We describe five patiens with autoimmune bullous der matosis who presented cutaneous lesions localized on the face. In these patients iuumunopathologically confirmed diagnoses were penphigus erythematosus, bullous pemphigoid, cicatricial pernphigoid(Brunsting-Perry variant), epidemic lysis bullosa acquisita and bullous systemic lupus erythematosus in each. Except, for the case of pemphigus erythematosus, facelimited cutnious lesions can be seen rarely with these imrnunobullous diseases. We suggest that some external, in malfactors such as UV light and photosensitivity might be regarded as the precipitating factors fo the clinical rnanifestations of those skin lesions, and which should be included in the differential diingosis of vesiculobullous diseases involving the face.
Diagnosis ; Humans ; Lupus Erythematosus, Systemic ; Pemphigoid, Bullous ; Pemphigus ; Precipitating Factors ; Skin ; Ultraviolet Rays

Backgraund : Most of the inflammatory bullous lesions in bullous pamphigoid(BP) and epidermolysis bullosa acquisite(EBA) demonstrate similar clinical and histological features. However, the specificities of the autoantibodiea reactive to the dermo-epidermal junction antigeins are different. In these two bullouk dermat loses, there are no remarkable differences in symptomaiology, there are no unique predilection sites for the lesions and they do not usually leave scars after nvolution. Considering these similarities, dermatologists could encounter some clinical confuiion in maling a provisional diagnosis. OBJECTIVE: Authors examined three patients of BP with classic inflammatory bullous eruptions and threa patients of EBA with inflammatory bullous lesions to see if here were any differences in the morphology of the immuno-fluorescence(IF) pattern. METHODS: For direct IF of vertical or semi-vertical sections, perilasional skin was obtained in each patient for indirect IF of horizontal or semi-horizonta1 sections, Ig(bound substrates were prepared from all these autoantibody-positive individuals. All specimens veri; observed at 200/400-magnification fields through an ordinary fluorescence microscopy. RESULTS: Patterns of fluorescence observed by direct IF were characterized as thin-linear in BP and thick/coarse-linear in EBA along the basement membrane zone. On the horizontal section preparations in indirect IF, the patterns of fluorescence were clean-linear in E3P hnd more fluffy-linear in EBA at the dermoepidermal junction, These patterns could be recognized aim/larly in the fields of both 200 and 400-magnification, however each minor differences were not easy to distinguish each other. CONCLUSION: The above findings of subtle differences in the pattern of IF may provide some suggestions to the examiners for the need of differential diagnosis in theae riseases.
Basement Membrane ; Blister ; Cicatrix ; Diagnosis ; Diagnosis, Differential ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescence* ; Humans ; Male ; Microscopy, Fluorescence ; Pemphigoid, Bullous* ; Skin

Backgraund : Most of the inflammatory bullous lesions in bullous pamphigoid(BP) and epidermolysis bullosa acquisite(EBA) demonstrate similar clinical and histological features. However, the specificities of the autoantibodiea reactive to the dermo-epidermal junction antigeins are different. In these two bullouk dermat loses, there are no remarkable differences in symptomaiology, there are no unique predilection sites for the lesions and they do not usually leave scars after nvolution. Considering these similarities, dermatologists could encounter some clinical confuiion in maling a provisional diagnosis. OBJECTIVE: Authors examined three patients of BP with classic inflammatory bullous eruptions and threa patients of EBA with inflammatory bullous lesions to see if here were any differences in the morphology of the immuno-fluorescence(IF) pattern. METHODS: For direct IF of vertical or semi-vertical sections, perilasional skin was obtained in each patient for indirect IF of horizontal or semi-horizonta1 sections, Ig(bound substrates were prepared from all these autoantibody-positive individuals. All specimens veri; observed at 200/400-magnification fields through an ordinary fluorescence microscopy. RESULTS: Patterns of fluorescence observed by direct IF were characterized as thin-linear in BP and thick/coarse-linear in EBA along the basement membrane zone. On the horizontal section preparations in indirect IF, the patterns of fluorescence were clean-linear in E3P hnd more fluffy-linear in EBA at the dermoepidermal junction, These patterns could be recognized aim/larly in the fields of both 200 and 400-magnification, however each minor differences were not easy to distinguish each other. CONCLUSION: The above findings of subtle differences in the pattern of IF may provide some suggestions to the examiners for the need of differential diagnosis in theae riseases.
Basement Membrane ; Blister ; Cicatrix ; Diagnosis ; Diagnosis, Differential ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescence* ; Humans ; Male ; Microscopy, Fluorescence ; Pemphigoid, Bullous* ; Skin

BACKGROUND: In autoimmune bullous dermatoses, such as pemphigus and bullous pemphigoid, variations in the expression of the antigen in different body locations are recognized. OBJECTIVE: The degree of expession of epidermolysis bullosa acquisita (EBA) antigen in different sites on the body surface was estimated from the highest dilution factor of EBA sera that gave a positive reaction at a site by indirect immunofluorescence (IF). METHODS: Two sera, obtained from EBA patients with inflammatory and mechanobullous skin lesions, having antihody titers of 160 against the dermal component of the NaCl split skin, were used by indirect IF techniques with 20 specimens (2 from each of 10 locations) of normal human skin from different sites. These 20 skin samples were obtained from 10 healthy adults (1-3 from each individual). RESULTS: The greatest expression of the antigen was in the skin taken from the upper back with the titer of 160. EBA antigen was least recognized in skin specimens from the inner thigh and calf. Skin from the scalp, abdomen, and anterior chest and others demonstrated intermediate degrees of expression. CONCLUSION: There was some moderate degree of variation in the expression of EBA antigens in skin samples obtained from different locations on the body. It seems however that there is not any positive correlation between the degree of expression of EBA antigen in each location and predilection sites (possibly the trunk) of clinical lesions in EBA.
Abdomen ; Adult ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Fluorescent Antibody Technique, Indirect ; Humans ; Pemphigoid, Bullous ; Pemphigus ; Scalp ; Skin ; Skin Diseases, Vesiculobullous ; Thigh ; Thorax

BACKGROUND: Polyarteritis nodosa(PAN) is a disease of necrotzing vasculitis which has a clinical spectrum encompassing those cases of multisystem involveme it and skin-limited variant. The clinical course of cutaneous PAN has been considered to be a benign one, however there is sorne controversy regarding its nosological entity. OBJECTIVE: To characten the clinical course of the patients with cutaneous PAN and determine whether or not it is a benign disease or something more sever. METHODS: Clinical and laboratory findings in 8 patients with Logy-proven cutaneous PAN but without any detectable visceral involvement were observed periochelly during a follow-up period over 18 months. Meticulous physical and pertinent laboratory earinations were performed each time during their visits. RESULTS: Livedo reticulari, on the lower legs was the mostorenon cutaneous findings with these patients. During the follow-up periods there were no suspected abnormal physical and laboratory findings to have other organ involvement in all 8 patients Serologic examinations for antineutrophil cytoplasmic antibody and hepatitis-B surface antigen revealed negative in all patients tested. CONCLUSION: The follow-up periods of 2 years or so does not seem to be long enough in characterizing the clinical features of PAN. However, the cutaneous: on of PAN can be regarded as a benign cutaneous variant of the disease having no visceral lesion of arteritis, even though mild degrees of constitutional symptoms and a few laboratory abnormlites could be seen.
Antibodies, Antineutrophil Cytoplasmic ; Antigens, Surface ; Arteritis ; Follow-Up Studies ; Humans ; Leg ; Polyarteritis Nodosa* ; Vasculitis

Edwards syndrome is first introduced by Edwards and characterized by facial anomalies, multiple cardiovascular, gastrointestinal, urogenital, and skeletal malformations. It results from triplication of part or all of chromosome 18 in some or all of the patient's cells. It has an incidence of 1 in 4,500 live births or less and short life expectancy. Recently we experienced a case of 3-day-old female new born infant with this syndrome. Post mortem examination showed progeric face with prominent occiput, large flabby ears, microphthalmia, and micrognathia, bilateral clenched hands with flexion contraction of middle fingers, and bilateral rockerbottom feet. Internal examination revealed horseshoe kidney, esophageal atresia with tracheoesophageal fistula, two accessory spleens, and multiple cardiac anomalies. A trisomy 18 was confirmed by the cytogenetic study.
Infant ; Male ; Female ; Infant, Newborn ; Humans ; Incidence

A 56-year-old woman presenting a 10-year history of atrophic discoid patches with underlying subcutaneous nodules was seen. These skin lesions were distributed on the upper arms bilaterally. Biopsy specimens taken from the lesional skin showed epidermal and dermal changes consistent with the discoid lesions of the cutaneous lupus erythematosus. Deep dermis and subcutaneous fat tissue revealed sclerosis and fibrinoid alterations of the collagen and necrosis of fat cells, the features corresponding to the lupus ei ythematosus profundus. She had no laboratory evidences suspective of systemic lupus erythematosus or other connective tissue diseases. This patient with lupus erythernatosus profundus which cleveloped subsequent to the discoid lesion is consiclered to be a rarely encounteririg observation in lupus erythematosus.
Adipocytes ; Arm ; Biopsy ; Collagen ; Connective Tissue Diseases* ; Connective Tissue* ; Dermis ; Female ; Humans ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Systemic ; Middle Aged ; Necrosis ; Panniculitis, Lupus Erythematosus* ; Sclerosis ; Skin ; Subcutaneous Fat

No abstract available.
Femoral Neck Fractures* ; Femur Neck*

Rhabdomyosarcoma which was first described by Weber has generally been considered asan uncommon tumor. Pack and Eberhart were of the opinion that rhabdomyosarcoma represented 13.9 percent of soft tissue sarcomata. Recently it was classified as four types such as pleomorphic, embryonal, botryoid and alveolar type by many authors. We have experienced two cases of embryonal rhabdomyosarcoma, one arised within left leg and the other within right foot. The former(3 months old, female) showed tumor mass at birth, the latter(13 months old, male) showed at 1 month after birth.
Chungcheongnam-do ; Foot ; Leg ; Parturition ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal

No abstract available.
Lung Neoplasms* ; Lung*