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Biomedical subjects

K E Harman

Publications and source records attributed to K E Harman.

At least 19 recordsLinked to original sources
PubMed2006

Evidence of an association between desmoglein 3 haplotypes and pemphigus vulgaris.

BACKGROUND: Pemphigus vulgaris (PV, OMIM 169610) is a severe blistering disorder of the skin and mucous membranes, caused by the production of autoantibodies directed against the epithelial adhesive protein desmoglein 3. Although an association between PV and HLA class II alleles has been established, the genetic factors predisposing to the disease remain poorly understood, the rarity of PV hampering the recruitment of substantial patient cohorts. OBJECTIVES: To investigate DSG3 as a candidate PV susceptibility gene. METHODS: We examined five DSG3 single nucleotide polymorphisms (rs8085532, rs3911655, rs3848485, rs3794925 and rs1466379) in two case-control datasets respectively originating from the U.K. (62 PV patients, 154 controls) and northern India (28 patients, 98 controls). RESULTS: In the U.K. sample, we observed a significant association between PV and the DSG3*TCCTC haplotype (Fisher's exact test P = 0.002). A related haplotype (DSG3*TCCCC) was associated with PV in the Indian dataset (P = 0.002). We also found that all British and Indian patients bearing DSG3 risk haplotypes carried at least one copy of a PV-associated HLA allele. CONCLUSIONS: These results suggest that genetic variation of DSG3 may be an additive risk factor predisposing to PV and warrant further investigations of this gene.

Case-Control Studies↗
PubMed2006

Acquired haemophilia heralded by bleeding into the oral mucosa in a patient with bullous pemphigoid, rheumatoid arthritis, and vitiligo.

Acquired haemophilia is rare and potentially fatal, with a mortality of 20% if left untreated. There is a strong association with other autoimmune diseases. This report describes a patient with rheumatoid arthritis, vitiligo, and bullous pemphigoid where the diagnosis of acquired haemophilia was made after an extensive bleed into a bullous lesion in the buccal mucosa. This case highlights some of the potential complications of acquired haemophilia and its treatment.

Aged↗
PubMed2004

Trends in the presentation of cutaneous malignant melanoma over three decades at King's College Hospital, London.

The aim of this study was to examine trends in the presentation of cutaneous malignant melanoma at King's College Hospital (KCH) over the last three decades (1970-2000). KCH was one of seven centres that participated in the 1987 Cancer Research Campaign (CRC) publicity campaign aimed at promoting earlier self-recognition of melanoma. Data included patient age at presentation, sex, tumour site, Breslow thickness and histological subtype. The late 1980s saw a threefold increase in the annual number of melanomas and an eightfold increase in thin melanomas compared to the 1970s. The increase occurred in both sexes and was particularly marked after the CRC campaign but numbers had already begun to increase prior to this. The increase has predominantly been thin (Breslow < 1.5 mm) tumours of the superficial spreading variety with a resultant fall in mean Breslow thickness. There has been a decline in the annual number of melanomas since the peak in 1992 which is not explained by increased proportion of in situ tumours. The CRC campaign may have contributed to the documented increase in thin tumours but this trend had begun prior to 1987 suggesting factors other than public awareness and earlier presentation are important. It is encouraging that the number of melanomas has declined over the last 5 years at KCH but it is yet to be seen whether this reflects a real decrease in the incidence of melanoma.

Abdominal Neoplasms↗
PubMed2003

An evaluation of the usefulness of mycophenolate mofetil in pemphigus.

BACKGROUND: Pemphigus is a group of autoimmune blistering diseases of the skin and/or mucous membranes requiring management with immunosuppressive therapy. The optimal therapeutic regimen would rapidly induce remission and maintain effectiveness with minimal adverse effects in the long term. OBJECTIVES: The present study describes our experience of the addition of mycophenolate mofetil (MMF) to prednisolone in the management of severe, refractory pemphigus. METHODS: Patients with active, refractory pemphigus were treated with MMF. Our series included 12 cases of pemphigus vulgaris, four cases of pemphigus foliaceous and one case of paraneoplastic pemphigus. All patients were monitored to assess disease control and mycophenolate toxicity. RESULTS: Of the 17 cases, MMF has been of benefit to 12. MMF was well tolerated and there were no treatment withdrawals because of safety concerns. CONCLUSIONS: We found that MMF permitted a reduction in prednisolone dosage without disease relapse.

Adult↗
PubMed2003

Persistent anticonvulsant hypersensitivity syndrome responding to ciclosporin.

The drug hypersensitivity syndrome is a severe, multisystem reaction that typically develops within 2 months of starting a drug. We describe a case which has been unusually persistent, requiring prolonged treatment with systemic corticosteroids and after 1 year, ciclosporin. To the best of our knowledge, the use of ciclosporin for the treatment of the drug hypersensitivity syndrome has not been described previously.

Adult↗
PubMed2003

Guidelines for the management of pemphigus vulgaris.

These guidelines for management of pemphigus vulgaris have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.

Dermatologic Agents↗
PubMed2002

New laboratory techniques for the assessment of acquired immunobullous disorders.

This review aims to summarize investigations that are used in the assessment of patients with suspected immunobullous disorders. Emphasis is placed upon new adaptations of existing techniques and the advent of recombinant technology, which has enabled the production of synthetic antigens. The use of recombinant antigens has provided the latest generation of diagnostic techniques that are likely to make a major impact on routine diagnosis of immunobullous disorders.

Autoantigens↗
PubMed2002

Psoriasis bullosa acquisita.

We report a 51-year-old man with a 20-year history of chronic plaque psoriasis who developed an autoimmune subepidermal blistering eruption that had clinical features of bullous pemphigoid, erythema multiforme and epidermolysis bullosa acquisita. Investigations revealed a 1 : 400 titre circulating and in vivo bound IgG autoantibody that mapped to the dermal side of 1 m NaCl-split skin and localized to the lower lamina lucida/upper lamina densa on immunogold electron microscopy. Immunoblotting, using dermal extracts, showed serum binding to antigens of approximately 200- and approximately 260 kDa. Indirect immunofluorescence microscopy, using the patient's serum on archival skin sections taken from selected individuals with different forms of inherited epidermolysis bullosa as substrate, showed normal basement membrane labelling on all samples apart from recessive dystrophic epidermolysis bullosa skin (with inherent mutations in the type VII collagen gene): in these cases there was a complete absence of immunostaining. Clinically, the patient responded rapidly to combination treatment with intravenous immunoglobulin and oral corticosteroids, dapsone and mycophenolate mofetil. Autoimmune subepidermal blistering has been reported in other patients with psoriasis, although no specific target antigen has ever been determined. Our study provides preliminary evidence that, for this patient at least, the autoantibody may be targeted against a skin component closely associated with type VII collagen (the epidermolysis bullosa acquisita antigen). Therefore, we propose the term 'psoriasis bullosa acquisita' for this and possibly other patients with similar skin eruptions.

Autoimmune Diseases↗