Résumé
Kasabach-Merritt phenomenon does not occur with common hemangioma, rather it is associated with the more aggressive Kaposiform hemangioendothelioma and rarely with other vascular neoplasm. We report the case of an adult who was diagnosed as Kaposiform hemangioendothelioma complicated by Kasabach-Memtt phenomenon. This is the first report in Korea of an adult with Kasabach-Merritt phenomenon who has osteolytic changes of femur, pelvic bone, and lumbar spine.
Sujets)
Adulte , Humains , Fémur , Hémangioendothéliome , Hémangiome , Syndrome de Kasabach-Merritt , Corée , Os coxal , Rachis , Tumeurs vasculairesRésumé
Syndromal acanthosis nigricans is associated with cellular resistance to the action of insulin and other endocrinopathies. In the insulin resistant state, increased insulin binds to the receptors of insulin-like growth factors (IGFs). IGFs are growth prompting peptides, which act as direct mitogens on cells containing the receptors. Through these actions, increased cellular growth and metabolism cause the skin lesions of acanthosis nigricans. Hyperandrogenism is also found in syndromal acanthosis nigricans patients. When associated with insulin resistance, these states are proportionally related. Increased insulin prompts the synthesis of androgen and conversely, increased androgen elevates the insulin resistance. We present three cases of syndromal acanthosis nigricans with hyperpigmented skin lesions and obesity. Laboratory findings demonstrated normal fasting blood suga levels with increased insulin and C-peptide levels and decreased insulin receptors. The oral glucose tolerance test was normal. We suggest that these patients were in an insulin resistant state. Skin biopsies from the axilla in all three cases showed hyperkeratosis, irregular acanthosis and upward projection of dermal papillae as finger like fashion. Thus all three cases represent syndromal acanthosis nigricans associated with an insulin resistant state based on clinical, histopathological, and laboratory findings. The three patients were treated with dietary control.
Sujets)
Humains , Acanthosis nigricans , Aisselle , Biopsie , Peptide C , Jeûne , Doigts , Hyperglycémie provoquée , Hyperandrogénie , Insulinorésistance , Insuline , Métabolisme , Mitogènes , Obésité , Peptides , Récepteur à l'insuline , Peau , SomatomédinesRésumé
A 38-year-old man presented with a 2-year history of recurreit irticaria, abdominal pain and arthralgia of his finger joints. These symptoms were not con rolled in spite of taking medication and had a tendence to be aggravated in the winter. Laboratory findings revealed hvpocomlementemia, hypergammaglobulinemia and a positive rect on with an ice cube test. Skin biopsy specimen taken from the urticarial lesion of the left upper arm showed leukocytoclastic vasculitis with perivascular neutrophilic infiltrition, nuclear dusts, and extravasation of red blood cells. We thus made the diagnosis of hypocomplementemic urtirarial vasculitis syndrome with cold urticaria. The patient was treatcd ith systemic corticosteroids and antihistamines.
Sujets)
Adulte , Humains , Douleur abdominale , Hormones corticosurrénaliennes , Bras , Arthralgie , Biopsie , Diagnostic , Poussière , Érythrocytes , Articulation du doigt , Antihistaminiques , Hypergammaglobulinémie , Glace , Granulocytes neutrophiles , Peau , Urticaire , VasculariteRésumé
Bowenoid papulosis is manifested by clinically the benign-appearing papules of anogenital region and histopathologically similar features of squamous cellurar inoma in situ in human. A cause is thought to be Human Papillomavirus infection, type 16. The treatment is mainly through conservative management but others are topical application of podophyllin, shave excision, local excision, cryotherapy, electrodessication, topical applcation of 5-FU ointment and subcutaneous injection of recombinant interferon gamma. We present a case of Howenoid papulosis treated with DNCB imriunotherapy. The patient, had multiple papules on the penis with mild pruitus. Histopatnologic findings showed crowding and irregular arrangement of the nucleic many of which are large, hyperchromatic, and pleomorphic. Dyskeratotic and multinucleated keratinocytes wire also present, as were atypical mitoses, scattered in epidermal layer. We treated him with DNCB immunotherapy. Initially he was sensitized with 2,000 ug/0.1ml and two weeks later he received 50 ug/0.1ml per every week. After 3 weeks, he had showed no improvement and we elevated the dose to 100 ug/0.1ml. He was improved after 6th trial and his skir. lesions were almostly disappeared at present.