Multiple familial and pigmented basal cell carcinomas in early childhood - Bazex-Dupré-Christol syndrome.

BACKGROUND: Bazex-Dupré-Christol syndrome (BDCS) is an X-linked dominantly inherited disorder affecting hair follicle structures. Currently, hypotrichosis, follicular atrophoderma and basal cell carcinomas are considered frequent symptoms of the disorder whereas milia are supposed to reflect infrequent clinical signs. Usually, basal cell carcinomas in this disease manifest from the second decade of life onwards. CASE REPORT: Here, we studied a novel multigeneration family of German origin with BDCS. Interestingly, two family members developed pigmented basal cell carcinomas in early childhood, at the age of 3 and 5 years, respectively. The differentiation from other pigmented lesions was accomplished by both dermoscopy and histopathology. A thorough survey of the current literature revealed that milia were present in almost all patients with BDCS reported, as is the case in our family. CONCLUSIONS: We suggest that milia should also be considered frequent symptoms in BDCS. For the first time, to the best of our knowledge, we describe the occurrence of pigmented basal cell carcinomas in BDCS during the first decade of life. Our observation emphasizes the importance of screening for cutaneous malignancies in this disorder already at young age.

Photodynamic therapy of multiple actinic keratoses: reduced pain through use of visible light plus water-filtered infrared A compared with light from light-emitting diodes.

BACKGROUND: Photodynamic therapy (PDT) with methyl aminolaevulinate (MAL) is an effective treatment for multiple actinic keratoses (AKs). Pain, however, is a major side-effect. OBJECTIVES: To compare pain intensity, efficacy, safety and cosmetic outcome of MAL PDT with two different light sources in an investigator-initiated, randomized, double-blind study. METHODS: Eighty patients with multiple AKs grade I-II were assigned to two groups: group 1, MAL PDT with visible light and water-filtered infrared A (VIS+wIRA); group 2, MAL PDT with light from light-emitting diodes (LEDs), with a further division into two subgroups: A, no spray cooling; B, spray cooling on demand. MAL was applied 3 h before light treatment. Pain was assessed before, during and after PDT. Efficacy, side-effects, cosmetic outcome and patient satisfaction were documented after 2 weeks and 3, 6 and 12 months. Where necessary, treatment was repeated after 3 months. RESULTS: Seventy-six of the 80 patients receiving MAL PDT completed the study. Patient assessment showed high efficacy, very good cosmetic outcome and high patient satisfaction. The efficacy of treatment was better in the group of patients without spray cooling (P=0·00022 at 3 months, P=0·0068 at 6 months) and showed no significant differences between VIS+wIRA and LED. VIS+wIRA was significantly less painful than LED: the median of maximum pain was lower in the VIS+wIRA group than in the LED group for PDT without spray cooling. Pain duration and severity assessed retrospectively were less with VIS+wIRA than with LED, irrespective of cooling. CONCLUSIONS: All treatments showed high efficacy with good cosmetic outcome and high patient satisfaction. Efficacy of treatment was better without spray cooling. VIS+wIRA PDT was less painful than LED PDT for PDT without spray cooling.

[Necrotizing toxic contact dermatitis of the scalp from hydrogen peroxide]. / Nekrotisierende toxische Kontaktdermatitis der Kopfhaut durch Wasserstoffperoxid.

Skin reactions to chemicals used by hair dressers are usually reported as irritative or allergic contact dermatitis of the hands. We describe a 15-year old girl who suffered an unusually severe injury to her scalp with necrosis of the galea aponeurotica as a result of highlighting her hair. Injury resulted in a scaring alopecia, which can only be treated by plastic reconstructive surgery. The cause for this injury might have been a higher than usual concentration of hydrogen peroxide used for highlighting the hair.

High-resolution optical coherence tomography as a non-destructive monitoring tool for the engineering of skin equivalents.

BACKGROUND: Three dimensional skin equivalents are widely used in dermatopharmacological and toxicological studies and as autologous transplants in wound healing. In pharmacology, there is tremendous need for monitoring the response of engineered skin equivalents to external treatment. Transplantation of skin equivalents for wound healing requires careful verification of their quality prior to transplantation. Optical coherence tomography (OCT) is a non-contact, non-destructive imaging technique for living tissues offering the potential to fulfill these needs. This work presents an analysis of OCT for high-resolution monitoring of skin equivalents at different stages during the culture process. METHODS: We developed a high-resolution OCT imaging setup based on a commercially available OCT system. A broadband femtosecond laser light source replaces the original superluminescence diode. Tomograms of living skin equivalents were recorded with an axial resolution of 3 mum and correlated with histology and immunofluorescence images. Comparison with standard low-resolution OCT is presented to emphasize the advantages of high-resolution OCT for this application. RESULTS: OCT is particularly able to distinguish between different layers of skin equivalents including stratum corneum, epidermal and dermal layer as well as the basement membrane zone. The high-resolution OCT scans correlate closely with two key benchmarks, histology and immunofluorescence imaging. CONCLUSIONS: This study clearly demonstrates the benefits of high-resolution OCT for identifying living tissue structure and morphology. Compared with the current gold standard histology, OCT offers non-destructive tissue imaging, enabling high-resolution evaluation of living tissue morphology and structure as it evolves.

Circulating clonal CLA(+) and CD4(+) T cells in Sezary syndrome express the skin-homing chemokine receptors CCR4 and CCR10 as well as the lymph node-homing chemokine receptor CCR7.

BACKGROUND: Adhesion molecules and chemokine receptors are involved in tissue-specific homing of T cells to the skin and play an important role in the pathophysiology of cutaneous lymphoma. It has recently been reported that the chemokine CCL27 expressed by keratinocytes attracts lymphocytes bearing the chemokine receptor CCR10. OBJECTIVES: To investigate the expression of CCR4, CCR7 and CCR10 on skin-homing CLA(+) and CD4(+) T cells in the peripheral blood of patients with Sezary syndrome (SS), a rare leukaemic variant of cutaneous T-cell lymphoma. METHODS: Lymphocytes from five patients with SS, six patients with mycosis fungoides and four healthy volunteers were isolated and analysed using flow cytometry. Additionally, the T-cell receptor (TCR)-Vbeta CDR3 regions were cloned and sequenced in two patients. RESULTS: We found that CCR4 is expressed on almost all CLA(+) and CD4(+) memory T cells. Using monoclonal antibodies specific for single TCR-Vbeta chains we identified malignant T cells in four patients with SS. Importantly, we found that most but not all malignant Sezary cells expressed the skin-homing chemokine receptor CCR10. Additionally, we found that a significant proportion of these cells also expressed the lymph node-homing chemokine receptor CCR7. CONCLUSIONS: Our results support the concept that chemokine receptors play an important role in the pathophysiology of SS and suggest that the malignant clone may represent an expansion of skin-homing cutaneous 'central' memory T cells in the peripheral blood of these patients.

Results of a surveillance programme for patients at high risk of malignant melanoma using digital and conventional dermoscopy.

Patients with a high number of atypical naevi and a personal and/or family history of melanoma are at high risk of malignant melanoma. The objective of this study was to design a special documentation and surveillance programme using epiluminescence microscopy (ELM) and digital epiluminescence microscopy (DELM) to improve the surveillance of these patients. High-risk patients (n=212) were categorized by the number and phenotype of their naevi and their personal and family history of melanoma. Then patients were screened by the unaided eye, conventional photography, ELM and, in selected cases of atypia, DELM. Median follow-up was 18 months, and 2939 pigmented lesions were followed by DELM. Examination on the first visit identified 17 cutaneous melanomas. During the following observation period, another 17 melanomas were identified. Fifteen of these follow-up melanomas were exclusively identified based upon DELM. In these cases, subtle lesional changes occurred over time, and ELM diagnostic algorithms for differentiating benign melanocytic lesions from melanoma did not score a suspicion of melanoma. All melanomas, either pre-existing or developing during follow-up, were identified in an early, curable phase of tumour growth. We conclude that DELM follow-up for patients at high risk allows the early detection of melanomas that have not yet acquired melanoma-typical ELM features.

[Unilateral cheek tumor. Case report and differential diagnoses]. / Unilateraler Wangentumor. Fallbericht und Differenzialdiagnosen.

A 56-year-old male presented with a tumor on the cheek that had developed over 5 months. Unexpectedly, histological and immunohistochemical studies revealed an adenocarcinoma of the salivary gland. We discuss the differential diagnoses of tumors of the cheek.

Current and future directions in the treatment of metastatic malignant melanoma.

Recently treatment strategies in advanced malignant melanoma have significantly changed. Due to high response rates (e.g. more than 50% for the Dartmouth-regimen), combination chemotherapy has been the standard therapy in several oncological and dermatooncological centers in the USA and Europe. For the last three years different prospective randomized phase III trials failed to achieve similar results. There was no benefit in overall survival and in response duration in comparison to single agent chemotherapy. Currently, randomized clinical trials seem to be the best approach for the clinical treatment of metastatic melanoma. In this review several novel strategies against malignant melanoma are discussed with focus on the role of single agent chemotherapy and biochemotherapy.

[Regression phase as therapeutic goal of cryosurgical treatment of growing capillary infantile hemangiomas. Treatment decision, treatment strategy and results of an open clinical study]. / Regressionsphase als therapeutisches Ziel der kryochirurgischen Behandlung wachsender kapillärer Säuglingshämangiome. Behandlungsentscheidung, Behandlungsstrategie und Ergebnisse einer offenen klinischen Studie.

BACKGROUND AND OBJECTIVE: Cryosurgery is a widely accepted modality for the treatment of capillary hemangiomas of the newborn. However, no studies clearly supporting its effectivity are available. PATIENTS AND METHODS: Out of 116 consecutive outpatients with 147 hemangiomas, 105 growing tumors in 91 patients were treated by cryosurgery. 13 further growing tumors were not treated because of lack of parental consent. Hemangiomas without growth within the last 2 weeks and with clinical signs of regression were not treated. After a follow-up of 1 year, the outcome of the treatment was classified as either regression (> 10% size reduction), no change (size alteration < 10%) or progression (> 10% size growth). The effectiveness of the treatment was assessed as clinical improvement of the hemangioma on an arbitrary scale per treatment session. RESULTS: Cryosurgery of growing hemangiomas efficiently initiated the regression phase (p < 0.05), in the most cases (68%) after a single treatment. Cryotherapy was most effective when employed in older children (p < 0.05) and for small hemangiomas (p < 0.05). Probably, this reflects associated spontaneous regression. CONCLUSIONS: Thus cryosurgery is a verifiably effective treatment modality for infantile capillary hemangiomas, shortening significantly their growth phase.