Leg ; Osteogenesis, Distraction

We report two cases of Darier's disease improved with Etretmate in the 44-year-old father and his 18-year-old son. Clinically, multiple dirty brownish, verrucous, discrete or confluent papules and plaques were noted on their back, abdomen, and face. Histopathologically, both of them demonstrated hyperkeratosis, suprabasal acantholysis, acantholytic cells in the lacunae, corpsronds, grains, and dyskeratotic cells. Oral Etretinate 30mg daily was given in both cases with remakable improvement 3 weeks after treatment. Six months later, they showed almost total clearin, of their skin lesions. No specific adverse effect was noted. There has been no evidence of recurrence for 6 months follow up period.
Abdomen ; Acantholysis ; Acitretin* ; Adolescent ; Adult ; Edible Grain ; Darier Disease* ; Etretinate* ; Fathers ; Follow-Up Studies ; Humans ; Recurrence ; Skin

A 29-year-old woman had multiple, umbilicated papules on her fingers for 2 months. Skin biopsy specimen showed transepidermal perforation filled with plug of cellular debris, degenerated collagen and mucinous material. In the dermis, there were necrobiosis and peripheral palisading of mononuclear cells. Special stainings including masson's trichrome, alcian blue, acid orcein, and PAS showed necrobiotic collagen fibers and acid mucopolysaccharides on the necrobiotic zone and trasepidermal elimination canal. Topical application of 0.05% Clobetasol-17-propionate ointment improved her lesion.
Adult ; Alcian Blue ; Biopsy ; Collagen ; Dermis ; Female ; Fingers ; Glycosaminoglycans ; Granuloma Annulare* ; Granuloma* ; Humans ; Mucins ; Necrobiotic Disorders ; Skin

A 49-year-old male had erythematous to rusky red papules, indurated plaques and lichenified patches with hyperpigmentation on sun-exposed areas for 6 years. Phototest revealed the decreased rninimal erythemal dose to UVA(10J/cm. Photopatch test with 5% Trandate ointment, 5% hydrochlorthiazide ointment and vaselin. as a control were all negative. Two weeks after cessation of Trandate, an oral challenge of hydrochlorthiazide followed by phototest was perfrirmed resulting in exacerbation of skin lesions and photosensitivity with a decreased MED to UVA(10J/cm) again. After the cesation of Trandate containing hydrochlorthiazide, the skin lesions were improved with complete loss of photosensitivity. But, improvement of the infiltrated or licheified plaques were delayed. Presenile cataract previously noted in the patient seemed to be related to his longstanding intake of hydrochlorthiazide.
Cataract ; Humans ; Hyperpigmentation ; Labetalol ; Male ; Middle Aged ; Skin

We report a case of a phototoxic reaction in a 48-year-old female induced by griseofulvin ingestion. The patient hac! erythematous papules, vesicles and patches on the sun-exposed areas. Phototest revealed a decreased minimal erythemal dose to UVA (10J/cm). Photopatch tests with 1%, 5%, 10% Griseofulvin ointment and vaseline as a control and photoingestion tests with Griseofulvin (50mg b.i.d.) were all negative. After the cessation of Griseofulvin, her skin lesions were markedly improved with complete loss of photosensitivity.
Eating ; Female ; Griseofulvin ; Humans ; Middle Aged ; Petrolatum ; Skin

Erythromelalgia is a rare disorder characterized by intense hun ing pain in the distal extremities associated with erythema and increased skin temperature. The patient was a 42- year-old woman with a 5 year history of burning pain, erythema, and warmth of the skin on both hands and feet. She had been suffering from bronchiali astalima since childhood. The onset of the symptoms were temporally related to the attacks of hei bronchial asthma. Physical examination revealed mottled dusky red patches on her both ha ids and feet, and purplish acrocyanosis on the finger and toe tips. We present a case of secondary erythromelalgia associated with bronchial asthma and acrocyanosis.
Asthma* ; Burns ; Erythema ; Erythromelalgia ; Extremities ; Female ; Fingers ; Foot ; Hand ; Humans ; Physical Examination ; Skin ; Skin Temperature ; Toes

Hypothalamic suprachiasmatic nucleus (SCN) is a circadian pacemaker which controls diurnal behavioral and hormonal rhythms in mammals. The SCN receives environmental light signals through the retinohypothalamic tract, and glutamate is the major excitatory neurotransmitter in the retinohypothalamic tract. In the present study, we investigated the developmental expression of the mRNAs for N-methyl-D-aspartate type glutamate receptor (NR)1, NR2A, NR2B and NR2C subunits in the rat SCN using in situ hybridization with specific riboprobes. At postnatal day 2 (P2), P8, Pl5 and P45, the high level of NRI transcripts was observed in both ventrolateral and dorsomedial subdivisions of the SCN, and the distinct expression of NR2C mRNA was principally found in the dorsomedial SCN. The weak NR2B mRNA expression was clearly found in both subdivisions of the SCN at P2 and P8, whereas specific NR2B hybridization signals were not found at Pl5 and P45. There was no specific hybridization signal of NR2A in the SCN throughout the postnatal life. These findings implicate that NR may play an important role in the neonatal SCN. In addition, this study suggests that NR1, NR2B and NR2C might be the major NR subunits in the developing SCN, whereas NRI and NR2C could be the subunit components of NR in the adult SCN.
Adult ; Animals ; Glutamic Acid ; Humans ; In Situ Hybridization ; Mammals ; N-Methylaspartate* ; Neurotransmitter Agents ; Rats* ; Receptors, Glutamate ; RNA, Messenger* ; Suprachiasmatic Nucleus

The "Dandy-Walker syndrome" has been the source of much controversy not only as to its embryogenesis but also concerning the correct diagnosis and surgical management of this disorder. The original description by Dandy and Blackfan emphasized the association of hydrocephalus, posterior fossa cyst and maldevelopment of the cerebellar vermis and later, Taggart and Walker described the genesis of these anomalities to atresia of the foramina of Magendie and Luschka. The Neuroradiologic findings associated with the presence of a Dandy-Walker cyst are pathognomonic. These findings relating to the cystic dilatation of the fourth ventricle which accompanies atresia and its outlets, gave a detailed clinicoanatomical information about the hydrocephalus in the CT scan. The CT scan contributed to the new advanced fields in the diagnosis and surgical management of hydrocephalus combining with the posterior fossa, lesion, because it was easily able to detect the lesion site stereoscopically and also find out the relationship to the involved surrounding area comparing with each of the slice in the CT scan. The purpose of this paper is to attempt to clarify the differentiation of the Dandy-Walker cyst variant from the Dancy-Walker cyst and anatomically detect the damaged brain correlated with the ventricular dilatation on the CT scan. A 7 year-old boy was admitted to the Department of Neurosurgery, Catholic Medical Center on Dec, 22, 1977 with one month history of progressive headache, nausea, vomiting and gait disturbance. On his examination he showed moderate enlargement of head, papilledema, diplopia, horizontal nystagmus, and gait disturbance. We confirmed the diagnosis under the Dandy Walker Cyst Variant by conray ventriclography and CT scan. He underwent lateral ventriculo-peritoneal shunt and then his symptoms progressively improved. He was discharged to his home after the 14th day of hospitalization.
Brain ; Child ; Dandy-Walker Syndrome ; Diagnosis ; Dilatation ; Diplopia ; Embryonic Development ; Female ; Fourth Ventricle ; Gait ; Head ; Headache ; Hospitalization ; Humans ; Hydrocephalus ; Male ; Nausea ; Neurosurgery ; Papilledema ; Pregnancy ; Tomography, X-Ray Computed ; Ventriculoperitoneal Shunt ; Vomiting

A patient with extraskeletal cervical epidural Ewing's sarcoma who presented with cervical radiculopathy is reported. A 58-year-old woman presented with right posterolateral neck pain and upper extremity radiculopathy. The computed tomography myelography showed epidural and paravertebral masses on right side, with widening of the ipsilateral neural foramina at the C2-C3 level. On magnetic resonance images, the mass was isointense to soft tissue on T1-weighted images, hyperintense on T2-weighted images, and showed diffuse enhancement on GdTA enhanced T1-weighted images. The staged, combined posterior and anterior approach were done and the tumor mass was subtotally removed. During postoperative adjuvant chemotherapy with Cytoxane, Adriamycin, Vincristine, Prednisolone regimen, the patient died of severe compression of upper cervical cord by recurrent, extended tumor mass in the spinal canal.
Chemotherapy, Adjuvant ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Female ; Humans ; Middle Aged ; Myelography ; Neck Pain ; Prednisolone ; Radiculopathy ; Sarcoma, Ewing* ; Spinal Canal ; Upper Extremity ; Vincristine

No abstract available.
Animals ; Burns* ; Lymphocyte Subsets* ; Lymphocytes* ; Mice* ; Spleen* ; Thymus Gland*