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Primary adrenocortical insufficiency (Addison's disease).

In the United States the etiology of most cases of adrenal gland atrophy or destruction is unknown. Autoimmune processes may be responsible. With rare exceptions, all three zones of the adrenal cortex are destroyed and there is reduced glucocorticoid, mineralocorticoid and androgen secretion. Although the deficiency of glucocorticoid is probably the most severe threat to life, treatment is available to correct all the deficiencies. Patient education and preparedness can prevent serious complications.

17-Hydroxycorticosteroids↗

Effects of cyclosporine in spontaneous, posterior uveitis.

Cyclosporin A (CsA) was administered to chicks of the Smyth delayed-amelanotic (SDA) line from day of hatch to 4, 8 or 12 weeks of age. Animals were evaluated at 8, 12, 16 or 20 weeks with regard to major features of the SDA-line disorder, including extent of feather and choroidal amelanosis, choroidal inflammation, and histopathology of the retinal pigment epithelium and outer retina. A suppression and delay in the onset of both amelanosis and ocular histopathology occurred during CsA administration. However, a rebound enhancement of symptoms occurred 4-8 weeks after withdrawal of CsA that was closely associated with the duration of CsA treatment. These results indicate that CSA may yield therapeutic effects during the period of treatment, but its withdrawal may lead to more severe symptoms that would have occurred without treatment.

Animals↗

Clinical course of bimatoprost-induced periocular skin changes in Caucasians.

PURPOSE: To describe the demographic and clinical characteristics of bimatoprost-induced periocular skin hyperpigmentation in Caucasians. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Thirty-seven Caucasian patients (29 female, 8 male) with a diagnosis of primary open-angle glaucoma (n = 28) or ocular hypertension (n = 9) in whom cosmetically noticeable periocular skin pigmentation developed with bimatoprost therapy. METHODS: An unbiased examiner performed a retrospective chart analysis of patients in whom periocular skin hyperpigmentation developed after starting bimatoprost therapy. Data collected included patient demographics, diagnosis, medication history, dates of starting and stopping bimatoprost treatment, and subjective assessment of the periocular hyperpigmentation at initial detection and follow-up visits. MAIN OUTCOME MEASURES: Periocular hyperpigmentation was graded using an arbitrary scale from 0 to 3. The number of days to the onset of hyperpigmentation and to pigment resolution was determined and their associations to demographic and other clinical parameters were analyzed. RESULTS: Patients had variable grades of periocular hyperpigmentation at presentation (mean, 1.27+/-0.50; range, 1-2.5). Bimatoprost-induced periocular hyperpigmentation appeared most frequently between 3 and 6 months after initiation of bimatoprost therapy (277+/-138 days). Resolution of skin hyperpigmentation was noted most frequently between 3 and 12 months (205+/-97 days); however, there was a wide range of 61 to 472 days. Thirty-three of the 37 patients had complete resolution of the periocular hyperpigmentation. CONCLUSIONS: Bimatoprost use is associated with periocular skin hyperpigmentation in Caucasians with variable time of onset. The periocular hyperpigmentation appears gradually, but in this series was completely reversible on discontinuation of bimatoprost.

Aged↗

Clinical manifestations of terminal osseous dysplasia and pigmentary defects in a young girl.

Terminal osseous dysplasia and pigmentary defects is a rare X-linked dominant disorder with prenatal male lethality. Affected females display multiple systemic abnormalities such as limb deformities and pigmented lesions of the face and scalp. Phenotypic expression of the syndrome varies among the affected individuals. In this case report, we describe the syndromic dental and oral abnormalities in a female child aged 3 and 1/2 years. A widened bigonial width of the mandible and a brachyfacial pattern are observed. Intraoral findings include multiple frenulae, shallow mucobuccal fold, hypodontia, conical incisors, and other developmental structural defects.

Abnormalities, Multiple↗