Professional self respect. Professional foul?
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Biomedical subjects
Publications and source records attributed to S K Goolamali.
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Lichen sclerosus is commonly seen in middle-aged and elderly women but it affects both sexes, can occur at any age and anywhere on the body and has been reported in most races. Its aetiology is uncertain but there is an increased incidence of auto-immune antibodies in lichen sclerosus and an association with auto-immune disease such as vitiligo, thyroid disease and alopecia areata. Lichen sclerosus has been observed in different generations of a family but no consistent genetic pattern has emerged. Lichen sclerosus typically affects the anogenital region and presents with an intractable 'burning' pruritus. Extragenital lesions do not itch. Oral lesions occur in lichen sclerosus and may resemble lichen planus. Lichen sclerosus exhibits the isomorphic or Koebner phenomenon. The literature supports the view that lichen sclerosus is not a precancerous lesion but there is a significant association with vulval squamous cell carcinoma. Patients with inactive, treated lichen sclerosus should be reviewed annually. The treatment of choice of uncomplicated genital lichen sclerosus is a topical steroid of a potent or super-potent category. In males, circumcision may be indicated if topical measures are unsuccessful. In women, surgery is considered predominantly for cancer confirmed by tissue examination.
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Three infants with X-linked ichthyosis have been observed following pregnancies in which placental sulphatase deficiency (PSD) was suggested prenatally by low oestrogen excretion and an abnormal urinary steroid excretion pattern. This was confirmed in two cases by the absence of placental enzyme activity. In one case labour was spontaneous but all deliveries required Caesarean section. At 8 months the first infant showed an eczema in an atopic distribution but when seen at 5 years had typical X-linked ichthyosis. The skin of the second child peeled extensively at the age of 2 days but was not troublesome for 2 years, when ichthyosis vulgaris was diagnosed on clinical grounds. This pattern has persisted for 3 years. The third infant showed a mildly scaly skin in the neonatal period but at 3 months the features and distribution of X-linked ichthyosis were apparent. X-linked ichthyosis may have a variable presentation which is not always apparent at birth.
Intestinal permeability was investigated in adult patients with atopic eczema by in vivo and in vitro techniques. Patients with symptoms of 'immediate' food allergy were specifically excluded. A 51Cr-labelled ethylenediaminetetraacetate absorption test was carried out in eighteen patients. Their mean (+/- s.d.) 24-hour urine excretion following oral administration of the test substance (2.1 +/- 0.9%) did not differ significantly from that of thirty-four normal controls (1.9 +/- 0.5%). Small bowel permeability was estimated directly in jejunal mucosal samples in ten patients with three permeability probes of differing molecular weight. Mucosal permeability did not differ significantly from that of fifteen control patients for any of the test substances. Two patients had abnormal results by both tests and in one this was due to coeliac disease. These results suggest that altered intestinal permeability is not important in the pathogenesis of eczema. Patients demonstrating increased intestinal permeability should undergo jejunal biopsy to exclude significant small bowel disease.
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We describe the case of an otherwise healthy 7-year-old girl whose mother noticed that she intermittently smelt of fish. This was due to the intermittent excretion of trimethylamine which could be precipitated by choline ingestion and by eating fish. Excluding eggs, liver and salt-water fish from the diet relieved the symptom. After a standard 15 g choline load, the child's father, but not her mother, excreted amounts of trimethylamine which were intermediate between those excreted by the patient and normal control subjects.
We report a patient with an eccrine carcinoma who developed localized blistering which clinically resembled pemphigoid, histologically showed subepidermal blistering with features of both dermatitis herpetiformis and bullous pemphigoid, responded to dapsone and exhibited linear IgA deposition on direct immunofluorescence. The nosological position of patients with linear IgA deposition and subepidermal blistering is not clear. A review of the literature reveals that in adults linear IgA deposition may occur in three separate situations: dermatitis herpetiformis, bullous pemphigoid and a third condition of which our case is an example which is best termed IgA bullous pemphigoid. This condition is distinguished from cases of dermatitis herpetiformis with linear IgA by the clinical features and the site of IgA deposition on immunoelectronmicroscopy. It is distinguished from cases of bullous pemphigoid with linear IgA by the absence of circulating IgG antibasement membrane zone antibody, the therapeutic response to dapsone and the frequent occurrence of circulating IgA antibasement membrane zone antibody. IgA bullous pemphigoid has not previously been reported with a carcinoma but the association lends further support to the concept that this eruption represents a variant of pemphigoid.
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In a controlled study of barium swallow radiographs in systemic sclerosis, longitudinal oesophageal mucosal folds were absent in eight of 30 patients and in one of 30 control subjects (P less than 0.03). Patients without mucosal folds developed Raynaud's phenomenon at an earlier age than those who retained their mucosal folds (P less than 0.05). A loss of oesophageal mucosal folds did not necessarily signify more severe visceral or cutaneous disease.
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Plasma immunoreactive beta-melanocyte-stimulating hormone (beta-MSH) concentrations were normal in schizophrenic patients on prolonged and high dosage phenothiazine therapy. A group of patients treated with chlorpromazine 25 mg three times daily for pruritic dermatoses also showed no significant change in plasma beta-MSH concentration.
Sebum excretion rates (SER) were measured before and after treatment in patients with hypothyroidism and thyrotoxicosis. The mean SER in the former was significantly less than that in normal controls but there was no correlation between SER and the severity of the disease as indicated by serum thyroid-stimulating hormone levels. After treatment with L-thyroxine the SER increased but remained subnormal. By contrast the SER was not increased in patients with thyrotoxicosis and it was unaffected by treatment. The human sebaceous gland seems to respond to thyroid hormone mainly in the hypothyroid range.
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