Bart W. Boom

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We describe a patient with long-standing severe leukocytoclastic vasculitis of the skin and essential mixed cryoglobulinemia type II, who showed a limited reaction to immunosuppressive drugs, plasmapheresis, and colchicine. Therapy with high-dose gamma-globulin intravenously (IV) for five days resulted in disappearance of vasculitis lesions within three(More)
In order to determine the effect of chronic skin disorders on sexuality a cross-sectional study was carried out in the Dermatological Outpatient Clinic of Leiden University Hospital. Fifty-two patients with psoriasis and 25 patients with atopic dermatitis filled in a questionnaire which included items on sexual responsiveness and satisfaction. The response(More)
We describe a 27-year-old Dutch woman with the hyperimmunoglobulinemia D and periodic fever syndrome. During febrile attacks she occasionally presented with skin lesions on the distal parts of her upper and lower extremities, with the histologic picture of a leukocytoclastic vasculitis. Clear perivascular deposits of IgD and C3 were presented in early(More)
The presence of the membrane attack complex of complement (MAC) was studied by a two-step immunofluorescence method in 15 patients with leukocytoclastic vasculitis of the skin, using an antibody against MAC neoantigen. Perivascular deposits of MAC were present in 13 specimens of lesional skin and only two specimens of clinically uninvolved skin, suggesting(More)
At present, initial high-dose prednisone is the treatment of choice for patients with pemphigus and bullous pemphigoid. To reduce the risks associated with long-term corticosteroid treatment, other immunosuppressants are often given as steroid-sparing agents. Occasionally, the dose of steroids cannot be reduced. In this study, we report six patients with(More)
Activation of the complement system is an important element in our concept of the pathomechanism of immune complex (IC) vasculitis. Both deposition of IC and attraction of polymorphonuclear leukocytes (PMN) are effected by products of complement activation. Actual tissue damage, however, is believed to be caused by PMN penetrating the vessel wall. Our(More)
We describe a girl with photosensitivity (P), ichthyosis (I), brittle hair (B), impaired intelligence (I), possibly decreased fertility (D), and short stature (S). The clinical findings fit into the PIBI(D)S syndrome and trichothiodystrophy. A remarkable and probably unique observation for this disorder was the intermittent character of the scalp hair loss(More)