ABSTRACT
Dyskeratosis congsnita is a rare congenital disorder characterized by the triad of reticular pigmentation of the skin. dystrophic naila, and leukoplakia of the mucous membrane, and is often associated with severe pancytopenia. A 9-year-old boy had reticular pigmentation of the skin, dystropbic changes of the finger and toe nails, white patches of the buccal mucosa, mild hyperkeratosia of the palms and soles, excesaive lacrimation, dysphagia and severe pancytopenia, Bone marrow showed hypoplastic anemia and decreased cell mediated immunity was noticed.
Subject(s)
Child , Humans , Male , Anemia, Aplastic , Bone Marrow , Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Deglutition Disorders , Dyskeratosis Congenita , Fingers , Immunity, Cellular , Leukoplakia , Mouth Mucosa , Mucous Membrane , Pancytopenia , Pigmentation , Skin , ToesABSTRACT
Malignant schwannoma is a rare spindle cell tumor derived from schwann cells. There is no significant age or sex distribution. The typical clinical presentation is that of a painless slowly enlarging soft tissue mass, occasionally associated with a peripheral neuropathy. Tumors tend to recur lacally and eventually metastasize, commonly to the lung. We report a case of solitary malignant schwannoma with metastsis to the regional axillary lymph nodes. The patient is a 50-year-old male who had the recurred tumor on right upper arm and child fist sized palpable painless mass on right axilla, which were confirmed to be malignant schwannoma and. lymph node metastasis. But we could not find any evidence of pulmonary metastasis. Tumor and metastatic lymph nodes were widely excised and skin graft was performed.
Subject(s)
Child , Humans , Male , Middle Aged , Arm , Axilla , Lung , Lymph Nodes , Neoplasm Metastasis , Neurilemmoma , Peripheral Nervous System Diseases , Schwann Cells , Sex Distribution , Skin , TransplantsABSTRACT
Epidermolysis bullosa aimplex is a dominantly inherited syndrome characterized by the dissolution of the basal epidermal cells with mechanical stress. A 23 year-old man developed recurrent bullous eruptions with pain on hands, feet and face since childhood, which healed spontaneously without scarring. Grandmother of his father's side, father, aunt of his father's side and two of his three brothers had suffered similar skin lesions. Light and electron microscopy showed degeneration and bulla formation in the basal cell layer of epidermis and intact basement membrane attached to dermis.
Subject(s)
Humans , Young Adult , Basement Membrane , Cicatrix , Dermis , Epidermis , Epidermolysis Bullosa Simplex , Epidermolysis Bullosa , Fathers , Foot , Hand , Microscopy, Electron , Siblings , Skin , Stress, MechanicalABSTRACT
Acute febrile neutrophilic dermatosis, which was introduced to the dermatologic literature by Sweet in 1964, is characterized by persistent high fever and preceding upper respiratory infection like symptoms, polymorphonuclear leukocytosis in the peripheral blood, raised painful plaques on the limbs, face and neck, histologically a dense dermal infiltration with polymorphonuclear leukocyte, dramatic response to corticosteroids, and the absence of scarring. Case 1 was a 35 year-old female who had suffered from malaise, sore throat, and fever 3 to 7 days before the each episode of the cutaneous manifestation. Painful, red, raised, well-demarcated plaques appeared on the face with tendency of recurrences, and was accompanied by a few, small, shallow ulcerations on the lower Iip and tongue, and conjunctivitis. Polymorphonuclear leukocytosis (10400 with 65%, 11600 with 75%) and elevated ESR(23mm/hr, 19mm/hr) were found on two occasions. Histologically a dense inflammatory cell infiltration composed predominantly of polymorphonuclear neutrophils, small round cells, and a few eosinophils were seen in the dermis and subcutis, especially around the dermal capillaries. Alao there were many nuclear debris and marked endothelial cell proliferations. (countinued...)
Subject(s)
Adult , Female , Humans , Adrenal Cortex Hormones , Capillaries , Cicatrix , Conjunctivitis , Dermis , Endothelial Cells , Eosinophils , Extremities , Fever , Leukocytosis , Neck , Neutrophils , Pharyngitis , Recurrence , Sweet Syndrome , Tongue , UlcerABSTRACT
There have been no standard treatments for recurrent aphthous stomatitis, and clinical management is usually directed toward symptomatic relief. Recent immunological investigations have focused on possible imrnunopathogenesis of the disease. Several reparters suggested that levamisole, nonspecific immune-stimulator, had a beneficiaI effect in controlling attacks of recurrent a,phthous stomatitis and in reducing subsequent episodes. The present study was undertaken to evaluate the therapeutic effectiveness of levamisole in patients with recurrent aphthous stomatitis. A total of 8 patients who had had recurrent aphthous stomatitis for 2 to 20 years and who had experienced at least one episode per month were selected for this study from the department of dermatology, National Medical Center, through March 1979 to September 1979, Levamisole (Decaris') was given 150mg, p.o., once daily on 3 consecutive days every week for 2 months. Tbe results were as follows. 1) One patient had to have levamisole discontinued due to a high fever and exacerbations of tbe oral ulcerations. 2) 5 patients showed beneficial effects in reducing tbe number, frequency, pain and severity of recurrent aphthous stomatitis. 3') One patient showed no therapeutic response. 4) One patient, who has experienced new oral ulcerations continuously before starting levamisole, showed no recurrences of the lesions during the follow-up period of 3 months. 5) Side effects during levamisole administration were transient and generally mild, They included nausea, headache, dizziness and high fever.