Plexiform neurofibroma is considered a pathognomic of Von Recklinghousen's disease, which involves the deep and large nerve trunk. These are large irregular nerve fascicles which result from an increase in endoneural matrix within individual nerve facicles, without an increased number of nerve fibers. We experenced a case of Von Recklinghausen's disease in a 24 year-old male who had variable cutaneous skeletal, and CNS lesions. He presented multiple neurofibromas, cafe-au-lait spots, and axillary freckles as common cutaneous lesions of NF-I and giant pigmentation, sacral hypertrichosis, and plexiform neurofibroma as unusual cutaneous lesions. Also he had a scoliosis, bowing deformity of the humerous and wedging deformity of the body of the 5th cervical spine as a skeletal manifestation and cortical calcification in the occipital area as a CNS manifestation.
Cafe-au-Lait Spots ; Congenital Abnormalities ; Humans ; Hypertrichosis ; Male ; Melanosis ; Nerve Fibers ; Neurofibroma, Plexiform* ; Neurofibromatoses ; Neurofibromatosis 1 ; Pigmentation* ; Scoliosis ; Spine ; Young Adult

We have experienced a 14 year old female patient who had suffered from headache, dizziness, exertional dyspnea and chest pain during 6 months. She was diagnosed as primary pulmonary hypertension by ultrasonogram and cardiac cathererization. On the cardiac catheterization, there was elevated pulmonary artery pressure and normal pulmonary wedge pressure. We report this case with related literature review.
Adolescent ; Cardiac Catheterization ; Cardiac Catheters ; Chest Pain ; Dizziness ; Dyspnea ; Female ; Headache ; Humans ; Hypertension, Pulmonary* ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Ultrasonography

The antiphospholipid antibody syndrome is an acquired multisystemic disorder characterized by persistent elevated antiphospholipid antibodies and/or hypercoagulation in veins or arteries, or both. The clinical manifestations of the antiphospholipid antibody syodrome are recurrent thrombosis, fetal loss, thrcenbocytopenia, and various cutaneous lesions. Skin lesions are the first sign of this syndrome in 41% of patients and systemic thrombosis develops in 40% of them. Livedo reticularis is the most common cutaneous finding of the antiphosphotipid antibody syndrome. Although vasculitis has not been frequently noted in antiphospholipid antibody syndrome, some vasculitis such as polyarteritis nodosa, giant cell arteritis, and other nonspecific vasculitides have been found in association with antiphospholipid antibody syndrome. We present a male patient with typical manifestations of leukocytoclastic vasculitis with deep vein thrombosis and positive antiphospholipid antibodies. It suggests that a case of antiphospholipid antibody syndorme was accompanied with cutaneous leukocytoclastic vasculitis.
Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Arteries ; Giant Cell Arteritis ; Humans ; Livedo Reticularis ; Male ; Polyarteritis Nodosa ; Skin ; Thrombosis ; Vasculitis* ; Vasculitis, Leukocytoclastic, Cutaneous ; Veins ; Venous Thrombosis

BACKGROUND: Bentonite clay, which is a major component of mud pack, has been used for various purposes in cosmetics. Glycolic acid is known to be effective in the treatment of acne. Al-though those products are used widely, information on the mode of action and effects on the skin are little and controversial till now. OBJECTIVE: To investigate whether bentonite alone, or bentonite with glycolic acid in mixed formulation affect the stratum corneum leading to alteration on cutaneous barrier function and whether those products alter the lipid lamellae and desmosomes of corneocytes. MATERIALS AND METHODS: Mud pack-type ointment of bentonite, bentonite and 5% glycolic acid formulation, bentonite and 10% glycolic acid formulation were applied on the volar fore-arm of the five healthy men and flank skin of five 6-8 week old hairless mice. Transepidermal water loss and capacitance were measured. Electron microscopic examination after ruthenium tetroxide postfixation was performed on the flank skin of the mice. RESULTS: Transepidermal water loss(TEWL) increased immediately and normalized 4 to 6 hours later after removal of vapor permeable membrane in both mouse and human. Capacitance did not show any evidence of change in the water content of the stratum corneum. Electron microscopic examination revealed that lipid lamellae and desmosome of corneocytes were not de-graded, but lamellar body secretion and partially electron-lucent material was-increased in 10% glycolic acid and bentonite mixture-treated area. CONCLUSION: Barrier function of stratum corneum is not disturbed by bentonite and glycolic acid formulations at the concentration used. Barrier structures are not disrupted, but lamellar body secretion and partially electron-lucent material was increased by bentonite and glycolic acid formulations at higher concentration.
Acne Vulgaris ; Animals ; Bentonite* ; Desmosomes ; Humans ; Male ; Membranes ; Mice ; Mice, Hairless ; Mud Therapy ; Ruthenium ; Skin ; Water

PURPOSE: Recently developed BMS(TM) (Zassi Bowel Management System(TM): Hollister Inc., Illinois, USA) can provide effective nonsurgical fecal diversion without the risks associated with colostomy creation and subsequent closure. Our aim is to evaluate the effectiveness of the BMS in diverting feces from the perianal wide surgical wound in patients with Fournier's gangrene. METHODS: BMS(TM) was applied in five patients (male: 2, median age; 44) with Fournier's gangrene from January 2000 to September 2001. The treatments consist of three times a day wound dressing after wide surgical debridement and intravenous antibiotic therapy. For evacuation of feces, twice daily warm saline irrigation was administered via BMS(TM) or low daily doses of polyethylene glycol solutions were orally taken in. An endoscopic and anorectal manometric study was done to evaluate possible mucosal complications and anorectal functional changes. RESULTS: The average duration of the BMS application was 41 (range, 22~63) days. The result of a manometric study after immediate removal of the BMS(TM) showed a decreased mean resting pressure (range: 22~36 mmHg) and a decreased mean squeezing pressure (range: 32~39 mmHg). After 3 days, the sphincter pressure had improved markedly: mean resting pressures of 38, 45, 60, and 63 mmHg and mean squeezing pressure of 78, 89, 91, and 101 mmHg respectively. Fecal incontience was not noted in any patient. Other possible mucosal complications were not noted. There were no mortalit. CONCLUSIONS: BMS(TM) application in Fournier's gangrene patients after surgery successfully avoids a defunctioning colostomy. Furthermore, no significant complications were noted over a prolonged period up to 63 days.
Bandages ; Colostomy ; Debridement ; Fasciitis, Necrotizing ; Feces ; Fournier Gangrene ; Humans ; Illinois ; Polyethylene Glycols

BACKGROUND: Topical irritants disrupt the cutaneous permeability barrier through the removal of stratum comeum lipids. This perturbation of barrier integrity stimulates a variety of homeostatic repair responses that ultimately result in the normalization of bamer function. Object To measure the effect of desoxymethasone ointment, vaseline and hydrobase on the barrier recovery of benzalkonium chloride (BKC) imtated skin. MATERIALS AND METHODS: The left flank skin of 2-3 monthold hairless mice was treated with BKC and then desoxymethasone ointment, vaseline and hydrobase were applied. Transepidermal water loss (TEWL) was checked after 0, 3, 6, 9, 12, 15, 18, 21 and 24 hours. Electron microscopic examination was performed after 3 and 24 hours after desoxymethasone, vaseline and hydrobase had been applied. RESULTS: The recovery of TEWL was most prominantly observed in the desoxymethasone ointment treated group followed by vaseline and hydrobase. Electron microscopic examination using ruthenium tetroxide fixation revealed that secretion and numbers of lamellar bodies and complete formatice of lipid bilayers were most prominent at desoxymethasone ointment and vaseline treated group. CONCLUSION: Desoxymethasone ointment, vaseline and hydrobase can be good agents in improving bamer recovery after exposure to irritant material.
Animals ; Benzalkonium Compounds* ; Desoximetasone* ; Irritants ; Lipid Bilayers ; Mice ; Mice, Hairless* ; Permeability ; Petrolatum* ; Ruthenium ; Skin*

PURPOSE: To report a case of Charles Bonnet syndrome that developed after resection of a meningioma. CASE SUMMARY: The authors reviewed the medical record, brain magnetic resonance image, and Goldmann visual field test of a 56-year-old male patient who was diagnosed with a meningioma in the right parietal and occipital lobe and underwent resection of the tumor. The preoperative Goldmann visual field test showed homonymous left inferior quadrantanopsia. Subtotal resection of the mass in the right parietal and occipital lobe was performed, and postoperative histopathologic examination confirmed the diagnosis of a meningioma. Postoperatively, the patient complained of visual hallucination in an area of the eye with visual field defects. However, his consciousness and orientations were intact, and other cognitive functions were also normal. CONCLUSIONS: Visual hallucination can manifest in an area with visual field defects after resection of abrain tumor. In such a case, a diagnosis of Charles Bonnet syndrome should be considered.
Brain ; Brain Neoplasms ; Consciousness ; Eye ; Hallucinations ; Hemianopsia ; Humans ; Magnetic Resonance Spectroscopy ; Male ; Medical Records ; Meningioma ; Middle Aged ; Occipital Lobe ; Orientation ; Visual Field Tests ; Visual Fields

A 4-year-old boy has had a solitary sclerotic depressed plaque on the right anterior chest since birth. The histopathologic findings are consistent with morphea profunda: thickening, hyalinization, and homogenization of collagen bundles in the dermis and subcutaneous tissues, admixture with a prominent lymphocytic and plasma cell infiltrate, and sweat glands en-trapped between the thickened collagen bundles. We report a case of congenital solitary morphea profunda.
Child, Preschool ; Collagen ; Dermis ; Humans ; Hyalin ; Male ; Parturition ; Plasma Cells ; Scleroderma, Localized* ; Subcutaneous Tissue ; Sweat Glands ; Thorax

Deep skin and soft tissue defects with exposed bone and tendon is difficult to treat, because skin graft rarely survives and flap surgery is sacrifice of donor site. Since "Stage I" membrane was developed by Yannas and Bruke in 1980, numerous kinds of artificial skin have been developed. The adaptability of "Terudermis", developed by the Terumo Co., as an artificial skin composed of sponge made of a fibrillar atelocollagen and a heat-denatured atelocollagen, was clinically evaluated on application to 13 cases presenting deep skin and soft tissue defect with exposed bones and tendons from October 1997 to march 1998. Terudermis has the advantage of allowing early incorporation of fibroblasts and capillaries into its collagen sponge due to very weak dehydrothermal cross-linking. Before Terudermis graft, several days of wet dressing and debridement were required to prepare healthy well-vascularized bed because Terudermis was weak on unsanitary wounds. After bed preparation, Terudermis was grafted like usual skin graft. Tie-over bolster dressing or compressive dressing was used case by case. The dressing was opened 2~3 days after Terudermis grafting. Wet dressing was done daily until the skin graft was done. Autologous skin graft was done 2-3 weeks after Terudermis graft. Our clinical results indicated that Terudermis was beneficial in treating 77% of our patients. Through the use of this new method, treatment of severe skin and soft tissue defects that are usually treated by musculocutaneous or other conventional skin flaps can be replaced by Terudermis as an new artificial dermis.
Bandages ; Capillaries ; Collagen ; Debridement ; Dermis ; Fibroblasts ; Humans ; Membranes ; Porifera ; Skin ; Skin, Artificial ; Tendons ; Tissue Donors ; Transplants ; Wounds and Injuries*

Tegafur [1-(tetrahydro-2-furyl)-5-fluorouracil], the prodrug of 5-fluorouracil, is an anticancer agent. Several cutaneous reactions have been reported following systemic 5-fluorouracil for the treatment of malignancies. We report a patient with marked inflammation of the actinic keratosis following the use of tegafur for stomach carcinoma. The side-effect with 5-fluorouracil was beneficial as most actinic keratosis cleared following the inflammatory reaction. Dermatologists and oncologists should be aware of this potential side-effect, not only because it may become more prevalent but, most importantly, because it is not an allergic reaction to 5-fluorouracil but a dose-dependent response, and the chemotherapy may be continued in most patients.
Actins* ; Drug Therapy ; Fluorouracil* ; Humans ; Hypersensitivity ; Inflammation ; Keratosis, Actinic* ; Stomach ; Tegafur