Multi-systemic Langerhans cell Histiocytosis in an adult

The etiology of Langerhans cell histiocytosis (LCH), a mix between immune dysregulation, inflammation, and malignancy, remains unclear.1; 2; 3 ; 4 In half of the patients, an oncogenic BRAF mutation is found. 3 Because of the diversity of symptoms, the diagnosis of LCH, as defined by the Histiocyte Society, is often made with considerable delay. Multisystemic LCH, affecting 2 or more organ systems and “risk organs,” like the hematopoietic system, the spleen, liver, and central nervous system, implies a worse prognosis. 1; 3; 4; 5 ; 6 To raise the awareness of multisystemic LCH, the case of an elderly patient is presented and a new therapeutic scheme with pioglitazone (peroxisome proliferator–activated receptor-γ [PPAR-γ] agonist), etoricoxib (COX-2 inhibitor), and trofosfamide (alkylating medium) is described

Kyrle Disease and Acquired Perforating Collagenosis Secondary to Chronic Renal Failure and Diabetes Mellitus

A 59-year-old man with chronic renal failure and diabetes mellitus presented with pruritic crusted lesions which histologically were perforating disorders, showing features of both Kyrle disease and acquired perforating collagenosis. The mechanisms of transepidermal elimination and the classification of perforating disorders are briefly discussed. Additionally, we question the concept of perforation, as epidermal damage and exposure of subepidermal substances may artificially present as perforation

Congenital ichthyosiform erythroderma with epidermolysis due to a novel frameshift mutation in KRT10

Neonatal erythroderma (NE) is an erythema that covers at least 90% of the body surface and occurs at birth or in the first 4 postnatal weeks.1 The incidence of NE in patients in the Netherlands has been estimated by dermatologists to be 10 per 100,000 newborns.1 In congenital ichthyosiform erythroderma (CIE), the presence or absence of a collodion membrane and/or extracutaneous findings is crucial to narrow down possible differential diagnoses. Here, we describe a keratinopathic form of NE or CIE due to a mutation in the KRT10 gene. Additionally, 2 variants, 1 pathogenic and 1 of unclear significance, of FLG were detected

Impact of a Glycolic Acid-Containing pH 4 Water-in-Oil Emulsion on Skin pH

The skin pH is crucial for physiological skin functions. A decline in stratum corneum acidity, as observed in aged or diseased skin, may negatively affect physiological skin functions. Therefore, glycolic acid-containing water-in-oil (W/O) emulsions adjusted to pH 4 were investigated regarding their effect on normal or increased skin pH. A pH 4 W/O emulsion was applied on three areas with pathologically increased skin surface pH in diabetics (n = 10). Further, a 28-day half-side trial (n = 30) was performed to test the long-term efficacy and safety of a pH 4 W/O emulsion (n = 30). In summary, the application of a pH 4 W/O emulsion reduced the skin pH in healthy, elderly and diabetic subjects, which may improve epidermal barrier functions

Nodular Cutaneous Amyloidosis at the Temple

A 52-year-old woman presented with a large partially yellow and erythematous tumor on her right temple. She reported that it had grown over the last 4 years. Regional lymph nodes were impalpable. A punch biopsy showed eosinophilic material in the dermis and subcutis. Immunohistochemistry showed positive staining for kappa and lambda light chains. Electron microscopy showed the typical amyloid fibrils (7–10 nm in diameter). There was no evidence of systemic amyloidosis, paraproteinemia or underlying plasmacytoma. The tumor was completely removed via curettage. At follow-up, the patient presented in good health with no signs of relapse

Impact of Age and Body Site on Adult Female Skin Surface pH

Background: pH is known as an important parameter in epidermal barrier function and homeostasis. Aim: The impact of age and body site on skin surface pH (pH(SS)) of women was evaluated in vivo. Methods: Time domain dual lifetime referencing with luminescent sensor foils was used for pH(SS) measurements. pH(SS) was measured on the forehead, the temple, and the volar forearm of adult females (n = 97, 52.87 +/- 18.58 years, 20-97 years). Every single measurement contained 2,500 pH values due to the luminescence imaging technique used. Results: pH(SS) slightly increases with age on all three investigated body sites. There are no significant differences in pH(SS) between the three investigated body sites. Conclusion: Adult pH(SS) on the forehead, the temple and the volar forearm increases slightly with age. This knowledge is crucial for adapting medical skin care products. Copyright (C) 2012 S. Karger AG, Base

Review Intense Pulsed Light (IPL): A Review

Background: Intense pulsed light (IPL) devices use flashlamps and bandpass filters to emit polychromatic incoherent high-intensity pulsed light of determined wavelength spectrum, fluence, and pulse duration. Similar to lasers, the basic principle of IPL devices is a more or less selective thermal damage of the target. The combination of prescribed wavelengths, fluences, pulse durations, and pulse intervals facilitates the treatment of a wide spectrum of skin conditions. Objective: To summarize the physics of IPL, to provide guidance for the practical use of IPL devices, and to discuss the current literature on IPL in the treatment of unwanted hair growth, vascular lesions, pigmented lesions, acne vulgaris, and photodamaged skin and as a light source for PDT and skin rejuvenation. Methods: A systematic search of several electronic databases, including Medline and PubMed and the authors experience on intense pulsed light. Results: Numerous trials show the effectiveness and compatibility of IPL devices. Conclusion: Most comparative trials attest IPLs similar effectiveness to lasers (level of evidence: 2b to 4, depending on the indication). However, large controlled and blinded comparative trials with an extended follow-up period are necessary. Lasers Surg. Med. 42:93-104, 2010

Systemic therapy of necrobiotic xanthogranuloma: a systematic review

Background Even though a plethora of systemic therapies have been proposed for necrobiotic xanthogranuloma (NXG), there is no systematic review on this topic in literature. Objective To review all existing literature on the systemic therapy of NXG in order to identify the most effective therapies. Methods All reported papers in the literature were screened for systemic treatments of NXG. Papers without proper description of the therapies, papers describing topical therapy, and articles without assessment of effectiveness were excluded. Subsequently, we analyzed 79 papers and a total of 175 cases. Results The most effective treatments for NXG are intravenous immunoglobulins (IVIG), corticosteroids, and combination therapies including corticosteroids. Conclusions Corticosteroids and IVIG should therefore be considered first-line treatments in patients with NXG

Autofluorescent Imaging in Patients With Peritoneal Carcinomatosis

Background and objectives. Autofluorescence imaging (AFI) is mainly used to detect (pre)cancerous colorectal and pulmonal lesions. This is the first report establishing the feasibility of AFI in patients with peritoneal carcinomatosis (PC). Methods. This is a prospective analysis of 10 patients undergoing conventional white-light laparoscopy (WL) and AFI for PC of different gastrointestinal tumors and 1 ovarian cancer. Before taking biopsies, suspicious peritoneal lesions were first detected by WL and then investigated by AFI. The intraoperative findings were photographed and then correlated with histological results. Results. Conventional WL and AFI evaluation was successful in all patients. A total of 38 biopsies were taken. The neoplasm detection rate under WL was 66% and increased to 86% when using AFI. The positive tumor detection rate was slightly higher in low AF lesions (83 vs 88%) and higher in tumor nodules (94%) than in flat peritoneal lesions (75%). For tumor nodules, the sensitivity was 94%, and the specificity was 100%. For flat lesions, the sensitivity was 75% and specificity 50%. Conclusions. We demonstrate the feasibility and effectiveness of AFI in patients with PC

The Impact of 10% α-Hydroxy Acid Emulsion on Skin pH

Background/Aims: The effects of a 10% α-hydroxy acid (AHA) oil/water (O/W) emulsion on the pH of human skin surface (pHss) and stratum corneum (SC; pHsc) were evaluated in vivo. Methods: The AHA O/W emulsion was applied to an area on the volar forearm of male volunteers (n = 12), and then wiped off after 10 min. Prior to application and over the following 3 h, the pHss was measured. We used glass electrode measurements and time domain dual lifetime referencing (tdDLR) with luminescent sensor foils. In another experiment (n = 5), the impact of the AHA O/W emulsion on the pHsc gradient was measured by tape stripping of the SC of the volar forearm after application of the AHA O/W emulsion. Results: Compared with pHss values prior to treatment [5.2 ± 1.7 (tdDLR) or 4.8 ± 0.5 (electrode)], the pHss was significantly reduced 10 min after application [4.0 ± 0.3 (tdDLR) or 4.1 ± 0.1 (electrode)] and the pHss remained significantly reduced over the measurement period of 3 h [after 3 h: 4.4 ± 0.2 (tdDLR) or 4.5 ± 0.3 (electrode)]. The AHA O/W emulsion significantly reduced the pHsc even down to deep layers of the SC. Conclusion: After a 10-min application time, the 10% AHA O/W emulsion reduces the pHss (for at least 3 h) and pHsc in deep layers of the SC