Dermatofibrosarcoma protuberans: 35 patients treated with Mohs micrographic surgery using paraffin sections.

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 cm(2) (range 0·75-54·8) and the median postoperative wound size was 46·8 cm(2) (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.

A case of focal dermal hypoplasia (Goltz) syndrome with exophytic granulation tissue treated by curettage and photodynamic therapy.

Focal dermal hypoplasia (Goltz) syndrome is a rare genetic disorder characterized by cutaneous, ectodermal and mesodermal defects. We present a case in which painful, exophytic granulation tissue has been the main symptom over the past 15 years. After unsatisfactory results with a number of treatment modalities including topical steroids, silver-nitrate applications, cryotherapy, curettage, excision and pulsed-dye laser, we achieved significant benefit with curettage in combination with photodynamic therapy. Although impaired wound healing has been described in focal dermal hypoplasia, this is, to our knowledge, the first time that pyogenic granuloma-like lesions have been reported.

Treatment of Darier's disease with photodynamic therapy.

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.

Guidelines for topical photodynamic therapy: report of a workshop of the British Photodermatology Group.

Topical photodynamic therapy (PDT) is effective in the treatment of certain non-melanoma skin cancers and is under evaluation in other dermatoses. Its development has been enhanced by a low rate of adverse events and good cosmesis. 5-Aminolaevulinic acid (ALA) is the main agent used, converted within cells into the photosensitizer protoporphyrin IX, with surface illumination then triggering the photodynamic reaction. Despite the relative simplicity of the technique, accurate dosimetry in PDT is complicated by multiple variables in drug formulation, delivery and duration of application, in addition to light-specific parameters. Several non-coherent and coherent light sources are effective in PDT. Optimal disease-specific irradiance, wavelength and total dose characteristics have yet to be established, and are compounded by difficulties comparing light sources. The carcinogenic risk of ALA-PDT appears to be low. Current evidence indicates topical PDT to be effective in actinic keratoses on the face and scalp, Bowen's disease and superficial basal cell carcinomas (BCCs). PDT may prove advantageous where size, site or number of lesions limits the efficacy and/or acceptability of conventional therapies. Topical ALA-PDT alone is a relatively poor option for both nodular BCCs and squamous cell carcinomas. Experience of the modality in other skin diseases remains limited; areas where there is potential benefit include viral warts, acne, psoriasis and cutaneous T-cell lymphoma. A recent British Photodermatology Group workshop considered published evidence on topical PDT in order to establish guidelines to promote the efficacy and safety of this increasingly practised treatment modality.

Bowen's disease, solar keratoses and superficial basal cell carcinomas treated by photodynamic therapy using a large-field incoherent light source.

BACKGROUND: Photodynamic therapy (PDT) has not yet been demonstrated to be superior to conventional treatment in the treatment of superficial skin cancers and premalignant skin conditions. A limitation for PDT is the absence to date of a light source suitable for the treatment of larger lesions or 'field changes' where several lesions are present on one anatomical site. OBJECTIVES: To investigate the safety and efficacy of a large field light source, the Waldmann PDT 1200, in the treatment of Bowen's disease (BD), superficial basal cell carcinomas (BCCs) and solar keratoses (SKs). METHODS: After application of 5-aminolaevulinic acid for 4-6 h, each lesion was irradiated with 105 J cm-2 of incoherent red light centred on 640 nm. Eighty-eight patients with 239 lesions were recruited. RESULTS: Within two treatments, 88% of BD lesions, 95% of BCCs and 99% of SKs showed complete clinical clearance. At 12 months the complete response rates were 69% for BD, 82% for BCC and 72% for SK. CONCLUSIONS: This study confirms that PDT is a useful treatment and that selected superficial BCCs and SKs respond well to PDT. The PDT 1200 light source proved capable of treating multiple lesions amounting to a 'field change' and also lesions up to 10 cm in diameter within an acceptable treatment time. Thus far, PDT has failed to become established as a routine treatment for small premalignant and malignant skin lesions as it has not proved superior to simple cheaper conventional therapies such as cryotherapy, curettage and cautery, topical chemotherapy with 5-fluorouracil, or surgery. However, PDT has become established as a treatment for selected cases in some centres. This study suggests a role for PDT in the treatment of large premalignancies, superficial BCCs and field change where existing treatments may be problematic.

Objective assessment of port-wine stains following treatment with the 585 nm pulsed dye laser.

Previous studies assessing the treatment of port-wine stains (PWS) with the 585 nm pulsed dye laser have relied on either subjective clinical assessment or in vivo measurement of skin colour alone. The aim of the present retrospective study was to develop an objective method of assessing available pre- and post-treatment photograph pairs. Port-wine stains depicted in photographs of 23 patients following six or more treatment sessions were assessed for changes in colour (DeltaH*) and PWS size by computer image analysis and were compared with a subjective assessment of PWS reduction by a "blinded" physician examining the same images. The post-treatment mean reduction in the PWS assessed by the physician was 39.7%. A global assessment score incorporating values of DeltaH* and PWS size by computer analysis showed a mean reduction of 12%, with a more significant correlation with the physician assessment (Spearman's rank correlation coefficient 0.627; P=0.001) than changes in size or colour alone.

Single-episode photodynamic therapy and vulval intraepithelial neoplasia type III resistant to conventional therapy.

BACKGROUND: Photodynamic therapy (PDT) using topical 5-aminolaevulinic acid (5-ALA) has been suggested as an effective and tissue-conserving method of treating carcinoma in situ of the vulva. OBJECTIVES: To evaluate PDT in patients with vulval intraepithelial neoplasia type III (VIN III). METHODS: Topical PDT was performed in six patients with VIN III. Five of the six patients had persistent disease following treatment with other modalities including 5-fluorouracil cream, cryotherapy, carbon dioxide laser ablation and excision. Each patient was treated once with a fluence of 150 J cm-2 using a broad-band light source (580-740 nm) 4 h after topical application of 20% 5-ALA. Patients were reviewed clinically at 1 month and 6 months after treatment. RESULTS: All of the patients developed initial erythema of treated sites, three with subsequent erosions. All patients had clinically evident persistent VIN III at 1-month review. Five patients have subsequently undergone surgical treatment and one is regularly reviewed. CONCLUSIONS: This small uncontrolled study indicates that, as currently administered, a single episode of topical PDT is not effective in the management of treatment-resistant VIN III.

A randomized paired comparison of photodynamic therapy and topical 5-fluorouracil in the treatment of actinic keratoses.

BACKGROUND: Photodynamic therapy (PDT) has not been compared with topical 5-fluorouracil (5-FU) in the treatment of epidermal dysplasia. OBJECTIVE: The purpose of this study was to assess the efficacy and tolerability of these two treatment modalities in 17 patients with actinic keratoses on the backs of the hands. METHODS: Each patient's right and left hands were randomized to receive either a 3-week course of topical 5-FU applied twice per day or PDT using topical 5-aminolevulinic acid (5-ALA) and then, after 4 hours, irradiation with an incoherent light source consisting of a 1200 W metal halogen lamp emitting red light (580 to 740 nm). Each hand randomized for PDT received 150 J/cm(2). The observed median fluence rate was 86 mW/cm(2) (interquartile range, 53 to 100 mW/cm(2)). All patients were reviewed at 1, 4, and 24 weeks after starting treatment. RESULTS: Fourteen of 17 patients (82%) completed the study. The mean lesional area treated with topical 5-FU decreased from 1390 mm(2) (standard deviation [SD], 1130) to 297 mm(2) (SD, 209). This represents a mean reduction in lesional area of 70% (confidence interval [CI], 61%-80%). The mean lesional area treated with topical PDT decreased from 1322 mm(2) (SD, 1280) to 291 mm(2) (SD, 274), representing a mean reduction in lesional area of 73% (CI, 61%-84%). The reduction in lesional area elicited by the two treatment methods was similar (CI, -25% to 17%). There was no statistically significant difference between the treatment methods in overall symptom scores for pain and redness. CONCLUSION: One treatment with PDT using topical 5-ALA appears to be as effective and well tolerated as 3 weeks of twice-daily topical 5-FU, a cheap and widely available alternative.

The role of photodynamic therapy in dermatology.

Photodynamic therapy (PDT) involves selective photosensitization of a target tissue by means of a topically or systemically administered agent which is then activated by light to effect an oxygen dependent cytotoxic reaction. The production of reactive oxygen intermediates, including singlet oxygen, is localized to where the photosensitizer accumulates and induces apoptosis and vascular endothelial damage. First-generation photosensitizers are haematoporphyrin derivatives and are effective in treating certain nonmelanoma skin cancers. However, they induce cutaneous photosensitization for at least 4-6 weeks and have a limited role in dermatology. Many second-generation photosensitizers, such as 5-aminolaevulinic acid, are associated with less prolonged photosensitization. Although PDT remains largely experimental, it has potential applications in both benign and malignant skin disease.

Photosensitivity associated with the Smith-Lemli-Opitz syndrome.

A case of severe photosensitivity in a girl with the Smith-Lemli-Opitz syndrome is reported. Children with this recessively inherited metabolic disorder of cholesterol metabolism present with a variety of congenital abnormalities of the nervous system and internal organs in association with varying degrees of mental retardation. Photosensitivity is a feature which has previously only briefly been mentioned in the literature in association with this syndrome. However, more recently, it has become apparent that photosensitivity is not uncommon among children with the Smith-Lemli-Opitz syndrome, although the nature of the photosensitivity in these patients has remained undefined. Our patient has suffered from sunlight intolerance since early infancy, with redness and pruritus of sun-exposed skin developing within minutes of sun exposure. Monochromator ultraviolet (UV) radiation and visible light testing revealed an immediate and persistent reaction to low-dose UVA at 350 nm, and an abnormal erythemal response to visible light at 400 nm.

Dermatology in-patient management greatly improves life quality.

The aim of this study was to measure the effect of in-patient management on the quality of life of adult dermatology patients, and to identify the diagnostic categories which show the greatest improvement. Over a 6-month period, all 230 patients admitted to the dermatology ward of the University Hospital of Wales were invited to complete a Dermatology Life Quality Index (DLQI) questionnaire on admission, and again 4 weeks after discharge. Two hundred and seventeen (93%) of these patients entered the study, and 181 (83.4%) returned both questionnaires. The mean DLQI on admission was 13.2 (standard deviation [SD] 7.6; n = 181), and 4 weeks after discharge it was 7.7 (SD 6.8; P < 0.001). Seventy-three per cent of the 181 patients showed improvement, 5.5% remained unchanged, and 21.5% worsened. Patients with psoriasis improved from 13.7 (SD 6.5) to 6.7 (SD 5.6; n = 63; P < 0.001), and those with eczema improved from 16.2 (SD 6.3) to 9.6 (SD 7.6; n = 56; P < 0.001). Patients with pruritus showed little improvement, as did those admitted for liver biopsy. Patients with psoriasis and severe eczema showed, overall, a significant decrease in impairment of life quality following in-patient treatment. Severe eczema has a greater adverse impact on the quality of life than severe psoriasis. The parameters for which most improvement was seen were those which were of most concern to the patients, i.e. their symptoms (score after discharge = 1.2; DS 0.9; P < 0.001) and their embarrassment (0.9; SD 1.0; P < 0.001).

Protracted cutaneous disorders in association with low CD4+ lymphocyte counts.

We report two patients with skin disorders usually associated with severe immunosuppression, who had low CD4+ lymphocyte counts but normal immunoglobulin levels. The patients were HIV negative, and had CD4+ lymphocyte counts just above 300/mm3, but they presented with cutaneous manifestations of profound immunodeficiency. Idiopathic CD4+ lymphocyte deficiency is a recently described syndrome which may present with dermatological disease. We discuss the symptom complex of our patients in relationship to the diagnosis of idiopathic CD4+ lymphocyte deficiency.