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ABSTRACT: Graft-versus-host disease (GVHD) is a major complication after allogeneic hematopoietic stem cell transplantation (HSCT) and skin is involved in acute and chronic disease. Immune-mediated vessel attack and subsequent microvessel loss have been observed in skin of patients with chronic GVHD.
To test whether long-term survivors (LTS) after allogeneic HSCT without cutaneous GVHD show signs of persistent vascular remodeling.
Microvessels in skin biopsies were investigated in a cohort of 32 LTS with a median follow-up of 17 years (range 11-26). Five were currently classified as having chronic GVHD other than skin involvement.
LTS showed no significant difference in median microvessel density and relative vessel size distribution pattern compared to healthy controls. Past experience of GVHD and current status of chronic GVHD other than skin involvement had no impact on vessel density. In contrast, recipients with chronic cutaneous GVHD of sclerotic type and patients with lichen sclerosus have significant microvessel loss in the upper dermis.
The complex therapy of allogeneic HSCT had no sustained effect on the microvascular architecture of LTS when clinicopathological evidence of cutaneous GVHD is absent. Microvascular remodeling as observed during chronic GVHD recovers completely after resolution of chronic cutaneous GVHD.
Dermatology 05/2009; 219(1):32-41. · 2.05 Impact Factor
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ABSTRACT: Cutaneous drug reactions occur with a frequency of 1-8% and can be higher for certain classes of drugs. They can range from mild morbilliform eruptions to more severe forms such as drug-hypersensitivity syndrome, toxic epidermal necrolysis or anaphylaxis. Acute generalized exanthematous pustulosis (AGEP) is considered to be a clinical reaction pattern, which is induced in over 90% of the cases by systemic drugs. It is a rare presentation of an adverse drug reaction most frequently triggered by anti-infectious drugs. A high proportion of these cases have been attributed to aminopenicillins and macrolides. We report a terbinafine-induced AGEP in a 68-year-old male confirmed by lymphocyte stimulation in vitro, and review the published cases induced by antimycotic drugs with special emphasis on terbinafine-triggered cases.
British Journal of Dermatology 05/2005; 152(4):780-3. · 3.67 Impact Factor
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ABSTRACT: Cryopyrin-associated periodic fever syndrome (CAPS) is a rare inherited disease due to mutations in the NLRP3 (also called CIAS1) gene on chromosome 1q44 resulting in overproduction of interleukin-1. CAPS comprises three clinically overlapping disorders including Muckle-Wells syndrome. We report on two half-siblings with Muckle-Wells syndrome who were successfully treated with the interleukin-1 beta antibody canakinumab. Despite reduced dosing and longer treatment intervals compared to the recommended dosing schedule (e.g. 150 mg every 8 weeks), the efficacy and tolerability of canakinumab was impressive in both patients. The pharmacologic properties of canakinumab are reviewed and the clinical and economical aspects highlighted. We show that with individualized ‘reflare-guided’ administrations of canakinumab overall costs could hypothetically be reduced by 50% (approx. USD 60,000/patient/year) and therefore could have a major impact on treatment costs.
Dermatology 08/1970; 223(2):113-118. · 2.05 Impact Factor
