Search

Summary Background Solid organ transplant recipients are at increased risk of skin cancer. Melanoma is less common than nonmelanoma skin cancer (NMSC) although the relative proportion of melanoma among skin cancers has been shown to be higher in paediatric than adult recipients. Multiple melanocytic naevi and/or atypical naevi may be a risk factor for the development of melanoma. The relationship between naevus counts and phenotypic characteristics, disease-related variables and sun exposure has not been explored in paediatric transplant patients. Objectives To determine the prevalence of premalignant and malignant skin lesions and to identify known risk factors associated with benign and atypical melanocytic naevi in a U.K. paediatric transplant population. Methods Paediatric (≤ 19 years) renal and liver transplant patients, who were 5 or more years post-transplantation, were reviewed over 12 months. Lifetime history of sun exposure, episodes of sunburn, sunny holidays, sunscreen use, sun bed use, demographic and transplantation details were collected using interview, questionnaire and case note review. A skin examination was performed for regional counts of malignant lesions, benign and atypical naevi. Results Ninety-eight patients (82 liver, 13 renal, three multiorgan) with a median follow up of 9 years (range 5–16) were reviewed. Neither skin cancer nor premalignant lesions for NMSC were detected in this group. Eighty-five patients had benign naevi (median 6, range 1–57). Clinical risk factors for increased counts of benign naevi included increasing age (P = 0·03), more episodes of sunburn (P = 0·003) and prolonged treatment with ciclosporin (P = 0·009). The presence of atypical naevi in six patients was significantly associated with more episodes of sunburn (P = 0·006) and more transplants (P = 0·04). Other variables including phenotype, skin type, sun exposure, holidays abroad, residence abroad and total duration of immunosuppression did not correlate with benign or atypical naevus counts. Conclusions Skin cancer was not observed in paediatric solid organ transplant recipients who were 5–16 years post-transplantation. Both benign and atypical naevus counts were higher in children with frequent episodes of sunburn. As both naevi and sunburn are risk factors for melanoma, we should target fair-skinned transplant recipients with naevi for intensive sun avoidance education. A prospective, longitudinal follow-up study should determine the onset of skin cancer post-transplantation and the significance of benign and atypical naevus counts in this cohort.

We report a 12-year-old boy with dental, auricular, nasolacrimal duct and unique eyelid anomalies as well as cribriform scrotal atrophy. We believe this is the first description of such a case, although many of the features fit within the spectrum of the ankyloblepharon/ectodermal dysplasia/clefting (AEC) syndrome.

A patient with an atypical presentation of cutaneous T-cell lymphoma is reported. For many years, there were perianal lesions only, which were initially diagnosed as a non-specific severe perianal dermatitis, possibly due to psoriasis. There was little response, however, to potent topical steroids. Further patch and plaque lesions then developed elsewhere and plaque stage mycosis fungoides was diagnosed, allowing effective treatment with superficial radiotherapy and PUVA to be given. The difficulties that may be encountered in the diagnosis of cutaneous T-cell lymphoma are discussed.

Summary The development of extensive cutaneous necrosis in a patient with tumour-stage mycosis fungoides is described. Skin biopsies showed a lymphomatous infiltrate, and thrombosis of dermal blood vessels. Investigation revealed the presence of anticardiolipin antibodies, a lupus anticoagulant, and low free protein S, which contributed to a prothrombotic state. Antiphospholipid antibodies have been detected in non-Hodgkin's lymphoma, but clinical manifestations are uncommon. Such autoantibodies may be produced by neoplastic lymphoid cells. The frequency with which antiphospholipid antibodies occur in mycosis fungoides is currently unknown.