Hailey-Hailey disease(benign familial chronic pemphigus) is a rare autosomal dominant disorder characterized by blisters at sites of friction such as the neck, axillae and groin which are caused by suprabasal epidermal acantholysis. We report two cases of Hailey-Hailey disease in the one family. One of the two cases has asymptomatic multiple longitudinal white bands in the fingernails associated with typical skin lesions. The nail lesions have not been described until reported by Burge in 1992 and it may be a characteristic finding in Hailey-Hailey disease.
Acantholysis ; Axilla ; Blister ; Friction ; Groin ; Humans ; Nails ; Neck ; Pemphigus, Benign Familial* ; Skin

We report two cases of recurrent extramarnmary Pagets disease after wide local excision. Both cases involved the scrotum and the penoserotal junction, respectively. On histopathological examination, we could observed many Paget cells confined to the epidermis and the hair follicle, but not invading the underlying dermis in both cases. There was no evidence of internal malignancy. Both cases were treated with wide local reexcision.
Dermis ; Epidermis ; Hair Follicle ; Paget Disease, Extramammary* ; Scrotum

Kaposis sarcoma is a multicentric neoplastic vascular tumor involving the skin or internal organs. We report a case of classic Kaposis sarcoma in a 69-year-old male who had positive serum cytomegalovirus antibodies and disseminated multiple erythematous to purplish colored confluent papules and edema on both lower legs. The histopathological finding showed abnormally proliferated and dilated vessels, vascular spaces, spindle cells, and extravasated erythrocytes. He was treated with radiotherapy, but died due to ventricular tachycardia.
Aged ; Antibodies ; Cytomegalovirus ; Edema ; Erythrocytes ; Humans ; Leg* ; Male ; Radiotherapy ; Sarcoma, Kaposi* ; Skin ; Tachycardia, Ventricular

The monoclonal antibody Ki-1(CD30) was first described in 1982 and was reported to react selectively with Reed-Sternberg cells in Hodgkins disease, highly activated B and T cells and large cell lymphomas of both T-and B-cell origin. Lymphomatoid papulosis(LyP) is characterized by recurrent erythematous papules or nodules that undergo spontaneous healing with hyperpigmentation and scarring. Histologically, two major types of LyP can be distinguished: type A is characterized by the presence of variable numbers of Ki-1+ large, atypical lymphocytes with some Reed-Sternberg like cells and type B is characterized by Ki-1- atypical cerebriform mononuclear cells similar to those in mycosis fungoides. We report a case of Ki-1+ lymphomatoid papulosis which was histopathologically recognized as a type B lesion but represented a Ki-1 positive reaction.
B-Lymphocytes ; Cicatrix ; Hodgkin Disease ; Hyperpigmentation ; Lymphocytes ; Lymphoma ; Lymphomatoid Papulosis* ; Mycosis Fungoides ; Reed-Sternberg Cells ; T-Lymphocytes

No abstract available.
Eosinophilia*

BACKGROUND: Although therapeutic plasmapheresis (TP) is a useful procedure in removing pathogenic antibodies and toxic substances from the patient, adverse reactions could arise from the use of replacement fluids and anticoagulants. Comprehensive analysis on those adverse effects had been rarely reported in Korea. METHODS: We retrospectively investigated the clinical records and the TP records from 3,962 TP sessions for 581 patients between January 1995 and October 2008 at Asan Medical Center, and we analyzed the adverse reactions related to TP. RESULTS: Adverse reactions were seen in 142 patients (24.4%) in 348 TP procedures (8.8%). Citrate toxicity was most frequently seen in 83 procedures (23.9%) followed by chills in 72 procedures (20.7%), allergic reactions in 69 procedures (19.8%) and hypotension in 60 procedures (17.2%). Citrate toxicity, chills and allergic reactions were seen more frequently in the TP procedures using FFP than in the TP procedures using albumin (P=0.001). The prevalence of citrate toxicity was significantly lower in the cases where calcium gluconate was administered (P<0.001), while it was significantly higher in the patients whose hematocrit was below 28.5% (P<0.001). In terms of severity, the mild, moderate and severe adverse reactions were 36.8%, 56.3% and 6.9%, respectively. CONCLUSION: TP is a relatively safe method of treatment, but it is important to predict and prevent adverse reactions and to respond appropriately to these adverse reactions.
Antibodies ; Anticoagulants ; Calcium Gluconate ; Chills ; Citric Acid ; Gluconates ; Hematocrit ; Humans ; Hypersensitivity ; Hypotension ; Plasmapheresis ; Prevalence ; Retrospective Studies

Lipoid pneumonia is chronic, interstitial, proliferative inflammation resulting from aspiration of lipoid material. Mineral oil is a hydrocarbon that physicians often use to treat chronic constipation in children and adults. Mineral oil may not elicit a normal protective cough reflex and may impair mucociliary transport. We experienced a case of exogenous lipoid pneumonia caused by aspiration of mineral oil given as a laxatives confirmed by fiberoptic bronchoscopy with bronchoalveolar lavage and bronchial biopsy in a 9-month-old boy with chronic cough and radiologic evidence of parenchymal lung disease.We reported this case with a brief review of related literatures.
Adult ; Biopsy ; Bronchoalveolar Lavage ; Bronchoscopy ; Child ; Constipation ; Cough ; Humans ; Infant ; Inflammation ; Laxatives* ; Lung ; Male ; Mineral Oil ; Mucociliary Clearance ; Pneumonia* ; Reflex

Background: The highly lipid soluble opioid, fentanyl, has a rapid onset and short duration of action. The present study was designed to examine the analgesic efficacy and side effects of the combination of fentanyl with morphine in patients using intravenous PCA. Methods: Patients were randomly assigned to receive one of three PCA regimens: M4 group (40 mg morphine+90 mg ketorolac+1.5 mg dorperidol), M2F2 group (20 mg morphine+200 ug fentanyl+90 mg ketorolac+1.5 mg dorperidol), or M2F4 group (20 mg morphine+400 ug fentanyl+90 mg ketorolac+1.5 mg dorperidol). All patients were given initial loading dose of 0.1 mg/kg morphine plus 1 mg droperidol at the end of surgery. Pain score, side effects, and overall satisfaction were assessed at 30 min, 1 hr, 8 hr, 24 hr, and 48 hr postoperatively. Results: The pain score was significantly higher in the M2F2 group than in the M4 group and M2F4 group during 1 hr and 8 hr postoperatively. The total opioid consumption was significantly greater in the M2F4 group than in the M4 group. Patient satisfaction was better in the M2F4 than other two groups. There were no differences in the overall incidence of side effects among three groups. Conclusions: The present results suggest that the combination of fentanyl with morphine for intravenous patient-controlled analgesia is a useful method, and the double dose of fentanyl in comparison with the equipotent morphine dose is recommended in the early postoperative period.
Analgesia, Patient-Controlled* ; Droperidol ; Fentanyl* ; Humans ; Incidence ; Morphine* ; Passive Cutaneous Anaphylaxis ; Patient Satisfaction ; Postoperative Period

Background: We hypothesized that intravenous lidocaine mixed with propofol may have an influence on anesthesia induction and hemodynamic responses to propofol induction and endotracheal intubation as well as propofol-induced pain on injection. Methods: Seventy-five patients were allocated to group L1 (2% lidocaine 1.5 mg/kg, n=25), group L2 (2% lidocaine 2 mg/kg, n=25) or group C (normal saline 0.05 mL/kg, n=25) according to the lidocaine dosage mixed with propofol 2 mg/kg. The pain on injection was scored as none, mild, moderate, and severe. The site of pain and recall of pain were also recorded. Loss of verbal response was observed during induction. Mean arterial blood pressure (MAP) and heart rate (HR) were recorded before anesthetic induction (baseline value), immediately before and after endotracheal intubation, and every min until 5 min thereafter. Results: Ninety-two percent of patients reported pain upon injection in group C, whereas 8% of the patients in group L1 and no patient in group L2. Loss of verbal response before injection of total dose of propofol was observed in 44% in group L2, 36% in group L1 and 28% in group C. Lowered MAP caused by propofol increased significantly after endotracheal intubation in all three groups (p<0.05). HR increased immediately and 1 min after endotracheal intubation in all three groups (p<0.05). Conclusions: Our results indicate that intravenous lidocaine 1.5 mg/kg or 2 mg/kg mixed with propofol 2 mg/kg significantly reduces the incidence and the degree of pain, but does not affect anesthesia induction and hemodynamic responses to propofol and tracheal intubation.
Anesthesia ; Arterial Pressure ; Heart Rate ; Hemodynamics* ; Humans ; Incidence ; Intubation* ; Intubation, Intratracheal ; Lidocaine* ; Propofol*

We report a case of Darier's disease with typical cutaneous and nail lesions in a 50-year-old male patient. He has brownish hyperkeratotic papules on the ant.chest, back, both axilla and groins and verrucous surfaced hypertrophic plaques on the scalp, forehead and both postauricular areas, which were accompanied by a severe itching sensation and malodor. Also he has finger nail lesions such as subungual hyperkeratotic papules, distal wedge shaped notching and multiple longitudinal red lines on the nail plates. Histopathologic features taken from the inguinal area revealed hyperkeratosis, suprabasal clefts and lacuna, protruded villi lined with one layer of basal cells into the cavity and dyskeratotic cells such as corps ronds in the epidermis. Hyperkeratotic papules on the ant.chest, back, both axilla and groins disappeared after systemic treatment withe a low dose of oral etretinate but hypertrophic plaques on the scalp and both postauricular areas still remained clinically and histopathologically. We then the treated postauricular hypertrophic plaques with CO2 laser vaporization and severe malodor with oral isotretinoin and it resulted in a marked improvement.
Acitretin* ; Axilla ; Darier Disease* ; Epidermis ; Etretinate* ; Fingers ; Forehead ; Groin ; Humans ; Isotretinoin* ; Lasers, Gas* ; Male ; Middle Aged ; Pruritus ; Scalp ; Sensation ; Volatilization*