Résumé
We report a case of type IIb hyperlipoproteinemia associated with xanthoma tuberosum, xanthoma planum and xanthoma striatum. Serum lipoprotein electrophoresis showed increased beta fraction and pre-beta fraction, suggesting type IIb hyperlipoproteinemia.
Sujets)
Électrophorèse , Hyperlipoprotéinémies , Lipoprotéines , Témazépam , XanthomatoseRésumé
Lovastatin(Mevacor) belongs to a new generation of potent drugs for the treatment of hypercho- lesterolemia. It acts by competitive inhibition of 3-hydroxyl-3-methylglutaryl coenzyme A(HMG-CoA) reductase, diminishing in that way the cholesterol synthesis in the liver. HMG-CoA reductase inhibitors also affect cutaneous lipid content. This can induce cutaneous side effects such as eczernatous rashes or xerotic, ichthyosiform lesions due to abnormality in the barrier function in some patients. We report a 54-year-old male patient who developed ichthyosiform skin lesions under treatment with lovastatin(Mevacor) for hypercholesterolemia.
Sujets)
Humains , Mâle , Adulte d'âge moyen , Cholestérol , Exanthème , Inhibiteurs de l'hydroxyméthylglutaryl-CoA réductase , Hypercholestérolémie , Foie , Lovastatine , Oxidoreductases , PeauRésumé
A 21-year-old woman, who had a one-year history of pancytopenia with histiocytic necrotizing lymphadenitis and hepatosplenomegaly, presented with a 5 day history of tense bullae, which were localized on the face. These clusters of tense bullae occurred on clinically normal skin, she did not have other skin lesions. A diagnosis of bullous systemic lupus erythematosus (BSLE) was established based on clinical, laboratory, histological, and immunological findings. The bullae showed good responses to dapsone (100mg, daily) and resolved within 10 days with-out scaring. Bullous lesions of SLE may be the first cutaneous manifestation in some patients with SLE and should be considered in the differential diagnosis of the other subepidermal bullous disorders.
Sujets)
Femelle , Humains , Jeune adulte , Cloque , Dapsone , Diagnostic , Diagnostic différentiel , Lymphadénite nécrosante histiocytaire , Lupus érythémateux disséminé , Pancytopénie , Peau , Neurostimulation électrique transcutanéeRésumé
Epithelioid hemangioendothelioma(EH) is a low-grade tumor of vascular endothelial cell origin, which most commonly occur in the lower extremities of middle-aged patients with an equal gender distribution. It may occur on internal organs such as the liver or lung or bones. A 54-year-old woman who had a history of metastatic adenocarcinoma of unknown origin on the right inguinal lymph node, was presented to us because of a 1.0 x 1.0cm-sized and 2 x 2cm-sized two subcutaneous nodule on her right shin. On H & E stain, there were nests of round nucleated, polygonal, vacuolated cells, some of which contained fragmented erythrocytes. Factor VIII related antigen, CD31 was positive. A diagnosis of epithelioid hemangioendothelioma was established based on histologic, immunohistochemical studies. Wide excision was performed. There was no recurrence nor the development of new lesions so far.
Sujets)
Femelle , Humains , Adulte d'âge moyen , Adénocarcinome , Diagnostic , Cellules endothéliales , Érythrocytes , Hémangioendothéliome épithélioïde , Foie , Membre inférieur , Poumon , Noeuds lymphatiques , Récidive , Facteur de von WillebrandRésumé
Phakomatosis pigmentovascularis is a rare cutaneous malformation characterized by a combination of nevus flammeus and pigmentary nevus such as nevus of Ota, nevus of Ito, mongolian spots, nevus spilus. It is often associated with systemic organ involvement. We report two cases of phakomatosis pigmentovascularis. The first was a 11-year-old boy, who had had diffuse nevus flammeus, mongolian spots since birth, and congenital glaucoma. The second case was a 9-year-old boy who had had nevus flammeus, mongolian spots, nevus of Ota, and nevus of Ito over various parts of his body since birth. He also had leg-length discrepancy, avascular necrosis of the femur head, congenital temporal alopecia and varicose veins.
Sujets)
Enfant , Humains , Mâle , Alopécie , Tête du fémur , Glaucome , Tache mongoloïde , Nécrose , Syndromes neurocutanés , Naevus , Naevus de Ota , Parturition , Tache lie de vin , VaricesRésumé
Necrotizing fasciitis is a life-threatening, invasive soft-tissue infection that is characterized by widespread, rapidly developing necrosis of the subcutaneous tissue and fascia with relative sparing of the skin and underlying muscle. In type I necrotizing fasciitis, anaerobes and gram negative bacteria are predominant; in the type II form, Group A p-hemolytic streptococcus is a major etiologic agent. It needs immediate surgical intervention for a favorable outcome. A 64-year-old male presented with extremely painful, erythematous, symmetric, tense swelling with yellowish or hemorrhagic blisters on his right upper extremity. Two weeks before the admission, pain on his elbow made him take acupuncture therapy, There was severe tenderness, pitting edema, and his right elbow showed limited flexibility. The patient underwent wide debridement and drainage immediately. Liquefactive necrosis was seen in the subcutaneous fat and superficial fascia without muscular involvement. Group A B-hemolytic streptococci were grown from the tissue culture specimen.