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Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas. Management of vascular tumors complicated by KMP is challenging, and it is common for referral centers to receive patients in critical medical condition after multimodality treatment failure of vascular anomalies. Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP. A full-term male newborn with KHE complicated by KMP is reported. Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids.

Abstract Background: Vulvar diseases are common in the general population and have a negative impact on the quality of life. Objectives: To describe our experience as dermatologists in the management of vulvar dermatosis consultations. Methods: A retrospective observational study was conducted with patients who attended monographic vulvar consultations over a 5-year period. Clinical information was obtained from the patient’s charts. Results: 148 women were studied. Their mean age was 43.24 years (standard deviation: 15.15 years), with ages ranging from 4 months to 80 years. 53.4% of patients took between 2 and 5 years to seek medical attention for the first time. The most frequent diagnosis was lichen sclerosus (41.9%), irritative eczema of the vulva (14.9%), and lichen simplex chronicus (10.1%). 83.8% reported anogenital itching, 66.2% pain, and 45.9% dyspareunia. The most frequently prescribed treatment was ultra-potent topical corticosteroids (clobetasol propionate; 41.2%). Patients with lichen sclerosus were significantly older than those who presented with any of the other diseases. No differences were found in terms of either the time of disease evolution or in symptom presentation. Study limitations: Retrospective study. Vulvar diseases with an infectious cause are usually managed in primary care, therefore, were not included. All patients were recruited from a single private hospital which limits the comparisons with the public health system. Conclusions: Vulvar diseases frequently occur and are associated with high morbidity. It is essential to promote the development of specific vulvar consultations in hospitals. Specialties such as dermatology, gynecology, urology, or physiotherapy must be part of these units.