Mastocytoses are a heterogenous group of diseases characterized by proliferation and accumulation of mast cells in the skin and other organs.They are subdivided into cutaneous mastocytoses; systemic forms, which may a...Mastocytoses are a heterogenous group of diseases characterized by proliferation and accumulation of mast cells in the skin and other organs.They are subdivided into cutaneous mastocytoses; systemic forms, which may appear with or without skin lesions; mast cell sarcomas and extracutaneous, localized, benign mastocytomas.Systemic mastocytoses apart from the skin mainly involve bone marrow, gastrointestinal tract, bones, lymph nodes, spleen and liver.Whereas indolent forms of systemic mastocytosis are mainly treated with antihistamines, glucocorticosteroids and PUVA therapy, the more aggressive forms, including mast cell leukemia, often require cytostatic chemotherapy.A 53-year old patient with beginning “smoldering systemic mastocytosis”failed to respond to high-dose systemic glucocorticosteroids and interferon-α.Treatment with cladribine led to an impressive improvement of skin lesions, a significant decrease in tryptase serum levels and stabilization of bone marrow infiltrates.展开更多
The clinical features of necrobiotic xanthogranuloma (NX)have been presented in a series of case reports, but there are hardly any reports on the clinical long- term course of this rare and usually chronic entity. The...The clinical features of necrobiotic xanthogranuloma (NX)have been presented in a series of case reports, but there are hardly any reports on the clinical long- term course of this rare and usually chronic entity. Therapeutic recommendations are mostly based on individual observations, lacking general therapeutic guidelines. To illustrate a typical chronic and progressive course of NX, we report the case of a 64- year- old woman with periorbital NX, ocular involvement and IgG paraproteinemia. Diagnosed with NX for the first time in 1993, the patient was already presented in this journal in 1995. Since then a series of therapeutic options including medication with dapsone, chlorambucil, interferon- alpha, clofazimine, melphalan, fumaric acid esters, surgical treatment and other physical therapies such as radiation, plasmapheresis, and photodynamic therapy have been applied. None of these therapeutic approaches, however, showed a satisfying long- term effect. At present the patient is undergoing cyclophosphamide- dexamethasone pulse therapy.展开更多
Ammonium persulphate is a potent bleach and oxidizing agent that is commonly present in hair bleaches. Because bleaching is so commonly performed, hairdressers often develop allergic contact dermatitis to ammonium per...Ammonium persulphate is a potent bleach and oxidizing agent that is commonly present in hair bleaches. Because bleaching is so commonly performed, hairdressers often develop allergic contact dermatitis to ammonium persulphate. In addition to this delayed reaction, asthma and rhinitismay develop as immediate reactions in those exposed to the fumes. Severe anaphylactic reactions are rare. We report a 24- year- old woman who acquired dermatitis following contact with bleaching substances while working as a hairdresser. After changing her profession, the dermatitis disappeared. Following the private use of a hairdressing bleach containing ammonium persulphate, she suffered a severe anaphylactic reaction with unconsciousness. The patient also developed an anaphylactic reaction three hours following patch testing with the hairdresser battery. The rub test with ammonium persulphate (2.5% ) in a 1:100 solutionwas positive.展开更多
文摘Mastocytoses are a heterogenous group of diseases characterized by proliferation and accumulation of mast cells in the skin and other organs.They are subdivided into cutaneous mastocytoses; systemic forms, which may appear with or without skin lesions; mast cell sarcomas and extracutaneous, localized, benign mastocytomas.Systemic mastocytoses apart from the skin mainly involve bone marrow, gastrointestinal tract, bones, lymph nodes, spleen and liver.Whereas indolent forms of systemic mastocytosis are mainly treated with antihistamines, glucocorticosteroids and PUVA therapy, the more aggressive forms, including mast cell leukemia, often require cytostatic chemotherapy.A 53-year old patient with beginning “smoldering systemic mastocytosis”failed to respond to high-dose systemic glucocorticosteroids and interferon-α.Treatment with cladribine led to an impressive improvement of skin lesions, a significant decrease in tryptase serum levels and stabilization of bone marrow infiltrates.
文摘The clinical features of necrobiotic xanthogranuloma (NX)have been presented in a series of case reports, but there are hardly any reports on the clinical long- term course of this rare and usually chronic entity. Therapeutic recommendations are mostly based on individual observations, lacking general therapeutic guidelines. To illustrate a typical chronic and progressive course of NX, we report the case of a 64- year- old woman with periorbital NX, ocular involvement and IgG paraproteinemia. Diagnosed with NX for the first time in 1993, the patient was already presented in this journal in 1995. Since then a series of therapeutic options including medication with dapsone, chlorambucil, interferon- alpha, clofazimine, melphalan, fumaric acid esters, surgical treatment and other physical therapies such as radiation, plasmapheresis, and photodynamic therapy have been applied. None of these therapeutic approaches, however, showed a satisfying long- term effect. At present the patient is undergoing cyclophosphamide- dexamethasone pulse therapy.
文摘Ammonium persulphate is a potent bleach and oxidizing agent that is commonly present in hair bleaches. Because bleaching is so commonly performed, hairdressers often develop allergic contact dermatitis to ammonium persulphate. In addition to this delayed reaction, asthma and rhinitismay develop as immediate reactions in those exposed to the fumes. Severe anaphylactic reactions are rare. We report a 24- year- old woman who acquired dermatitis following contact with bleaching substances while working as a hairdresser. After changing her profession, the dermatitis disappeared. Following the private use of a hairdressing bleach containing ammonium persulphate, she suffered a severe anaphylactic reaction with unconsciousness. The patient also developed an anaphylactic reaction three hours following patch testing with the hairdresser battery. The rub test with ammonium persulphate (2.5% ) in a 1:100 solutionwas positive.
