Microscopic polyangiitis is a systemic small-vessel vasculitis, which may involve multiple organs, but cardiac involvement is relatively rare. We report a case of microscopic polyangiitis with multiple organ involvement, in which myocardial infarction was the early manifestation of the disease. A 53-year-old man presented with sudden papulovesicular eruptions and swellings on the face, posterior neck, dorsa of both hands and fingers, and with diffuse erythematous patches on the back. He had suffered from renal dysfunction, arthralgia, and hypertension for more than 8 years. He had been admitted to the department of cardiology for acute myocardial infarction and had suffered from recurrent gastrointestinal bleeding, renal failure, acute pancreatitis and sepsis during the admission. Histopathologically, small-vessel leukocytoclastic vasculitis with out granuloma was seen. Direct immunofluorescence showed no immune deposite. A high serum level of P-ANCA was detected by ELISA.
Acute Kidney Injury ; Antibodies, Antineutrophil Cytoplasmic ; Arthralgia ; Cardiology ; Enzyme-Linked Immunosorbent Assay ; Fingers ; Fluorescent Antibody Technique, Direct ; Granuloma ; Hand ; Hemorrhage ; Humans ; Hypertension ; Microscopic Polyangiitis* ; Middle Aged ; Myocardial Infarction* ; Neck ; Pancreatitis ; Sepsis ; Vasculitis

Sjogren's syndrome has been considered to be an autoimmune disease affecting various organs including salivary and lacrimal glands. It occurs most commonly in middle-aged women, and less than 20 cases with primary Sjogren's syndrome have been reported in children. An 11-yim-old boy presented with recurrent annular erythema on the face that had been present for 2 years. A schirmers test showed a positive result. ANA was detected at a dilution of 1:640. Anti-Ro/SSA and anti-La/SSB antibodies were also detected. On histological examination, lymphocytes were infiltrated in the periappendegeal areas as well as the papillary and reticular dernis. The skin findimg is uncommon in children, but has become a characteristic feature of childhood Sjogrens syndrome with anti-Ro and/or anti-La antibodies.
Antibodies ; Autoimmune Diseases ; Child ; Erythema* ; Female ; Humans ; Lacrimal Apparatus ; Lymphocytes ; Male ; Sjogren's Syndrome* ; Skin

BACKGROUND: In eczematous skin it is expected that altered defense mechanisms are employed against an injury, which mechanisms include alterations in the stratum corneum barrier function and lipid contents. OBJECTIVE: To investigaten aspect of pathophysiology of hand eczema by comparing the water holding capacities and transepidermal water loss(TEWL) after injuries on the skin and by comparing the epidermal lipid contents between hand eczema and normal subjects. METHODS: The water holding capacities were checked by corneometer, and the TEWL were checked after epidermal barrie! destruction with acetone or adhesive tapes. The epidermal lipids were extracted with acetone, ther (1: 1 v/v) mixture solvent and analysed by HP- TLC. RESULTS: The water holdingapacities were decreased in eczema of the palm, but the recoveries of TEWL and the lipid content did not differ between hand eczema and normal subjects. CONCLUSION: The water holding capacities were decreased in the eczematous skin. But there were no significant differencies in the recovery of destructed epidermal barrier and the epidermal lipid contents.
Acetone ; Adhesives ; Defense Mechanisms ; Eczema* ; Hand* ; Skin ; Water

A 26-year-old man presented with a 7-month history of hyperkeratotic skin lesions on both elbows and heels. He also had marked subungal hyperkeratosis and splinter hemorrhage on the finger and toe nails. A biopsy specimen from the elbow revealed lichenoid re-actions with areas of epidermal atrophy with alternating acanthosis and foci of parakeratosis. We made a diagnosis of keratosis lichenoides chronica based on the characteristic clinicopathologic findings. We have treated the patient with etretinate, but only the skin lesions on the elbows and heels responded to treatment.
Acitretin ; Adult ; Atrophy ; Biopsy ; Diagnosis ; Elbow ; Etretinate ; Fingers ; Heel ; Hemorrhage ; Humans ; Keratosis* ; Nails, Malformed* ; Parakeratosis ; Skin ; Toes

No abstract available.
Female ; Mammaplasty*

Intramuscular lipomas are benign soft-tissue mesenchymal tumors which rarely occur in the region of the head. These tumors present as slow-growing, generally painless masses and are easily misdiagnosed initially as epidermal inclusion cysts. We describe a 44-year-old woman who presented with an intramuscular lipoma of the frontalis muscle.
Adult ; Female ; Head ; Humans ; Lipoma*

Bullous pemphigoid(BP) and herpes gestationis(HG) are subepidermal bullous diseases which show clinical and immunological similarities. Both diseases show immune deposits along the basement membrane zone and their autoantibodies bind a common antigenic site within the non-collagenous stretch of the 180 kDa BPAG2 ectodomain. Besides its association with pregnancy, HG has some characteristic features that distinguish it from BP. The serum of patients with HG often contains an IgG that avidly fixes complement, and showes IgG1 subclass predominance. We report here two cases of non-pregnant young women presenting clinical and histological features of bullous pemphigoid or herpes gestationis. The immunopathology, IgG subtyping and immunoblotting studies showed that the autoantibodies in the patients were the characteristic ones of herpes gestationis. The patients might be a subtype of BP that have characteristics of autoantibodies of HG patients.
Autoantibodies* ; Basement Membrane ; Complement System Proteins ; Female ; Humans ; Immunoblotting ; Immunoglobulin G ; Pemphigoid Gestationis* ; Pemphigoid, Bullous* ; Pregnancy

Infantile myofibromatosis is an uncommon, benign, probably hamai tomatous proliferation of myofibroblasts. This lesions are most often present before the age of 2 years, show a male predomenance and may be multiple or solitary. The solitary lesions arise in the dermis, subcutis, or deep soft tissue, most commonly in the head and neek region or trunk. The elinical coirse is benign and spontaneous regression is not infrequent. A female newborn presented with a turmr lesion on the upper portion of the right eyelid. Physical examination showed a firm, non-tender, fixed, erythematous, 3 x 3cm sized tumor. Histologic examinatign revealed a spindle cell tumor in the deimis with a whorled arrangemant and grouped fescicles. There was no evidence for potential visceval and bony involvement. Follow-up examination at 6 months of aige revealed a remarkable degree of spoitaneous involution.
Dermis ; Eyelids ; Female ; Follow-Up Studies ; Head ; Humans ; Infant, Newborn ; Male ; Myofibroblasts ; Myofibromatosis ; Physical Examination

Pachydermodactyly is a rare form of digital fibromatosis mostly affecting young men. It is characterized by asymptomatic, protracted, bulbous soft tissue swellings on proximal phalangeal and proximal interphalangeal joint areas of fingers. Histopathologic findings represent hyperkeratosis, acanthosis, and thickening of the dermis due to increased collagen bundles There is also mucin deposition in the dermis. Intralesional infection of steriods is helpful in relieving the skin lesions. We report a case of pacgydermodactyly manifested as soft tissue swellings unilaterally on the lateral aspects of fingers. The lesions showed a good response to triamcinolone intralesional infections.
Collagen ; Dermis ; Fibroma ; Fingers ; Humans ; Joints ; Male ; Mucins ; Skin ; Triamcinolone

Heterotopic brain tissue is defined as displaced neuroglial tissue that has no connection with the central nervous system. As there is no communication with underlying structures, serious anomalies rarely occur. A 6-year-old Korean male presented an erythematous, non tender nodule measuring 1.5X1.5X0.5cm on right parietal scalp since birth. The lesion was covered with thickened alopetic skin. There was no neurologic defect. The mass was not connected with intracranial structures on a magnetic resonance imaging.
Brain* ; Central Nervous System ; Child ; Humans ; Magnetic Resonance Imaging ; Male ; Parturition ; Rabeprazole ; Scalp* ; Skin