What Can Be Expected on Irradiated Skin? A Dermatologic Focus on Acute and Chronic Radiotherapeutic Effects, Treatment, and Benign Dermatoses.

Radiotherapy has been a common and well-known treatment for several cancers and benign dermatoses. An overview of the characteristics of acute and chronic cutaneous effects of radiotherapy and its treatment is presented. A current overview of benign dermatoses after radiotherapy, presently only dispersedly published in the literature, is given with the mean age of occurrence, dose of radiotherapy, and their latency time calculated for those described more than 8 times. Benign dermatoses occurring most often after radiotherapy (>20 times described), ie, morphea, bullous pemphigoid, pemphigus vulgaris, and acneiform eruptions are discussed in more detail. Finally, dermatoses with a specific distribution related to the irradiated area are highlighted. This review provides an overview of cutaneous side effects of radiotherapy, especially of the benign dermatoses, as a supplement to the clinical knowledge of dermatologists, oncologists, and wound care specialists.

Post radiation skin tumors: basal cell carcinomas, squamous cell carcinomas and angiosarcomas. A review of this late effect of radiotherapy.

This review gives an overview of radiotherapy-induced malignant skin tumors as described in the present medical literature. Basal cell carcinomas are the most frequent post-radiation malignant skin tumors; however, specific incidence ratios are few and show ratios of 2%. Squamous cell carcinomas are briefly discussed, followed by post-radiation sarcomas. Most cases of post-radiation cutaneous sarcomas are angiosarcoma, malignant fibrous histiocytoma, leiomyosarcoma and fibrosarcoma. In cases of radiotherapy for breast cancer, angiosarcomas are the most frequently found malignant sarcomas worldwide (incidence 0.5%) in the irradiated area. We present 192 cases of angiosarcomas after radiotherapy for breast cancer. Also, the atypical vascular lesion, a benign vascular skin lesion occurring after radiotherapy, and the important differential diagnosis of angiosarcoma will be presented and discussed. Other skin tumors supposedly related to radiotherapy are occasionally published and summarized in this review. Because most radiation-induced malignant tumors occur many years after the initiation of radiotherapy and incidences are low, we suggest good instruction of patients regarding self control of the skin rather than a yearly follow-up.

Proposal for updating the pseudoxanthoma elasticum classification system and a review of the clinical findings.

Pseudoxanthoma elasticum (PXE) is a systemic disorder affecting elastic tissues most markedly in skin, retina, and blood vessels. It is caused by mutations in the ABCC6 gene and is transmitted in an autosomal recessive fashion. In 1994 a new classification system for PXE was published as the result of a consensus conference. Since then the ABCC6 gene has been discovered. We propose that there is a need for a classification system incorporating all relevant systemic symptoms and signs, based on standardized clinical, histological, and molecular biological examination techniques. We re-evaluated the histopathologic PXE signs and propose a classification system with unambiguous criteria leading to a consistent diagnosis of definitive, probable, or possible PXE world-wide. We put this proposed classification forward to encourage further debate on the diagnosis of this multi-organ disorder.

Pseudoxanthoma elasticum: Wide phenotypic variation in homozygotes and no signs in heterozygotes for the c.3775delT mutation in ABCC6.

PURPOSE: : Pseudoxanthoma elasticum is an autosomal recessive disorder of elastic tissue in the skin, eyes, and cardiovascular system, caused by mutations in the ABCC6 gene. The purpose of this study was to check variability in expression within one genotype and look for pseudoxanthoma elasticum signs in heterozygotes. METHODS: : We examined a relatively large, in comparison with the present literature, group of adult persons homozygous or heterozygous for the c.3775delT mutation in the ABCC6 gene, from a genetically isolated population in the Netherlands. All participants filled out a questionnaire and underwent standardized dermatologic and ophthalmologic examinations with photography of skin and fundus abnormalities. Skin biopsies from affected skin or a predilection site and/or a scar were examined and compared with biopsies from controls. RESULTS: : Skin abnormalities, ophthalmologic signs, and cardiovascular problems varied greatly among the 15 homozygous participants. There was no correlation among severity of skin, eyes, or cardiovascular abnormalities. None of the 44 heterozygous participants had any sign of pseudoxanthoma elasticum on dermatologic, histopathologic, and/or ophthalmologic examination, but 32% had cardiovascular disease. CONCLUSION: : Individuals homozygous for the c.3775delT mutation can have a highly variable phenotype. We did not find pseudoxanthoma elasticum eye or skin abnormalities in the heterozygous family members.

Müllerian serous cystadenoma of the scrotum following orchiopexy.

A 24-year-old man presented himself with a nodular lesion of about 1 cm diameter at the site of a previous orchiopexy associated with surgery for cryptorchism. Histopathology revealed the lesion to be adenomatous and confined to the scrotum. Histological and immunohistological features were not consistent neither with median raphe cysts or cutaneous adenomas nor with the intrascrotal adenomas of the rete testis, epididymis, nor with (malignant) mesotheliomas. However, the lesion did compare well with serous (papillary) cystadenomas of the testis or paratestis. These adenomas are thought to originate in remnants of the Müllerian system or of peritoneal lining altered by Müllerian metaplasia. This implies that the scrotal adenoma may have developed from an implant of such elements during orchiopexy 14 years ago. Complete excision of the lesion appears to be an adequate therapy.

Eponyms of Sir Jonathan Hutchinson.

Sir Jonathan Hutchinson was an extraordinary man. He was trained as a surgeon and a pathologist, but was also keenly interested in dermatology, the study of syphilis, ophthalmology, and neurology. His observations with detailed descriptions of skin diseases were remarkable. His medical bibliography staggeringly consists of over 1000 published reports. This description of the eponyms attributed to Hutchinson--illustrated with clinical images, an old plate, and a portrait--demonstrates his important contribution to dermatology.

Giant ceribriform intradermal nevus on the back of a newborn.

A male newborn had a large cerebriform tumor covering his shoulders and almost the entire surface of his back. After exclusion of further abnormalities, the diagnosis of cerebriform intradermal nevus was made. This particular variant of giant melanocytic nevus should always be differentiated from cutis verticis gyrata, if located on the vertex. The clinical manifestation of cerebriform intradermal nevi as giant melanocytic nevi on the back is extremely rare, with only one instance reported to date. Such nevi are a therapeutic challenge, particularly if the skin lesion covers a large surface of the body, as in the patient presented here.

Disruption of Abcc6 in the mouse: novel insight in the pathogenesis of pseudoxanthoma elasticum.

Pseudoxanthoma elasticum (PXE) is a heritable disorder of connective tissue, affecting mainly skin, eye and the cardiovascular system. PXE is characterized by dystrophic mineralization of elastic fibres. The condition is caused by loss of function mutations in ABCC6. We generated Abcc6 deficient mice (Abcc6-/-) by conventional gene targeting. As shown by light and electron microscopy Abcc6-/- mice spontaneously developed calcification of elastic fibres in blood vessel walls and in Bruch's membrane in the eye. No clear abnormalities were seen in the dermal extracellular matrix. Calcification of blood vessels was most prominent in small arteries in the cortex of the kidney, but in old mice, it occurred also in other organs and in the aorta and vena cava. Newly developed monoclonal antibodies against mouse Abcc6 localized the protein to the basolateral membranes of hepatocytes and the basal membrane in renal proximal tubules, but failed to show the protein at the pathogenic sites. Abcc6-/- mice developed a 25% reduction in plasma HDL cholesterol and an increase in plasma creatinine levels, which may be due to impaired kidney function. No changes in serum mineral balance were found. We conclude that the phenotype of the Abcc6-/- mouse shares calcification of elastic fibres with human PXE pathology, which makes this model a useful tool to further investigate the aetiology of PXE. Our data support the hypothesis that PXE is in fact a systemic disease.

Narrowband UVB and psoralen-UVA in the treatment of early-stage mycosis fungoides: a retrospective study.

BACKGROUND: Treatment of early-stage mycosis fungoides (MF) consists of topical steroids, phototherapy (UVB), photochemotherapy (psoralen plus UVA [PUVA]), topical nitrogen mustard, or total skin electron-beam irradiation. It has been reported that the same effective UVB dose is safer than PUVA regarding carcinogenicity and produces fewer side effects. Narrowband UVB (311 nm) results in less irritation and erythema and is more effective compared with broadband UVB. OBJECTIVE: Our purpose in this retrospective study was to analyze the response to treatment, relapse-free interval, and irradiation dose in 56 patients with early-stage MF (stage Ia and Ib). A total of 21 patients were treated with narrowband UVB (311 nm); 35 patients were treated with PUVA. RESULTS: Narrowband UVB treatment led to complete remission in 17 of 21 patients (81%), partial remission in 4 of 21 (19%), and none showed progressive disease. PUVA treatment led to complete remission in 25 of 35 patients (71%), partial remission in 10 of 35 (29%), and none showed progressive disease. The mean relapse-free interval for patients treated with UVB was 24.5 months (range, 2-66 months) and for patients treated with PUVA, 22.8 months (range, 1-43 months). CONCLUSION: Narrowband UVB therapy for patients with early-stage MF is an effective treatment modality. It has several advantages over treatment with broadband UVB and PUVA. When treating patients with early-stage MF it may be beneficial to start with narrowband UVB therapy and, if there is progression or no response, switch to PUVA therapy.