Search

Summary Background Dermatofibrosarcoma protuberans (DFSP) has conventionally been treated with wide local excision. More recently Mohs micrographic surgery (MMS) has been advocated. Objectives To assess our departmental experience with DFSP in the context of a literature review relating to DFSP treated with MMS. Methods This was a case review of 35 patients with DFSP treated between 1998 and 2009 with MMS using paraffin-embedded sections. Results Seventeen patients required one horizontal layer to clear their tumour, 10 patients needed two and eight patients needed three layers or more. The median preoperative clinical size was 6 cm2 (range 0·75–54·8) and the median postoperative wound size was 46·8 cm2 (range 4–145·2). Tumour persistence has not been observed in any of our patients after a median follow-up duration of 29·5 months (range 6–146). Conclusions We present 35 DFSP patients, none of whom showed persistent tumour after treatment with ‘slow’ MMS using paraffin sections. We advocate MMS as the treatment of choice for DFSP, especially for tumours over the head and neck region where tissue conservation is particularly important.

Summary Background Photodynamic therapy (PDT) using topical 5-aminolaevulinic acid (5-ALA) as a photosensitizer has been reported in the treatment of both neoplastic and benign cutaneous disorders. Objectives To evaluate the efficacy of photodynamic therapy in selected patients with Darier's disease (keratosis follicularis). Methods Six patients with Darier's disease were assessed before and after treatment with PDT using 5-ALA and mean fluence rates of 110–150 mW cm−2. Results Of the six patients, one was unable to tolerate the treatment. Of the remaining five, all experienced an initial inflammatory response that lasted two to three weeks. In four of the five patients, this was followed by sustained clearance or improvement over a followup period of six months to three years. Three of these four patients were on systemic retinoids and the fourth had discontinued acitretin prior to PDT. In the fifth patient partial improvement was followed by recurrence after etretinate therapy was discontinued. Biopsy specimens taken immediately after the procedure in two patients demonstrated a mild inflammatory cell infiltrate in the dermis. A biopsy obtained eighteen months after PDT from a successfully treated area showed no signs of Darier's disease and a subtle increase of collagen in the upper dermis. Conclusions Photodynamic therapy can be viewed as a potential adjunctive modality for Darier's disease but should not be considered as a substitute for retinoids in patients who require systemic treatment.