Christine I. Harrington

Interleukin 1 receptor antagonist gene polymorphism association with lichen sclerosus

Cytokines play key roles in immune responses, inflammation and fibrosis. The balance between levels of cytokines, their receptors and specific inhibitors controls inflammatory reactions in tissues. The pathogenesis of lichen sclerosus is unknown but probably involves cytokine mediators such as interleukin 1 (IL-1) and interleukin 1 receptor antagonist (IL-1ra). The IL-1ra is a competitive inhibitor of IL-1α and IL-1β, and therefore is a powerful endogenous anti-inflammatory molecule. The gene encoding IL-1ra (designated IL-1RN) has a variable number tandem repeat polymorphism in intron 2. There are five alleles of the gene corresponding to 2, 3, 4, 5 and 6 repeats of an 86-bp sequence. We have determined allele frequencies in a control population and a group of 78 patients with lichen sclerosus. The frequency of one of the alleles is related to increasing disease severity. Thus, IL-1RN may be a candidate gene or severity factor for lichen sclerosus or may possibly be a linked marker to another, as yet undefined, gene.

Skin cancer in renal transplant recipients is associated with increased concentrations of 6-thioguanine nucleotide in red blood cells

Of 108 renal transplant recipients (53 men and 55 women) treated with azathioprine (0.8‐2.9 mg/kg/day) and prednisolone (10 mg daily), 10 men had actinic keratoses, and five of these had squamous cell carcinoma, on light‐exposed areas of skin. The time from transplantation to diagnosis of these skin lesions varied from 1.2 to 9.0 (mean 5.1) years.

Occupational argyria; light and electron microscopic studies and X-ray microanalysis.

Microscopic studies have been performed on skin biopsies from five patients with occupational argyria.

Psychological difficulties within a group of patients with vulvodynia

This study reviews and reports on some of the psychological difficulties seen in a group of women with vulval pain (vulvodynia). The investigation involved 164 women (82 suffering with Vulvodynia and 82 women in a control group with general dermatology conditions) to establish the prevalence of psychological difficulties using validated questionnaires (SCL-90R and the IBQ) and reports on the possible effect these may have on sexual and relationship function and satisfaction. The level of psychological difficulties revealed significantly higher levels of psychological distress in the vulvodynia group within the domains of somatisation, obsessive-compulsive, depression, anxiety & phobic symptoms as well as with interpersonal sensitivity hostility and paranoia.

The association between lichen sclerosus et atrophicus and HLA-B40

Fifty Caucasian females with lichen sclerosus et atrophicus were investigated to determine their HLA types. HLA‐B40 was the only antigen found to be significantly more common in these patients when compared with a control population.

An investigation into the incidence of auto-immune disorders in patients with localized morphoea

Patients with localized morphoea have an increased incidence of auto‐immune disorders, but this is not related to the age of onset, duration of disease nor the site or number of lesions. The patients have an increased incidence of tissue antibodies and these are more common when more than one lesion is present. The relatives of the patients have also been found to have an increased incidence of auto‐immune disorders.

Coexistence of bullous pemphigoid and pemphigus foliaceus

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.

Sexual difficulties within a group of patients with vulvodynia

Dyspareunia, caused by vulvodynia, has, like other sexual problems, evolved from being conceptualized as a psychological problem through a period when it became viewed by the medical world as a physical problem. It is now seen as being a combination of psychological and physical factors. However, no research to our knowledge has explored in depth the level of sexual and relationship difficulties of this population. This investigation involved 172 women (85 suffering with vulvodynia and 87 women in a control group with general dermatology conditions) to establish the prevalence of relationship and sexual difficulties, using validated questionnaires, a newly devised symptom checklist and a structured interview. The results demonstrated increased levels of sexual and relationship difficulties within the vulvodynia group that need further exploration.

Herpes gestationis: immunopathological and ultrastructural studies*

Eleven patients with the clinical picture of herpes gestationis were investigated. Biopsies were taken from involved and uninvolved areas of skin and the immunopathological and microscopic changes studied. Direct immunofluorescence showed a deposition of C3 and/or IgG at the basement membrane zone in the involved skin of nine patients and the uninvolved skin of five. Immuno‐electron microscopy using a multistep peroxidase antiperoxidase method revealed the in vivo deposition of IgG at the basal plasma cell membrane that extended into the lamina lucida. Light microscopy of urticarial and vesicular lesions showed a marked oedema of the papillary dermis with an inflammatory cell infiltrate that was mainly perivascular. There was spongiosis of the epidermis with oedema and necrosis of the basal cells and in several specimens sub‐epidermal clefts with bulla formation. Electron microscopy confirmed the marked degenerative and necrotic changes of the basal cells in the involved areas of skin.

Esophageal Lichen Planus Presenting with Dysphagia

A 79-year-old woman presented with a 7-year history of dysphagia and odynophagia to solids and to a lesser extent liquids. Pain on swallowing radiated to the right jaw. She also complained of an unpleasant taste, and of a 12 kg weight loss over the preceeding 6 months. There was a long history of pruritus vulvae. Hyperthyroidism had been diagnosed 10 years previously and was treated initially with carbimazole and thiouracil. Because of poor compliance, however, radioiodine therapy was instituted 2 years later. There was no history of dyspepsia. At presentation she was clinically and biochemically euthyroid. At onset, the dysphagia was clinically investigated. Indirect laryngoscopy and barium swallow at that time revealed no abnormality. Re-examination 6 years later revealed a white patch on the floor of the mouth and direct laryngoscopy and pharyngo-esophagoscopy demonstrated a raised lacey pattern of striae on the posterior pharyngeal wall and other multiple raised white areas in the upper esophagus up to 20 cm from the incisors. An upper esophageal lesion was biopsied. Histology revealed a mildy thickened squamous epithelium but no significant atypia. In the upper dermis there was a lymphohistiocytic inflammatory infiltrate most marked at the epithelial-submucosal interface (Fig. 1), Several colloid bodies were present. These findings confirmed the clinical diagnosis of lichen planus. There were no cutaneous signs of lichen planus, although there was a bluish discolouration of the labia majora. Treatment with oral prednisolone and a steroid mouth wash produced considerable improvement in the dysphagia. She is currently controlled on 12,5 mg daily of prednisolone, eating normally, and gaining weight.