S. Hesse

Tumeur vasculaire géante de l’adulte : angiome en touffe ou hémangioendothéliome kaposiforme

Resume Introduction L’angiome en touffe et l’hemangioendotheliome kaposiforme sont deux tumeurs vasculaires rares, benignes mais agressives decrites essentiellement chez l’enfant. Observation Un homme de 72 ans avait depuis 10 ans une tumeur vasculaire de l’epaule droite qui augmentait de taille progressivement et atteignait, lors de la consultation, 50 cm de grand axe. L’examen histologique montrait que cette tumeur vasculaire associait par place des aspects d’angiome en touffe et d’hemangioendotheliome kaposiforme. Discussion La tumeur chez ce malade etait atypique par sa taille tres importante et jusqu’alors jamais decrite. L’association histologique d’aspects pouvant relever a la fois d’un angiome en touffe et d’un hemangioendotheliome kaposiforme va dans le sens des publications recentes qui tendent a reunir ces deux lesions et a en faire des stades evolutifs d’une meme entite.

Congenital and Disseminated Pyogenic Granuloma-like Vascular Lesions

We report an exceptional case of multiple cutaneous and visceral neonatal pyogenic granuloma (PG) initially suggestive of a diffuse neonatal haemangiomatosis. CASE REPORT A full-term female newborn, with no significant past medical history, was referred to our department for treatment of an acute respiratory distress syndrome of neurological origin at day 8 of life. At birth, she presented with 3 small angiomatous papules and 4 subcutaneous nodules suggestive of neonatal hae-mangiomatosis (NH) (Fig. 1). A brain MRI revealed a highly vascularised brain stem tumour suggestive of glioma (Fig. 2), associated with 2 abnormal hepatic lesions consistent with infantile haemangiomas (IH) on ultrasound and CT scan. Methylprednisolone was started for the suspected glioma-associated oedema, and vincristine and propranolol were introduced for NH. After initial improvement, an acute intracranial hypertension related to cystic evolution of the disease necessitated surgical resection at the age of 2 months. Pathological examinations of the brain, cutaneous and subcutaneous lesions were similar, showing a vascular lobular proliferation of capillaries highly suggestive of PG. The misdiagnosis of glioma was eliminated. The GLUT-1 antigen marker was negative, ruling out the diagnosis of NH-like infantile haemangioma (Fig. 3). Lymphatic marker (D2-40) was also negative and eliminated a multifocal lymphangioendotheliomato-sis with thrombocytopaenia (MLT). Cutaneous and hepatic lesions gradually regressed. She is currently in complete remission after completing a treatment over 18 months with propranolol but a spontaneous improvement can not be excluded. DISCUSSION

Persistent telangiectatic erythema following nostril piercing

reinitiated; within two months her pruritus ceased, her facial lesions diminished, and eyebrows began to reappear (Fig. 2). In summary, in contrast to lepromatous leprosy and cutaneous T-cell lymphoma or mycosis fungoides, actinic reticuloid and systemic mastocytosis are uncommon conditions associated with leonine facies and eyebrow loss. Topical and systemic treatment of the condition associated with the eyebrow loss may result in diminishing the related symptoms. However, in most patients, the facial skin lesions persist and loss of eyebrows is permanent. Yet, in a patient with systemic mastocytosis-associated leonine facies and eyebrow loss, the infiltrative cutaneous facial papules decreased in size and the eyebrows reappeared.