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At least 1,135 records · Page 63Linked to original sources
PubMed1979

[Traumatically induced junctional activity of nevocellular nevi (author's transl)].

Forty papillomatous nevi, mainly with junctional nests, were partially removed surgically in such a way that a small margin was left intact on one side and the deep nevus cells were retained on the flor of the wound. After healing of the wound, a small layer of scar tissue separated the regenerated epidermis from the deep nevus cells, which were left in the dermis. After very differing intervals, as early as 30 days after the partial surgical removal, typical alterations could be observed in the epidermis above the cicatrical tissue. Most frequently, there was a hyperpigmentation with an increase in the number of basal fluorescing dendritic cells, which could also be found along the uppermost parts of epidermal appendages. Moreover, nest-like conglomerations of pigment-producing cells appeared in the form of junctional nevus cell nests. About 2-3 month after the partial surgical removal "Abtropfung" was visible, indicating a regeneration of the nevus. Fluorescence-histochemical and enzyme-histochemical results revealed that marked alterations can be found not only in the newly formed epidermis above the scar, which morphologically show minor alterations.

Adult↗
PubMed1987

A clinico-pathological study of actinomycotic mycetomas caused by Actinomadura madurae and Actinomadura pelletierii.

Twenty seven cases of actionomycotic mycetoma caused either by Actinomadura madurae or Actinomadura pelletierii have been described. Infection by A. madurae has been more common than A. pelletierii. Left foot in A. madurae and right foot in A. pelletierii infections were involved more commonly in adult males, whereas right foot of the females was frequently affected in A. madurae infection. Large, soft, white grains in A. madurae and small, firm, red grains in A. pelletierii were consistently seen. Deep hematoxylin stained grains with scalloped margin and prominent eosinophilic club in A. madurae and such deep stained grains with smooth margin and horizontal cracks appearing as portions of a spherical mass in A. pelletierri were diagnostic. Large numbers of plasma cells and Russel bodies were also characteristic of A. madurae infection. Both the grains were stainable with Von Kossa method for calcium. Bone changes were similar in both the infections. Oral tetracycline produced soft tissue and bone resolution to almost normalcy in those who regularly consumed the drug any time from 2 to 6 years. Mild glucose intolerance, facial hyperpigmentation and urticaria were the side effects observed in a few. Two patients developed cataract following tetracycline therapy. The value of medical therapy with oral tetracycline in Actionomadura mycetomas is emphasized.

Actinomycetales↗
PubMed1985

De Barsy syndrome--an autosomal recessive, progeroid syndrome.

We report two families with seven siblings with de Barsy syndrome. Characteristic features include severe mental retardation, hypermobility with athetoid movements, grimacing, muscular hypotonia, laxity of small joints and brisk deep tendon reflexes, progeroid aspect with cutis laxa, atrophy of skin with hyperpigmentation, isolated depigmentations, reduction of subcutaneous fatty tissue, translucent vein pattern, short stature, frontal bossing in the young child, large prominent ears with dysplastic helices and corneal clouding or cataracts. The syndrome probably has autosomal recessive inheritance.

Abnormalities, Multiple↗
PubMed1982

Primary adrenocortical nodular dysplasia with Cushing's syndrome and cardiac myxomas. A peculiar familial disease.

In a family with 4 children, 2 had slowly developing Cushing's syndrome and were adrenalectomized between the ages of 18 and 28 years. As in other cases with familial Cushing's syndrome, primary adrenocortical nodular dysplasia, the so-called adrenocortical adenomatosis, was demonstrated. The brother, now 39 years old, is in good health. The sister, however, died at the age of 36 years. Autopsy revealed a cardiac myxoma of the left atrium. A cardiac myxoma had been found incidentally at autopsy of these two patients' oldest brother, a boy who died at the age of 4 years. Both siblings with Cushing's syndrome presented additional Peutz-Jegher-like hyperpigmentation and myxomatous tumours of the skin. Identical observations of familial adrenocortical nodular dysplasia, Cushing's syndrome and cardiac myxomas have not been reported to date. However, the familial occurrence of cardiac myxomas and adrenocortical dysplasia in combination with bilateral large cell calcifying Sertoli cell tumours of the testis has been published recently.

Adrenal Cortex Diseases↗
PubMed1982

Redistribution of melanosomal complexes within keratinocytes following UV-A irradiation: a possible mechanism for cutaneous darkening in man.

In contrast to other ultraviolet (UV) wavelengths, UV-A can induce long-term or "true" pigmentation rapidly with little or no latency. The response cannot be clearly separated from immediate pigment darkening and is too rapid in onset to be explained by neomelanogenesis. In order to investigate possible mechanisms for this phenomenon, UV-irradiated skin was examined microscopically and ultrastructurally 18 h postirradiation. Specimens from skin sites tanned by exposure to melanogenic doses of UV-A showed a paradoxical reduction in the degree of basal melanization by light microscopy compared to unirradiated skin. Ultrastructurally, there was migration and dispersion of packaged melanosomes within keratinocytes from their normal, aggregated location around the nucleus towards the periphery of the cell. These changes were not observed in specimens exposed to melanogenic doses of UV-B. We propose that UV-A wavelengths can selectively cause redistribution of melanin-laden organelles within human keratinocytes in vivo and that this phenomenon accounts for the visually observed hyperpigmentation that develops soon after single exposures to these wavelengths. Dispersion of melanosomal complexes may be another mechanism by which UV-radiation (UVR) can induce tanning in human skin.

Adult↗
PubMed1976

The effect of X-ray irradiation upon the epithelial melanin unit of the hair bulb in hooded rat.

A marked increase in the quantity of pigment in the upper bulb, penetration of melanosomes into the papillae and vacuolisation of the bulb keratinocytes occurred as early as 4--6 h after irradiation with a dose 600--1200 R. Afterwards considerable hyperpigmentations appeared in the proximal segments of dystrophic and catagen hairs and melanin was found in the catagen columns and beyond them. In most animals within 4--6 h after irradiation occurred a distinct vacuolisation of the Malpighian layer and the granular layer became more prominent.

Animals↗
PubMed1993

Acute mucocutaneous toxicity following high-dose hydroxyurea.

Three patients with advanced acute myeloid leukemia were treated with oral high-dose hydroxyurea at a dose of 10 g daily for 8-10 days. Severe acute stomatitis developed in all three patients. In addition, two of the patients developed a peculiar acute cutaneous type of toxicity associated with soreness, violet erythema, and edema of the palms and foot soles followed by intense universal hyperpigmentation of the skin. Apparently, the pronounced acute mucocutaneous toxicity was caused by the sustained high daily dose of hydroxyurea, indicating that myelosuppression may not be the dose-limiting toxicity of this drug.

Acute Disease↗
PubMed1997

Transforming growth factor-alpha (TGF alpha)-producing gastric carcinoma with acanthosis nigricans: an endocrine effect of TGF alpha in the pathogenesis of cutaneous paraneoplastic syndrome and epithelial hyperplasia of the esophagus.

A case of well-differentiated adenocarcinoma (Borrmann type 3) of the stomach in a 76-year-old man associated with the typical skin manifestations of acanthosis nigricans and with multiple protruding lesions showing epithelial hyperplasia of the esophagus is reported. The advanced tumor was located in the cardiac region of the stomach, and measured approximately 8 cm in diameter, with partial invasion to the esophagus. The associated cutaneous lesions were characterized by hyperpigmentation and by protruding verrucous papules on the torso, head, face, neck, upper extremities, perineum, and inguinal region. Histologically, the protruding skin lesions showed keratinocytes proliferation throughout the epidermis, resulting in diffuse hyperkeratosis, papillomatosis, and acanthosis of the skin. Immunohistological analysis showed coexpression of transforming growth factor alpha (TGF-alpha) and epidermal growth factor (EGF) receptors in the tumor from the stomach. It is reasonable to conclude from this evidence that gastric carcinoma cells secrete TGF alpha in an autocrine for auto-stimulation. EGF receptor expression was also noted on the papillomatous hyperplasia of the cutaneous lesion. Serum level of TGF alpha, determined by an enzyme-linked immunosorbent assay, was high (144 pg/ml; normal, 22.0 +/- 16 pg/ml (Mean +/- SD)). Serum TGF alpha abruptly decreased to 49 pg/ml on day 7 after the total gastrectomy, and then gradually increased to 77 pg/ml within 28 days. Amelioration of the cutaneous lesions and the protruding lesions in the esophagus was observed after surgical resection of the gastric carcinoma. This suggests that the TGF alpha stimulates the proliferation of keratinocytes involved with EGF receptor. Large amounts of circulating TGF alpha in the blood over a long period released by the primary tumor seem to act as an endocrine-like mechanism causing epidermal and esophageal epithelial cells to proliferate. There is a possible link in the pathogenesis of the acanthosis nigricans as a cutaneous paraneoplastic syndrome, and epithelial hyperplasia of the esophagus.

Acanthosis Nigricans↗
PubMed1979

[Chronic mercury poisoning following topical application of skin bleachers (author's transl)].

In two female patients chronic mercurialism following topical application of skin bleachers for the treatment of freckles was diagnosed. Over 20 to 25 years 3 to 10% mercurial ointments were applied twice daily. Recurrent attacks of headache, dyspnoea and abdominal colic-like cramps had lead repeatedly to emergency hospitalisations, multiple medical check-ups and surgical exploratory procedures. The diagnosis of mercurialism was made clinically because of a slate-grayish skin hyperpigmentation in the presence of freckles, and the drug-history. Metallic deposits in facial biopsies were demonstrated by electron microscopy. On admission 15.4 microgram% and 5.0 microgram % total Hg respectively were found in the two patients (normal values up to 2.0 microgram %). Following D-penicillamine treatment the mercury excretion via the urine could be elevated up to 25 microgram %. Neither the slate-gray facial discoloration nor the neurasthenic complaints were affected by this therapeutic trial. In one patient, a maculo-papular drug-induced skin rash to D-penicillamine developed. Persistent unsolved neurological complaints and cramp-like abdominal pains should remind that percutaneous mercury intoxication through intact skin following skin bleachers is still possible today.

Adult↗
PubMed1990

Dyskeratosis congenita (Zinsser-Cole-Engman syndrome). An autopsy case presenting with rectal carcinoma, non-cirrhotic portal hypertension, and Pneumocystis carinii pneumonia.

A 24-year-old Japanese man presented with dyskeratosis congenita (DC, Zinsser-Cole-Engman syndrome) complicated by non-cirrhotic portal hypertension, signet ring carcinoma of the rectum and Pneumocystis carinii pneumonia. At the age of 9 years, he was diagnosed as having DC on the basis of typical clinical manifestations including atrophic lingual papillae, hyperpigmentation of the skin, thrombocytopenia, and ophthalmological abnormalities. A few years later pancytopenia and splenomegaly developed. At 24 years, signet ring carcinoma of the rectum was detected but could not be resected because of the severity of the pancytopenia. Death was due to respiratory failure from P. carinii pneumonia. At autopsy the case illustrated several unique findings for DC, including non-cirrhotic portal hypertension, atrophy of frontal lobe and markedly slender folia of the cerebellum and superimposed infections with herpes zoster virus and P. carinii. Striking lymphocyte depletion and atrophy of lymphoid parenchyma in lymph nodes, tonsils, spleen, gastrointestinal tract, or thymus were seen histologically. The morphological picture supports the suggestion that there is a defect in the cell-mediated immune system in patients with DC, although immunoglobulin levels in the blood are normal. The cell-immune deficiency is a major factor in the poor prognosis.

Adenocarcinoma, Mucinous↗
PubMed1982

[Side-effects of polychemotherapy in metastatic testicular neoplasms (author's transl)].

25 Patients with metastatic non-seminomatous testicular neoplasms were treated by surgery and cytostatic therapy using a combination consisting of Velban, Bleomycin, Cis-Platinum and/or Ifosfamid. In 22 patients this procedure induced a persistant complete remission with a mean observation time of 23 months. 2 patients died because of post-surgical complications after a second-look-lymphadenectomy. They suffered from rapidly progressive tumor disease. One patient died in a septicemia during chemotherapy. Our experience is that morbidity of an effective chemotherapy should not be underestimated. Transient bone marrow suppression, anorexia, alopecia and hyperpigmentation are unavoidable. However, severe vomiting, disturbed electrolyte metabolism, hemorrhagic cystitis, anemia and septicemia can well be managed by respective supportive care. Septicemia, for instance, may be treated with appropriate antibiotics without inducing tubular necrosis. Supportive measures also will avoid severe chronic defects of ear and kidney function.

Adult↗
PubMed1985

Trans-sphenoidal microsurgical treatment of Nelson's syndrome.

Clinical features and the operative results in the author's personal series of ten cases with Nelson's syndrome are presented. There were nine females and one male patient with ages ranging from 20 to 44 years (mean 36 y). All patients presented with typical hyperpigmentation of the skin and had abnormally raised plasma ACTH levels. X-ray polytomography detected sellar abnormalities in 80% of the cases. Computerized tomography was positive for pituitary tumour in three patients. Trans-sphenoidal microsurgical removal of Nelson's ACTH adenomas were successful in seven of ten patients. The operative technique of radical-selective resection to achieve endocrinological cure is described. Pertinent literature is reviewed and the incidence of Nelson's syndrome, an issue of controversies regarding the pathogenesis, and the management of Nelson's adenomas are discussed.

Adenoma↗
PubMed1996

Case report: gastric carcinoma as a complication of dyskeratosis congenita in an adolescent boy.

Dyskeratosis congenita (DC), or the Zinsser-Engman-Cole syndrome, is a rare X-linked heritable disorder, affecting primarily the ectodermal tissues, with hyperpigmentation of the skin, leukoplakia of the buccal and anal mucosa, and nail dystrophy (1, 2). Aplastic anemia (3) and a variety of neoplasms (4, 5) are some of the extraectodermal manifestation of this disorder, which although X-linked recessive, has also been described in a few females (6, 7). Mental retardation, diarrhea, and gastrointestinal bleeding have been considered to be less frequent features (8). We report an adolescent Indian male who presented with all the ectodermal manifestations, as well as mental retardation, bone marrow aplasia, and gastrointestinal hemorrhage secondary to adenocarcinoma of the stomach.

Adenocarcinoma↗
PubMed1995

Addison disease 10 years after bone marrow transplantation for Wiskott-Aldrich syndrome.

UNLABELLED: We report a 10-year-old boy with familial Wiskott-Aldrich syndrome (WAS) who underwent successful bone marrow transplantation (BMT) at the age of 9 months. With the exception of auto-immune haemolytic anaemia due to warm antibodies lasting 15 months there had not been any complication after BMT. Ten years later the patient presented with diarrhoea, hyperpigmentation of skin and oral mucosa, fatigue and polyuria. Diagnosis of Addison disease was confirmed by typical electrolyte imbalance and absent cortisol response to adrenocorticotrophic hormone. Adrenal antibodies were positive. On therapy with oral gluco- and mineralocorticoids, the symptoms disappeared and electrolytes normalized. To our knowledge auto-immuno endocrinopathy after BMT for WAS has not yet been reported. CONCLUSION: This is the first report of auto-immune adrenal insufficiency after BMT for WAS. The aetiopathogenesis of this condition remains unknown since auto-immune diseases as toxic side-effects of the ablative treatment before BMT have not yet been reported, and a relapse of WAS and cotransplantation of auto-immune adrenal insufficiency have been ruled out.

Addison Disease↗
PubMed1982

Early skin responses of hibernating and nonhibernating ground squirrels to topical applications of DMBA.

Skin patches on hibernating and nonhibernating ground squirrels were treated with multiple topical applications of the carcinogen 7,12-dimethylbenza(a)anthracene. Nonhibernators showed blistering, peeling, drying, hair loss, increased vascularization and hyperpigmentation in proportion to DMBA concentration. The latter was apparently due to a) an increased number of dermal and epidermal melanocytes and b) the appearance of melanocytes with large coarse cytoplasmic granules. Notably, hibernators remained free of gross skin changes and were histologically similar to untreated controls.

9,10-Dimethyl-1,2-benzanthracene↗
PubMed1990

High-dose multi-agent chemotherapy followed by bone marrow 'rescue' for malignant astrocytomas of childhood and adolescence.

Between April 1986 and March 1989, ten patients under 21 years of age with histologically confirmed malignant astrocytoma, received marrow-ablative chemotherapy with either thiotepa and Etoposide (five patients) or thiotepa, Etoposide and BCNU (five patients), followed by bone marrow 'rescue'. Nine patients had glioblastoma multiforme (GBM), and one patient had an intrinsic brain stem anaplastic astrocytoma (AA). Seven patients were treated for recurrent tumor. Two patients who developed GBM as second malignancies were treated directly following surgical resection. One patient had received irradiation only for recently diagnosed cervical spinal cord GBM. Thiotepa was administered at a total dose of 600-900 mg/M2 over three days, Etoposide was administered at a total dose of 1500 mg/M2 over three days, and BCNU was administered at a total dose of 600 mg/M2 over four days. Non-hematopoietic toxicities have been mainly transient, predictable and acceptable, consisting of oropharyngeal mucositis, cutaneous hyperpigmentation, erythema and desquamation. Four patients achieved complete responses (CR), as determined by radiographic evaluation (CT and/or MRI) on day 28 post-marrow infusion. The mean remission duration of those with CR is 290+ days; two patients presently remain in remission. Two patients achieved partial responses (PR, greater than 50% tumor shrinkage) by day 28 post-marrow infusion; both developed disease progression, at day 61 and 94 post-marrow infusion, respectively. One patient, with a brain stem AA, had stable disease maintained for 13 months post-marrow infusion. With a total (CR + PR) response rate of 60%, these regimens merit evaluation in broader categories of recurrent brain tumor patients, as well as in patients with newly-diagnosed GBM.

Adolescent↗
PubMed1980

Congenital retino-pigment epithelial malformation: a clinicopathological case report.

An unusual unilateral fundus lesion in a 16-year-old boy is described consisting of hyperpigmentation and presumed hypertrophy of the retinal pigment epithelium combined with malformation and thickening of the overlying sensory retina. Based on its clinical and histological characteristics, this lesion is considered to be a congenital retinal pigment epithelial malformation.

Adolescent↗
PubMed1996

Early diagnosis of Nelson's syndrome.

Nelson's syndrome is a specific form of Cushing's disease treated by bilateral adrenalectomy, presenting with a deep hyperpigmentation caused by a pituitary adenoma (corticotropinoma). These ACTH-secreting tumors are frequently aggressive, so early diagnosis is of prime importance. We have studied 33 patients with Nelson's syndrome, 28 women and 5 men, aged 14-56 yr at the time of adrenalectomy and 16-58 yr at the time of Nelson's syndrome diagnosis (observed for 5-32 yr). Methods of examination included simultaneous adrenocorticotropic hormone (ACTH) and cortisol measurements during routine hydrocortisone replacement therapy, computed tomography (CT), pituitary magnetic resonance imaging (MRI), and visual field examination. The results obtained in a group of six patients diagnosed in the last 3 yr were compared with those obtained in a group of 27 patients examined before 1992. High plasma ACTH levels accompanied by normal serum cortisol concentration were characteristic for a late stage of the disease. Absolute temporal scotomas were an early finding. MRI, especially with the gadolinium enhancement, was superior to CT in demonstrating pituitary microadenomas in Nelson's syndrome. Thus, MRI diagnosis allowed for an early neurosurgical treatment of the patients with Nelson's tumors.

Adenoma↗