Pigmentary anomaly caused by mosaic 3q22.2q29 duplication.

A 39-year-old woman sought advice regarding potential risks to her offspring due to previous possible diagnosis of incontinentia pigmenti. She had linear hyperpigmentation along the lines of Blaschko affecting the upper and lower limbs, and skin-coloured papules on the left palm. Ophthalmoscopy revealed hypopigmented spots in the macular region of the retina in each eye due to focal areas of depigmentation of the retinal pigment epithelium. An array comparative genomic hybridization on DNA extracted from a skin biopsy revealed a 63.63-Mb duplication, arr[GRCh37] 3q22.2q29(134212001_197837069)x3, on the long arm of chromosome 3. This case is an example of genetic mosaicism resulting from a de novo genetic defect arising at some point in embryonic development. Click here for the corresponding questions to this CME article.

Acantholytic dyskeratosis of the vulva presenting with clinical features of vulval lichen sclerosus-A possible rare collision entity.

We present a challenging case of chronic, erosive, scarring dermatosis of the vulva with clinical features of long standing lichen sclerosus (LS), namely pallor and loss of vulval architecture, but with histopathology consistently showing features of an acantholytic process. The history and clinical features of this case do not resemble other acantholytic conditions such as pemphigus vulgaris, Hailey-Hailey disease, Darier disease, or the entities described as acantholytic dermatoses affecting the vulva. As far as we are aware, the combination of the clinical features and histopathologic findings in our case do not fit with any previously described condition and we propose that this is a rare entity of a collision of LS and an erosive acantholytic process occurring together.

Time in motion--testing efficiency in the dermatology procedure setting.

BACKGROUND: Time-in-motion studies are validated means of determining efficiency and identifying sources of delay in a complex working environment. This analysis technique was applied to the dermatological surgical services provided by a university hospital department of dermatology. OBJECTIVE: To identify any sources of low efficiency in dermatological surgical service provision and to make recommendations for increasing efficiency. METHOD: Three types of dermatology surgical sessions were observed (consultant-led procedure list, nurse-led procedure list, and dermatology day-surgery list). Activities of staff were noted every 2 minutes throughout the sessions, and the data were compiled for processing in Microsoft Excel. RESULTS: The results show that treatment of dermatology patients with day surgery was less efficient and cost effective than the other settings. Consultant-led procedure lists were the most efficient. Nurse-led sessions were less expensive but also less efficient than the consultant-led lists. Paperwork and generic communication were identified as major constituents of non-operating time. CONCLUSIONS: The most efficient setting for dermatological procedures is the consultant-led procedure list. Paperwork and generic communication are identified as sources of service delay. Suggestions are made regarding possible solutions to these problems. We demonstrate the advantages of broad, objective appraisal of practice as opposed to numerical target-driven approaches that fail to address specific aspects of efficiency.

Successful treatment of high-grade vulval intra-epithelial neoplasia with imiquimod 5% in a renal transplant recipient.

Organ transplant recipients are at a higher risk of pre-malignant human papillomavirus-associated lesions due to immunosuppression. The efficacy of immunomodulants such as imiquimod 5% is not yet fully evaluated in this population. We describe a case of vulval intra-epithelial neoplasia in a renal transplant recipient which was successfully treated with topical imiquimod.

Vulvodynia.

CONTEXT: Vulvodynia is a term used to describe chronic burning and/or pain in the vulva without objective physical findings to explain the symptoms. The terminology and classification of vulvodynia continue to evolve, and much remains to be understood about the prevalence, pathogenesis, natural history, and management of this distressing condition. STARTING POINT: James Aikens and colleagues showed that chronic vulval pain (vulvodynia or vulvar dysaesthesia) is associated with worse depressive symptoms (Am J Obstet Gynecol 2003; 189: 462-66). However, the increased scores for depression in this case-control study were attributed to sexual disinterest and experience of chronic pain rather than to features of depressive disorder. These results lend weight to the increasing need for better understanding of the pathogenesis of vulval pain and how to manage it appropriately. WHERE NEXT? The aetiology of vulvodynia and effectiveness of treatments need further study. Appraising the available literature, we have formulated a useful approach to patients with chronic vulval pain. There is a pressing need for further case-control studies of potential causes of vulvodynia and for randomised trials of interventions.

Erosive lichen planus of the vulva and vagina.

BACKGROUND: Erosive lichen planus causes erosion of the vulva and vagina and characteristic oral lesions. Dyspareunia is usual, and vaginal stenosis may occur. This report highlights the clinical features and the response to medical therapy. CASES: We report the case histories of three women who presented to the Vulvovaginal Disorders Clinic of the University of Iowa with long histories of dyspareunia and advanced vaginal scarring. In each case, the clinical diagnosis of erosive lichen planus was obvious but had not been made previously. All three women have responded well to topical treatment with tacrolimus 0.1% ointment. CONCLUSION: Erosive lichen planus should be suspected in a case of vaginal erosion or narrowing. Surgical management is inappropriate when the mucosa is eroded. Inspection of the mouth may confirm the diagnosis.

Dicaprylyl maleate--an emerging cosmetic allergen.

Dicaprylyl maleate (DCM) has been reported rarely as a cause of allergic contact dermatitis. The objectives of this study were to identify patients from multiple centres with allergy to DCM in cosmetic products confirmed by patch testing and, in addition, to investigate the effect of testing with aged DCM. This is an international multicentre study of 22 patients with 26 reactions to products containing DCM. Patch testing was carried out to ingredients including DCM obtained from the manufacturer. Further testing was carried out with deliberately aged DCM in a sample of patients. 22 patients had clinical and positive patch test reactions at 4 days to a total of 26 cosmetic products containing DCM. 5 patients did not react to DCM prepared by the manufacturer from concurrent factory stock but did have positive reactions to a deliberately aged batch of DCM. DCM is an emerging cosmetic allergen. Testing with aged material yields a greater number of positive results. Co-operation between cosmetics manufacturers and clinicians is important in the identification of new allergens.

Ulcerative vulvitis in atypical Reiter's syndrome.

We report a case of atypical Reiter's syndrome occurring in a female patient who had severe, ulcerative vulvar disease develop in association with conjunctivitis, low back pain, stomatitis, and psoriasiform skin lesions. Vulvar lesions have rarely been described in Reiter's syndrome and are not well characterized.