Recurrent urticarial skin eruption since infancy. Muckle-Wells syndrome (MWS).
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Biomedical subjects
Publications and source records attributed to G Desirello.
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The authors report the case of a 64-year-old female with a pemphigus vulgaris that relapsed on her left cheek following X-irradiation. The lesion developed not only on the irradiated area but also along a linear streak. Blaschko lines seem to represent the linear systematization pattern more likely to account for this unusual distribution.
The effectiveness and tolerance of isotretinoin have been assessed in 72 cases (34 male and 38 female) suffering from papulo-pustular acne with nodulo-cystic component recalcitrant to traditional treatments. The drug confirmed its noteworthy effectiveness and speed of action in all cases. Stress is laid in particular on the lack of recurrence even after a very long-term follow-up. Tolerance was also very good with a prevalent incidence of mucocutaneous signs (desquamation, cheilitis, dermatitis) and the consequent acceptability of treatment on the part of patients.
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A patient with Richner-Hanhart syndrome had thigh skin grafted onto her heel in an attempt to improve her walking. The graft seemed to be spared by the hyperkeratosis that arrested at the periphery of the graft and formed a keratotic wall. A low-tyrosine, low-phenylalanine diet was effective in clearing hyperkeratosis.
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A patient is described with the clinical and histopathological features of both Dowling-Degos disease and Kitamura's acropigmentatio reticularis. The possible identity of the two genodermatoses is discussed on the basis of the similarity of their clinical and histopathological features and of the presence of the two diseases in the family of the propositus.
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Plasma levels of Zn, Cu and Ni were determined in psoriatic patients by atomic absorption spectrophotometry. In psoriatic patients, plasma Cu levels were significantly higher, whilst plasma Zn and Ni were significantly lower than in healthy controls.