A multi-centre audit on genital lichen sclerosus in the North West of England.

BACKGROUND: Guidelines for the management of genital Lichen sclerosus (LS) have recently been updated. OBJECTIVE: To look at the audit points suggested by the updated guidelines: performance of biopsies in active LS not responding to treatment; clear follow-up arrangements for patients with active disease; patient awareness of need to report suspicious lesions; and use of an appropriate topical steroid regieme. METHOD: Patients with a diagnosis of genital LS seen over the preceding 12 months were identified from eight hospital Trusts. In this study, 194 patients participated, 178 females and 16 males. RESULTS: The diagnosis was purely clinical in 62 patients - the remainder required biopsies. The commonest reason for performing a biopsy was to clarify the diagnosis (116), followed by to rule out malignancy (11). The majority (98%) were offered follow-up after the initial consultation and only 19 patients were discharged to primary care. In this study, 37% patients had documented evidence that a patient information leaflet had been given. 112 were treated with the clobetasol propionate 0.05% regieme quoted in the guideline. CONCLUSION: We conclude biopsies should be done as indicated in the guideline and the reason for biopsy documented. Discharge may be possible at 6 months for stable uncomplicated disease, although this may prove difficult if adequate follow-up arrangements are not available in the community. We advocate that all patients should receive a patient information leaflet and must be made aware of the increased risk of SCC. Topical corticosteroid treatment should be simplied to the regieme documented in the guidelines unless contraindicated.

Cutaneous manifestations of the malignant carcinoid syndrome.

BACKGROUND: The incidence of carcinoid tumours is approximately 1.5 per 100 000 of the population. The malignant carcinoid syndrome, which is caused by circulating neuroendocrine mediators produced by the tumour, occurs in less than 10% of patients. Cutaneous involvement, although recognized in this rare syndrome, has rarely been reported. OBJECTIVES: To examine a series of patients with the malignant carcinoid syndrome, to establish the prevalence and types of cutaneous involvement and to assess whether these could be used as indicators of disease activity, progression and prognosis. METHODS: Patients with the malignant carcinoid syndrome who attended a multidisciplinary clinic for neuroendocrine tumours over a 23-month period between February 2001 and December 2002 were invited to participate in the study. This involved completion of a standard history proforma and a detailed cutaneous examination with findings recorded by clinical photography. RESULTS: Twenty-five patients were enrolled. All but one had experienced flushing, three had rosacea, five had clinical features of pellagra and two had scleroderma. Flushing was generally an early manifestation of the syndrome, whereas both the pellagra and scleroderma tended to occur in more advanced disease. CONCLUSIONS: This descriptive case series indicates that cutaneous features are not uncommon in the syndrome. They are readily diagnosed on clinical examination, and may be useful indicators of disease activity and prognosis.

Localized morphoea preceded by a pigmented purpuric dermatosis.

Localized morphoea uncommonly occurs in a linear distribution and may present following trauma, although most cases are idiopathic. Pigmented purpuric dermatoses such as lichen aureus may also rarely occur in a linear distribution and have been associated with trauma. A middle-aged man is described who initially presented with lesions typical of lichen aureus in a linear distribution at a site exposed to chronic low-grade trauma. This eruption was transient and clinically underwent spontaneous complete resolution. Several months later he developed localized morphoea in an identical distribution. To our knowledge this is the first reported case of a pigmented purpuric dermatosis, presumably precipitated by trauma, evolving into linear morphoea.

Papular acantholytic dyskeratosis of the vulva.

We describe two patients with unusual asymptomatic, papular lesions on the vulva, clinically resembling lichen planus, the histology of which revealed unexpected findings of suprabasilar clefting, acantholysis and dyskeratotic cells giving rise to corps ronds and grains together with hyperkeratosis and parakeratosis, features originally associated with a diagnosis of Darier's disease. Focal acantholytic dyskeratosis has been described in a wide variety of inflammatory and neoplastic processes including those involving mucous membranes and has been attributed various diagnostic labels. We feel that the findings in our patients are consistent with a diagnosis of papular acantholytic dyskeratosis of the vulva, a rare condition, which was first described in 1984.

Upper urinary tract abnormalities in multiple sclerosis patients with urinary symptoms.

OBJECTIVE: To determine the prevalence of upper urinary tract complications in multiple sclerosis (MS) patients with urinary symptoms, and to determine if an association exists between degree of physical impairment and upper urinary tract complications. DESIGN AND SETTING: A cohort study of MS patients seeking treatment at a freestanding, university-affiliated rehabilitation hospital. PARTICIPANTS: A referred sample of 48 patients with MS, exacerbation-free for 6 months with symptoms of neurogenic bladder dysfunction. For each patient, demographic data, disease characteristics, and urologic history was obtained. Using the Kurtzke Expanded Disability Status Scale (EDSS), participants were divided into a control (EDSS < 7) and study (EDSS >/= 7) group. INTERVENTION: Ultrasound examination of the upper urinary tract. MAIN OUTCOME MEASURE: Significant MS-related abnormalities of the upper respiratory tract. RESULTS: Ten of 48 patients (21%) had significant MS-related upper urinary tract abnormalities, which were evenly distributed between control and study groups. In the more disabled study group, abnormalities were associated with the symptom of urinary hesitancy (p < .05) and form of bladder management (p < .05). CONCLUSIONS: Routine screening for upper urinary tract complications appears indicated in a select group of MS patients with urinary symptoms.