Biomedical subjects
Publications and source records attributed to I B Sneddon.
A patient with bullous pemphigoid developed pemphigus foliaceus 5 years later. Mixed bullous diseases are rare and this is the first reported case of coexisting bullous pemphigoid and pemphigus foliaceus.
An account is given of the manner in which patients suffering from psychoneurosis and psychoses present to the Dermatologist. Cases of monosymptomatic hypochondriasis, trichorrhexis nodosa in an obsessional neurotic, acne excoriée, schizophrenia, depression and delusions of infestation are described. The value of modern neuroleptic drugs in the management of monosymptomatic hypochondriasis in cluding delusions of infestation is confirmed.
Cell-mediated cytotoxicity was examined in thirty-seven patients with systemic sclerosis using both whole blood and purified peripheral blood mononuclear cells (PBM) to measure antibody-dependent (ADCC) and phytohaemagglutinin (PHA) induced lymphocyte cytotoxicity to 51Cr-labelled Chang liver cells. In twenty-three mildly affected patients, ADCC and PHA-induced cytotoxicity did not differ from that found in control populations. By contrast, fourteen patients severely affected by extensive visceral disease showed reductions in both ADCC and PHA-induced cytotoxicity which were more marked in whole blood assays (P less than 0.001) than in those performed with PBM (P less than 0.05). The addition of patient's sera to control cytotoxicity assays suggested that blocking or suppressive serum factors could only account for some of the disproportionate reduction in whole blood cytotoxicity which, in the main, must be due to a lack of circulating effector cells. These results are in agreement with previous findings of reduced numbers of circulating thymus-dependent lymphocytes in patients with severe disease, a defect of cell-mediated immunity that may result from the chronic antigenic stimulation of an autoimmune disease process.
Seventy-one patients with systemic sclerosis (SS) were typed for twenty-seven HLA alleles of the A and B loci, and the findings were related to both the extent of visceral disease and tests of cellular immune competence in a subgroup of fifty-two of these patients. Nineteen pa;ients with widespread visceral involvement and more rapidly progressive disease had an increased frequency of HLA-B8 (relative risk = 4.14; P less than 0.05) when compared to thirty-three less severely affected patients and 3000 controls. Patients with severe and progressive disease also had defective cell-mediated immunity with reductions in both the numbers of circulating thymus-dependent (T) lymphocytes and in the lymphocyte transformation response to phytohaemagglutinin. These findings suggest that a genetic factor, such as an abnormal immune response gene, may be involved in the progression of the disease.
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Pemphigus vulgaris is uncommon in adolescence and only ten well documented cases in this age group were found in a recent review (5). Because the condition is often more severe in the younger age group it is important to consider it in the differential diagnosis of bullous eruptions of childhood and to perform direct and indirect immunofluorescence studies.
Subcorneal dermatosis is a chronic relapsing pustular eruption which has been recognised for 20 years. The diagnosis can be made only by combining the clinical features of a recurrent eruption mainly on the trunk which spares the mucosae and has the histological appearance of a subcorneal bullae filled with polymorphonuclear leucocytes situated on the surface of normal epidermis. No immunofluorescence either direct or indirect similar to that seen in pemphigus, pemphigoid or dermatitis herpetiformis has been reported. No jejunal abnormality has been found in any case but some, though not all, patients respond to treatment with Dapsone (DDS; diaminodiphenylsulfone). The condition can be distinguished from other cases of subcorneal pustular eruptions only by combining the clinical and histological features. The etiology remains unknown.
This case of benign mucous membrane pemphigoid (BMMP) is unusual in that blistering, scarring lesions were confined to the skin for 15 years before mucous membranes were involved. The onset of this disorder at the age of 38 is also unusual. Detailed immunological investigation was performed on this patient but the results in no way clarify the present confusion regarding the immunopathological processes in BMMP related to those operative in bullous pemphigoid.
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Description of adverse effects of strong corticosteroids in rosacea. Perioral dermatitis also seems to be caused by treatment with strong corticosteroids. The rebound effect after weaning the patient from strong corticosteroids might be obviated by hydrocortisone 17-butyrate or hydrocortisone 21-acetate. Continued oral tetracycline treatment is recommended.
The action of topical corticosteroids is anti-inflammatory and antimitotic. By the use of laboratory and clinical trials they can be divided into four grades of potency. Weak steroids may be made more potent by better penetration which can be achieved by plastic occlusion or by alteration of the vehicle in which they are suspended. Local side-effects of topical corticosteroids are described and attention is drawn to the face as a particularly vulnerable area. Use of large amounts of the most potent steroids has produced Cushingoid states normally associated only with systemic steroids, and this is more likely if the patient has liver damage. Practical advice on the choice of steroid for particular disorders is given and the importance of intermittent treatment both for safety and effectiveness is stressed.
Large numbers of milia were seen in areas of skin affected with bullae in a 68-year-old woman who had extensive lichen sclerosus et atrophicus. Although milia are frequently seen in other sub-epidermal bullous disorders, they have not previously been reported in lichen sclerosus et atrophicus.
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