ABSTRACT
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.
Subject(s)
Female , Humans , Young Adult , Blister , Dapsone , Diagnosis , Diagnosis, Differential , Histiocytic Necrotizing Lymphadenitis , Lupus Erythematosus, Systemic , Pancytopenia , Skin , Transcutaneous Electric Nerve StimulationABSTRACT
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.
Subject(s)
Child , Humans , Male , Alopecia , Femur Head , Glaucoma , Mongolian Spot , Necrosis , Neurocutaneous Syndromes , Nevus , Nevus of Ota , Parturition , Port-Wine Stain , Varicose VeinsABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Acupuncture Therapy , Blister , Debridement , Drainage , Edema , Elbow , Fascia , Fasciitis, Necrotizing , Gram-Negative Bacteria , Necrosis , Pliability , Skin , Streptococcus , Subcutaneous Fat , Subcutaneous Tissue , Transcutaneous Electric Nerve Stimulation , Upper ExtremityABSTRACT
We report a case of angiocentric lymphoma in a 41-year-old female who complained chiefly of a genital ulcer and a bulging conjunctiva on the right eye. She had a history of a re-current oral ulcer over a 5 year period and a genital ulcer over 3 years. The above manifested clinical findings may suggest Behcet's disease. However, a biopsy from the upper vaginal wall and the inferior turbinate showed that atypical small and large lymphocytes with hyper-chromatic, elongated and convoluted nuclei had aggregated around the thickened blood vessels, which is consistent with angiocentric lymphoma. Immunophenotypically, the atypical cells were positive for pan T-cell markers not B-cells. Serologically, the patient showed a high titer of i?B virus viral capsid antigen(VCA) IgG.
Subject(s)
Adult , Female , Humans , B-Lymphocytes , Biopsy , Blood Vessels , Capsid , Conjunctiva , Immunoglobulin G , Lymphocytes , Lymphoma , Oral Ulcer , T-Lymphocytes , Turbinates , UlcerABSTRACT
BACKGROUND: Nerve growth factor(NGF), brain derived neurotrophic factor(BDNF), neurotropin 3(NT-3) and neurotropir-4/5 are neurotrophic factors necessary for the development and maintenance of specific neurors. The tyrosine protein kinase(trk) receptors exhibit specificity for differ ent neurotrophins. NGF is the cognate ligand for the trk A receptor, BDNF binds to trk B receptor and NT-3 binds to irk A, trk B and trk C receptors, Since melanoma cells are devived from neural ectoderm, growth factors which affect neuronal tissue may have a role in melanoma biology. OBJECTIVE: The purpose of this study is to demonstrate the presence of trk receptors in rnelanoma cells and observe th effect of K-252a on these melanoma cells growth and differentiation. METHODS: After K252a over a range of 0-200nM was added into their cell lines, we exam ined cell viability of SK 28 and SK 30 cells. We performed this to examine the expression of the trk by flow cytometry and immunoblotting. RESULTS: 1. The incubation of SK 28 cells and SK 30 cells with K 252a resulted in a dose dependent inhibition of cell proliferation. 2. In the flowcytometry, SK 28 cells and SK 30 cells showed a high expression of trk A and trk B, not trk C. 3. Using immunoblottiiig, trk in SK 28 cells and SK 30 cells was not expressed. CONCLUSIONS: These results indicate that the identification of tyrosine protein kinase reeeptors and their inhibitor which affect differentiation and growth of a melanoma may provide an additional therapeutic option for treatment of melanoma.
Subject(s)
Biology , Brain , Cell Line , Cell Proliferation , Cell Survival , Ectoderm , Flow Cytometry , Immunoblotting , Intercellular Signaling Peptides and Proteins , Melanoma , Nerve Growth Factor , Nerve Growth Factors , Neurons , Protein-Tyrosine Kinases , Receptor, trkB , Sensitivity and Specificity , TyrosineABSTRACT
Maffuccis syndrome was first reported by Maffucci, in 1881. This syndrome is manifested by multiple enchondromas and hemangiomas, characterized by non-hereditary mesenchymal neoplasia that has a propensity for malignant transformation. Involvement of long bones, particularly the bones of the hands and feet, is most common. The most important complication of this syndrome is the high frequency of chondr osarcomatous metaplasia. We report a case of Maffuccis syndrome in a 56-year-old woman with multiple hemangioma on both the hands and forearms, left foot and enchondromas of multiple bones. The disease started at the age of 8 and was associated with deformity, but malignant transformation was not found.
Subject(s)
Female , Humans , Middle Aged , Chondroma , Congenital Abnormalities , Enchondromatosis , Foot , Forearm , Hand , Hemangioma , MetaplasiaABSTRACT
We report a case of Hunter' s syndrome with characteristic nodules on the upper back. The patient was a 7-year-old Korean boy who presented with ivory-colored papules and nodules on both sides of the scapula, pectoral regions and lateral aspects of the upper arms. These lesione are regarded as pathognomonic cutaneous markers for Hunter s syndrome. He also presented with truncal hypertrichosis, retarded growth, short neck, round face, claw like contractures of hands, multiple joint contractures, and a clear cornea. Severely elevated glycosaminoglycan levels were present in the patient s urine samples. The patient s 5-year-old brother had similar clinical features.