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1/84. IgA multiple myeloma presenting as an acquired bullous disorder.

    A 63-year-old man presented with an intensely pruritic vesiculo-bullous eruption on the limbs and was subsequently found to have an IgA kappa multiple myeloma. The eruption clinically and histologically was suggestive of linear IgA disease (LAD), dermatitis herpetiformis (DH), epidermolysis bullosa acquisita (EBA), or bullous lupus erythematosus (LE), with the skin biopsy revealing subepidermal bullae and dermal papillary micro-abscesses. However, direct immunofluorescence showed a unique pattern of diffuse dermal IgA staining. Although chemotherapy produced a dramatic resolution of the lesions, which paralleled the fall in serum IgA paraprotein level, the myeloma later became progressive and the resulting paraprotein increase was accompanied by recurrence of the eruption. We propose that this patient's rash was the presenting manifestation of his multiple myeloma, and was a consequence of transudation of IgA paraprotein into the dermis.
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ranking = 1
keywords = dermatitis herpetiformis, herpetiformis, dermatitis
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2/84. Bazex syndrome mimicking a primary autoimmune bullous disorder.

    Bazex syndrome is a paraneoplastic condition that is most frequently associated with squamous cell carcinoma of the upper aerodigestive tract. The lesions affect acral areas of the skin, including hands, feet, ears, nose, and, to a lesser extent, elbows and knees. Lesions mimic psoriasis and dermatitis. paronychia and nail dystrophy are frequent. Bullous lesions have been reported only rarely. We report a patient with Bazex syndrome with predominantly bullous lesions that mimicked a primary autoimmune bullous disorder.
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ranking = 0.0016870424985208
keywords = dermatitis
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3/84. mercury-induced nummular dermatitis.

    We report 2 cases of relapsing nummular dermatitis according to mercury sensitivity, which was confirmed by patch testing. Removal of the amalgam from dental metal alloys markedly improved their skin eruptions. One of the patients, a dentist, experienced exacerbation of the eruptions on his lower legs after handling dental amalgam. hypersensitivity to haptens such as metals is possibly involved in, at least in some patients, the pathogenesis of nummular dermatitis.
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ranking = 0.010122254991125
keywords = dermatitis
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4/84. dapsone-induced photodermatitis in a patient with linear IgA dermatosis.

    dapsone (4, 4' diaminodiphenylsulfone) is an efficient antiinflammatory agent. Its therapeutic use may result in a variety of adverse effects. The most frequent unwanted reactions are hemolytic anemia and methemoglobinemia. By oral route dapsone is mainly metabolized to monoacetyldapsone (MADDS) and hydroxylamine dapsone (DDS-NOH). We report a 76-year-old female patient with linear IgA dermatosis who developed a dapsone-induced photosensitivity 8 weeks after initiation of sulfone therapy. She showed a widespread erythematous eruption in UV-exposed skin area. After clearing of skin lesions the photopatch test revealed positive reactions to dapsone, MADDS and DDS-NOH. dapsone-induced photosensitivity to date has been described only in leprosy patients. We demonstrate for the first time that this adverse reaction is not restricted to leprosy and that dapsone metabolites may also contribute to the mechanism of photosensitivity like the parent sulfone. dapsone-induced photosensitivity is a rare, not dose-related adverse effect of the sulfone and can also occur in patients with inflammatory skin disorders.
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ranking = 0.006748169994083
keywords = dermatitis
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5/84. linear iga bullous dermatosis responsive to a gluten-free diet.

    dermatitis herpetiformis is associated with a gluten-sensitive enteropathy in >85% of cases. Both the skin lesions and the enteropathy respond to gluten restriction. linear iga bullous dermatosis has a much lower prevalence of histological small bowel abnormalities, and lesions are not known to respond to gluten restriction. We report a patient with linear iga bullous dermatosis and gluten-sensitive enteropathy. This report addresses the issue of whether linear iga bullous dermatosis can be associated with gluten-sensitive enteropathy. We evaluated the response to gluten restriction and normal diet by following the status of the patient's jejunal biopsies and skin lesions. The patient responded to gluten restriction, as shown by resolution of jejunal abnormalities and skin lesions and subsequently by recurrence of jejunal abnormalities and skin lesions with reinstitution of a gluten-containing diet. This report demonstrates that linear iga bullous dermatosis can respond to gluten restriction if an underlying gluten-sensitive enteropathy is present.
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ranking = 0.091608344328708
keywords = herpetiformis
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6/84. Linear IgA dermatosis presenting with erythema annulare centrifugum lesions: report of three cases in adults.

    Linear IgA dermatosis presented with erythema annulare centrifugum lesions in three elderly women. Search for underlying malignancy revealed low-grade B-cell lymphoma in one case. In addition to subepidermal blistering, histology showed a typical mixed infiltrate of granulocytes and eosinophils and, occasionally, papillar microabsesses in one case. In the two other subjects, characteristic subepidermal lining with granulocytes was observed. Immunofluorescence studies confirmed the diagnosis, while autoantibodies characteristic for dermatitis herpetiformis were absent. To our knowledge this is the second report of adult linear IgA dermatosis in association with erythema annulare centrifugum lesions. Our observations concord with several other reports of figurate erythema associated with autoimmune blistering disease and other immune disorders. Common antibody-related immunological mechanisms indicate that the two distinct clinical pictures are probably stages of the same pathogenic entity.
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ranking = 1
keywords = dermatitis herpetiformis, herpetiformis, dermatitis
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7/84. Erosive pustular dermatosis of the leg: report of three cases.

    Erosive pustular dermatosis of the leg is a distinct form of spongiform amicrobial pustulosis. The disorder typically affects the lower limbs of elderly patients presenting with chronic venous insufficiency and stasis dermatitis, and has a chronic course. Three elderly patients with chronic venous ulcers are described, who developed pustules and moist eroded lesions on the leg. The clinical and histological features were typical for erosive pustular dermatosis. The lesions rapidly responded to topical treatment with either tacrolimus or corticosteroids. Of note, this condition was associated with a diverticular disease in two patients, while in another patient an epidermoid carcinoma of the tongue was present. Erosive pustular dermatosis of the leg is an uncommon but distinct skin disorder typically associated with trophic changes of the lower limbs. Our observations raise the question of the relation of erosive pustular dermatosis of the leg with the group of neutrophilic dermatoses. Topical immunotherapy with tacrolimus may constitute a novel therapeutic option for this frequently recalcitrant condition.
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ranking = 0.0016870424985208
keywords = dermatitis
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8/84. Clinical resemblance of widespread bullous fixed drug eruption to stevens-johnson syndrome or toxic epidermal necrolysis: report of two cases.

    Widespread bullous fixed drug eruption (FDE) is the most severe form of FDE and may be mistaken clinically for stevens-johnson syndrome or toxic epidermal necrolysis (SJS/TEN). We report two cases of generalized bullous drug eruption with extensive epidermal necrosis and detachment mimicking SJS/TEN overlap and TEN, respectively. The first patient, a 78-year-old man, developed SJS/TEN-like eruption with widespread dusky red patches and denuded areas shortly after taking multiple nonsteroidal antiinflammatory drugs (NSAIDs). Histopathology showed vacuolar interface dermatitis with numerous necrotic keratinocytes and a superficial and deep perivascular infiltrate containing lymphocytes, eosinophils, neutrophils and melanophages. These findings are consistent with FDE. The second patient, a 61-year-old woman, had three episodes of near-total body epidermal detachment shortly after taking NSAIDs. TEN was diagnosed clinically in all three episodes without pathologic confirmation. FDE was suspected due to lack of involvement of two mucosal sites and uneventful recovery. These cases highlight the importance of considering severe bullous FDE in the differential diagnosis of SJS and TEN, and the necessity of skin biopsy in such cases.
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ranking = 0.0016870424985208
keywords = dermatitis
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9/84. A subepidermal blistering dermatosis associated with coexistent IgG and IgA anti-dermal basement membrane zone antibodies; demonstration of IgG antibodies reactive against a 200-kDa dermal antigen.

    We report a 66-year-old woman presenting with an annular erythematous and bullous eruption. Her clinical and histological findings were similar to those of linear IgA bullous dermatosis or dermatitis herpetiformis. Direct immunofluorescence revealed linear deposition of IgA, IgG and C3 along the basement membrane zone (BMZ). Indirect immunofluorescence detected IgG and IgA antibodies against the BMZ. Salt-split skin technique demonstrated that IgG antibodies bound exclusively to the dermal side, while IgA antibodies bound not only to the dermal side, but also to the epidermal side with relatively weak intensity. On immunoblot analysis, the patient's IgG antibodies exclusively reacted with a band of 200-kDa, while the antigenic target of IgA antibodies was not identified. The present case is thought to be a unique bullous dermatosis mediated by both the IgG antibodies to a novel 200-kDa antigen and IgA antibodies against undetermined antigens.
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ranking = 1
keywords = dermatitis herpetiformis, herpetiformis, dermatitis
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10/84. tea tree oil dermatitis associated with linear IgA disease.

    tea tree oil dermatitis is an increasingly common finding, reflecting the strong demand for natural remedies and aromatic substances. Linear immunoglobulin a (IgA) disease is a rare acquired subepidermal blistering disorder, characterized by basement membrane zone IgA deposition. We describe a patient in whom linear IgA disease appears to have been precipitated by a contact reaction to tea tree oil.
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ranking = 0.0084352124926038
keywords = dermatitis
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