Juvenile hyaline fibromatosis (JHF) is a rare, progressive autosomal recessive disease that's characterized by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. We present here the case of a 2-yr-old boy with JHF along with a review of the relevant literature. This case demonstrates that JHF should be considered in the differential diagnosis when multiple subcutaneous nodules are observed in the face, head and neck.
Contracture ; Diagnosis, Differential ; Fibroma ; Gingiva ; Gingival Hypertrophy ; Head ; Hyalin ; Hyalinosis, Systemic ; Joints ; Neck ; Skin

Phenytoin is a commonly used antiepileptic drug, and its narrow therapeutic index causes various toxicities. Although acute toxicity commonly causes ataxia, few cases have been reported of permanent cerebellar atrophy. A 49-year-old female with epilepsy was prescribed oral phenytoin. After three years of medication, seizure was controlled, but she developed unsteady gait and dysarthria. On admission, the patient showed severe bilateral ataxia, gingival hypertrophy, nystagmus, and dysarthria. Phenytoin toxicity was the impression, and phenytoin was omitted. Serum phenytoin level was 46.9 mg/L (therapeutic range being 10~20 mg/L). Brain magnetic resonance imaging (MRI) was performed to rule out other brain lesions, and diffuse cerebellar atrophy was revealed. After rehabilitation, mild bilateral ataxia remained, standing balance was poor, and the patient was able to walk 70 meters with minimal assist using roller walker. We review a case of chronic phenytoin toxicity that manifested as cerebellar ataxia and later showed atrophy of cerebellum.
Ataxia ; Atrophy ; Brain ; Cerebellar Ataxia ; Cerebellum ; Dysarthria ; Epilepsy ; Female ; Gait Disorders, Neurologic ; Gingival Hypertrophy ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Phenytoin ; Seizures ; Walkers

Sebaceous hyperplasia occurs on the face in persons over 40 years of age. Cyclosporine in dermatologic diseases can be used for the treatment of psoriasis, pyoderma gangrenosum, lichen planus, graft-versus-host disease, epidermolysis bullosa acquisita, Behcet's disease, and paraneoplastic pemphigus. etc. The side effects of cyclosporine in mucocutaneous tissue, were hypertrichosis, gingival hypertrophy, acneiform eruption, keratosis pilaris, flushing, angioedema, urticaria, and anaphylaxis have also been reported with the use of cyclosporine. Development of sebaceous hyperplasia is related to the process of dysplastic epithelial proliferation. We experienced a case of sebaceous hyperplasia in a renal transplant patient receiving cyclosporine. After carbon dioxide laser was done, lesions were cleared without any scar.
Acneiform Eruptions ; Anaphylaxis ; Angioedema ; Cicatrix ; Cyclosporine* ; Epidermolysis Bullosa Acquisita ; Flushing ; Gingival Hypertrophy ; Graft vs Host Disease ; Humans ; Hyperplasia* ; Hypertrichosis ; Keratosis ; Lasers, Gas ; Lichen Planus ; Pemphigus ; Psoriasis ; Pyoderma Gangrenosum ; Urticaria

BACKGROUND: As the patients who undergo heart transplantation have achieved better survival in recent years, a growing number of recipients are at a risk for experiencing surgical complications in addition to rejection and infection. In this paper, we report on our experience with the surgical complications that occurred in heart transplant recipients. MATERIAL AND METHOD: From April 1994 to September 2003, 37 heart transplantations were performed at our center by a single surgeon. The indications for transplantation were dilated cardiomyopathy, ischemic cardiomyopathy, valvular cardiomyopathy and familial hypertrophic cardiomyopathy. RESULT: Twenty postoperative complications required surgeries in 15 patients (41%). The types of operations required were; redo-sternotomy for bleeding (5), pericardiostomy for effusion (4), implantation of a permanent pacemaker (1), right lower lobe lobectomy for aspergilloma (1), removal of urinary stone (1), cholecystectomy for gall bladder stone (1), drainage of a perianal abscess (1), paranasal sinus drainage (1), total hip replacement (1), partial gingivectomy due to gingival hypertrophy (1), urethrostomy (1), herniated intervertebral disc operation (1) and total hysterectomy for myoma uteri (1). The locations of the complications were mediastinal in 10 (27%) cases and extramediastinal in 10 (27%) cases. CONCLUSION: The relatively high incidence of extrathoracic complications associated with heart transplantation emphasizes the importance of a multidisciplinary approach to the improve long-term survival when managing those complex patients.
Abscess ; Arthroplasty, Replacement, Hip ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Cardiomyopathy, Hypertrophic, Familial ; Cholecystectomy ; Drainage ; Gingival Hypertrophy ; Gingivectomy ; Heart ; Heart Transplantation ; Hemorrhage ; Humans ; Hysterectomy ; Incidence ; Intervertebral Disc ; Myoma ; Pericardial Window Techniques ; Postoperative Complications ; Rejection (Psychology) ; Transplants ; Urinary Bladder Calculi ; Urinary Calculi ; Uterus

Prolymphocytic leukemia is a chronic lymphoproliferative disorder, characterized by prominent splenomegaly, prolymphocytes accounting for more than 55% of circulating lymphocytes, no significant peripheral lymphadenopathy and short term survival with terminal fatal multi-organ failure. We report a case of B-cell prolymphocytic leukemia in a 57-year-old woman who presented with easy bruising and arthritis for 1 year and low abdominal pain for 2 months. Physical examination revealed gingival hypertrophy and mild splenomegaly. On peripheral blood smears the leukocytes were markedly increased in number due to leukemic cells that count about 62% of leukocytes. The bone marrow aspiration smear and biopsy revealed diffuse infiltration of medium to large prolymphocytes having moderate amount of basophilic cytoplasm, round to oval nuclei with coarse chromatin, and prominent nucleoli. Abdominal pain aggravated despite chemotherapy, and pelvic computed tomography (CT) revealed a huge lobular pelvic mass which had increased in size on the follow-up CT. Total hysterectomy with bilateral adnexectomy was performed. Microscopic findings included massive infiltration of prolymphocytic cells in the uterus, upper vaginal wall, bilateral ovaries, and bilateral mesosalpinges. On immunohistochemistry, the leukemic cells showed B cell gamma light chain phenotype.
Abdominal Pain ; Arthritis ; B-Lymphocytes* ; Basophils ; Biopsy ; Bone Marrow ; Chromatin ; Cytoplasm ; Drug Therapy ; Female* ; Follow-Up Studies ; Gingival Hypertrophy ; Humans ; Hysterectomy ; Immunohistochemistry ; Leukemia, Prolymphocytic ; Leukemia, Prolymphocytic, B-Cell* ; Leukocytes ; Lymphatic Diseases ; Lymphocytes ; Lymphoproliferative Disorders ; Middle Aged ; Ovary ; Phenotype ; Physical Examination ; Splenomegaly ; Uterus