Dermatomyositis* ; Skin*

A 45-year-old woman presented with a six-month history of Hailey-Hailey disease, which was recalcitrant to many dermatologic therapies including systemic antibiotics, dapsone, and topical corticosteroids. We precribed tacrolimus (0.1%) ointment for application to the lesions twice a day and examined weekly for the efficacy; the lesions were much improved after 6 weeks of treatment.
Adrenal Cortex Hormones ; Anti-Bacterial Agents ; Dapsone ; Female ; Humans ; Middle Aged ; Pemphigus, Benign Familial* ; Tacrolimus*

Intravenous immunoglobulin therapy has been used to treat various immune-mediated diseases. Its utility in dermatology continues to expand, including treatment for autoimmune blistering disease, graft-versus-host disease, and dermatomyositis. It is generally considered safe, and serious adverse effects such as anaphylaxis, renal tubular necrosis or aseptic meningitis rarely occur. Furthermore, adverse skin reactions very rarely occur, but can include urticaria, pruritus, eczema, alopecia, lichenoid dermatitis or pompholyx. We describe a case of a 48-year-old man who developed vesicular eczema on his palms and soles after administration of intravenous immunoglobulin therapy for encephalomyelopathy.
Alopecia ; Anaphylaxis ; Blister ; Dermatitis ; Dermatology ; Dermatomyositis ; Eczema ; Eczema, Dyshidrotic* ; Graft vs Host Disease ; Humans ; Immunization, Passive* ; Immunoglobulins* ; Meningitis, Aseptic ; Middle Aged ; Necrosis ; Pruritus ; Skin ; Urticaria

BACKGROUND: The differential diagnosis of bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) presents some difficulties since both diseases have overlapping clinical and histological features, as well as immunopathological findings. Confocal laser scanning microscopy (CLSM) has been developed and has shown to be a promising tool for dermatological investigations, giving a higher degree of resolution and available co-localization analysis. OBJECTIVE: The purpose of this study was to evaluate whether the new technique of CLSM could reliably identify and differentiate the binding sites of disease specific-autoantibodies (Abs) at the basement membrane zone (BMZ), with the sera from BP and EBA. METHODS: An indirect immunofluorescence (IF) assay was performed to localize the binding sites of circulating Abs from 5 cases of both BP and EBA, as well as the sites of 3 BMZ markers (integrin beta4, laminin-5, and type IV collagen). To facilitate identification and topographic differentiation between the two groups, patients' Abs were labeled with fluorescein isothiocyanate, whereas the BMZ markers were labeled with Texas red. The tissue specimens were observed under both conventional IF microscopy and CLSM. RESULTS: Owing to superposition of antigens and marker labels, double immuno-labeled sections under IF microscopy revealed limitations for the differentiation of patient's sera from BMZ markers even with high magnification (x1,000). However, CLSM was able to eliminate much of the antigen overlap. In BP, the circulating autoantibody' deposits were recognized on the epidermal side of laminin-5 and type IV collagen, and codistributed with integrin beta4. On the other hand, the binding of autoantibodies in EBA was on the dermal side from that of integrin beta4, laminin-5 and type IV collagen. These spatial relationships are compatible with their known microstructural locations. CONCLUSION: Our study indicates that CLSM examination may provide more precise localization of the antigens in BP and EBA than conventional IF microscopy. CLSM would not only be an efficient tool to identify circulating anti-BMZ autoantibodies for diagnosis and differential diagnosis of blistering diseases, but also a great addition to examining tissue specimens in patients who do not have detectable circulating Abs.
Autoantibodies ; Basement Membrane ; Binding Sites ; Blister ; Collagen Type IV ; Diagnosis ; Diagnosis, Differential* ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescein ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Hand ; Humans ; Integrin beta4 ; Microscopy ; Microscopy, Confocal* ; Pemphigoid, Bullous* ; Texas

OBJECTIVES: The purpose of this study was to investigate the clinical utility of the Korean Wechsler Intelligence Scale for Children-Fourth Edition (K-WISC-IV) and General Ability Index (GAI) in children with attention-deficit hyperactivity disorder (ADHD). METHODS: The GAI and Full Scale IQ (FSIQ) in K-WISC-IV were compared for a group of 79 children with ADHD and 234 normal children. The mean differences within each group were testified by the t-test and Cohen's d. The Wechsler's descriptive classifications for each GAI and FSIQ score were analyzed in the children with ADHD. Additionally, the critical value for the difference between the means of the FSIQ and GAI was calculated in the normal children. RESULTS: The score on the GAI was higher than that on the FSIQ in the children with ADHD, with a mean difference of 2.19 points. The implications of these findings are discussed regarding the cognitive characteristics of the children with ADHD. According to Wechsler's descriptive classification, the application of the GAI showed a lower (7.59%), equal (61.65%) or higher (60.76%) prevalence of ADHD compared to the application of FSIQ. CONCLUSION: The implications of these findings are discussed regarding the cognitive characteristics of the children with ADHD. The GAI reflecting the attention and behavioral problems in children with ADHD could be utilized as an alternative global intelligence estimate that considers their potential for development.
Child ; Classification ; Humans ; Intelligence* ; Prevalence ; Problem Behavior

The prediction of functional outcome in patients with acute cerebral infarction depends on many factors. Various techniques have been applied to predict severity and outcome after cerebral infarction. Neuron-specific enolase (NSE) is a component of a specific brain enzyme and a useful marker of brain injury. We evaluated the relation between initial serum NSE level and short- and long-term clinical outcome in 59 patients with acute cerebral infarction and in 38 age-matched healthy controls. Serum NSE levels were determined in patients with carotid artery (CA) territory cerebral infarction within 24 hours of onset. Brain MRI was performed four to seven days after stroke. Patients were divided into two groups: large CA territory infarction with a lesion extending cortex (cortex group), and small subcortical CA territory infarction (subcortical group) with a lesion confined to the subcortical white matter. We compared the initial serum NSE levels of the two groups. National Institute of Health Stroke Scale (NIHSS) was determined at admission and seven days after onset and the modified Rankin's scale was used at the 3 months follow-up after onset. Serum NSE levels were significantly elevated in patients with acute cerebral infarction compared with the normal controls (13.88 +/- 5.47 ng/dl vs. 8.15 +/- 1.53 ng/dl, p < 0.05). The initial ( < 24 h) serum NSE level was higher in the cortical group than in the subcortical group (16.68 +/- 5.70 ng/dl vs. 10.98 +/- 3.34 ng/dl, p < 0.05). NIHSS on admission and on the 7th day correlated with the initial serum NSE level (p < 0.05), as were more severe functional outcomes, as determined 3 months after onset (p < 0.05). This study shows that initial serum NSE level may be a useful marker for severity in acute ischemic stroke, and that it may be well correlated with short-term and long-term functional outcomes.
Acute Disease ; Aged ; Carotid Artery Diseases/*physiopathology ; Cerebral Infarction/*physiopathology ; Female ; Human ; Male ; Middle Age ; Phosphopyruvate Hydratase/*blood ; Severity of Illness Index

Amyloidosis is a rare disease characterized by deposition and accumulation of insoluble fibrils in various organs and tissues. Variations in the precursor proteins composing each fibril account for the diverse character of amyloidosis. Amyloidosis light chain (AL) and amyloid A amyloidosis (AA) are two clinical entities representative of this diversity. We present a case of a 58-year-old woman presenting with refractory diarrhea and abdominal pain. She was ultimately diagnosed with intestinal amyloidosis diffusely involving the small and large intestines. Even though a definitive therapeutic strategy has not been established for gastrointestinal amyloidosis, this particular patient has been successfully managed with high-dose steroids and azathioprine.
Abdominal Pain ; Amyloid ; Amyloidosis ; Azathioprine ; Diarrhea ; Female ; Humans ; Intestines ; Light ; Middle Aged ; Proteins ; Rare Diseases ; Steroids

No abstract available.
Arteriovenous Malformations* ; Prostate-Specific Antigen ; Urinary Bladder Neck Obstruction* ; Urinary Bladder*

OBJECTIVE: The typical clinical feature of chronic cutaneous lupus erythematosus (CCLE) is 'discoid erythema'; however, several different clinical variants exist such as hypertrophic LE, LE profundus, chilblain LE, tumid LE and so on. The heterogeneity-spectrum of the lesional morphology of the cutaneous lesions and the predilection sites of the lesions of twenty Korean patients with CCLE were examined and tried to compare to those of CCLE patients of Caucasian origin. METHODS: Clinical examinations of twenty patients with CCLE were carried out by dermatologists to define the morphologic characteristics of cutaneous lesions and the anatomic predilection sites of the lesions. RESULTS: The most common cutaneous lesion-morphology noted in Korean patients with CCLE was classic discoid erythema. Three cases had concurrent cutaneous lesions of other clinical variants of CCLE. The predilection sites of all lesions were the face (cheeks) and head (frontal area). These clinical findings observed among this group of patients are similar to those features seen in people of Caucasian origin. CONCLUSION: The most frequent clinical feature of CCLE was discoid erythema and the predilections sites of the lesions found were the face and the scalp; indicating that the morphological heterogeneity-spectrum of cutaneous lesions and the predilection sites observed in Korean patients of CCLE seem to have no remarkable differences when compared with Caucasian cases with the same diagnoses.
Chilblains ; Diagnosis ; Erythema ; Head ; Humans ; Lupus Erythematosus, Cutaneous* ; Scalp

BACKGROUND: Apoptosis, or programmed cell death, may participate with pathogenesis of intercellular detachment and loss of cell-matrix interaction. Autoimmune bullous dermatoses is an entity charaterized by bullous lesions of the skin and mucosa, and autoantibodies to the specific tissue components. OBJECTIVE: The purpose of this study was to investigate the induction of apoptosis in the lesional skin of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), and epidermolysis bullosa acquisita (EBA). METHODS: Hoechst 33342 (bisbenzimide) staining and terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL) staining was performed to determine the induction of apoptosis in the lesional skin of each disease. RESULTS: In PV and PF, typical findings of apoptosis were observed in the lesional epidermis showing acantholysis. However, in BP and EBA, no apoptosis of the epidermis was observed. CONCLUSION: These results suggest that apoptosis is only associated with acantholysis of the epidermal keratinocytes, one of many components of pathogenesis in bullous disease, in patients with pemphigus.
Acantholysis ; Apoptosis ; Autoantibodies ; Blister ; Cell Death ; Epidermis ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Humans ; Keratinocytes ; Mucous Membrane ; Pemphigoid, Bullous* ; Pemphigus* ; Skin ; Skin Diseases, Vesiculobullous