1/237. Treatment of linear iga bullous dermatosis of childhood with colchicine.linear iga bullous dermatosis of childhood is the most common immunobullous disorder of childhood. First-line treatment includes dapsone or sulfapyridine. We report a child with linear IgA dermatosis who was treated successfully with colchicine after developing hemolytic anemia from dapsone. We recommend colchicine as an alternative therapy for linear IgA dermatosis of childhood in patients in whom first-line treatment is contraindicated or who fail first-line treatment or develop side effects from it.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
2/237. 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 = 0.85714285714286keywords = dermatosis (Clic here for more details about this article) |
3/237. linear iga bullous dermatosis in a patient with acute lymphocytic leukemia: possible involvement of granulocyte colony-stimulating factor.We describe a case of linear iga bullous dermatosis (LABD) in a patient with acute lymphocytic leukemia during treatment with granulocyte colony-stimulating factor (G-CSF). After a drug eruption due to imipenem cilastatin sodium had disappeared, bullous lesions appeared on the trunk. Results of histopathological studies and direct immunofluorescence studies of the lesion were consistent with LABD. Reinstitution of G-CSF after the resolution, however, did not reproduce the bullous eruptions. This suggests that in addition to G-CSF, the presence of precipitating factors that can synergistically enhance or accelerate the outbreak of the disease is required for the development of bullous lesions. Various cytokines, such as interleukin-2 (IL-2) and interferon-gamma (IFN-gamma), endogenously produced from activated lymphocytes during the drug eruption might have provided a favorable milieu for the onset of G-CSF-induced LABD. We suggest that patients with LABD will need special attention with respect to the type of cytokines or combination of cytokines given as therapeutic modalities.- - - - - - - - - - ranking = 0.71428571428571keywords = dermatosis (Clic here for more details about this article) |
4/237. linear iga bullous dermatosis in a patient with chronic renal failure: response to intravenous immunoglobulin therapy.linear iga bullous dermatosis is a blistering disease with a heterogeneous clinical manifestation, characterized by deposition of IgA along the basement membrane zone of perilesional skin on direct immunofluorescence. We describe a patient with chronic renal failure who experienced linear iga bullous dermatosis. Long-term administration of intravenous immunoglobulin therapy was associated with clinical remission lasting more than 12 months.- - - - - - - - - - ranking = 0.85714285714286keywords = dermatosis (Clic here for more details about this article) |
5/237. Pseudoporphyria induced by propionic acid derivatives.BACKGROUND: Pseudoporphyria is a photosensitive bullous skin disease that is distinguished from porphyria cutanea tarda (PCT) by its normal porphyrin profile. Drugs are a major cause of this disease, and the list of culprits is continually expanding. Nonsteroidal antiinflammatory agents (NSAIDs), especially naproxen and other propionic acid derivatives, appear to be the most common offenders. OBJECTIVE: The study was carried out to increase awareness about the etiology and characteristic features of pseudoporphyria. methods: We report two cases of pseudoporphyria caused by naproxen and oxaprozin. We review the current English language literature on this entity and discuss its clinical features, histology, ultrastructure, etiology, and pathophysiology. RESULTS: A 44-year-old man taking naproxen for chronic low back pain and a 20-year-old woman on oxaprozin for rheumatoid arthritis presented with tense bullae and cutaneous fragility on the face and the back of the hands. In both, skin biopsy showed a cell-poor subepidermal vesicle with festooning of the dermal papillae. Direct immunofluorescence revealed staining at the dermal-epidermal junction and around blood vessels with IgG in the first case and with IgG, IgA, and fibrin in the second case. urine collections and serum samples yielded normal levels of uro- and coproporphyrins. CONCLUSIONS: Most cases of pseudoporphyria are drug-induced. naproxen, the most common offender, has been associated with a dimorphic clinical pattern: a PCT-like presentation and one simulating erythropoietic protoporphyria in the pediatric population. Other NSAIDs of the propionic acid family can also cause pseudoporphyria.- - - - - - - - - - ranking = 0.013793669941667keywords = skin disease (Clic here for more details about this article) |
6/237. linear iga bullous dermatosis associated with ulcerative colitis.A 41-year-old woman developed skin vesicles one week before examination. She had been suffering from ulcerative colitis since the age of 30. Histologic examination of a vesicle showed a subepidermal bulla accompanied by mononuclear leukocyte infiltration intermingled with eosinophils and neutrophils. eosinophilia was also noted. Direct immunofluorescent test revealed linear deposits of IgA in the basement membrane zone.- - - - - - - - - - ranking = 0.57142857142857keywords = dermatosis (Clic here for more details about this article) |
7/237. Subcorneal pustular dermatosis treated with puva therapy. A case report and review of the literature.BACKGROUND: Subcorneal pustular dermatosis (SPD) is a chronic recurrent pustular dermatosis of unknown etiology. Many treatments have been proposed, none of which has been uniformly successful. OBJECTIVE: Our purpose is to report a patient with SPD successfully treated by PUVA and to review the literature concerning phototherapy treatment of SPD. methods: A patient suffering from SPD resistant to diaminodiphenylsulphone (dapsone) responded well to a combination therapy consisting of dapsone and PUVA. He received 50 mg/day and 3 PUVA sessions a week. photographs were taken at baseline and after 15 sessions. RESULTS: The lesions were virtually cleared after 15 sessions. The patient remained free of lesions with a maintenance therapy of dapsone (50 mg/ day) and 1 PUVA session a week. CONCLUSION: The therapeutic value of phototherapy for the treatment of SPD still has to be confirmed and could be a valuable alternative for treatment-resistant patients.- - - - - - - - - - ranking = 0.85714285714286keywords = dermatosis (Clic here for more details about this article) |
8/237. Eosinophilic pustular folliculitis in infancy: report of two new cases.Eosinophilic pustular folliculitis (EPF) is a cutaneous inflammatory follicular disorder of unknown etiology. The diagnosis is made on the basis of clinical and histopathologic features. We describe two patients who had recurrent episodes of pruritic follicular papular and pustular lesions on the face, extremities, and trunk. The eruptions lasted for 1 month with intermittent remissions. Laboratory tests disclosed no infectious or parasitic etiology in patient 2. In patient 1 we isolated methicillin-resistant staphylococcus aureus in a blood culture. He had sepsis with lung and liver involvement. EPF is a self-limited dermatosis. On occasion, skin lesions may become superinfected, resulting in localized pyoderma or rarely systemic infection (sepsis). Histologically both of our patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Their lesions responded well to topical corticosteroids.- - - - - - - - - - ranking = 0.14285714285714keywords = dermatosis (Clic here for more details about this article) |
9/237. Drug-induced linear iga bullous dermatosis probably induced by furosemide.linear iga bullous dermatosis (LABD) is an autoimmune disease, characterized by linear deposition of IgA along the basement membrane zone. Drug-induced LABD is rare but increasing in frequency. A new case of drug-induced LABD associated with the administration of furosemide is described.- - - - - - - - - - ranking = 0.71428571428571keywords = dermatosis (Clic here for more details about this article) |
10/237. Subepidermal autoimmune bullous skin diseases associated with B-cell lymphoproliferative disorders.The development of several types of autoimmune disorders is well known in the course of B-cell lymphoproliferative diseases. In order to investigate whether these autoimmune diseases may target cutaneous antigens, especially those of the dermal-epidermal junction, we conducted a retrospective analysis of all cutaneous direct immunofluorescence studies performed in patients with B-cell neoplasia who presented with cutaneous lesions requiring a skin biopsy. patients with linear deposits of immunoglobulin or C3 molecules at the dermal-epidermal junction were studied further, using immunoblotting and immunoelectron microscopy. patients with no overt hematologic malignancies but with cutaneous lesions requiring direct immunofluorescence were studied as a control population. During the study period, the diagnosis of autoimmune blistering diseases of the dermal-epidermal junction was established in 9/102 (8.9% /- 2%) patients with a B-cell lymphoid disorder, compared with 288/8,189 (3.5% /- 0.04%) patients without underlying hemopathy (p < 0.01). Among the 9 patients, 7 were studied in detail. Immunologic studies demonstrated epidermolysis bullosa acquisita in 6 patients and cicatricial pemphigoid in 1. Autoimmune blistering diseases, especially epidermolysis bullosa acquisita, are part of the spectrum of autoimmune disorders associated with B-cell neoplasia.- - - - - - - - - - ranking = 0.055174679766668keywords = skin disease (Clic here for more details about this article) |
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