Epidermolysis bullosa is the name given to a group of rare, genetically determined diseases characterized by cutaneous blistering and subsequent scarring. It presents a challenge to the anaesthetist managing patients who often require multiple operations. This review article includes a retrospective audit of cases treated at St Thomas' Hospital between 1993 and 1998. We studied the case notes and anaesthetic records of patients with dystrophic epidermolysis bullosa requiring anaesthesia and recorded the anaesthetic management and complications that occurred. Methods Patients From January 1993 to January 1998, 51 patients with dystrophic epidermolysis bullosa had a total of 306 anaesthetics. The ratio of males to females was 3:2, with an age range of 1-51 yr; 44% were from the UK, while the remainder were from Italy, Serbia, Croatia, Greece and Portugal. Only 12% of the foreign patients spoke English.

Epidermolysis bullosa (EB) refers to a group of hereditary mechano-bullous conditions, many of which are associated with chronic scarring. Several forms of the disease have been reported in association with cutaneous malignancy. We present a series of 10 EB patients (eight generalised recessive dystrophic EB, one dominant dystrophic EB, one non-lethal junctional EB) aged 24-55 years with a total of 29 squamous cell carcinomas (SCC). Three patients died from metastatic disease associated with invasive, poorly differentiated SCC. Six cases had multiple primary SCC, including three patients with simultaneous multifocal disease. Twenty-eight of the 29 SCC arose on the limbs. Histology revealed that most of the SCC were well or moderately differentiated (22/29). Unusual histological findings included two verrucous SCC, as well as a spindle cell (angiosarcoma-like) SCC. Most of the SCC developed in areas of chronic non-healing ulceration (10/29) or longstanding hyperkeratotic crusting (14/29). The dermis around or beneath the carcinomas was densely scarred, more so than in non-malignant areas. In some cases it was difficult to distinguish the clinical appearances of certain areas of chronic ulceration, scarring, and crusting typical of dystrophic EB from many of the SCC. This study underlines the need for constant vigilance for the development of carcinomas in this group of patients, the occasional diagnostic difficulty, and the potential for metastasis.

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SummaryThe case notes and anaesthetic charts of 44 patients with dystrophic epidermolysis bullosa were reviewed retrospectively. A total qf 390 general anaesthetics were performed for 469 Key wordsComplications; dystrophic epidermolysis bullosa. Anaesthesia.Dystrophic epidermolysis bullosa (DEB) is a rare, hereditary mucocutaneous disorder whose primary feature is the formation of blisters following minor shearing trauma to the skin. Treatment of DEB involves surgery which has a number of challenging implications for the anaesthetist.In the last 30 years there have been 17 case reports of the anaesthetic management of epidermolysis bullosa (EB) but only two series (both retrospective). One looked solely at airway management during anaesthesia [I] and the other was a review of the anaesthetic management of eight patients [2].St. Thomas' Hospital (which includes the Institute of Dermatology) has been a national referral centre for EB for 15 years and so has built up a large experience of the surgical and anaesthetic management. Patients and methodsThe case notes of patients with DEB who had surgery at St. Thomas' or the Lister Hospital, London SWl , were scrutinised to determine their anaesthetic management and to record any complications that had arisen. Results PatientsFrom 1982 to January 1992, 44 patients (26 female, 18 male) with DEB had a total of 390 anaesthetics given by 20 anaesthetists. Forty patients were diagnosed as having autosomal recessive DEB and four patients had either dominant DEB or localised recessive DEB.The age range of the patients at the time of operation is shown in Figure 1. The youngest patient to have a general anaesthetic was one year old and the oldest 48 years. Surgical procedures

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Histological interpretation of frozen sections made during Mohs' micrographic surgery may be difficult, depending on the morphological and staining characteristics of the tumour and on the nature of the associated inflammatory infiltrate. We have employed an adaptation of micrographic surgery in which horizontal, formalin-fixed, paraffin-embedded sections were used to improve histological assessment in the excision of 18 non-melanoma skin tumours in which frozen sections had been or were likely to be unsatisfactory. We describe our experience of this method in the management of squamous cell carcinomas (11), extramammary Paget's disease (two), microcystic adnexal cell carcinomas (two), dermatofibrosarcoma protuberans (two), and primary cutaneous neuroendocrine carcinoma (Merkel cell carcinoma) (one). The use of horizontal paraffin-embedded sections lengthens the duration of the procedure but facilitates accurate assessment of histological sections in selected tumours.

A prospective study of children with large congenital melanocytic naevi (CMNs) is in progress. Its aims are to observe the natural history and inheritance of CMNs, to record associated pathologies, and eventually to evaluate the effects of treatment. Information is obtained by patient-completed questionnaires and clinical assessment. One hundred and thirty-three children have been followed for a total of 452 patient years. The female/male ratio is 3:2. The characteristics, size and distribution at birth of the CMNs in this group of patients, and their development and associated abnormalities, are documented. After 5 years, affected children were compared with a matched cohort of normal children. Although the prevalence of neurological and developmental defects was higher in affected children, no malignant melanomas have been reported to date. The families of affected children had higher numbers of naevi and café-au-lait spots than those of normal children, suggesting a possible hereditary component. The mothers of affected children also reported an increased incidence of influenza-like illness and of severe nausea and vomiting in pregnancy. This study will continue for a further 4 years, in the first instance, after which we will seek relationships between characteristics of the naevi and outcome.