No abstract

PpIX production by ALA and its esters was induced on both normal and malignant cell lines derived from the skin, and cell death of PDT responses is closely related to the amount of PpIX synthesis rather than to the irradiation dose.

Clear cell sarcoma (CCS), also known as malignant melanoma of soft parts, is a rare malignancy constituting approximately 1% of all soft-tissue sarcomas. It occurs predominantly in the lower extremities of young adults, manifesting as a deep, painless, slow-growing mass. CCS is sometimes confused with other types of melanoma because of its melanocytic differentiation. Although BRAF and KIT mutations are well-known melanocytic tumour-promoting mutations frequently found in cutaneous melanoma, they are rare or absent in CCS. We present two cases of CCS with different clinical and genetic features. Both female patients, aged 25 and 20 years, presented with a palpable nodule on a lower extremity. Biopsies of both tumours revealed features diagnostic of CCS. Each tumour cell was positive for S100 protein and HMB-45. However, one patient's tumour was localized to the dermis, with many multinucleated giant cells, whereas the other was located in the deep subcutaneous fat layer near bone. Fluorescence in situ hybridization demonstrated the presence of a characteristic Ewing sarcoma RNA-binding protein (EWSR)1 gene rearrangement in both cases. Reverse-transcription polymerase chain reaction (PCR) and sequencing of the PCR product revealed an EWSR1-activating transcription factor 1 type 1 fusion transcript in both cases. In addition, we detected BRAF mutation in the dermal type and KIT mutation in the subcutaneous type. It is of interest that the BRAF and KIT mutations are known to be very rare in CCS. On the basis of our observations, we suggest that mutation inhibitors may be useful in selected patients with mutated CCS lineages.

A multitude of factors significantly influence the QoL of patients with vitiligo. A better appreciation of these factors would help the management of these patients.

SummaryIn vitiligo, areas of skin lose their colour and become completely white. These white areas, which are often irregular like islands on a map, usually stand out against the normal skin around them, and are very obvious in individuals with a naturally pigmented (brown or black) skin, or in white-skinned people with a tan. People with vitiligo can become very embarrassed and withdrawn, not wanting to go out or to meet people; as a result, the quality of their day-to-day life can suffer. Vitiligo affects between 0.5% and 2% of the population and treatment does not work very well. This study from South Korea looked at what factors to do with vitiligo most affected patients' quality of life. It was a large study involving more than 1,100 adults from 21 different hospitals. Patients filled in a detailed questionnaire called the Skindex-29 questionnaire and members of the study team examined the patients' skin. From the questionnaire results, what concerned patients most were whether the vitiligo was going to get worse and whether it was a serious condition. They were also concerned about how loved ones felt about it, and over half the patients felt depressed about their condition. Involvement of large areas of the body, or areas of the body that other people could easily see, particularly affected quality of life. Vitiligo is far from just a cosmetic problem: over a third of patients were affected emotionally even if the areas of vitiligo were normally covered up.