No abstract available.
Darier Disease*

Cutaneous pseudolymphoma is a benign reactive lymphoproliferative process that clinically and histologically simulates cutaneous lymphomas. Cutaneous pseudolymphoma usually develops as solitary or multiple erythematous papules or nodules. The face, scalp, and neck are the most common sites for pseudolymphomas. Several cases of pseudolymphomas on the lip have been reported previously in the literature but are rare. A 52-year-old woman presented with an asymptomatic, solitary, indurated, erythematous swelling of the mucosa of the upper lip, which had been present for several years. A punch biopsy was performed, and histologically, the lesion was diagnosed as cutaneous pseudolymphoma. Despite treatment with triamcinolone acetonide intralesional injection and systemic steroids, the lesion waxed and waned for 1 year. After stopping treatments for several months, the lesion spontaneously resolved. Herein, we report a rare case of pseudolymphoma on the lip without causative history.
Biopsy ; Female ; Humans ; Injections, Intralesional ; Lip* ; Lymphoma ; Middle Aged ; Mucous Membrane ; Neck ; Pseudolymphoma* ; Scalp ; Steroids ; Triamcinolone Acetonide

Motor neuron diseases (MNDs) refer to a heterogeneous group of progressive neurologic disorders caused by degeneration of motor neurons. The diseases affect either the upper motor neurons, lower motor neurons, or both, and are characterized by weakness, atrophy, fasciculation, spasticity, and respiratory failure. We report a case of a 61-year-old male patient with no past history of cardiovascular or pulmonary disease, who presented with only dyspnea, and no indication of any other symptom such as muscle weakness, atrophy, or bulbar dysfunction. Neuromuscular conduction study, including a study of the phrenic nerve, confirmed the diagnosis of MND. The patient greatly improved giving respiratory assistance at night, using a noninvasive ventilator. This case indicates that MNDs should be considered as differential diagnoses for patients showing acute respiratory failure of unknown causes. This report will aid in the prompt diagnosis and treatment of MNDs.
Atrophy ; Diagnosis ; Diagnosis, Differential ; Dyspnea ; Fasciculation ; Humans ; Lung Diseases ; Male ; Middle Aged ; Motor Neuron Disease* ; Motor Neurons* ; Muscle Spasticity ; Muscle Weakness ; Nervous System Diseases ; Phrenic Nerve ; Respiration, Artificial ; Respiratory Insufficiency* ; Ventilators, Mechanical

BACKGROUND: There have been a few reported cases of congenital great toenail dystrophy (GTND), described as a congenital malalignment of the great toenails. However, acquired GTDN is rare, and has not been documented extensively. This study aimed to describe the clinical features of 21 patients with acquired GTND. METHODS: Twenty-one patients with acquired GTND who visited Yeouido St. Mary's Hospital between June 2005 and August 2012 were retrospectively reviewed. RESULTS: The mean patient age was 43.1 years (range, 17 to 88 years), and the cohort predominantly comprised women (18/21). In our experience, all acquired GTND patients presented with yellow or yellow-brownish chromonychia, onychotrophy, and onycholysis. Conservative treatment with tape methods and grinding, as well as nail extraction, was provided and yielded little improvement in any case. CONCLUSION: This study provides initial data on the nail changes affecting the great toenail, such as yellowish chromonychia, onychomadesis, and onycholysis. These data may help physicians to distinguish various nail disorders, including onychomycosis, congenital malalignment of the great toenails, and yellow nail syndrome.
Cohort Studies ; Female ; Humans ; Nails* ; Onycholysis ; Onychomycosis ; Retrospective Studies ; Yellow Nail Syndrome

A nevus with cyst is defined as a single lesion of a melanocytic nevus, and this is commonly associated with an epidermal cyst. There have been rare reported cases of adnexal origin. The combination of nevus and cyst could possibly be misdiagnosed clinically as a malignant melanoma at initial examination. A 51-year-old woman presented with a painful, solitary, dome-shaped, erythemaous nodule on the forehead which had been present for several years. The histopathologic specimen of the nodule showed a solitary cyst with a melaocytic nevus. There were nevus cells without junctional activities in the upper dermis and melanin pigments in the upper dermal nevus nest. It contained lamellated keratin materials and vellus hair shafts. We report here a rare case of melanocytic nevus combined with an vellus hair cyst in a 51-year-old woman.
Bone Cysts ; Dermis ; Epidermal Cyst ; Erythema ; Female ; Forehead ; Hair* ; Humans ; Melanins ; Melanoma ; Middle Aged ; Nevus ; Nevus, Pigmented*

Cerebral venous thrombosis (CVT) is quite rare and underlying risk factors are diverse. We describe that 60-year-old man developed CVT after use of cyclosporine. He had suffered from leukocytoclastic vasculitis and urticarial vasculitis. Initial MR venogram revealed thrombosis in superior sagittal sinus, right transverse sinus and sigmoid sinus with multiple cortical and deep collateral veins. Anticoagulation was started and maintained for one year, then follow up MR venogram was normal. We suggest that cyclosporine might increase a risk of CVT.
Colon, Sigmoid ; Cyclosporine ; Follow-Up Studies ; Risk Factors ; Superior Sagittal Sinus ; Thrombosis ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous ; Veins ; Venous Thrombosis

BACKGROUND: Rosacea is characterized by erythema of the central face that persists for several months or longer. Reports of the histological changes in rosacea are scarce, and few attempts have been made to correlate such changes with clinical findings and pathophysiology. OBJECTIVE: This study aimed to elucidate the clinical manifestations of rosacea and investigate its histological features. METHODS: We performed a retrospective analysis of 278 patients with histologically confirmed rosacea who visited the Department of Dermatology at the Catholic Medical Center between January 2008 and May 2013. Clinical subtypes, disease severity, and precipitating factors were evaluated. In 115 randomly selected patients, histopathological features were evaluated as well. RESULTS: The ratio of males to females was 1:1.8. The age distribution showed a peak incidence in the fifth decade. The most common subtype was papulopustular rosacea (52.9%) followed by erythematotelangiectatic rosacea (34.9%), ocular rosacea (4.0%), and phymatous rosacea (2.9%). Granulomatous rosacea accounted for 5.4% of rosacea cases. Precipitating factors included hot weather (54.7%), stress (51.8%), sun exposure (37.4%), alcohol (37.4%), and hot baths (33.1%). Histological analysis of skin biopsies from 115 patients revealed solar elastosis in 62 patients (53.9%) and telangiectasia in 85 patients (73.9%). CONCLUSION: In this study, Korean rosacea patients were predominantly female with a peak age in the fifth decade and the majority suffered from the papulopustular and erythematotelangiectatic types of rosacea. Histological observations pertaining to each rosacea type were also discussed.
Age Distribution ; Baths ; Biopsy ; Dermatology ; Erythema ; Female ; Humans ; Incidence ; Korea ; Male ; Precipitating Factors ; Retrospective Studies ; Rosacea* ; Skin ; Solar System ; Telangiectasis ; Weather

Hypersensitivity to mosquito bites is defined as the appearance of intense skin reactive lesions and systemic symptoms subsequent to mosquito bites. Most cases of hypersensitivity to mosquito bites reported thus far have been associated with chronic Epstein-Barr virus infection or natural killer cell leukemia/lymphoma. In this study, we describe the case of an 18-year-old Korean boy who had hypersensitivity to mosquito bites associated with primary systemic anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. After a mosquito bite, the patient developed a progressive cutaneous nodule on his left lower leg and regional lymphadenopathy in the left inguinal area. The histopathological and immunohistochemical findings suggested anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. Positron emission tomography-computed tomography revealed increased fluorodeoxyglucose uptake in the left T4 vertebrae, left external iliac lymph nodes, left inguinal lymph nodes, and lateral subcutaneous region of the left lower leg. According to the clinical, histopathological, and immunohistochemical findings, as well as the imaging data, the patient was diagnosed with primary systemic anaplastic lymphoma kinase-positive anaplastic large cell lymphoma. Consequently, the patient received a total of 6 cycles of cyclophosphamide + doxorubicin + vincristine + prednisolone chemotherapy at 3-week intervals, after which the lesions regressed.
Adolescent ; Culicidae* ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Electrons ; Herpesvirus 4, Human ; Humans ; Hypersensitivity* ; Killer Cells, Natural ; Leg ; Lymph Nodes ; Lymphatic Diseases ; Lymphoma* ; Lymphoma, Large-Cell, Anaplastic* ; Male ; Prednisolone ; Skin ; Spine ; Vincristine

Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is one of the mitochondrial disorders that can present as a stroke-like episode or seizure. Although the pathophysiology of MELAS remains inconclusive, the main possibilities are thus far thought to be mitochondrial cytopathy and angiopathy. This case report describes a 61-year-old woman diagnosed with MELAS who presented simultaneously with vascular hyperemia and crossed cerebellar diaschisis.
Acidosis, Lactic ; Female ; Humans ; Hyperemia ; Kearns-Sayre Syndrome ; Mitochondrial Diseases ; Mitochondrial Encephalomyopathies ; Mitochondrial Myopathies ; Seizures