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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., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists.)

Background: Melanoma in situ/lentigo maligna (LM) is a potential precursor of LM melanoma. It occurs most commonly in elderly individuals on sun-exposed skin of the head and neck. Although surgical excision is the treatment of choice, this may not be desirable or feasible for large lesions at functionally or cosmetically important sites. Imiquimod is a topical immunomodulator which can generate a local cytotoxic response with potentially antiviral and antitumour effects., Objectives: To present our experience of LM treated with imiquimod., Methods: A retrospective review was performed of all patients with facial LM treated in our unit with topical imiquimod between January 2001 and December 2006. Pretreatment diagnostic biopsies were also reviewed and histologically graded., Results: Forty-eight patients were treated with imiquimod. There were 37 responders and 11 treatment failures (of whom two were 'partial responders'). Of the 37 responders, 31 showed a clinical inflammatory response to imiquimod. One patient in whom treatment failed subsequently developed invasive disease. The mean follow-up duration was 49 months. We could not identify histological features of prognostic significance. However, the ability to develop an inflammatory reaction to imiquimod was a strong predictor of therapeutic benefit., Conclusions: We consider imiquimod to have a role in the treatment of LM in patients in whom surgery may be contraindicated or for those in whom the cosmetic or functional consequences may be considerable. Until better characterized, its use should probably be confined to centres with experience in the detection and treatment of LM and melanoma.

Disfiguring nodular nasal lupus pernio is a rare condition that responds poorly to medical management. We have treated three affected patients with carbon dioxide laser remodelling, and report their progress 6 years, 2 years and 16 months following treatment, respectively. Although the abnormal granulomatous tissue was debulked rather than completely excised, the wounds healed within 4 weeks in all patients. The cosmetic results are acceptable and have been maintained in two of the patients, perhaps a reflection of their overall systemic sarcoidosis control.

Melanoma in situ of the lentigo maligna (LM) type is a precursor lesion of LM melanoma. It most commonly occurs in elderly individuals, on the head and neck. Although surgical excision is recommended, this may not be practical for large lesions at cosmetically sensitive sites. In addition, histological changes commonly extend beyond the clinical margins of the lesion. This study describes the use of imiquimod 5% cream as topical immunotherapy in the management of lentigo maligna. Twelve patients (average age 63 years, 10 female), of biopsy-proven facial LM were treated with topical imiquimod, three times a week for 6 weeks. In the absence of an inflammatory response, patients were asked to apply the treatment daily. Seven showed clearance of the LM clinically and histologically. A further three patients showed clearance histologically with persisting pigmentation due to dermal melanin and melanophages. Thus, 10 of 12 patients cleared with no relapse after a median follow-up of 6 months. Two patients failed to respond to imiquimod and their lesions were treated with surgical excision. Imiquimod was well tolerated, except in three patients who experienced an intense inflammatory response. Two of these also developed secondary infection. Imiquimod 5% cream appears to offer a potential noninvasive method for the treatment of lentigo maligna.

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.

'Nonablative laser resurfacing' is a new treatment for photoaged skin, the aim of which is to wound the upper dermis in order to induce dermal fibrosis and improve the clinical appearance. Unlike conventional laser resurfacing or dermabrasion, the epidermis is protected and retained to avoid the problems associated with open wounds and reepithelialization. A number of lasers and light sources have been developed or adapted for this purpose.

Background: It remains questionable whether micrographic surgery with frozen sections is an appropriate technique for excision of melanoma in situ (MIS) of the lentigo maligna type. Advocates of the technique have interpreted MIS as being histologically defined by nests and contiguous atypical melanocytes on the basal layer. Others, however, have viewed the periphery of MIS as consisting of scattered single atypical melanocytes, a finding that may be difficult or impossible to establish on frozen sections., Objectives: To examine the reliability of micrographic surgery using frozen sections interpreted by an experienced Mohs' surgeon, in the excision of MIS., Methods: From a total of 154 specimens, frozen sections from the 50 specimens with margins that were considered difficult to interpret were thawed, sent for routine processing and then examined 'blind' by a dermatopathologist., Results: Using the dermatopathologist's report on paraffin-embedded sections as a reference point, the sensitivity and specificity of frozen sections were calculated to be 59% and 81%, respectively., Conclusions: Using these histological criteria, micrographic surgery with frozen sections alone is unreliable in the excision of MIS.

These guidelines for management of cutaneous melanoma present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation. To reflect the collaborative process for the U.K., they are subject to dual publication in the British Journal of Dermatology and the British Journal of Plastic Surgery.

Training in aspects of skin surgery is now a requirement of general dermatology registrars and residents in the UK and USA, respectively. We compare this component of the training programmes at St John's Institute of Dermatology in London and at New York Medical College in New York City. Because of their understanding of cutaneous pathology, dermatologists are often best qualified to assess patients for skin procedures and should therefore be involved, directly or indirectly, in their surgical management. In our view, the curricula should be changed to place more emphasis on the supervised assessment and treatment of patients with skin tumours as well as to reflect the growing importance of laser treatments and the demand by patients for cosmetic interventions.

We present four renal transplant patients who developed hypertrophic scars following carbon dioxide laser ablation of recalcitrant plantar warts. All of the patients were on long-term treatment with cyclosporin, which we believe to be responsible. We discuss several possible mechanisms by which cyclosporin may influence wound healing and scarring.

We present a 49-year-old patient with a congenital superficial vascular malformation of port-wine stain (PWS) type which has partially re-emerged in the 2.5 years since it was almost completely obliterated with the flashlamp-pumped short pulse pulsed-dye laser (FPDL). This observation is discussed with respect to the possible pathogenesis of PWS, with particular reference to the underlying autonomic nerve supply. The latter would not be expected to respond to FPDL and may explain re-emergence of the lesion.

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.

This article reviews the current uses of lasers in dermatological practice. It outlines safety issues and legislation and attempts to summarize the relevant physics and light interactions. The article is divided into sections pertaining to selective photothermolysis of structures containing the major skin chromophores: haemoglobin, melanin and water. The lasers used and conditions treated are discussed with reference to a literature review.

Background: Histamine-releasing activity (HRA) is detectable in up to 50% of patients with chronic ordinary urticaria., Objectives: To determine the effect of cyclosporin on clinical features and HRA in patients with chronic urticaria., Methods: Thirty patients with severe unremitting disease, responding poorly to antihistamines and showing a positive autologous serum skin test (ASST) as a marker of HRA, were randomized to 4 mg kg-1 daily of cyclosporin (Sandimmun, n = 20) or placebo (n = 10) for 4 weeks. Non-responders were offered open-label cyclosporin for 4 weeks. All were followed for up to 20 weeks or until clinical relapse; all took cetirizine 20 mg daily throughout the study. The primary measure of efficacy was a daily urticaria activity score (UAS) of weal numbers and itch (maximum score 42 per week). A positive response was defined as a reduction to < 25% of baseline weekly UAS and relapse as a return to > 75%. The effect of cyclosporin on serum HRA was assessed by in vitro basophil histamine release assays and ASSTs before and after treatment., Results: Twenty-nine patients (19 active, 10 controls) completed the randomized trial medication. Eight of 19 on active treatment but none on placebo had responded at 4 weeks (P < 0.05). Three others on active drug met the criterion for response at 2 weeks but not at 4 weeks. Mean reduction in UAS between weeks 0 and 4 was 12.7 (95% confidence interval, CI 6.6-18.8) for active and 2.3 (95% CI - 3.3-7.9) for placebo (P = 0.005). Seventeen non-responders (seven randomized to active and 10 to placebo) chose open-label cyclosporin and 11 responded after 4 weeks. Six of the eight randomized active drug responders relapsed within 6 weeks. Of the 19 responders to randomized and open-label cyclosporin, five (26%) had not relapsed by the study end-point. Mean in vitro serum HRA fell from 36% (95% CI 22-49%) to 5% (95% CI 1-8%) after cyclosporin treatment (n = 11, P < 0.0001). The ASST response to post-treatment serum was also reduced (P < 0.05)., Conclusions: This study shows that cyclosporin is effective for chronic urticaria and provides further evidence for a role of histamine-releasing autoantibodies in the pathogenesis of this chronic 'idiopathic' disease.

The major histocompatibility complex (MHC) acts as a marker for self during T-cell ontogeny and is associated with the pathogenesis of many autoimmune diseases. Recent investigations have shown about 30% of patients with chronic idiopathic urticaria (CIU) have IgG autoantibodies against the high-affinity IgE receptor, FcepsilonRI, or IgE. A link between MHC class II alleles and CIU has not been reported previously. DNA was extracted from blood of 100 Caucasian patients with CIU, and the MHC class II type determined using the polymerase chain reaction with sequence-specific primers, testing for DRB and DQB1 alleles. The frequency of alleles in CIU patients was compared with that found in 603 controls. Further human leucocyte antigen (HLA) typing on patient subsets, classified by the patients' responses to intradermal injection of autologous serum and their serum-induced histamine release from basophil leucocytes of healthy donors, was undertaken. HLA DRB1*04 (DR4) and its associated allele, DQB1*0302 (DQ8), are raised in CIU patients compared with a control population (P = 2 x 10-5 and P = 2 x 10-4, respectively). HLA DRB1*15 (DR15) and its associated allele, DQB1*06 (DQ6), are significantly less frequently associated with CIU. The HLA DRB1*04 association is particularly strong (corrected P = 3.6 x 10-6) for patients whose serum has in vivo and in vitro histamine-releasing activity. HLA class II typing is consistent with the concept that CIU is a heterogeneous disease, and supports an autoimmune pathogenesis in a subset of patients.

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.

Histamine releasing autoantibodies play a central role in the pathogenesis of chronic urticaria (CU) in approximately 30% of affected patients. We investigated the therapeutic effect of high-dose intravenous immunoglobulin (IVIG) on disease activity in patients with severe CU of autoimmune aetiology. Autoimmune urticaria was diagnosed by the development of a weal-and-flare reaction to the intradermal injection of autologous serum and by serum-induced histamine release from the basophil leucocytes of healthy donors in vitro. Ten patients with severe, autoimmune CU, poorly responsive to conventional treatment, were treated with IVIG 0.4 g/kg per day for 5 days. The outcome on cutaneous wealing and itch was monitored using urticaria activity scores, visual analogue scales and autologous intradermal serum tests. Clinical benefit was noted in nine of 10 patients: three patients continue in prolonged complete remissions (3 years follow-up), two had temporary complete remissions, and symptoms in four patients improved subsequent to treatment. There was significant improvement in the urticaria activity scores and visual analogue scores at 2 (P < 0.01) and 6 weeks (P < 0.01) post-IVIG compared with the baseline values (Wilcoxon matched pairs). The diminution in urticarial activity in the majority of patients corresponded with a reduced weal-and-flare response to the intradermal injection of autologous post-treatment serum compared with the pretreatment serum. Minor side-effects were common, but there were no serious or long-term adverse effects. IVIG represents a novel therapeutic option in selected patients with recalcitrant CU associated with histamine releasing autoantibodies.

Histological interpretation of frozen sections made during Mohs' micrographic surgery may be difficult, depending on the morphological and staining characteristics of the tumour and on the nature of the associated inflammatory infiltrate. We have employed an adaptation of micrographic surgery in which horizontal, formalin-fixed, paraffin-embedded sections were used to improve histological assessment in the excision of 18 non-melanoma skin tumours in which frozen sections had been or were likely to be unsatisfactory. We describe our experience of this method in the management of squamous cell carcinomas (11), extramammary Paget's disease (two), microcystic adnexal cell carcinomas (two), dermatofibrosarcoma protuberans (two), and primary cutaneous neuroendocrine carcinoma (Merkel cell carcinoma) (one). The use of horizontal paraffin-embedded sections lengthens the duration of the procedure but facilitates accurate assessment of histological sections in selected tumours.

Haemangiomas usually develop within the first few weeks of life, most regressing spontaneously before the age of 7 years. Some may ulcerate or compromise a vital function, in which case systemic corticosteroids, surgery or radiotherapy may be helpful. All of these treatment modalities are associated with significant morbidity. Treatment with the 585 nm flashlamp pulsed dye laser is safe and effective in the management of superficial vascular malformations. We report seven patients, under 12 months of age, who presented with proliferative haemangiomas, causing functional impairment. Ulcerated lesions were present in four patients. The patients were treated with the 585 nm pulsed dye laser (fluences 7.0-9.25 J/cm2), at intervals of 4-8 weeks. All of the lesions showed a significant reduction in size, together with improvement in the colour and integrity of the overlying skin. Treatment with the 585 nm pulsed dye laser should be considered in the management of infants with early proliferative haemangiomas, in whom intervention is indicated.

Treatment with the 585-nm pulsed dye laser was performed in seven patients with viral warts (six with plantar warts and one with a periungual wart) who had failed to respond to conventional therapeutic modalities, including keratolytics, formalin soaks, cryotherapy, curettage, CO2 laser and systemic retinoids. Each patient was and treated on six occasions, separated by intervals of 3 weeks, with fluences of 8.5-9.5 J/cm2. Final follow-up was performed 3 weeks after the final treatment. All patients showed some decrease in the thickness of their warts, this being of a small degree in five of the patients. In the remaining two, there was a moderate reduction in both thickness and wart size. None of the patients experienced complete resolution of their lesions. symptomatic improvement was obtained in all three patients who had previously complained of pain. These data indicate that treatment of resistant viral verrucae of the periungual and plantar skin with the pulsed dye laser is not curative although partial resolution and symptomatic improvement may occur.

We have investigated the kinetics of the leucocyte infiltrate in delayed pressure urticaria (DPU) in relation to the in vivo expression of the cytokine-regulated cell surface adhesion molecules, E-selectin (endothelial adhesion molecule-1, ELAM-1), intercellular adhesion molecule-1 (ICAM-1), and vascular adhesion molecule-1 (VCAM-1). Immunohistochemical analysis was performed on biopsies taken from unchallenged skin, and at 0, 2, 6, 24, 48 and 120 h after weighted rods had been applied to 13 patients with DPU. There was moderate to marked upregulation of E-selectin at 6 and 24 h after application of pressure. At 24 h, more patients showed expression of VCAM-1 on perivascular cells than before pressure. Moderate expression of ICAM-1 was present in some biopsies from both unchallenged and pressure-challenged skin, but there was no clear trend. In DPU, there was a significant increase in the neutrophil count at 2 h after a pressure challenge, with further increases at 6 and 24 h. The median cell counts per high-power field of eosinophils and monocyte/macrophages increased significantly at 24 h after pressure. Biopsies from four normal controls subjected to an identical pressure challenge showed no detectable changes in adhesion molecule expression or in the cell infiltrate. The findings in four patients with chronic idiopathic urticaria not associated with DPU were qualitatively similar to (but intermediate in severity between) the findings in DPU weals at 6 and 24 h. These results suggest that vascular endothelial activation is an early response to pressure challenge in DPU, and is also present in chronic idiopathic urticaria.(ABSTRACT TRUNCATED AT 250 WORDS)

A rare, but distinctive chronic eruption in six female black South Africans is reported. The original diagnosis of subcorneal pustular dermatosis of Sneddon and Wilkinson in these patients was refuted by the subsequent histological observation of both spongiform pustules and an underlying vasculitis. This may represent a previously undocumented form of generalized pustular psoriasis.

A clinically mild recurrent rash in a young male homosexual with acquired immune deficiency syndrome (AIDS)-related complex was found on histological examination to consist of a severe necrotizing folliculitis with underlying vasculitis. Follicular eruptions are amongst the seemingly banal dermatological disorders which may accompany AIDS or its prodromes.

The dermatological findings in five patients with thallium poisoning are described. Cutaneous involvement consisted mainly of an acneiform eruption, pellagra-like dyssebacea, crusted eczematous lesions and dry scaling of the distal parts of the extremities. Alopecia was not evident initially but later became apparent. The patients also had gastrointestinal and neurological symptoms and signs.

We report pathognomonic skin lesions of malignant atrophic papulosis, accompanied by lethal central nervous system and gastrointestinal disease in a 22-year-old black man. Histopathological studies of the brain and colon showed areas of infarction and an underlying vasculitis with leukocytoclasis.