercellular or basement membrane antibodies were present in the serum or epidermis. The disease responded favorable to dapsone (diaminodiphenylsulfone), which could be discontinued after 2 1/2 years. The literature is briefly reviewed, and there is a discussion of whether the patient was suffering from pemphigus or dermatitis herpetiformis. The conclusion is drawn that the diagnosis cannot be established with certainty and that we are perhaps dealing with a new disease. It is stressed that in such aberrant cases a therapeutic trial with dapsone should be made.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
8/61. pemphigus foliaceus successfully treated with mycophenolate mofetil as a steroid-sparing agent.pemphigus foliaceus is an autoimmune blistering disease of unknown origin with antibodies produced against desmoglein 1, an adhesive protein found in the desmosomal cell junction in the suprabasal layers of the epidermis. The disease is primarily treated with corticosteroids and corticosteroid-sparing immunosuppressive agents. We report a case of pemphigus foliaceus successfully treated with mycophenolate mofetil. It remains to be seen whether this agent has a significant effect on the course of the disease and remission induction.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
9/61. Use of mycophenolate mofetil in the treatment of paraneoplastic pemphigus.Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease with circulating antibodies that bind the cell surface of the epidermis and other non-stratifying epithelia, and immunoprecipitate a complex of four or five proteins (250 kDa, 230 kDa, 210 kDa, 190 kDa and occasionally 170 kDa).1,2 Combinations of immunosuppressive agents are usually required to obtain even partial control of the skin lesions.3 mucous membrane lesions are refractory to treatment. We describe a patient with PNP whose skin and oral lesions are quiescent following treatment with oral mycophenolate mofetil.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
10/61. 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 = 1.5keywords = epidermis (Clic here for more details about this article) |
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