DP10: A case of reactive angioendotheliomatosis with resolution following improved glycaemic control
Florence Garty, Kiran Kaur Gurdial Singh, Ahmed Basiouni, Sarah Rasool and Sarah Woodrow
Royal United Hospital, Bath, UK
An 88-year-old immunocompetent man with type 5 skin presented with a 6-month history of multiple firm, raised, pruritic skin lesions affecting his trunk and limbs. The lesions occurred spontaneously and over time; some resolved without treatment. He has a history of ischaemic heart disease, poorly controlled diabetes mellitus, previously treated prostate cancer and hypertension. At presentation, his HbA1c was significantly raised, despite being on two oral hypoglycaemic agents. At the time of initial review, there were hyperpigmented polymorphic plaques, and multiple smooth round and oval papules affecting the arms, trunk and thighs. A skin biopsy from a forearm lesion showed changes in keeping with reactive angioendotheliomatosis (RAE). There was prominent intravascular congestion with red blood cell rouleaux formation and no atypical lymphocytes. It was negative for human herpesvirus 8. His work-up was negative for causes of RAE, including paraproteinaemia, renal disease and systemic infection. His prostate-specific antigen level was normal. His RAE was attributed to underlying poorly controlled diabetes and atherosclerosis. Treatment with mometasone furoate 0·1% ointment was not effective and at follow-up 4 months later he was still experiencing new skin lesions. Optimization of glycaemic control was achieved with insulin therapy and on review 1 year later, most of the skin lesions had flattened significantly leaving postinflammatory hyperpigmentation and no new skin lesions were seen. RAE is a rare, benign, usually self-limiting condition classically described histologically by intravascular endothelial cell proliferation. However, histological findings in reported cases can be variable. It is frequently idiopathic, but may be associated with systemic disease in up to 75% of cases, including chronic infection, lymphoproliferative disorders and renal failure. A diffuse dermal angiomatosis (DDA) variant has been reported more commonly in the breast, but is often associated with cardiovascular disease. To date, approximately 101 cases of RAE have been reported in the literature, 75 of which were of the diffuse dermal angiomatosis subtype, with three cases reported in black skin. The clinical presentation is often of erythematous nodules and plaques with overlying ecchymosis on the lower legs. In DDA this can progress to painful ulceration. Hypotheses for pathogenesis have included circulating angiogenic factor causing capillary proliferation and hypoxia-induced increase in vascular endothelial growth factor. One could postulate that chronic vascular inflammation caused by diabetes could play a role in this condition. Treatment tends to focus on resolution of the underlying systemic condition, but case reports for isotretinoin have shown some improvement.
