No abstract available.
Carcinogenesis* ; Oenothera biennis*

No abstract available.
Immunohistochemistry ; Keratins

Bilateral segmental neurofibromatosis is a rare disease characterized by bilateral neurofibromas, with or without pigmented lesion, or unilateral neurofibromas with contralateral pigmented lesion, limited to a body segment. Partial unilateral lentiginosis is characterized by numerous lentigines localized to a body segment, often corresponding to one or more dermatome. Bilateral segmental neurofibromatosis combined with partial unilateral lentiginosis is very rare, and to our knowledge, only 2 cases have been reported in English literature. We herein report another case of bilateral segmental neurofibromatosis with partial unilateral lentiginosis in a 46-year-old woman.
Female ; Humans ; Lentigo* ; Middle Aged ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1 ; Rare Diseases

No abstract available.
Thanatophoric Dysplasia*

We present 2 cases of invasive extramammary Paget disease occuring in the vulva area of a 60 year old female, and in the scrotal and penile area of a 63 year old male patient. The histologically typical Paget cells were not only seen in the surface epithelium but were also involved in the outer root sheath of the hair follicles. Stromal infiltration of tumor cells into the upper dermis were present in both cases, however, no underlying primary sweat gland carcinoma was present. Metastatic foci of inguinal lymph nodes showed apocrine-type epithelium with abundant eosinophilic granular cytoplasm, which were positive for anti-CEA and GCDFP-15, as well as eccrine-type epithelium containing mucinous secretory materials in the lumen and the cytoplasm. Ultrastructural findings showed interdigitating plasma membranes with prominent desmosomes between the Paget cells, intracytoplasmic tonofibrils, intracellular tubules, lipid vacuoles, and enlarged mitochondria. Histological, immunohistochemical, and ultrastructural findings suggested that Paget cells showed both eccrine and apocrine differentiation.
Female ; Male ; Humans ; Neoplasm Metastasis

Epidermal nevus syndrome is a rare disease which includes disorders of bone, central nervous system, eye, kidney, vasculature, body symmetry, skin, and rarely, hypophosphatemic rickets which have been observed in association with epidermal nevi. We experienced a case of epidermal nevus syndrome. He presented with epidermal nevi and bilateral abdominal wall hernias at birth and developed right hemiparesis, mental retardation, and multiple fractures due to hypophosphatemic rickets. He died of pneumonia at the age of 6.
Abdominal Wall ; Central Nervous System ; Hernia ; Intellectual Disability ; Kidney ; Nevus* ; Paresis ; Parturition ; Pneumonia ; Rare Diseases ; Rickets, Hypophosphatemic* ; Skin

Epidermal nevus syndrome is a rare disease which includes disorders of bone, central nervous system, eye, kidney, vasculature, body symmetry, skin, and rarely, hypophosphatemic rickets which have been observed in association with epidermal nevi. We experienced a case of epidermal nevus syndrome. He presented with epidermal nevi and bilateral abdominal wall hernias at birth and developed right hemiparesis, mental retardation, and multiple fractures due to hypophosphatemic rickets. He died of pneumonia at the age of 6.
Abdominal Wall ; Central Nervous System ; Hernia ; Intellectual Disability ; Kidney ; Nevus* ; Paresis ; Parturition ; Pneumonia ; Rare Diseases ; Rickets, Hypophosphatemic* ; Skin

In the case of a soft tissue defect which requires thin & pliable tissues, the choice of donor site is limited due to flap bulkiness and donor site morbidity. To overcome these problems, a variety of perforator-based flaps such as paraspinous and parasacral perforator flap, deep inferior epigastric perforator flap, gluteal perforator flap, and thoracodorsal artery perforator flap have recently been introduced. We experienced 8 cases of soft tissue defects from December 1996 to March 1999 using the thoracodorsal artery perforator flap for reconstruction. We could elevate the cutaneous flap with preservation of the latissimus dorsi muscle flap only when it was based on one cutaneous perforator. Defatting procedure was possible for further thinning of skin flap. In one case, axillary defect after release of postburn scar contracture was repaired with island perforator flap and the other seven cases were repaired with free flap. The results were satisfactory. We believe the thoracodorsal artery perforator flap is useful for reconstruction of soft tissue defects which are large or under conditions requiring thin flap.
Arteries* ; Cicatrix ; Contracture ; Free Tissue Flaps ; Humans ; Perforator Flap ; Skin* ; Superficial Back Muscles ; Tissue Donors

A possible therapeutic effect of acetyl-L carnitine (ALCAR) on peripheral nerve injuries and the expression of Jun, the protein products of immediate-early genes(IEGs), in the spinal cord were investigated after sciatic nerve injury in Sprague-Dawley rats. Experimental animals were divided into 3 groups: intact sciatic nerve as a control group, surgical repair alone, and surgical repair with ALCAR treatment. Toe-spreading response, pinprick response, and compound action potential were measured to evaluate the recovery of sciatic nerve injury. Recovery of behavioral function and electrophysiological function both improved in the surgical repair with ALCAR treatment group. Weak basal expression of Jun was shown in the ventral horn the of spinal cord in the control group. In the surgical repair alone group or the surgical repair with ALCAR treatment group, Jun expression persisted until 28 days after injury in the ventral horn of the spinal cord. Moreover, the surgical repair with ALCAR treatment group showed more increased expression of Jun than the surgical repair alone group. These results suggest that ALCAR facilitates the functional recovery after peripheral nerve injury.
Acetylcarnitine ; Action Potentials ; Animals ; Carnitine* ; Horns ; Peripheral Nerve Injuries ; Rats* ; Rats, Sprague-Dawley ; Sciatic Nerve* ; Spinal Cord

Capsaicin, the pungent principle of hot peppers, is a neurotoxin that depletes unmyelinated primary sensory neurons (polymodal nociceptors) of neuropeptides like tachykinins. However, the role of capsaicin-sensitive sensory nerve in the production of cytokines, penicillin V (PEV)-induced active fatal anaphylaxis and other immune responses is not yet fully established. Neonatal mice were pretreated s.c. with a single injection of 10 ug of capsaicin per mouse in volume of 20 ul within 5 days of age. Using 5-8 week old mice pretreated as neonates with capsaicin, the capsaicin- pretreated and vehicle-treated control mice were examined for various parameters of immune responses described above. For the induction of active fatal anaphylaxis with PEV, 8 week old mice pretreated as neonates and age-matched capsaicin- untreated control mice were sensitized i.p. with 500 ug of PEV-ovalbumin conjugate plus 2*10(9) B. pertussis and 1.0 mg alum and challenged i.v. with PEV-bovine serum albumin conjugate 14 days later. It was found that neonatal capsaicin-pretreatment significantly enhanced contact hypersensitivity to TNCB and hemagglutination response to SRBC, but significantly inhibited the proliferation response of rnurine splenocyte to Con A and LPS. Interestingly, neonatal capsaicin pretreatment significantly inhibited the intensity of PEV-induced active fatal anaphylaxis and decreased the mortality due to anaphylactic shock. It also significantly inhibited LPS- induced production of cytokines such as TNF-a, IL-1B, IL-6, IL-10, and IL-12. The capsaicin-pretreatment also resulted in an inhibition of the activation of NF-kB. Taken together, these data showed for the first time that neonatal capsaicin-pretreatment significantly inhibited an antibiotic (PEV)-induced anaphylaxis and production of various cytokines, and suggest that capsaicin-sensitive primary sensory nerve may play an important regulatory role in active fatal anaphylaxis and cytokine production, thus potentially presenting tools for immune intervention. In particular, the data presented also indicated the possibility to selectively down-modulate cytokine production and NF-kB activation may offer a broad application for therapeutic intervention in neuroimmunological diseases and other pathological situations.
Anaphylaxis ; Animals ; Capsaicin* ; Cytokines ; Denervation* ; Dermatitis, Contact ; Hemagglutination ; Humans ; Infant, Newborn ; Interleukin-10 ; Interleukin-12 ; Interleukin-6 ; Mice ; Mortality ; Neuropeptides ; NF-kappa B ; Penicillin V ; Sensory Receptor Cells ; Serum Albumin ; Tachykinins ; Whooping Cough