Infliximab-induced lupus-like reaction in a patient with psoriatic arthritis.
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Biomedical subjects
Publications and source records attributed to G Cianchini.
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Cutaneous infiltration of activated CD4(+) T cells and eosinophils is an early event in blister formation during bullous pemphigoid (BP), suggesting that the trafficking of circulating leucocytes through the sites of inflammation, their activation and cytokine release is crucial in the pathogenesis of the disease. IL-16 is a major chemotactic factor able to recruit CD4(+) cells in the skin during inflammation and to induce the expression of functional high-affinity interleukin (IL)-2 receptors, thus contributing to cellular activation and proliferation. We performed a study in order to evaluate the presence of IL-16 in skin samples and sera and blister fluids of patients affected with BP in active phase of the disease (n = 39), compared with healthy donors studied as control group. Ten patients were also evaluated before and after steroid therapy. Our results demonstrated that IL-16 was expressed strongly by keratinocytes and by dermal infiltrating CD4(+) T lymphocytes in lesional skin of BP patients. High levels of IL-16 were detected in sera and blisters of BP, significantly higher in respect to healthy donors. When patients were investigated for the presence of eosinophil cationic protein (ECP) and soluble CD30 (sCD30) to reveal signs of eosinophils and Th2-cells activation, we found a positive correlation between IL-16 serum levels and both ECP and sCD30, suggesting that IL-16 is involved in Th2 lymphocytes and eosinophils recruitment during BP.
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We describe the clinical and laboratory findings of a young man with mycosis fungoides. The disease was associated, since the early stages, with autoimmune purpura. Interferon alfa (IFN-alpha) administration improved this patient's condition, both the purpuric eruption and patchy cutaneous lesions, thus suggesting T-cell abnormalities may be responsible for the development of the disease.
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Bullous pemphigoid (BP) is an autoimmune blistering skin disease in which autoantibodies are directed against hemidesmosomal proteins of basal keratinocytes. The presence of activated T helper cells in lesions and peripheral blood of BP patients, the eosinophilia, the high levels of serum IgE, eosinophil cationic protein and soluble immune products such as IL-2, sIL-2R, IL-5, soluble CD23 (sCD23) strongly suggest the involvement of a cell-mediated immune reaction in which Th2 lymphocytes could play a pivotal role. To seek evidence to support this hypothesis we evaluated serum levels of IL-4 and sCD30, a specific activation marker of cells able to produce Th2-like cytokines, in 25 patients affected with BP. Serum from both healthy donors and pemphigus vulgaris (PV) patients were used as controls. Our results demonstrated significantly higher levels of IL-4 and sCD30 in patients with BP in relation to both normal individuals (16.6 +/- 7.9 vs 4.5 +/- 2.2 pg/ml, P < 0.0001; 30.3 +/- 10 vs 10.5 +/- 4 U/ml, P < 0.0001) and PV patients (6.2 +/- 4 pg/ml, P < 0.0001; 16 +/- 8.5 U/ml, P < 0.0001). Furthermore, a positive correlation between IL-4 and sCD30 was found (R = 0.85, P < 0.0001). In a subset of seven patients observed after systematic immunosuppressive therapy, we detected a significant reduction in sCD30 serum level (36.9 +/- 7.3 vs 16.3 +/- 6.8 U/ml, P = 0.002), with a parallel improvement in their clinical condition. These results seem to be consistent with the systematic involvement of Th2 lymphocytes in the pathogenesis of BP and suggest a role for sCD30 as a serological marker of disease activity.
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The case of a 7-year-old boy affected by Dubowitz syndrome is described. The characteristic features of the syndrome--intrauterine growth retardation, low neonatal weight, short stature, characteristic facies, atopic dermatitis and mental retardation--are discussed with reference to the absence of microcephaly and the presence of spontaneous keloids.
Two familial cases of perianal streptococcal dermatitis in a 3-year-old girl and her 5-1/2-year-old brother are reported. The clinical features of well-demarcated perianal erythema variably associated with itching, painful defecation with subsequent constipation, rectal bleeding, and proctitis are reviewed, together with suggestions for the best therapy.
Perianal streptococcal dermatitis is a childhood disorder caused by group A beta-hemolytic streptococci which was first described by Amren in 1966. The incidence of this dermatosis, characterized by well defined erythema in the perianal area, has certainly been underestimated and to the authors' knowledge there have still been no reports of this pathology in Italy. Perianal streptococcal dermatitis merits attention given that affected subjects do not always receive appropriate treatment and on average there is a 6-month lapse between the appearance of symptoms and diagnosis. The authors present two cases which were recently referred to their attention and discuss the methods of contagion, the difficulties of clinical diagnosis, associations with other streptococcal disorders and the treatment of this morbid condition.
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The treatment of condyloma acuminatum in children is still controversial. The ideal treatment should take into account the efficacy of eradicating infection, but also the child's tolerance of treatment and its ease of use. Twenty cases observed by the authors over the course of the past 10 years are described. A treatment protocol is drawn up on the basis of a review of the latest reports on this topic and the availability of new products.
The authors review the literature on the subject of the questions raised by the finding of condyloma acuminatum in children. These questions concern its epidemiology, the methods of transmission, the existence or otherwise of sexual abuse and predisposing conditions, its relationship with various viral serotypes and with the onset of neoplasia in adulthood. The most appropriate modes of behaviour in relation to individual cases are also discussed and the authors make a few comments regarding prevention.
We report an 11-year-old girl with both piebaldism and neurofibromatosis type 1 (NF1). The patient had large depigmented patches on her lower limbs and a white forelock since birth. In addition, some café au lait spots were present on her trunk at birth and had increased in number and size during childhood in concomitance with the appearance of axillary and inguinal freckling. Neither neurofibromas nor Lisch nodules were detected and the patient was otherwise healthy. Pedigree analysis revealed inheritance for piebaldism on the paternal side. To our knowledge, the association of piebaldism and NF1 has been described previously in only three patients. Awareness of this rare association is relevant to ensure early diagnosis and adequate follow-up for NF1.
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Schnitzler syndrome is a rare condition characterized by chronic non-pruritic urticaria, recurrent fever, bone pain, osteocondensation, and monoclonal IgM gammopathy without features of lymphoproliferative disease. We describe the case of a 44-year-old man with an 8-year history of bone pain with hyperostosis and a 5-year history of chronic non-pruritic urticaria, associated with fever, hyperleukocytosis, hepatomegaly, serum monoclonal IgM-kappa. Systemic treatment with steroids was effective against bone pain but was ineffective in controlling the urticaria. We also review 35 cases. No adequate treatment has yet been found. The pathogenesis is unclear and the role of the IgM component in the induction of urticaria has not been established.
A 45-year-old man presented with a unilateral, papulopustular, rosacealike, chronic dermatitis which involved the left portion of the forehead and the eyelids unilaterally. Many Demodex mites were found in the follicles of the affected area. Topical crotamiton cleared the lesions after an unsuccessful trial with oral metronidazole. This observation provides further evidence that demodicidosis is a condition distinct from common rosacea.