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Biomedical subjects

S M Burge

Publications and source records attributed to S M Burge.

At least 19 recordsLinked to original sources
PubMed1997

Solar urticaria in an infant.

A 2-year-old girl presented with a history of an erythematous rash which occurred immediately after exposure to sunlight and had been a problem since birth. Extensive laboratory investigations to exclude genophotodermatoses, photosensitivity secondary to metabolic disorders and photoaggravated dermatoses were negative. Monochromator irradiation phototesting demonstrated immediate erythematous flares to all ultraviolet B (UVB), UVA and visible wavelengths up to 500 nm. A diagnosis of solar urticaria was made and she responded to loratidine 10 mg daily. We believe this is the first report of solar urticaria, confirmed by phototesting with a monochromator so early in life.

Child, Preschool↗
PubMed1996

Handicap in Darier's disease and Hailey-Hailey disease.

One hundred and thirty-seven patients with Darier's disease and 66 patients with Hailey-Hailey disease completed the Dermatology Life Quality Index (DLQI). The average DLQI score for Darier's disease was 5.89 (19.6% of the maximum score), median = 4, quartiles: 1.9. The average DLQI score for Hailey-Hailey disease was 6.06 (20.2% of the maximum score), median = 5, quartiles: 2.9. The DLQI score did not correlate with the physician's assessment of clinical severity in both diseases, thus emphasizing the importance of remembering the potential for handicap even in patients with disease that seems to be mild. The domains of the questionnaire with the highest mean scores in both diseases were for questions relating to symptoms and feelings. Domains relating to personal relationships and work obtained low mean scores even in severe disease. It is encouraging that most patients with Darier's disease and Hailey-Hailey disease maintain good relationships at work and home and seem to be able to adapt to their skin disease.

Adolescent↗
PubMed1996

Cutaneous manifestations of systemic lupus erythematosus.

We have assessed the cutaneous signs in 73 patients with systemic lupus erythematosus (SLE), seen during a 5-year period in an English hospital. Most previous information about the cutaneous manifestations of SLE has been obtained from studies performed in the U.S.A. We classified lesions as specific cutaneous and mucosal LE (acute, subacute and chronic) or non-specific LE-related, e.g. photosensitivity, urticaria, erythema, Raynaud's phenomenon or vasculitis. Acute cutaneous LE lesions included a butterfly rash with erythematous macules, telangiectasia or papulosquamous lesions, seen in 37 patients (51%) and facial oedema seen in four patients (5%). Five patients (7%) had psoriasiform subacute cutaneous LE. Chronic cutaneous LE was common: 18 patients (25%) had chronic discoid lesions (DLE) and, in 12 (15%), these had preceded systemic disease. One patient had facial lupus profundus. Ten patients (14%) had scarring alopecia secondary to DLE. Fifteen patients (20.5%) had chronic chilblain lupus. Twenty-three patients (31.5%) had a history of mouth ulceration. Of these, 11 (15%) gave a history of ulcers at the onset of their disease. Three (4%) had erythema and superficial ulceration of the palate, not typical of aphthous ulcers, and three (4%) had chronic buccal plaques. Cheilitis due to DLE was seen in three (4%), episcleritis in three (4%), five (7%) had nasal disease, six (8%) bullous skin eruptions, one 'the bullous eruption of SLE', four bullae associated with cutaneous vasculitis, and one bullae associated with ultraviolet radiation. Forty-six (63%) observed photosensitivity. A non-scarring alopecia occurred in 29 (40%). Vascular phenomena were common: three patients (4%) had chronic palmar erythema, Raynaud's phenomenon occurred in 44 patients (60%), chronic urticaria, worsened by sun exposure, was noted by 32 (44%) (in whom the lesions often lasted more than 36 h), eight (11%) had cutaneous vasculitis and three (4%) livedo reticularis. Skin changes play a prominent part in SLE and may provide helpful diagnostic information. In this British population, chilblains and urticaria were particularly common. Lesions of subacute cutaneous LE were relatively unusual in this group of patients with SLE.

Adolescent↗
PubMed1995

Vasculitis in a fixed drug eruption due to paracetamol.

A 48-year-old man presented with a history of recurrent erythematous lesions on the trunk and limbs. Examination suggested a fixed drug eruption and this was confirmed by oral challenge with paracetamol. The histology was unusual as it showed a leucocytoclastic vasculitis. Patients may have difficulty avoiding the offending drug because many drugs bought over the counter have similar names but different constituents. In addition, it may be difficult to persuade the patient that a specific drug is responsible for their skin problem.

Acetaminophen↗
PubMed1995

Topical isotretinoin in Darier's disease.

Topical 0.05% isotretinoin (Isotrex gel) was used to treat a test patch of skin in 11 patients with Darier's disease. Hyperkeratosis and papules improved in six patients after treatment for 3 months. Erythema, burning and irritation were common adverse effects, and these were severe in three patients, one of whom stopped treatment. Patients with mild Darier's disease may find topical isotretinoin helpful, but it is likely that most patients with widespread disease will require treatment with systemic retinoids.

Administration, Cutaneous↗
PubMed1995

Bullous systemic lupus erythematosus: revised criteria for diagnosis.

Blistering in systemic lupus erythematosus has been divided into three groups. A specific subgroup of 'bullous systemic lupus erythematosus' has been defined by Gammon et al. on the basis of a number of criteria. From our experience of seven patients with bullous systemic lupus erythematosus, and after reviewing the literature, we suggest that the current classification is too narrow. Our patients displayed clinical and immunohistological (based on direct and indirect immunofluorescence and Western immunoblotting) heterogeneity. Sera from two patients bound to epidermal epitopes in sodium chloride-split skin, but immunoblotting was negative. In neither of these patients could the target antigen be type VII collagen, the only antigen identified as pathogenic in this disease. Patients with epidermal binding should not be excluded from a diagnosis of bullous systemic lupus erythematosus. SLE is a disease in which there is a genetic predisposition to form antibodies to type VII collagen, along with other autoantibodies, many of which may be implicated in blistering. We suggest that the criteria for the diagnosis of BSLE should be revised. We define this disease as an acquired subepidermal blistering disease in a patient with SLE, in which immune reactants are present at the basement membrane zone on either direct or indirect immunofluorescence.

Adult↗
PubMed1994

Cantharidin-induced acantholysis: adhesion molecules, proteases, and related proteins.

Acantholysis is a feature of disorders such as Hailey-Hailey disease and Darier's disease. Immunocytochemical studies have shown internalization of desmosomal components after acantholysis. Basal cytokeratins show suprabasal expression in lesional Darier's disease. The exact mechanisms of acantholysis are still unclear. Cantharidin induces blistering, with suprabasal keratinocyte acantholysis, possibly by protease activation. Plasmin has been implicated in the pathogenesis of acantholysis in Darier's disease and Hailey-Hailey disease. We examined the distribution of desmosomal components, proteases and cytokeratins in cantharidin blisters, to compare them with those previously found in Darier's disease and Hailey-Hailey disease. Two drops of cantharidin collodion were applied to the skin of five normal volunteers. A 4-mm punch biopsy of the blister was taken, and snap frozen. Sections were stained with antibodies to desmosomal proteins (dp) 1/2, dp 3, desmosomal glycoproteins (dg) 1, 2/3, extracellular carbohydrate residues, using the lectins peanut agglutinin (PNA) and soybean agglutinin (SBA), proteases and cytokeratins. Acantholytic cells were stained diffusely with dp1/2; there was markedly reduced or absent peripheral staining for dp3, dg1, dg2/3, PNA and SBA. There was no clumping of stain. Plasminogen, fibrinogen and urokinase were expressed in some acantholytic cells. Basal keratin markers were expressed suprabasally in acantholytic cells. These results are similar to those previously obtained in Darier's disease, but different from the staining obtained in Hailey-Hailey disease. Extracellular glycosylated portions of adhesion molecules may be lost after acantholysis, perhaps as a result of conformational changes, internalization of extracellular domains, or proteolysis. The changes in the expression of plasminogen, fibrinogen, urokinase and cytokeratins in acantholytic cells in cantharidin-induced blisters are, as in Darier's disease and Hailey-Hailey disease, probably secondary to acantholysis, and changes in the shape of cells. We conclude that cantharidin blisters may be a useful model for the study of acantholysis in Darier's disease.

Acantholysis↗
PubMed1994

Cantharidin-induced acantholysis in Darier's disease: does acantholysis initiate dyskeratosis?

We have examined the action of cantharidin on the skin of patients with Darier's disease, and used immunohistological techniques to determine the distribution of desmosomal components, keratin intermediate filaments, and proteases in cantharidin-induced blisters. Cantharidin induced acantholysis, but the presence of acantholysis did not trigger the development of the characteristic warty, dyskeratotic papules in patients with Darier's disease. The distribution of desmosomal components, keratins and proteases within the acantholytic keratinocytes in the cantharidin-induced blisters was similar to that previously found in acantholytic cells within lesions of Darier's disease: peripheral staining for extracellular desmosomal components was reduced; some desmosomal components were detected diffusely in the acantholytic cells; basal cell keratin markers were expressed by some suprabasal acantholytic cells, and plasminogen was detected in association with acantholytic cells. Cleavage of desmosomes did not reveal the underlying abnormality in Darier's disease.

Acantholysis↗
PubMed1993

An immunohistological study of desmosomal components in pemphigus.

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune diseases in which there is loss of cohesion between keratinocytes (acantholysis) and blistering within the epidermis. PV is characterized by acantholysis predominantly between the epidermal basal cells and suprabasal layers, whereas in PF intraepidermal cleavage is higher in the epidermis. Adhesion between keratinocytes is dependent on the function of transmembrane glycoproteins of the cadherin family present in specialized adhesion junctions, the desmosomes. The pathogenesis of acantholysis in pemphigus is uncertain, but the pemphigus autoantibodies bind to epithelial cadherins. We have used monoclonal antibodies to desmosomal components to investigate their distribution in different forms of pemphigus. Our results show that the localization of desmosomal components is abnormal in intact perilesional epidermis, intact epidermis above the blisters in PV and intact epidermis below the blisters in PF. We suggest that autoantibody binding may have a direct effect on the function of specific epithelial cadherins, but will only cause cell separation where the antigen is the principal adhesion molecule.

Acantholysis↗
PubMed1993

The effect of hormonal changes on cutaneous disease in lupus erythematosus.

The behaviour of cutaneous disease in systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE), under the influence of various hormonal states, was studied in 68 patients. In 28 pregnancies, cutaneous disease was essentially unchanged. In a total of 57 patients whose lupus erythematosus (LE) had been diagnosed prior to the menopause, 20% described a premenstrual cutaneous exacerbation. Only three patients had taken an oestrogen-containing contraceptive. The duration of oral contraceptive treatment before the onset of lupus varied: 1 month in a patient presenting with the acute malar rash of SLE, 2 months in a patient who presented with annular weals and later developed systemic features, and 12 months in a patient who developed generalized DLE. Thirty-three patients were menopausal at the time of the study; 4% had noticed a perimenopausal cutaneous flare. There was no deterioration in the skin of the five patients on hormone replacement therapy.

Adolescent↗
PubMed1993

Warts and lupus erythematosus.

The human papilloma virus is implicated in causing several diseases, ranging from the common wart to malignancy. We describe a high prevalence of cutaneous warts in lupus erythematosus. The presence of warts did not correlate with the taking of immunosuppressive drugs. This observation suggests that there is a primary immunological defect among patients with lupus erythematosus. We found this high rate among patients with discoid as well as systemic lupus erythematosus. We found no correlation between the prevalence of cutaneous warts and cervical dysplasia, or malignancy. Discoid lupus erythematosus is often considered to be a different disease from systemic lupus erythematosus, running a more benign course. The high prevalence of cutaneous warts in both conditions highlights yet another similarity between these two diseases.

Adolescent↗