Cases reported "Pigmentation Disorders"

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1/86. Photoinduced dermal pigmentation in patients taking tricyclic antidepressants: histology, electron microscopy, and energy dispersive spectroscopy.

    Two patients had been taking long-term tricyclic antidepressant therapy. Each developed a blue to slate-gray hyperpigmentation in sun-exposed areas. On histologic examination there were refractile golden brown granules free in the dermis along collagen bundles. Similar pigment was present in macrophages and along the basement membrane zone. The granules stained for melanin, but not for iron, and were bleached by the permanganate method. Electron microscopy showed varying size and shaped electron-dense granules within lysosomes and free in the dermis, which, in unstained sections, showed a less dense peripheral halo. This peripheral halo was also evident on light microscopy. Energy dispersive spectroscopy showed these granules to be rich in copper and sulfur (elements present in tyrosinase and pheomelanin, respectively). We believe that this represents a drug-melanosome complex, which is most likely caused by chronic photoactivation.
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2/86. E.N.T. manifestations of Von Recklinghausen's disease.

    Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.
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ranking = 0.021978411247192
keywords = bone
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3/86. Pigmented paravenous retinochoroidal atrophy: a literature review supported by a unique case and insight.

    Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disorder of unknown origin characterised by bone corpuscle pigmentation accumulation along the distribution of the retinal veins. In addition there are peripapillary pigmentary changes as well as areas of chorioretinal atrophy adjacent to the perivenular pigmentary changes. The finding of PPRCA is usually incidental and does not affect vision. The literature regarding this condition is reviewed. Its natural course has been considered controversial. observation of the initial insult leading to PPRCA has not previously been reported. A case that provides a photographic record over 20 years of the development of this condition from the initial insult, including the gradual development of the characteristic fundus appearance, is described. The initial presentation was with a sudden reduction of vision and gross diffuse macular oedema in one eye, which was rapidly followed by similar involvement of the fellow eye despite treatment. Thus it was possible to examine the patient at the stage of the initial insult, 5 years before the development of the typical and pathognomonic retinal changes of PPRCA. The clinical and electrophysiological findings are discussed, as also is the relevance of this case to the literature reviewed.
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ranking = 0.010989205623596
keywords = bone
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4/86. Feminizing sertoli cell tumor associated with peutz-jeghers syndrome.

    peutz-jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the association of mucocutaneous pigmentation and multiple gastrointestinal hamartomatous polyps and with an increased risk of developing gonadal sex tumors besides other malignancies. We describe a 7 1/2 year-old boy with PJS and bilateral gynecomastia. He has had buccal pigmentation since 1.5 years and had been operated for rectal polyp excision at 3.5 years. On physical examination, his height was at the 90th percentile, and his height age and bone age were 9 and 10 1/2 years, respectively. Increased melanotic buccal pigmentation of the lips and bilateral gynecomastia were noticed. Both of the testes were firm, non-tender and smooth on the surface, and each measured 8 ml. Hormonal measurements were all in the prepubertal range. testis ultrasonography showed bilateral hyperechogenic areas within the glands. When he was operated for invagination and an ileum segment full of polyps was resected, bilateral testicular biopsies were also performed. Histopathological evaluation of the testes revealed bilateral multicentric benign Sertoli cell tumors. The aromatase inhibitor testolactone was started to slow skeletal maturation. On the basis of this and previous reports, PJS associated with sex-cord tumors is increasingly recognized in males as well as in females.
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ranking = 0.010989205623596
keywords = bone
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5/86. dyskeratosis congenita associated with three malignancies.

    dyskeratosis congenita is a rare inheritable disorder characterized by abnormalities of the skin, nails and oral mucosa. Aplastic anaemia resulting from bone marrow hypoplasia is a frequent cause of death. Squamous cell carcinoma developing from leukoplakia and visceral malignancies are other complications of the disease. We report here a case of dyskeratosis congenita in a man who developed three neoplasias of different systems over a period of many years. Squamous cell carcinoma and gastric adenocarcinoma manifested 17 years after the man was diagnosed with Hodgkin's disease.
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ranking = 0.010989205623596
keywords = bone
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6/86. minocycline-induced staining of torus palatinus and alveolar bone.

    BACKGROUND: minocycline hydrochloride, an analog of tetracycline, is widely used in the treatment of acne. Its use has been associated with discoloration of teeth, bone, and other tissues. methods: A case is presented involving a patient with minocycline-induced staining of the torus palatinus and alveolar bone. RESULTS: No treatment was rendered since the patient was not concerned with the appearance of the discoloration. The patient's dermatologist elected to change antibiotics. CONCLUSIONS: patients on long-term minocycline therapy should be made aware of the possibility of pigmentation of bone and soft tissue that may be reversible with discontinuation of therapy; however, minocycline-induced staining of the permanent dentition may not be reversible.
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ranking = 0.076924439365173
keywords = bone
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7/86. Accelerated phase at initial presentation: an uncommon occurrence in chediak-higashi syndrome.

    The authors describe an Indian child, who presented in the accelerated phase of the chediak-higashi syndrome. The disease usually presents in early childhood with recurrent skin and mucosal infections. This patient had subtle pigmentary abnormalities and no family history of the disease, which made the clinical diagnosis difficult. The cytopenias, hepatosplenomegaly, lymphohistiocytic infiltrate in the bone marrow, and the characteristic granules in the leucocytes clinched the diagnosis. This case underscores the importance of a bone marrow examination in patients with unusual presentations of rare disorders.
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ranking = 0.021978411247192
keywords = bone
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8/86. dyskeratosis congenita: delay in diagnosis and successful treatment of pancytopenia by bone marrow transplantation.

    dyskeratosis congenita is an inherited disorder characterized by nail dystrophy, skin pigmentary changes, mucosal leukoplakia, pancytopenia and an increased incidence of malignancy. Because of a widely held view that the outcome of bone marrow transplantation in dyskeratosis congenita is poor, this treatment option is sometimes not considered when pancytopenia develops. We present a child currently doing well 3 years after bone marrow transplantation, and review the literature.
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ranking = 0.065935233741576
keywords = bone
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9/86. Progressive multilayered banded skin in Winchester syndrome.

    Winchester syndrome is a rare genetic disorder, one of the inherited osteolysis disorders which are a group of diseases characterized by destruction and resorption of affected bones with consequent skeletal deformities and functional impairment. The syndrome is characterized by dissolution of carpal and tarsal bones with generalized osteoporosis, progressive joint contractures, short stature, peripheral corneal opacities, and coarse facial features, though there is variability within the clinical features. Phenotypic heterogeneity of cutaneous features are also reported to date of diffusely thickened leathery skin, hypertrichosis, patches of hyperpigmented, hypertrichotic leathery skin in annular or linear distribution, widespread acne, subcutaneous nodules, and gingival hypertrophy. We describe widespread progressive multilayered symmetrical restrictive banding of the skin developing in a woman with Winchester syndrome during her mid-twenties.
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ranking = 0.021978411247192
keywords = bone
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10/86. Oral-dental findings in dyskeratosis congenita.

    A 13-yr-old girl with dyskeratosis congenita is presented. Besides oral leukoplakia and nail dystrophies, there was evidence of pancytopenia, growth retardation, alopecia, mental retardation and microcephaly. The oral findings included caries, gingival recession, short-blunted roots, gingival bleeding, tooth mobility and severe alveolar bone loss resembling juvenile periodontitis.
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ranking = 0.010989205623596
keywords = bone
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