Subject(s)
Dermatology/history , History, 19th Century , History, 20th Century , Humans , Ireland , Male , Northern IrelandABSTRACT
The sclerodermatous variant of chronic graft-versus-host disease postallogeneic bone marrow transplantation is rare. We present four pediatric cases of sclerodermatous variant of chronic graft-versus-host disease describing their clinical appearance, management, and outcomes. We compare the pharmacologic and supportive therapies administered to these patients with the management suggested in the current literature. Several key findings were noted. There was a significantly higher mortality rate observed in this series compared with previous reports, with three of the four patients dying ultimately as a result of sclerodermatous variant of chronic graft-versus-host disease. The development of widespread ulceration, in two of the four patients, appeared to be associated with an overall deterioration in the clinical condition. In two patients high-dose thalidomide at 12 mg/kg/day seemed to halt the progression of cutaneous disease. Optimal care of sclerodermatous variant of chronic graft-versus-host disease patients required a multidisciplinary team. A lack of community services observed in this case series led to the need for unnecessarily prolonged inpatient admissions.
Subject(s)
Bone Marrow Transplantation/adverse effects , Graft vs Host Disease/diagnosis , Graft vs Host Disease/drug therapy , Scleroderma, Localized/diagnosis , Scleroderma, Localized/drug therapy , Adolescent , Child , Chronic Disease , Disease Progression , Fatal Outcome , Female , Graft vs Host Disease/etiology , Humans , Immunosuppressive Agents/therapeutic use , Male , Scleroderma, Localized/etiology , Thalidomide/therapeutic useABSTRACT
We report a 74 year old lady presenting with cutaneous leukocytoclastic vasculitis. The underlying aetiology was established as chronic hepatitis C infection with associated cryoglobulinaemia. This presented clinically as recurrent cutaneous vasculitic eruptions with absence of any other clinical manifestations. In this case, antiviral treatment to eradicate hepatitis C virus (HCV) was deemed inappropriate given the low necroinflammatory score determined by liver biopsy, absence of other systemic sequelae of cryoglobulinaemia and potential risks of therapy given her age. Currently her cutaneous disease is relatively well controlled with intermittent application of potent topical steroids. This case highlights the need to consider hepatitis C as a potential aetiological factor in all patients with cutaneous vasculitis. We suggest that viral hepatitis screening should be routine in all patients presenting with cutaneous vasculitis.
