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Biomedical subjects

S A Holme

Publications and source records attributed to S A Holme.

At least 19 recordsLinked to original sources
PubMed2006

Erythropoietic protoporphyria in the U.K.: clinical features and effect on quality of life.

BACKGROUND: Erythropoietic protoporphyria (EPP) is a rare inherited photodermatosis that causes lifelong painful photosensitivity. Neither its full clinical spectrum nor its impact on quality of life (QoL) has been investigated in a large cohort of patients. OBJECTIVES: To document the clinical features of EPP and its impact on QoL in a high proportion of all patients with EPP resident in the U.K. METHODS: Patients with EPP were identified from U.K. clinical databases and assessed by the same clinical investigator over a 7-month period using a standardized proforma and validated adult (Dermatology Life Quality Index, DLQI) and children's (Children's Dermatology Life Quality Index, CDLQI) QoL questionnaires. RESULTS: Three hundred and eighty-nine living patients with EPP were identified, of whom 223 [114 females, 109 males; median age 34 years (range: 5-87), from 193 families] were investigated. Total erythrocyte porphyrin (TEP) was higher in males (median: 25.3 micromol L1) than females (median: 19.3 micromol L1). The median ages at onset and diagnosis were 1 and 12 years, respectively. Median times for onset of symptoms after sun exposure, onset of signs (oedema, erythema) and resolution of symptoms were 20 min, 6 h and 3 days, respectively. Most patients reported absence of protection by glass (92%), priming (85%), exacerbation by wind (68%), no family history of photosensitivity (56%), no symptoms during winter (56%) and had chronic skin lesions (79%). Symptoms changed little with age but improved during pregnancy in 47% of gravid women. Most patients used protective clothing and a sunscreen; 28% were taking beta-carotene and a further 56% had taken it; 29% were not under regular medical care. Two patients (1%) had liver failure and 8% reported gallstone disease. QoL was markedly impaired, with scores similar to those in severe dermatological disease (mean DLQI score 14.0, n = 176; mean CDLQI score 12.8, n = 44), indicating a large effect on patients' lives. DLQI scores correlated weakly with TEP (rs = 0.228; P = 0.002) and time to onset of symptoms (rs = -0.233; P = 0.002) but not with age at onset. CONCLUSIONS: EPP is a persistent, severely painful, socially disabling disease with a marked impact on QoL. Its diagnosis is often overlooked. None of TEP, age at onset nor time to onset of symptoms is a useful predictor of impaired QoL in individual patients.

Adolescent↗
PubMed2004

Rosacea fulminans in pregnancy.

Rosacea fulminans is a rare condition with a female preponderance and unknown aetiology, characterized by the abrupt onset of papules, pustules and erythema affecting the face. Corticosteroids and isotretinoin are regarded as the two main therapeutic agents. We report a case associated with pregnancy, and discuss the therapeutic implications. This is the first published report of rosacea fulminans in pregnancy complicated by stillbirth.

Adult↗
PubMed2004

Variations in registration of skin cancer in the United Kingdom.

Cancer registries are used to compare incidences between regions, plan for service provision, and to assess the impact of health interventions. Significant variation in data capture for skin cancers and reporting of results was evident between regional cancer registries in the UK when assessed in 1991. Using a postal questionnaire we sought to document methods of recording skin cancer incidence in the UK in 2000, and to assess if practice has changed from 1991. All UK cancer registries were asked for details of their method of skin cancer case registration and latest available incidence figures. Methodology was assessed against recently implemented national standards. All registries responded to the survey. Sources of data were more uniform than was the case 9 years ago. All registries except one attained national standards for basal cell carcinoma data collection, but only half of the registries attained standards for squamous cell carcinoma. Ten of the 12 correctly recorded numbers of malignant melanomas, but three still failed to record the Breslow thickness or Clarke's level. Wide variation is evident in the recorded incidences for each of the malignancies, and the efficiency at which figures are made available. Thus, although there has been improvement since 1991, variability still exists between UK registries in methods of data capture, the data recorded, and efficiency of data processing in skin cancer registration.

Basal Cell Carcinoma↗
PubMed2004

Phototherapy and PUVA photochemotherapy in children.

The use of phototherapy and photochemotherapy in children has been limited due to concerns over their long-term carcinogenic potential. Furthermore, the method of administration is disconcerting to some children, particularly as phototherapy treatment units are seldom rendered 'child-friendly'. Despite these reservations, ultra-violet therapies can be useful treatment options for children with selected dermatological conditions provided they are used under carefully controlled conditions.

Child↗
PubMed2003

The Children's Dermatology Life Quality Index: validation of the cartoon version.

BACKGROUND: In 1995 the Children's Dermatology Life Quality Index (CDLQI) was developed as a tool to allow quality of life assessment of children with skin conditions. This initial questionnaire was in a written format. OBJECTIVES: Using the same validated questions, a full-colour cartoon version has been developed. The aim of this study was to validate this against the initial written questionnaire in a three-part study. METHODS: The first part of the study piloted the use of both versions in an outpatient setting. One hundred and one children completed both versions of the CDLQI in a random order. A further 66 children completed the cartoon CDLQI in outpatients, and subsequently completed the cartoon version on the same day at home, which was returned by post. The scores were compared. In the second part, in more controlled conditions to eliminate parental and investigator bias, 107 children with current dermatological problems were administered both versions of the CDLQI in a random order. The scores were analysed, and time to complete each version, and the child and parental preferences, were recorded. The third part assessed compliance by asking 546 children recently reviewed in dermatology clinics to return a single completed postal CDLQI. Half of the children were given the text, and half the cartoon version. RESULTS: The median age of participating children was 11 years. There was no significant difference in scores between the two versions in both parts 1 and 2, but the cartoon version was completed faster (median 90 s) than the written version (median 120 s) (P < 0.0001). Both children and their parents significantly preferred the cartoon version and found it easier to use. Forty-six per cent of the postal CDLQI questionnaires were returned; there was no difference in compliance between the two versions. CONCLUSIONS: The cartoon CDLQI is equivalent to the previously validated written CDLQI version, but is faster and easier for children to use, and is preferred by both children and parents.

Adolescent↗
PubMed2002

Cosmetic camouflage advice improves quality of life.

BACKGROUND: The subjective benefit of attendance at cosmetic clinics has not previously been reported. OBJECTIVES: To assess the effect on perceived quality of life (QoL) of cosmetic camouflage advice. METHODS: In a three-centre study, 135 individuals were invited to complete a dermatology-specific QoL measure, the Dermatology Life Quality Index (DLQI), before and 1 month after their first visit to a cosmetic camouflage clinic. RESULTS: Eighty-two completed DLQI questionnaires were returned before the camouflage clinic appointment, and 56 corresponding questionnaires were returned 1 month after. The mean age of responders was 50 years, and the mean duration of their skin conditions was 15 years. The main conditions seen were pigmentary disorders (29%), scars (22%) and vascular disorders (13%). There was a significant difference in mean DLQI scores before and after the clinic visit (9.1 vs. 5.8, P = 0.0001). CONCLUSIONS: When assessed at 1 month, attendance at a cosmetic camouflage clinic appears to improve QoL significantly.

Activities of Daily Living↗
PubMed2002

Erythrocyte thiopurine methyl transferase assessment prior to azathioprine use in the UK.

BACKGROUND: Individuals with low activity of a key metabolic enzyme, thiopurine methyl transferase (TPMT), are more susceptible to azathioprine-induced myelosuppression. AIM: To determine the pattern of use of TPMT activity estimation, with respect to azathioprine use, by medical practitioners in the UK. DESIGN: Retrospective analysis of assay use. METHODS: We analysed all test results (n=3291), and patient and practitioner details, from inception of TPMT assay in 1990 to the end of December 2000, held at the Purine Research Laboratory, Guy's Hospital, London. Patient details were anonymized. Repeat analyses and requests from outside the UK were excluded. RESULTS: The male:female ratio was approximately equal and the mean age was 46.6 (range 0.5-97) years. Thirteen different medical specialities requested assays; Dermatology and Gastroenterology were the most frequent users, together accounting for 86% of requests. The numbers of centres requesting the assay varied widely both within and between different specialities. Some 80% of individuals had normal TPMT activity, 9% enzymic activity above normal, and 10% low activity. Fifteen had no detectable enzymic activity: 0.45% (1:220) of the study population. DISCUSSION: This incidence of undetectable enzyme activity is significantly higher than the previously reported level of 1:300 derived from smaller studies, and makes the economics of screening more attractive.

Adolescent↗
PubMed2001

Audit of a melanoma screening day in the U.K.: clinical results, participant satisfaction and perceived value.

BACKGROUND: The incidence of cutaneous malignant melanoma (MM) has risen significantly over the last 50 years in caucasian populations. Mortality is related to the Breslow thickness of the lesion, and early detection followed by complete surgical excision is crucial to reducing this. Skin screening events have been advocated as a means of detecting greater numbers of MMs in the earlier and thinner stages. OBJECTIVES: To assess the feasibility and effectiveness of this approach in the U.K. METHODS: The Department of Dermatology, Singleton Hospital, Swansea, U.K. offered a 1-day melanoma screening event in summer 1998 at which 832 consecutive individuals were seen. A postal questionnaire 1 year after the event audited participant perceived value and satisfaction. RESULTS: Three MMs were identified (yield 1 : 277), all < 0.75 mm in thickness. Despite high participant satisfaction and perceived value, the pick-up rate of malignancy was significantly lower than at rapid access pigmented lesion clinics. CONCLUSIONS: In a country such as the U.K., with comprehensive health coverage and a low incidence of MM, triage for melanoma and referral to specialists by general practitioners may be more cost and time effective.

Adolescent↗
PubMed2001

Aquagenic pruritus responding to intermittent photochemotherapy.

Aquagenic pruritus is a rare but distinct entity in which intense itching develops after contact with water, in the absence of cutaneous signs or underlying disorders that could explain the symptoms. The aetiology is currently unknown, and treatment difficult, with a poor response to antihistamines. Psoralen-ultra violet A (PUVA) photochemotherapy can be effective, but frequent maintenance therapy may be required to prevent relapse of symptoms. We present a patient with typical aquagenic pruritus who responded well to PUVA, and who has remained well controlled on infrequent maintenance.

Administration, Oral↗
PubMed2001

Malignant melanoma in South Wales: changing trends in presentation (1986-98).

In 1990 we reported the trends in presentation of malignant melanoma (MM) from 1986 to 1988 in a defined population in South Wales. Ten years after this initial study we reassessed the incidence of invasive MM in this defined population over a similar 3 year period. Using the local skin cancer registry we identified 186 cases of MM representing a 74% increase. Age standardization to the world population gives an overall incidence of 11.8 per 100 000 population per annum. This is the highest published standard incidence of MM to date from the UK and certainly one of the highest in Europe. We also have observed a continuing trend for diagnosis of thinner lesions with a better prognosis. Information about mortality for the whole of Wales over the corresponding period is also presented.

Adolescent↗
PubMed2001

Nodular presentation of eosinophilic cellulitis (Wells' syndrome).

Eosinophilic cellulitis is a rare condition of unknown aetiology. The classical presentation is of a tender or mildly pruritic cellulitis-like eruption, that has typical histology characterized by tissue eosinophilia, oedema and "flame" figures. Other reported clinical presentations include papular and nodular eruptions. It may be recurrent, and preceded at a variable time by a pruritic papular eruption. We describe a patient with the rare nodular variant of eosinophilic cellulitis affecting the palms of the hands, which occurred 2 years after a nonspecific pruritic papular eruption, without an obvious precipitant and in the absence of the more typical cellulitis-like plaques.

Adult↗