Bullous pemphigoid is an autoimmune, subepidermal blistering disease commonly seen in the elderly. It is known as autoimmune bullous dermatoses with an estimated prevalence of 1 in 40,000 and shows no gender or racial predilection. It has an autoimmune nature with immunoglobulin (Ig) G and/or C3 complement targeting hemidesmosomal antigens located in the epidermal basement membrane zone. Drug-induced pemphigoid, although rare, can occur in patients taking penicillamine, furosemide, captopril, penicillin, or sulfasalazine, but infliximab-induced pemphigoid has not been reported. We experienced a case of infliximab-induced pemphigoid in 54-year-old woman with a 17-year history of rheumatoid arthritis.
Aged ; Antibodies, Monoclonal ; Arthritis, Rheumatoid ; Basement Membrane ; Blister ; Captopril ; Complement C3 ; Female ; Furosemide ; Humans ; Infliximab ; Immunoglobulins ; Middle Aged ; Pemphigoid, Bullous ; Penicillamine ; Penicillins ; Prevalence ; Skin Diseases, Vesiculobullous ; Sulfasalazine

Neuromyelitis optica (NMO) is an idiopathic, severe inflammatory demyelinating disease of the central nervous system targeting optic nerves and the spinal cord. It is characterized by acute bilateral visual loss (optic neuritis), acute transverse myelitis, and tends to spare brain early in the disease course. NMO can occur as an isolated condition or secondary to infection, toxin exposure, and autoimmune disease including systemic lupus erythematosus (SLE), sarcoidosis, and Behcet's disease. We experienced a case of SLE with myelitis and recurrent optic neuritis in a 28-year-old woman who presented with recurrent visual disturbance and sudden onset of paraplegia, and report here on this case along with a review of the relevant literature.
Autoimmune Diseases ; Brain ; Central Nervous System ; Demyelinating Diseases ; Female ; Humans ; Lupus Erythematosus, Systemic ; Myelitis ; Myelitis, Transverse ; Neuromyelitis Optica ; Optic Nerve ; Optic Neuritis ; Paraplegia ; Sarcoidosis ; Spinal Cord

Gitelman syndrome is a condition caused by a mutation of the thiazide sensitive Na-Cl cotransporter gene on the distal convoluted tubule. It results in a variety of clinical features, including hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. It is often diagnosed in asymptomatic adults presented with unexplained hypokalemia; however, it is sometimes associated with muscular cramps, numbness, fatigue, weakness, or paralysis. We experienced a case of rheumatoid arthritis accompanied by Gitelman syndrome, presented with hand tremor. We diagnosed her using renal clearance study and genetic analysis. Here, we report our experiences regarding this case along with a literature review.
Adult ; Alkalosis ; Arthritis, Rheumatoid* ; Fatigue ; Furosemide ; Genetic Testing ; Gitelman Syndrome* ; Hand ; Humans ; Hypesthesia ; Hypokalemia ; Muscle Cramp ; Paralysis ; Solute Carrier Family 12, Member 3 ; Thiazides ; Tremor

BACKGROUND: Aberrations of cardiovascular regulation have been reported in patients who suffer with fibromyalgia (FM). Abnormalities of the cardiovascular autonomic regulation, as well as the correlation between coronary heart disease and depression, have been considered to be the causative factors. The clinical features of transient left apical ballooning syndrome with the patients under acute stress have been clearly described, but the effect of chronic stress such as FM on the myocardium is unknown. We investigated the cardiac strain in FM patients by strain imaging with using the 2D grayscale images, and we quantified the regional myocardial deformation properties. METHODS: We investigated 30 consecutive postmenopausal women (mean age: 48+/-8 years) who satisfied the criteria for fibromyalgia with atypical chest pain by performing standard and 2-dimensional strain echocardiography (2DS). Those patients with hypertension, coronary heart disease or diabetes were excluded. The global and segmental longitudinal deformation parameters of the LV from 3 apical views were analyzed, and the patients underwent a manual tender point survey for determining the number of tender points and tender point counts, and the patients completed the fibromyalgia impact questionnaire (FIQ), the brief fatigue inventory (BFI), and Beck depression inventory (BDI). RESULTS: The global longitudinal LV strain was significantly reduced in the FM patients with a high FIQ score (>40) as compared to the patients with a low FIQ score (-18.61% vs. -22.72%). Also, both the global and segmental longitudinal LV strains were negatively associated with fatigue or the tender point counts. However, there was no significant association between depression and the LV strain. CONCLUSION: This study showed the reduced myocardial longitudinal deformation in FM patients. This suggested that strain imaging is a feasible approach to assess the regional ventricular function in FM patients.
Chest Pain ; Coronary Disease ; Depression ; Echocardiography ; Fatigue ; Female ; Fibromyalgia ; Humans ; Hypertension ; Myocardium ; Surveys and Questionnaires ; Sprains and Strains ; Takotsubo Cardiomyopathy ; Ventricular Function

Serotonin syndrome, an adverse drug reaction, is a consequence of excess serotonergic agonism of central nervous system receptors and peripheral serotonergic receptors. Serotonin syndrome has been associated with large numbers of drugs and drug combinations, and serotonin-norepinephrine reuptake inhibitor-induced serotonin syndrome is rare. It is often described as a sign of excess serotonin ranging from tremor in mild cases to delirium, neuromuscular rigidity, and hyperthermia in life-threatening cases. Diagnosis is based on the symptoms and patient's history, and several diagnostic criteria have been developed. We experienced a rare case of fibromyalgia accompanied by tremor, hyperreflexia, spontaneous clonus, muscle rigidity, and diaphoresis after 10 days of single use of duloxetine 30 mg. Only one case of serotonin syndrome resulting from administration of duloxetine has been reported in Korea, however that case resulted from co-administration of fluoxetine. We report here on this case along with a review of the relevant literature.
Central Nervous System ; Delirium ; Diagnosis ; Drug Combinations ; Drug-Related Side Effects and Adverse Reactions ; Duloxetine Hydrochloride* ; Felodipine ; Fever ; Fibromyalgia* ; Fluoxetine ; Humans ; Korea ; Muscle Rigidity ; Reflex, Abnormal ; Serotonin Syndrome* ; Serotonin* ; Tremor

Diffuse idiopathic skeletal hyperostosis (DISH) is a condition characterized by the calcification and ossification of soft tissue, and can lead to severe cervical kyphosis, presenting with a spectrum of physical complaints including dysphagia, hoarseness, stridor, aspiration pneumonia, and dyspnea due to airway compromise. Restrictive ventilatory impairment is very rare. We encountered a 73-year-old man with DISH presenting with progressive dysphagia and dyspnea over a few months. The symptoms were evaluated with a video fluoroscopy swallowing study and pulmonary function tests (PFT). The PFT revealed restrictive ventilatory impairment. A neck magnetic resonance imaging (MRI) study showed anterior cervical osteophytes causing upper airway compromise and compression of the esophagus. Osteophytes were removed surgically and the patient improved clinically. Here, we describe the case with a literature review.
Aged ; Deglutition ; Deglutition Disorders* ; Dyspnea ; Esophagus ; Fluoroscopy ; Hoarseness ; Humans ; Hyperostosis, Diffuse Idiopathic Skeletal* ; Kyphosis ; Magnetic Resonance Imaging ; Neck ; Osteophyte* ; Pneumonia, Aspiration ; Respiratory Function Tests ; Respiratory Sounds

Neurogenic bladder in systemic lupus erythematosus (SLE) has been considered to occur rarely. Myelopathy, cyclophosphamide treatment, vasculitis have been implicated as the causes of the bladder pathology in patients with SLE. In this report, we describe a 46-year- old female with SLE who simultaneously combined with the neurogenic bladder. The diagnosis of neurogenic bladder, attributed to peripheral neuropathy, was made on the basis of cystometrography and clinical symptom. Steroid therapy couldn't improve the clinical manifestation of neurogenic bladder despite the amelioration of the other lupus symptom.
Cyclophosphamide ; Diagnosis ; Female ; Humans ; Lupus Erythematosus, Systemic* ; Pathology ; Peripheral Nervous System Diseases ; Spinal Cord Diseases ; Urinary Bladder ; Urinary Bladder, Neurogenic* ; Vasculitis

OBJECTIVE: Fibromylagia (FM) is a rheumatic disorder characterized by widespread bodily pain, tenderness, and fatigue. It has a major impact on the quality of life including the quality of marital life. The objectives of this study were to assess the quality of marital life in patients with FM in Korea and to identify the correlation of the quality of marital life with disease severity. MEHTODS: Subjects were the patient group, composed of 50 married patients with FM, and a comparison group composed of 50 healthy married adults with similar demographic characteristics. The quality of marital life was measured using the Korean-Marital Satisfaction Inventory (K-MSI). The K-MSI consists of two valid scales, one global affective scale, and eleven additional scales measuring specific dimensions of relationship distress. The clinical parameters were assessed by Korean Fibromyalgia Impact Questionnaire (KFIQ) and tender point, respectively from 50 patients with FM. RESULTS: Of the 50 subjects, 48 (96%) were women with mean (+/-SD) age of 52.6(+/-8.8) years. The mean (+/-SD) duration of disease was 25.7 (+/-19.8) months. The mean (+/-SD) scores of KFIQ and tender point count were 52.9 (+/-14.9) and 13.1 (+/-1.6), respectively. The T-score of most scales of K-MSI of FM patients were higher than that of healthy controls (p<0.01). The scores of KFIQ were positively correlated with the eight scales of K-MSI and negatively correlated with one scale (Role orientation) of K-MSI. The tender point count was positively correlated with only one of scale (Conflict with in-law) of K-MSI. CONCLUSION: The results suggest that the quality of marital life in Korean patients of FM is lower than healthy subjects. In a simple correlation analysis, KFIQ was a meaningful variable correlated with the quality of marital life.
Adult ; Fatigue ; Female ; Fibromyalgia* ; Humans ; Korea ; Quality of Life ; Surveys and Questionnaires ; Weights and Measures

Focal dystonia is a neurological condition affecting a muscle or group of muscles in a specific part of the body, leading to involuntary muscular contractions. This condition is often treated with medications including muscle relaxants and injections of botulinum toxin. However, some cases do not respond to normal modes of treatment. Deep brain stimulation (DBS) can be a therapeutic option for patients who are resistant to medical treatment. We report a case of fibromyalgia accompanied by focal hand dystonia, where unilateral DBS improved the patient's focal dystonic movement. We also present a review of the relevant literature.
Botulinum Toxins ; Deep Brain Stimulation* ; Dystonia* ; Dystonic Disorders ; Fibromyalgia* ; Hand ; Humans ; Muscle Contraction ; Muscles

Thrombotic thrombocytopenic purpura (TTP) is a blood coagulation disorder that damages numerous organs, including the kidney, heart and brain. Features indicative of TTP include thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, kidney failure and fever. Infections, toxins, pregnancy and, rarely, autoimmune diseases are all known to be associated with TTP. We encountered a rare case of rheumatoid arthritis accompanied by TTP following tympanoplasty. The patient's confusion, thrombocytopenia and renal failure all improved after plasmapheresis and high-dose glucocorticoid therapy, but she eventually expired due to sepsis. We report on this case herein and also review the relevant literature.
Anemia, Hemolytic ; Arthritis, Rheumatoid* ; Autoimmune Diseases ; Blood Coagulation Disorders ; Brain ; Fever ; Heart ; Kidney ; Neurologic Manifestations ; Plasmapheresis ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic* ; Renal Insufficiency ; Sepsis ; Thrombocytopenia ; Tympanoplasty