Occasionally, patients with gastrointestinal Crohn's disease (CD) develop granulomatous skin lesions at sites remote from the gastrointestinal tract, separated from other ulcerations by normal skin, a phenomenon that has been referred to as metastatic cutaneous CD. Although metastatic CD of the vulval region has been often reported in English literature, we could not find such cases with family history. We report a case of vulval CD occurring in siblings.
Crohn Disease* ; Gastrointestinal Tract ; Humans ; Siblings* ; Skin ; Ulcer ; Vulva*

Dyschromatosis symmetrica hereditaria(DSH), also known as reticulated acropigmentation of Dohi, is a rare pigmentary disorder that has autosomal dominant inheritance. Characteristic lesion includes a mixture of hyperpigmented and hypopigmented macules with reticulate pattern, which are distributed on the dorsal aspects of the extremities. We report a 5-year-old boy who showed typical DSH with family history in four generations.
Child, Preschool ; Extremities ; Family Characteristics ; Humans ; Male ; Wills

An epidermal grafting using the top of suction blister has been used successfully for the treatment of depigmented skin, such as vitiligo, achromic areas after dermabrasion, and burns. A 9-year-old girl showed the delayed wound healing of epidermal grafting site for the treatment of vitiligo on popliteal fossa. Direct microscopic examination (KOH) from the wound showed numerous hyphae. Fungus culture and germ tube test confirmed Candida albicans from the wound surface. The wound healed by antifungal treatment, leaving scar.
Blister ; Burns ; Candida albicans ; Child ; Cicatrix ; Dermabrasion ; Female ; Fungi ; Humans ; Hyphae ; Skin ; Suction ; Transplants ; Vitiligo ; Wound Healing ; Wounds and Injuries

Pincer nail is a dystrophy characterized by an increase of transverse curvature along the longitudinal axis of the nail and reaches its greatest proportion at the distal part. We report a case of a 20-year-old woman with pronounced pincer nails on both toe nails. By median longitudinal incision with use of CO2 laser, her nail plate was separated, and aluminum splint was applied on nail plate. Her pain disappeared one month after treatment. The treated nail showed a normal shape even at the 12 months of follow-up. We recommend nail plate separation as a good method for treatment of pincer nail.
Aluminum ; Axis, Cervical Vertebra ; Female ; Follow-Up Studies ; Humans ; Lasers, Gas ; Splints ; Toes ; Young Adult

Ehlers-Danlos syndrome is a heterogeneous group of inherited generalized connective tissue disorders characterized by hyperextensibility and fragility of skin, joint hepermobility, abnormal scarring, easy bruisibility, visceral and neurological manifestations. It is classified into 10 different major subtypes based on genetic, biochemical, and clinical characteristics. We report a case of type II Ehlers-Danlos syndrome in a 19-year-old male who showed soft and velvety skin with marked hyperextensibility, thin atrophic scarring from trauma, and hypermobile finger joints.
Cicatrix ; Connective Tissue ; Ehlers-Danlos Syndrome* ; Finger Joint ; Humans ; Joints ; Male ; Molecular Biology ; Neurologic Manifestations ; Skin ; Young Adult

BACKGROUND: To investigate the role of alpha-adrenergic receptors in the development of delayed afterdepolarization, the effect of alpha-adrenoceptor stimulation and blockade on ouabain induced delayed afterdepolarization(DDAD) was examined in rabbit heart Purkinje fibers. METHODS: Purkinje fibers, taken from adult rabbit(1.8 - 2.0kg) heart anesthetized with penobarbital, were mounted in a Luicite chamber and superfused with Tyrode's solution. The transmembrane potentials were measured by the conventional microelectrode technique while the fibers were being stimulated with rectangular pulses of 50% above threshold voltage. The delayed afterdepolarizations were induced by overdrive excitation in the presence of ouabain. RESULTS: Delayed afterdepolarizations were not observed during superfusion of the control Tyrode's solution containing propranolol(5x10(-7)M). However, the addition of ouabain in the presence of propranolol elicited DADs which were dose-, time- and drive cycle length- dependent. Phenylephrine(PE ; 10(-7)M), and alpha-adrenoceptor agonist, potentiated the ouabain-induced DAD during the initial superfusion(for 10 or 20 min) of the test Tyrode's solution. However, it was followed by attenuating-effects after a superfusion time of 50 to 60 min. Both effects showed ouabain dose-dependence. Ouabain(2x10(-7)M), in the presence of propranolol, depolarized the maximum diastolic potential and shortened the action potential duration, and the addition of PE(10(-7)M) did not affect the characteristics of action potential except a decrease in velocity of phase 0 depolarization. Prazosin, an alpha1-adrenoceptor antagonist, inhibited the PE's enhancing effects of ouabaininduced DDAD at 20 min superfusion, but did not affect the attenuating-effects of PE at 60 min superfusion. On the other hand, yohimbine, an alpha2-adrenoceptor antagonist, did not affect the PE's DAD potentiating-effects at 20 min superfusion, but inhibited the attenunating-effects of PE at 60 min superfusion. CONCLUSION: It is inferred that alpha-adrenergic stimulation induce delayed afterdepolarization and triggered activity in the rabbits, being responsible for the arrhythmia development, and the effects are mainly due to the action of alpha1-subtpe adrenoceptor stimulation.
Action Potentials ; Adult ; Arrhythmias, Cardiac ; Hand ; Heart ; Humans ; Membrane Potentials ; Microelectrodes ; Ouabain ; Prazosin ; Propranolol ; Purkinje Fibers ; Rabbits ; Receptors, Adrenergic, alpha* ; Yohimbine

No abstract available.
Poland Syndrome* ; Poland*

No abstract available.
Blood Patch, Epidural ; Child ; Humans ; Post-Dural Puncture Headache

Segmental neurofibromatosis is rare disorder characterized by cafe-au-lait macules and neurofibromas, or only neurofibromas, limited to one region of the body. Typically the neurofibromas are unilateral and are occupied a single dermatome in most patients. We report a 62-year-old man with segmental neurofibromatosis on the right knee in the distribution of dermatomes L3 and L4.
Humans ; Knee* ; Middle Aged ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1

OBJECTIVES: Cord blood stem cells have been widely used as donor cells for bone marrow transplantation recently. These cells can give rise to a variety of hematopoietic lineages to repopulate the blood. Recent observations reveal that some bone marrow cells and bone marrow stromal cells(MSCs) can grow to become either neurons or glial cells. It is, however, unclear whether or not there exists stems cells which can differentiate into neurons in the blood during the early stages of postnatal life. METHODS: Human cord blood stem cells were prepared from human placenta after full term delivery. To induce neuronal differentiation of stem cells, beta-mercaptoethanol was treated. To confirm the neuro-glial characteristics of differentiated stem cells, immunocytochemical stain for NeuN, neurofilament, glial fibrillary acidic protein(GFAP), microtubule associated protein2(MAP2) was performed. RT-PCR was performed for detecting nestin mRNA and MAP2 mRNA. RESULTS: We showed in this experiment that neuro-glial markers(NeuN, neurofilament, MAP2, GFAP) were expressed and axon-like cytoplasmic processes are elaborated in the cultured human cord blood stem cells prepared from new born placenta after full term delivery. Nestin mRNA was also detected in fresh cord blood monocytes. Conclusions: These results suggest that human cord blood derived stem cells may be potential sources of neurons in early postnatal life.
Bone Marrow ; Bone Marrow Cells ; Bone Marrow Transplantation ; Cytoplasm ; Fetal Blood* ; Humans* ; Microtubules ; Monocytes ; Nestin ; Neural Stem Cells ; Neuroglia ; Neurons ; Placenta ; RNA, Messenger ; Stem Cells ; Tissue Donors ; Umbilical Cord*