Subject(s)
Skin Diseases, Papulosquamous/pathology , Acanthoma/diagnosis , Acanthoma/genetics , Acanthoma/pathology , Axilla/pathology , Biopsy, Needle , Cutis Laxa/diagnosis , Cutis Laxa/genetics , Cutis Laxa/pathology , Diagnosis, Differential , Female , Humans , Immunohistochemistry , Neck/pathology , Rare Diseases , Recurrence , Skin Diseases, Papulosquamous/diagnosis , Teaching Rounds , Young AdultABSTRACT
BACKGROUND: Swelling in an arteriovenous fistula (AVF) is commonly caused by thrombosis, aneurysm and infection. However, due to the increased risk of malignancy after transplantation, this should also be considered. PATIENTS: We discuss 4 patients with malignancy confined to an AVF after renal transplantation presenting in a 2-year period. Angiosarcoma was diagnosed in 3 patients and the other had post-transplant lymphoproliferative disorder (PTLD). Angiosarcoma behaves aggressively and 2 of our patients died within 6 months of diagnosis. There are 6 previous cases and 5 died within 16 months of diagnosis. PTLD at AVFs has not been documented previously. CONCLUSION: Malignancy at an AVF is a rare but important differential that can impact significantly on patient morbidity and mortality. Predilection for malignancy at an AVF is not understood. We review the literature and discuss possible aetiologies.
Subject(s)
Arteriovenous Shunt, Surgical , Hemangiosarcoma/diagnosis , Immunosuppressive Agents/adverse effects , Kidney Transplantation/adverse effects , Lymphoproliferative Disorders/diagnosis , Skin Neoplasms/diagnosis , Adult , Diagnosis, Differential , Epstein-Barr Virus Infections/complications , Female , Hemangiosarcoma/etiology , Hemangiosarcoma/pathology , Hemangiosarcoma/therapy , Herpesvirus 4, Human , Humans , Lymphoproliferative Disorders/pathology , Lymphoproliferative Disorders/therapy , Lymphoproliferative Disorders/virology , Male , Middle Aged , Skin Neoplasms/etiology , Skin Neoplasms/pathology , Skin Neoplasms/therapyABSTRACT
A 22-year-old man presented with recurrent palpable purpuric rash. His clubbing relates to underlying cystic fibrosis (CF) and his rash was identified as CF-related vasculitis, a rare extrapulmonary manifestation of the disease. It occurs predominantly on the lower limbs, mainly over the dorsa of the feet, ankles and tibial surfaces. The rash occurred while the patient had an infective exacerbation of CF (IECF), however, there had also been previous occurrences without worsening of his pulmonary symptoms, to which the rash remitted spontaneously. The patient responded well to immunosuppression, which was given on this admission due to worsening of his CF-related vasculitis. He died 18 months within the onset of his initial rash.
