Therapy of Chlorpromazine Melanosis: A Preliminary Report

Melanosis observed in association with prolonged chlorpromazine therapy has become a serious problem in mental institutions. Skin pigmentation has produced an appearance which is cosmetically undesirable. Ocular deposits have caused visual impairment. Diffuse visceral involvement has been accompanied by functional disturbances of the involved organs.Withholding chlorpromazine did not diminish the pigment deposits already present in eight patients with chlorpromazine-induced melanosis. Therefore therapy for existing cases and means of preventing this side effect were investigated. A method of blocking melanin synthesis by depressing tyrosinase activity was devised. A copperchelating agent, D-penicillamine, was administered for a period of four weeks (300 mg. three times daily for six days each week, with mineral supplement substituted on the seventh day). Four of six days patients thus treated improved markedly as evidenced by diminution of skin pigmentation. Urinary copper excretion was substantially increased during the trial period. An alternative method of treatment designed to stimulate melatonin production by the pineal gland was employed. Two patients were kept in darkness for a period of four weeks. One improved markedly, the other only slightly.     

Lipodermoid: A case report

Lipodermoids are epibulbar, developmental growths of normal adipose in an abnormal site, ie. near the lacrimal gland and extending between the superior rectus and lateral rectus muscles posteriorly. Differentiation of benign lipodermoids from non-benign lesions is essential. When other ocular anomalies or systemic conditions are found in conjunction with lipodermoids then Goldenhar-Gorlin syndrome must be considered. This paper presents a case of unilateral lipodermoid and scoliosis in an otherwise healthy adult male.     

骨髓中检出组织胞浆菌1例

组织胞浆菌（Histoplasma capsulatum）是一种深部真菌,可引起人体深部组织胞浆菌病。最近我们从1例患者骨髓涂片瑞氏染色、PAS染色、骨髓病理活检中检出组织胞浆菌,现予报道。     

Merkel cell carcinoma: case report

Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine carcinoma of the skin. Although it is 40 times less common than malignant melanoma, its mortality is much higher compared to melanoma. From 1986 to 2001 there was rapidly increasing incidence in reported cases of MCC, with a tripling in the rate over this 15-year period. The vast majority of MCC presents on sun-exposed skin. The head and neck area is the most common site of tumor occurrence. We present 70-year old female patient with painless red-colored nodule, size 2 x 2 x 2 cm on the dorsal side of mid left forearm. The surgical excision with negative margins was performed, and pathohistological analysis confirmed Merkel cell carcinoma. Sentinel lymph node biopsy was negative. In conclusion, as MCC is a very aggressive rare skin carcinoma with lethal outcome, it should be mandatory to perform biopsies of any suspected skin lesion.     

Pseudodiastrophic dwarfism: a case report

On the occasion of one new possible observation of the pseudodiastrophic dworfism observed in a femal newborn, first child of young, non-consanguineous parents, authors discuss the differential diagnosis as well as the problem of genetic counselling in the present case since the child's father is a technician in radiology and exposed to X-rays during 7 years.     

Robinow Syndrome: a case report

We report a case with Robinow syndrome which has been rarely reported in the literature. A male newborn who had fetal face appearance (broad and prominent forehead, hypertelorism, small saddle nose, anteverted nostrils, glabellar nevus flammeus, malar hypoplasia, down-turned mouth and retrognathia), mesomelic limb shortening, hemivertebra and genital hypoplasia was diagnosed as Robinow syndrome. Elevated levels of both basal and stimulated testosterone and dihydrotestosterone were found along with normal baseline levels of gonadotropins. These endocrinologic studies were suggestive for an androgen insensitivity. Mental and motor development of the infant were normal at 3 and 6 months of age. Because of the high level of consanguineous marriages in Turkey, we may expect a higher incidence of the autosomal recessive form of the syndrome. This gives a high recurrence risk and makes prenatal diagnosis an important option for future pregnancies in the families.     

Multicystic nephroma: a case report

BACKGROUND: Cystic nephroma is an uncommon pediatric renal neoplasm. It needs to be differentiated from cystic partially differentiated nephroblastoma and from other renal neoplasms showing extensive cystic change. It is scantily reported in the cytology literature. CASE: A 7-month-old female with a left-sided abdominal lump was diagnosed as having cystic Wilms' tumor on computed tomography. Fine needle aspiration cytology showed cellular smears composed of monomorphic, round to oval cells, suggestive of a small round cell tumor, possibly rhabdomyosarcoma. However, histopathologic examination showed it to be a multicystic nephroma. On review of the cytologic smears, the blastemal component was absent. CONCLUSION: This case highlights 1 extreme and unexpected cytologic appearance of cystic nephroma; it may result in misdiagnosis.     

Fetus-in-fetu: report of a case

A 5 month-old female was brought to our clinic because of diarrhea and abdominal distension. A plain radiograph demonstrated a mass with a vertebral column in the right upper quadrant of the abdomen. At operation a mass was found to be retroperitoneal, well encapsulated, and connected to the abdominal aorta of the host by two small vessels; no other connections and adhesions were seen between the mass and the host. The ovaries, uterus, and other pelvic and abdominal viscera of the host were normal. The mass was diagnosed as a fetus-in-fetu. The fetus-in-fetu, encapsulated with an amniotic capsule, was covered with skin and had a top with long hair, two protuberances, an amniotic hernial sac, upper limbs with syndactylic fingers, a gluteal region, and lower limbs with polysyndactylic toes. A brain mass and a spinal cord were identified in the cranial cavity and the vertebral canal. Several spinal ganglia and a nerve plexus were found. A noselike structure, upper lip, maxillalike bone with teeth, tonguelike structure, intestines, ribs, bones of the extremities, and skeletal muscles were also identified. A cloacalike cyst was observed to have an opening in the external female genitalia. Microscopically, a small number of motor neurons were seen in the brain mass and the anterior horn of the spinal cord. In the spinal ganglia, ganglion cells were differentiated. The submucosal and myenteric plexuses were seen in the intestinal wall. Well-differentiated muscle fibers were often accompanied with myelinated nerve fibers. Hematopoiesis was observed in the cranial bone marrow. The presence of the sex chromatin was confirmed in the nuclei of motor neurons and polymorphonuclear leukocytes. Thus, the present fetus-in-fetu, which was connected to the abdominal aorta of the host by two vessels, was a monozygotic twin which developed within its own amniotic cavity.     

须癣毛癣菌肉芽肿1例

须癣毛癣菌肉芽肿,是由须癣毛癣菌侵入皮肤真皮导致的一种浅部真菌的深在感染。也称为Majocchi肉芽肿,毛囊周围肉芽肿,临床上较少见。作者发现1例,现报告如下。     

Multifocal fibromatosis: a case report

We present of rare case of multifocal fibromatosis in a 52 year-old women. In 1996, she was first evaluated for a tumour of the right breast and on the basis of the surgical specimen the extra-abdominal fibromatosis was diagnosed. Four years later, she was reevaluated for the tumor of the right lung, and then in 2001 for the lesion of the right parietal pleura. Microscopic examination of pulmonary and pleural lesions revealed histological pattern almost identical with the breast tumor. The recurrent lesions were located proximally to the primary one.     

Cerebrotendinous xanthomatosis: a case report

BACKGROUND: Cerebrotendinous xanthomatosis is a rare, autosomal recessive, inherited lipid storage disease characterized by accumulation of cholestanol and cholesterol in most tissues. The disease is caused by mutations in the sterol 27-hydroxylase gene, leading to a block in bile synthesis, with accumulation of substrates for this enzyme, including cholesterol, resulting in an increase in the conversion of cholesterol to cholestanol. CASE: A 26-year-old woman presented with gradually increasing bilateral ankle swelling. She had a history of bilateral cataracts and left-sided hemiparesis. She had mental retardation, with a history of delayed milestone development. Her serum cholesterol levels were elevated. Aspiration of both ankle swellings revealed histiocytes and many foreign body giant cells. There were numerous rectangular to rhomboid crystals in the background. CONCLUSION: Very few articles are available on the cytologic features of tendinous xanthomas; hence we tried to highlight these features.     

Pharyngeal actinomycosis: a case report

Actinomycetes are saprophytic bacteria of the oral cavity. They can produce a rare, chronic, and suppurative process that usually originates from the teeth and mandible and then involve the cervical region, especially in the sub-mandibular area. A case of actinomycosis occurring in the retropharyngeal space in a 74 year-old man is reported. It arose as a swelling behind the third lower left molar that had no lesion. The patient was treated successfully with antibiotic therapy. The clinical presentation and management of the case are discussed and the relevant literature is reviewed.