1/33. 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 = 1keywords = membrane (Clic here for more details about this article) |
2/33. impetigo herpetiformis in a primigravida: successful treatment with etretinate.impetigo herpetiformis (IH) is a rare dermatosis which usually occurs during the third trimester of pregnancy. It is characterized by acute erythematosquamous plaques covered with tiny superficial pustules in a herpetiform distribution with less likely mucus membranes involvement. It can be associated with constitutional symptom s such as fever, chills, nausea, vomiting and diarrhea. impetigo herpetiformis can cause serious complications to the mother and fetus which include: maternal hypocalcemia leading to delirium, convulsions, and tetany in the mother, and placental insufficiency leading to still birth, neonatal death or fetal abnormalities. Lesions are expected to disappear after birth but may recur during subsequent pregnancies at an earlier gestational age. Presented here is a case of IH occurring during the 37th week of gestation in a primigravida who failed to respond to oral steroid but successfully cleared with oral etretinate.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
3/33. dermatitis herpetiformis presenting as chronic urticaria.Childhood dermatitis herpetiformis (DH) is an immunobullous disease associated with gluten-sensitive enteropathy. This disease is rare in children and is typically characterized by intensely pruritic vesicles on the extensor surfaces. Definitive diagnosis of DH depends on the direct immunofluorescence finding of granular or fibrillar IgA deposits along the basement membrane zone of biopsied perilesional skin. We report an 11-year-old boy with an unusual presentation of DH characterized by a 7-month history of chronic urticaria-like skin lesions. He had evanescent, largely asymptomatic, urticarial wheals on his trunk, face, and extremities that were unresponsive to conventional therapy for urticaria. skin biopsy specimen findings were consistent with DH and direct immunofluorescence of perilesional skin was diagnostic. The patient had no symptoms of gluten-sensitive enteropathy at the time of diagnosis, and his skin lesions rapidly cleared with dapsone therapy. This patient serves to highlight an unusual presentation of childhood DH and the need to consider this diagnosis when evaluating chronic urticarial lesions in children.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
4/33. Linear IgA disease and chronic active hepatitis--a coincidence or not?Linear IgA disease (granular type) associated with chronic active hepatitis in a sixteen-year-old male patient is described. The diagnosis was established by clinical and histopathological findings supported by immunofluorescence studies. The immunofluorescence pattern (granular IgA deposits along the basement membrane, more pronounced at tips of papillae) showed much transition in dermatitis herpetiformis and also to a lesser extent in pemphigoid (linear IgG and IgM deposits along the basement membrane). Chronic active hepatitis as linear IgA disease are significantly correlated with HLA B8. This is the first report of the association of linear IgA disease and chronic active hepatitis.- - - - - - - - - - ranking = 2keywords = membrane (Clic here for more details about this article) |
5/33. Bullous pemphigoid and dermatitis herpetiformis: mixed bullous disease or coexistence of two separate entities?We present a 73-year-old man with a 5-year history of dermatitis herpetiformis who developed lesions with the clinical, histologic, and immunologic features of bullous pemphigoid. Direct immunofluorescence testing of a skin biopsy demonstrated both granular deposition of IgA, predominantly in the papillary bodies, and linear deposition of IgG and C3 at the basement membrane zone. This mixed direct immunofluorescence pattern, typical for dermatitis herpetiformis in the type of IgA deposits, but also typical for pemphigoid in the linear localization of IgG and C3, is unusual. This case emphasizes that even after a specific diagnosis has been established, if the clinical morphology or response to therapy changes, repeat histologic and immunofluorescence studies may be indicated in diagnosis and management of patients with bullous disease.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
6/33. linear iga bullous dermatosis mimicking erythema multiforme in adult.This report describes a 49-year-old woman with an erythema multiforme--like rash and direct immunofluorescence showing linear IgA deposits at the dermoepidermal junction. light microscopy revealed features of bullous pemphigoid, dermatitis herpetiformis, and erythema multiforme; immunoelectron microscopy demonstrated IgA deposition beneath the lamina densa about anchoring fibrils. These data provide additional information about the variable clinical and histologic manifestations of the adult linear iga bullous dermatosis and emphasize the diagnostic dilemmas of light microscopy, which are resolved by immunohistochemical methods.- - - - - - - - - - ranking = 6.2273448306004keywords = lamina (Clic here for more details about this article) |
7/33. Circulating IgA anti-basement membrane antibodies in linear dermatitis herpetiformis (Duhring): immunofluorescence and immunoelectronmicroscopic studies.IgA deposits were observed by direct immunofluorescence in linear distribution along the basement membrane zone in a case of dermatitis herpetiformis (Duhring). In addition, in the serum of the same patient circulating IgA antibasement membrane zone antibodies were detected by indirect immunofluorescence, utilizing normal human skin and monkey esophagus as substrates. The ultrastructural localization of in vivo-bound IgA and circulating IgA antibasement membrane zone antibodies fixed to substrate tissue in vitro was found to be in the uppermost strata of the dermis below the basal lamina.- - - - - - - - - - ranking = 13.2273448306keywords = lamina, membrane (Clic here for more details about this article) |
8/33. Aggressive gluten challenge of dermatitis herpetiformis cases converts them from seronegative to seropositive for IgA-class endomysial antibodies.The responses to aggressive gluten challenge of two dermatitis herpetiformis patients with normal gut mucosa and negative serum findings for IgA endomysial antibodies while on normal diets indicate that these patients have a latent gluten-sensitive enteropathy. This was shown further by the control of skin lesions in both cases and in one case by the clearance of the induced gut lesions with a gluten-free diet. Specifically, for 12 to 20 weeks, aggressive gluten challenge (1 to 2 gm/kg/day) of these two patients was followed by both the appearance of and a rise in titer of IgA endomysial antibodies with an exacerbation of skin lesions. After 27 weeks of gluten challenge, histologic studies of the gut showed grade III flattening of the jejunal mucosa in the patient who developed IgA endomysial antibodies 19 weeks before biopsy was performed but not in the patient in whom IgA endomysial antibodies appeared 7 weeks before biopsy was performed. When both patients were placed on a gluten-free diet, IgA endomysial antibodies titers showed negative findings and the skin lesions subsided. It was possible to discontinue dapsone treatment after 30 weeks on a gluten-free diet in one patient and after 33 weeks in the other. It is important to note in the patient who developed grade III (significant) gut pathology after gluten challenge that a third biopsy taken 59 weeks after starting a gluten-free diet revealed a return to a grade II (insignificant) level of villus atrophy. Thus if sulfones are contraindicated in such cases, patients can be treated successfully with a gluten-free diet.- - - - - - - - - - ranking = 0.88662276134944keywords = mucosa (Clic here for more details about this article) |
9/33. The ultrastructural localization of IgA in skin of a patient with mixed form of dermatitis herpetiformis and bullous pemphigoid.A case with mixed features of dermatitis herpetiformis and bullous pemphigoid was investigated by immuno-electron microscopy. There were clinical, histological, and ultrastructural characteristics of both diseases, the response to sulfapyridine was dramatic at the beginning, but intestinal lesions were absent. Direct immunofluorescence tests were made 6 times in the 4 year period and demonstrated in all biopsies exclusively linear IgA deposits. The IgA deposits were shown to occupy the entire lamina lucida and to adhere to the basal cell membranes and lamina densa, very much like IgG deposits in bullous pemphigoid. antibodies against the basement membrane zone could not be demonstrated in the serum.- - - - - - - - - - ranking = 14.454689661201keywords = lamina, membrane (Clic here for more details about this article) |
10/33. Small bowel malignant lymphoma complicating celiac sprue and the mesenteric lymph node cavitation syndrome.Malignant small intestinal lymphoma may complicate or antedate clinical recognition of celiac sprue. However, histologic diagnosis of lymphoma is made especially difficult in the presence of small bowel ulceration. A 70-yr-old man with celiac sprue and a history of dermatitis herpetiformis was initially seen for recurrent diarrhea; panmalabsorption with steatorrhea and protein-losing enteropathy were documented. Subsequent studies showed ectopic gastric mucosa in the small bowel, hyposplenism with mesenteric lymph node cavitation, and small bowel erosions and ulceration. Despite strong clinical suspicion for more than 2 yr, only 1 of 88 small bowel biopsy specimens was positive for lymphoma. At autopsy, shortly after histologic diagnosis of lymphoma, extensive small bowel involvement and infiltration were observed. This is the first report of lymphoma complicating the recently described nonneoplastic lymphoreticular syndrome associated with celiac sprue characterized by splenic atrophy and mesenteric lymph node cavitation.- - - - - - - - - - ranking = 0.44331138067472keywords = mucosa (Clic here for more details about this article) |
| | Next -> |