A case of Maffucis syndrome is presented with a brief review of literature because of its rarity. An attempt was made for reconstructive surgery for the deformities involving right hand with rewarding result.
Congenital Abnormalities ; Hand ; Reward

During the past 11 years period from April, 1961 to March, 1972 171 cases of electrical burn patients were treated at Orthopedic Department of Han-Il Hospital. The author reviewed and discussed our clinical experience of electrical thermal injuries. Clinical analysis of these 171 cases of electrical burn patients was carried out and summarized as follows; 1) The majority of the patients ranged two to three decades of life(68.4%); The youngest 1 year old and oldest 53 years of age. Male were involved significantly more than female, the incidence being 163 cases (95.3%) and 8 cases. 2) Seasonal distribution showed more than about two third with 114 cases for rainy summer times and Autumn from May to October. 3) Among 171 cases of electrical burn patients, 131 patients (54.2%) were under 5% of body surface and 15 patients (11.3%) were over 20% of body surface. 4) In the regional distribution of burn wound, the upper extremity were 157(92.3%), 73(42.9%) in lower extremity, and 49 (28.9%) in other region. 5) Occupational distribution; The electrical line man and electrician were seen 136 cases (80%) which is two third of 171 patients, labor 12 cases(7%), 10 cases (5.9%) in painters, 8 cases (4.6%) in company employee and industrialist, 5 cases (2.9%) in house-wife, and 4 cases (2.4%) in motor man. 6) The most frequently early complication at electric burn of 171 cases were gastrointestinal symptom 111 cases (65%), fracture 18 cases (10.5%), head injuries (intracranial bleeding and basal bone fracture of skull) 15 cases (6.4%), spinal cord injury and kidney rupture 1 cases (0.6%), hemorrhage 2 cases (1.2%), and other 8 cases (4%). 7) The predominating organisms cultured out of burn wounds of 26 cases were staphylococcus aureus 9 (34.7%), pseudomonas auerginosa 5 (19.2%), E. coli 3 (11.5%), staphylococcus albus 1(4%), β-hemolytic streptococcus 1 (4%), and no growth were 7 (26.9%). 8) The type of current and the voltage in 102 cases among the 171 cases of electric burn patients was clear. Injury of the 99 cases out of the 102 cases were by indirect current and the 3 was by direct current. In 99 cases by indirect current, electric burn in accordance with high tension current in voltage was 81 cases (79.4%), low tension current injuries 18 cases (17.6%). 9) At the admission, laboratory findings of the electric burn patients showed hemoconcentration and leukocytosis. 10) Early excision of the necrotic tissue and skin grafting was performed in 94 times. The results was excellent in 71 times. 11) Among 171 cases of electric burn patients, later complication were seen 47 cases (27.4%), which is 25 cases (14.3%) in amputation, 8 cases in cicatrical contraction, 4 cases in serum hepatitis, 4 cases in paralysis, 2 cases in osteomyelitis, 2 cases in ankylosis of knee joint, 2 cases in drop foot, 1 case in anal stricture, and 1 case in cataract. 12) The overall motality in this series was 8% (14cases) and the most frerent cause of the death was due to head injury (9 cases) at the time of electrical burn injuries.
Amputation ; Ankylosis ; Burns ; Burns, Electric ; Cataract ; Constriction, Pathologic ; Craniocerebral Trauma ; Female ; Foot ; Fractures, Bone ; Hemorrhage ; Hepatitis ; Humans ; Incidence ; Kidney ; Knee Joint ; Leukocytosis ; Lower Extremity ; Male ; Orthopedics ; Osteomyelitis ; Paralysis ; Pseudomonas ; Rupture ; Seasons ; Skin Transplantation ; Spinal Cord Injuries ; Staphylococcus ; Staphylococcus aureus ; Streptococcus ; Upper Extremity ; Wounds and Injuries

PURPOSE: To evaluate the usefulness of positron emission tomography/computed tomography (PET/CT) for field modification during radiotherapy in esophageal cancer. MATERIALS AND METHODS: We conducted a retrospective study on 33 patients that underwent chemoradiotherapy (CRT). Pathologic findings were squamous cell carcinoma in 32 patients and adenocarcinoma in 1 patient. All patients underwent PET/CT scans before and during CRT (after receiving 40 Gy and before a 20 Gy boost dose). Response evaluation was determined by PET/CT using metabolic tumor volume (MTV), total glycolytic activity (TGA), MTV ratio (rMTV) and TGA ratio (rTGA), or determined by CT. rMTV and rTGA were reduction ratio of MTV and TGA between before and during CRT, respectively. RESULTS: Significant decreases in MTV (MTV2.5: mean 70.09%, p < 0.001) and TGA (TGA2.5: mean 79.08%, p<0.001) were found between before and during CRT. Median rMTV2.5 was 0.299 (range, 0 to 0.98) and median rTGA2.5 was 0.209 (range, 0 to 0.92). During CRT, PET/CT detected newly developed distant metastasis in 1 patient, and this resulted in a treatment strategy change. At a median 4 months (range, 0 to 12 months) after completion of CRT, 8 patients (24.2%) achieved clinically complete response, 11 (33.3%) partial response, 5 (15.2%) stable disease, and 9 (27.3%) disease progression. SUVmax (p = 0.029), rMTV50% (p = 0.016), rMTV75% (p = 0.023) on intra-treatment PET were found to correlate with complete clinical response. CONCLUSION: PET/CT during CRT can provide additional information useful for radiotherapy planning and offer the potential for tumor response evaluation during CRT. rMTV50% during CRT was found to be a useful predictor of clinical response.
Adenocarcinoma ; Carcinoma, Squamous Cell ; Chemoradiotherapy* ; Disease Progression ; Electrons ; Esophageal Neoplasms* ; Humans ; Neoplasm Metastasis ; Positron-Emission Tomography and Computed Tomography* ; Radiotherapy ; Retrospective Studies ; Tumor Burden

A 20-year-old female, who had been treated with topical PUVA therapy for her vitiligo, developed characteristic skin lesions of disseminated superficial artiric porokeratosis(DSAP). All of her three sisters had vitiligo and had been treated with topica PUVA, hut only one developed DSAP lesion. Her mother had had DSAP lesions on expcsec areas for 20 years. DSAP shows an autosomal dominant mode of inheritance, and is charaterized by multiple ciark brownish keratotic papaules surrounded by slightly elevatied order. The exacerbation of the lesion by exposure t.o UV lignt indicates that the regulatingene for DSAP is related to sunlight. 1-lerein we reported a case of DSAP induced by topica] PUVA therapy in one patient during management of familial vitiligo in 3 sisters.
Female ; Humans ; Mothers ; Porokeratosis* ; PUVA Therapy ; Siblings ; Skin ; Sunlight ; Vitiligo* ; Wills ; Young Adult

The epithelioid angiosarcoma is recently described ns a variant of ang-osarcoma based on its pathological feature, which is characterized by the epithelioid or histiocytoid morphology af the malignant tumor cells. The existence of vascular endothelial tumor with an epithelioid feature has been accepted for many years, most notablyn the forms of epithelioid hemangioma and epithelioid hemangioendo thelioma. In recent years, cutaneous angiosarcoma with epitheloid morphology have been reported as a cutaneos epithelioid angiosarcoma in the literatures. A 60-year-old man presente with a mild tender erythematous 2 x 2 x 2.5cm sized soft fluctuating single nodule with dark hemorrhagic crust on the left forehead for 3 months. He had had an abrasion wound on the lesion site of the forehead by an accidental trauma 5 month before the lesion appeared. Histopathological findings revealed that the tumor mass was chiefly composed of solid sheets of poorly differentiated malignant cells with an epithelioid feature. The characteristics of classical angiosarcoma such as primitive vascular spaces and clefts with malignat cells, and proliferating vessels were also found in some areas. The immunohistochemical stain wi.h the factor VIII related antigen ivas focally reactive in the tumor cells. Unfortunately he refused further treatment and expired 7 months after discharge at home without knowing the direct cause of his health. We report herein an interesting and rare case of the cutaneous epitheioid angiosarcoma which had typical clinical and histopathological findings and suggested as a case with a very aggressive course.
Forehead ; Hemangioma ; Hemangiosarcoma* ; Humans ; Middle Aged ; von Willebrand Factor ; Wounds and Injuries

No abstract available.
Child* ; Humans ; Purpura*

We report a case of MALT lymphoma in a 49-year-old woman. Her disease occurred simultaneously in the mucosa of her right upper eyelid conjunctiva and in her left nostril as ulcerating tumors associated with itchy ichthyosiform skin lesions on the trunk, hyperkeratotic palms and soles, and dystrophic nails. Histopathological examinations revealed consistent findings of MALT lymphoma with dissemination; i.e., diffuse infiltrates of lymphoplasmacytoid cells with a few Russel body-like structures, eosinophils, some shoddy granulomas under the irregularly hyperplastic epidermis, and diffuse infiltrates of CCL ( centrocyte-like ) cells and small lymphocytes inside and outside many lymphoid follicle-like structures in the subcutaneous tissue forming florid lymphoepithelial lesions. She died after 27 months duration of her disease with worsening of ichthyosiform skin lesions and dystrophic nails in spite of total excision of the tumors. We discuss the clinical and histopathologic features of MALT lymphoma with dissemination and the various similar diseases to differentiate.
Conjunctiva ; Eosinophils ; Epidermis ; Eyelids* ; Female ; Glycogen Storage Disease Type VI ; Granuloma ; Humans ; Lymphocytes ; Lymphoma, B-Cell, Marginal Zone* ; Middle Aged ; Mucous Membrane ; Skin ; Subcutaneous Tissue ; Ulcer

BACKGROUND: Metallothioneins(MTs) are ubiquitous low molecular weight cytosolic proteins with high contents of cystein that xhibit a selective binding affinity for zinc, copper and other group II heavy metal ions. In additi~on to the sequestration function and detoxification action, MTs probably participate in the cellirlar protection against. ionizing radiation and alkylating agent cytotoxicity and in cellular pn:liferation. OBJECT: The objective of this study was to evaIuate the expression of MTs and to clarify t.heir role in carcinogenesis and/or biological behaviour in the neoplasms of ectoderrnal origin. METHODS: We performed immunohistochemical studies on the surgical specimens of rnalignant rnelanoma and squamous cell carcinoma and basal cell carcinoma. The results were compared with the normal skin and mu osa and hair t,issues and raf't-on culture tissues of skin and mucosal keratinocytes. RESULTS: 1) MTs were strongly expressed in epidermal basal cells of the normal skin and raft-on cultured artificial skin They were also rnore weakly expressed in epithelial basal cells of the normal mucosa and raft on cultured artificial mucosa and in matrix cells of the normal hair. 2) In rnalignant melanoma, MTs were meaningfully expressed, i.e., a strong expression was seen in the lesions showing a rapid vertical growth phase with fatal out,comes, but a weak expression in the cases whose precursor lesions were thought as dysplastic nevi, compared to the negative finding of norrnal melanocyt,es. The strong expression in a case of congenit.al rnelanocyt.ic nevus is considered as the activation of nevoid melanocytes. 3) MTs were weakly expressed in squarnous cell carcinoma with a diffuse aattern within the tumor mass. St,rong expression of MTs was particularly apparent in the poorl. differentiated squamous cell carcinoma and at the growing border of well differentiated squamous cell carcinoma. 4) In basal cell carcinoma, MTs were rather weakly expressed in solid cireumscribed types with a diffuse pattern and in cystic type with a patchy pattern. However they were not expressed in the solid infiltrative type. CONCLUSION: From these resuilts, we concluded that MTs were ubiquitously present in possible stem cells of various epithelial tissues except melanocytes irrespective of normal or artificial skin and/or mucosa including norrnal hair tissues. In the neoplasms of ectodermal origin, MTs might be involved in the invasive growth of tumor cells in the malignant melanoma and squamous cell carcinoma. It was implied that the role of MTs in basal cell carcinoma might be related to early events of hasal cell carcinogeresis and its down regulating tendency could be possible in its infil trative stage.
Carcinogenesis ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Copper ; Cytosol ; Dysplastic Nevus Syndrome ; Ectoderm* ; Hair ; Ions ; Keratinocytes ; Melanocytes ; Melanoma ; Metallothionein* ; Molecular Weight ; Mucous Membrane ; Nevus ; Radiation, Ionizing ; Skin ; Skin, Artificial ; Stem Cells ; Zinc

A 50-year-old female, who had been suffered from diabetes rielitus, showed typical cutaneous findinhs of dermatomyositis with only transient and mild proximal muscle weakness at sometime during the course of disease. She had Gottrons papules, Gottrons sign, heliotrope rash, macular violsceous erythema involving neck, anterior chest, back, extensor site of forearms and arms and dorsa of hands. However, he serum CPK, LDH and sGOP levels were within normal limits. Her fasting and 2 hours potprandial blood glucose levels were elevated. There were no histological abnormality in muscle biopsy. Histopathologic findigs of the skin lesion on her back were that of poikiloderma atrophicans vasculare. We diagnosed our patient as Rebeccas type 2 amyopathic dematomyositis which is chracterized by skin disease with subjective myalgias and musle weakness, but without laboratory evidence of muscle disease.
Arm ; Biopsy ; Blood Glucose ; Dermatomyositis* ; Erythema ; Exanthema ; Fasting ; Female ; Forearm ; Hand ; Humans ; Middle Aged ; Muscle Weakness ; Myalgia ; Neck ; Skin ; Skin Diseases ; Thorax

No abstract available.
Diagnostic Tests, Routine* ; Hantaan virus*