界限类偏结核样型麻风病1例

患者男,38岁。右手腕皮疹伴麻木1年,加重1周。皮肤科检查见右手腕屈侧黄红色环形斑块。组织病理示真皮全层可见沿血管、神经和汗腺分布的类椭圆形结核样肉芽肿,以真皮下部为著。予氨苯砜和利福平联合化疗,现随访中。     

Histological evidence for skin rejuvenation using a combination of pneumatic energy,broadband light,and growth factor therapy

Abstract

Background and objective: A variety of laser or light-based devices have been developed for skin rejuvenation. This study evaluates the efficacy (at the microscopic level) of a combination of pneumatic energy, broadband light, and profusion tip-delivered epidermal growth factor (EGF). Methods: Healthy adult Japanese volunteers were recruited for this study. The posterior parts of the left and right arms were treated with a device that combines pneumatic energy and broadband light (Isolaz device). The left arms were also treated with EGF through a tip. Each subject received four treatments at 10-day intervals. Biopsy specimens obtained 3 weeks after the second treatment and 3 weeks after the fourth treatment were examined for histological study. Results: After two treatments, elastin fibers and fibroblasts in the dermal papillary layers of the left arms were increased compared to the right arms. A mean of 173.9 cells positive for proliferating cell nuclear antibody (PCNA) were observed on the left arm compared to 101.0 on the right arm. After the fourth treatment, angiogenesis and increased collagen fibers were observed in the dermal papillary layers of both arms. Conclusion: Four treatments with the addition of the profusion tip appear to hasten new collagen and elastin fiber formation, and the increase of PCNA positive cells so that dermal remodeling begins at an earlier stage than with the Isolaz alone.     

Recurrent bullous lesions associated with familial Mediterranean fever: a case report

Familial Mediterranean fever (FMF) is an inherited, recurrent, inflammatory disease. Of its various cutaneous features, erysipelas-like erythema is the best known and most common skin lesion. We present a new case of FMF with recurrent bullous lesions. A 41-year-old woman was admitted to our clinic with tense bullae, 20 × 20 mm in diameter on the left shin. The patient had a history of fever, abdominal pain, peritonitis attacks and infertility. A lesional skin biopsy revealed subepidermal bullae and neutrophilic infiltration around dermal vessels. Direct immunofluorescence analysis was negative. Over the period of investigation, the lesion regressed spontaneously; 1 month later, a similar lesion appeared on the right wrist. Diagnosis of FMF was made according to the Tel-Hashomer criteria. Recognition of this peculiar skin lesion may lead to an earlier diagnosis of the disease.     

Late-onset Ito's nevus: an uncommon acquired dermal melanocytosis

Dermal melanocytoses comprise a variety of congenital and acquired conditions characterized by a sparse population of intradermal dendritic, variably pigmented, spindle-shaped melanocytes. While Mongolian spot, Ota's and Ito's nevi are usually present at birth or appear around puberty; acquired dermal melanocytoses that appear in adult life are extremely rare. They include the facial lesions of acquired bilateral nevus of Ota-like macules, also named Hori's nevus, and the acquired unilateral nevus of Ota, also known as Sun's nevus. Uncommon extrafacial examples of acquired dermal melanocytoses include lesions involving upper extremities, wrist, back, lower extremities and dorsal aspects of the hands and feet. They are more prevalent among Asian women. In general, dermal melanocytoses are rare lesions in Caucasian patients and acquired variants are exceedingly uncommon. We report a rare example of acquired Ito's nevus that appeared in a Caucasian elderly woman and review the literature about acquired dermal melanocytoses.     

Unique skin changes in a case of Albright hereditary osteodystrophy caused by a rare GNAS1 mutation

Albright hereditary osteodystrophy (AHO) is a syndrome of short stature, obesity, brachydactyly and subcutaneous calcifications with pseudohypoparathyroidism (PHP; leading to hypocalcaemia, hyperphosphataemia and elevated levels of parathyroid hormone, PTH). It was first described over 60 years ago. Since then, much has been learned about the aetiology of AHO which has been shown to be caused by heterozygous loss‐of‐function mutations within the GNAS1 gene. GNAS1 is subject to imprinting leading to phenotypic heterogeneity within kindreds with one mutation. Patients with AHO often present with symptoms of hypocalcaemia and/or with subcutaneous calcifications. The latter is thought to be the typical skin abnormality in AHO. We describe a family with AHO and hormone resistance (PHP type Ia) resulting from a rare mutation in GNAS1. The proband presented with small subcutaneous calcifications in the helix of the right ear and concentrated in a sharply demarcated zone of subcutaneous and dermal hypoplasia. This abnormality has so far not been described in patients with AHO. We speculate on the mechanism of dermal hypoplasia and resistance to PTH and suggest that subcutanous or dermal hypoplasia might be another feature which can be present in patients with AHO.     

Agminated blue nevus with a GNAQ mutation: A case report and review of the literature

Agminated blue nevi are dermal melanocytic proliferations that classically present as dark blue macules or papules in a grouped, linear, or blaschkoid distribution. In their more common sporadic form, blue nevi manifest in young adulthood as solitary blue papules or macules on the scalp, face, hands, or feet. By contrast, agminated blue nevi tend to manifest earlier in life, and are distributed more evenly across anatomic sites. Recent studies have identified mutations in sporadic blue nevi in the genes encoding G Protein subunit alpha Q and G protein subunit alpha 11 (GNAQ and GNA11). It is unknown whether agminated blue nevi share the same genetic changes. In the present paper, we present a case of agminated blue nevus on the wrist, and identify an activating mutation (c.626A > T, p.Glu209Leu) in GNAQ. We hypothesize that GNAQ/GNA11 activating mutations arising earlier during development may trigger agminated blue nevi, explaining the broader field of involvement in these cutaneous lesions.     

Rupioid psoriasis with arthropathy

A 31-year-old male presented with limpet-like, cone-shaped skin lesions on the scalp, the extremities, and the trunk of 4 months' duration, and had had severe joint pain in his right fingers, wrist, and knee for 1 month. Radiological examination revealed arthritis of the above mentioned multiple joints. Rheumatoid factor was serologically negative. Histopathological findings showed dense inflammatory cell infiltration and remarkable Munro's microabscesses in the horny layer in addition to psoriasiform epidermal hyperplasia, and predominant dermal oedema. Based on clinicopathological findings, a diagnosis of rupioid psoriasis with arthropathy was made. Following treatment for arthralgia using low doses of systemic steroid, the effects of cyclosporin combined with topical steroids was seen to alleviate dramatically the skin lesions and arthritis within 2 weeks. There was no recurrence of such skin and joint lesions during a follow-up period of 1 year.     

Focal Dermal Hypoplasia Due to a Novel Mutation in a Boy with Klinefelter Syndrome

A boy was born with multiple anomalies, including right hemifacial microsomia, eye abnormalities, syndactyly, right hand ectrodactyly, hypoplastic nails, omphalocele, bladder exstrophy, renal dilatation, and splayed symphysis pubis. The skin was also abnormal, with atrophic skin plaques and areas of telangiectasia along the lines of Blaschko. The karyotype was 47,XXY (Klinefelter syndrome). He was found to have a heterozygous mutation in the PORCN gene. He exhibited the classical features of focal dermal hypoplasia. Fewer than 15% of reported cases are male when it is thought to be due to postzygotic mutation and thus mosaic. This is the first reported boy to have heterozygous mutation for Goltz syndrome who survived due to the extra X chromosome.     

Restorative effect of hair follicular dermal cells on injured human hair follicles in a mouse model

No model is available for examining whether in vivo‐damaged human hair follicles (hu‐HFs) are rescued by transplanting cultured hu‐HF dermal cells (dermal papilla and dermal sheath cells). Such a model might be valuable for examining whether in vivo‐damaged hu‐HFs such as miniaturized hu‐HFs in androgenic alopecia are improvable by auto‐transplanting hu‐HF dermal cells. In this study, we first developed mice with humanized skin composed of hu‐keratinocytes and hu‐dermal fibroblasts. Then, a ‘humanized scalp model mouse’ was generated by transplanting hu‐scalp HFs into the humanized skin. To demonstrate the usability of the model, the lower halves of the hu‐HFs in the model were amputated in situ, and cultured hu‐HF dermal cells were injected around the amputated area. The results demonstrated that the transplanted cells contributed to the restoration of the damaged HFs. This model could be used to explore clinically effective technologies for hair restoration therapy by autologous cell transplantation.     

In vivo measurement of the water content in the dermis by confocal Raman spectroscopy

Background/purpose: Dermal water plays an important role in the physical properties of the skin. Recently, researchers have attempted to directly measure the dermal water content in vivo using magnetic resonance imaging, near infrared spectroscopy, and Raman spectroscopy. However, these methods have limitations. Although confocal Raman spectroscopy has been developed to measure the water content in the skin, no reports have suggested that this instrument can measure the dermal water content. This report describes a method for measuring the dermal water content in vivo using confocal Raman spectroscopy. Methods: We used a confocal Raman spectrometer and adjusted the laser exposure time and depth increments according to the skin depth. Age‐related changes in the dermal water content of the forearm were examined in 30 young and 30 elderly male subjects. Diurnal changes in the dermal water content of the forearm were examined in 12 elderly male subjects. Results: Adjusting the exposure time and depth increment dramatically improved the signal‐to‐noise ratios of the Raman spectra. Elderly dermis had significantly higher water content than young dermis. Moreover, the dermal water content displayed a diurnal change. Conclusion: This study demonstrates that the dermal water content can be measured in vivo using confocal Raman spectroscopy.     

Keratinocytes express fibrillin and assemble microfibrils: implications for dermal matrix organization

Fibrillin–containing microfibrils are key architectural structures of the upper dermis and integral components of the dermal elastic fibre network. Microfibril bundles intercalate into the dermal—epithelial junction and provide an elastic connection between the dermal elastic fibre network and the epidermis. Immunohistochemical studies have suggested that they are laid down both at the dermal—epithelial junction and in the deep dermis. While dermal fibroblasts are responsible for deposition of the elastin and microfibrillar components that comprise the elastic fibres of the deep dermis, the cellular origin of the microfibril bundles that extrude from the dermal—epithelial junction is not well defined. We have used fresh tissues, freshlyisolated epidermis and primary human and porcine keratinocyte cultures to investigate the possibility that keratinocytes are responsible for deposition of these microfibrils. We have shown that keratinocytes in vivo and in vitro synthesize both fibrillin-1 and fibrillin-2, and assemble beaded microfibrils concurrently with expression of basement membrane collagen. These observations suggest that keratinocytes co-ordinate the secretion, deposition and assembly of these distinct structural elements of the dermal matrix, and have important implications for skin remodelling.     

Collagen cross-linking in sun-exposed and unexposed sites of aged human skin

A recently described nonreducible, acid-heat stable compound, histidinohydroxylysinonorleucine (HHL), is a collagen cross-link isolated from mature skin tissue. Its abundance is related to chronologic aging of skin. The present communication describes the quantity of HHL from aged human skin of the same individuals in sun-exposed (wrist) and unexposed (buttock) sites. Punch biopsies were obtained from these sites from nine people of age 60 or older. HHL contents (moles/mole of collagen) at these sites were for wrist 0.13 +/- 0.07 and for buttock 0.69 +/- 0.17 (mean +/- SD, p less than 0.001). In addition, it was found that acute irradiation of the cross-linked peptides with UVA (up to 250 J/cm2) and UVB (up to 1 J/cm2) had no effect on HHL structure. The same treatment significantly degraded another nonreducible, stable collagen cross-link, pyridinoline. The results suggest that chronic sunlight exposure may be associated with an impediment to normal maturation of human dermal collagen resulting in tenuous amount of HHL. Thus, the process of photoaging in dermal collagen is different from that of chronologic aging in human skin.     

A case of intraepidermal Merkel cell carcinoma within squamous cell carcinoma in-situ: Merkel cell carcinoma in-situ?

We report a case of a 79-year-old Caucasian male who presented with a wrist lesion of combined intraepidermal Merkel cell carcinoma and squamous cell carcinoma in-situ. The two tumors were tightly admixed and distinct, and both were without any dermal or invasive components. No features of transition between the two tumors were seen. We suggest the term Merkel cell carcinoma in situ for tumors that demonstrate exclusive intraepidermal proliferation of neuroendocrine cells.     

Wirksamkeit,Nebenwirkungen und Patientenzufriedenheit bei Leitungsanästhesie durch Handblock vor Behandlung der palmaren Hyperhidrose mit Botulinumtoxin A

Background: The treatment of palmar hyperhidrosis with botulinum toxin type A (BTX A) injections is one of the most effective therapeutic options. However, without adequate anaesthesia this treatment is not feasible in most patients. The anaesthesia of the radial, median and ulnar nerves (wrist block) is despite the absence of good data considered effective but risky. Patients and methods: Between 1. 10. 1999 and 30. 9. 2001 retrospectively the efficacy, side effects and the patient satisfaction regarding the overall performance of the wrist block prior to the treatment of the palmar hyperhidrosis with BTX A was investigated. The study took place at the department of dermatology in Mannheim. Results: Of the 38 patients treated, 33 were finally included into the trial. The patients chose the average score of 7,9 ± 2,1 (sd) on a scale from “0” to “10” (not at all effective‐maximally effective) for the efficacy of the wrist block in reducing pain. The patients were very content with the wrist block and granted it the average score of 7,7 ± 2,1 (sd) on a scale from “0” to “10” (not at all content‐maximum content). Unwanted effects could be seen in 31/33 patients (93,9 %): these were dizziness, headache or haematoma at the spot of injection as well as pain caused by the injections of the local anaesthesia or paresthesias such as tickling of the hand or arm. All side effects caused by the wrist block were reversible and lasted – except in one patient – only for a short time. 29/33 patients (87,9 %) recommended the wrist block prior to treating palmar hyperhidrosis with BTX A and 28/33 patients (84,9 %) could not imagine to agree to such a treatment without local anaesthesia. Conclusion: The wrist block effectively reduces pain due to the treatment of palmar hyperhidrosis with BTX A. Major side effects are rare; patient acceptance and satisfaction are high.     

Influence of subcutaneous adipose tissue mass on dermal elasticity and sagging severity in lower cheek

Background: Subcutaneous adipose tissue lies just beneath the dermal layer, but the interaction between the two is not well understood. Recently, we showed that the subcutaneous adipose layer affects dermal properties in an obese rodent model. Objective: To explore the influence of the subcutaneous adipose layer on dermal properties and morphology in humans. Methods: Subcutaneous adipose mass was measured by ultrasound using a Prosound alpha 5^®, dermal elasticity was measured using an in vivo suction skin elasticity meter (Cutometer MPA 580^®), and sagging severity were evaluated morphologically using photograph‐based grading criteria at the lower cheek in 70 Japanese female volunteers (age; 31–59, BMI; 17.1–36.2). The correlations among the results were examined. Results: Thickness of the subcutaneous adipose layer was significantly and negatively correlated with dermal elasticity parameters, i.e., elastic deformation, elastic deformation recovery, extensibility, total deformation recovery, ratio of viscoelasticity to elastic distention and overall elasticity, including creep and creep recovery. Furthermore, we investigated their influence on facial morphology, such as sagging. The subcutaneous adipose mass was significantly and positively correlated with sagging severity at the lower cheek (R=0.442, P<0.001), and there was a significant negative correlation between dermal elasticity and sagging severity (R=?0.358, P<0.01). Conclusion: These results indicate that increment of subcutaneous adipose mass impairs dermal elasticity, which in turn exacerbates sagging severity.     

Curative effects of microneedle fractional radiofrequency system on skin laxity in Asian patients: A prospective,double-blind,randomized, controlled face-split study

Background: To date, no studies compared curative effects of thermal lesions in deep and superficial dermal layers in the same patient (face-split study). Objective: To evaluate skin laxity effects of microneedle fractional radiofrequency induced thermal lesions in different dermal layers. Methods and Materials: 13 patients underwent three sessions of a randomized face-split microneedle fractional radiofrequency system (MFRS) treatment of deep dermal and superficial dermal layer. Skin laxity changes were evaluated objectively (digital images, 2 independent experts) and subjectively (patients’ satisfaction numerical rating). Results: 12 of 13 subjects completed a course of 3 treatments and a 1-year follow-up. Improvement of nasolabial folds in deep dermal approach was significantly better than that in superficial approach at three months (P=.0002) and 12 months (P=.0057) follow-up. Effects on infraorbital rhytides were only slightly better (P=.3531). Conclusion: MFRS is an effective method to improve skin laxity. Thermal lesion approach seems to provide better outcomes when applied to deep dermal layers. It is necessary to consider the skin thickness of different facial regions when choosing the treatment depth.     

Compound clear cell sarcoma misdiagnosed as a Spitz nevus

Clear cell sarcoma (CCS) typically presents as a tumor in the deep soft tissue of extremities, but when centered in the dermis it may be confused with a melanocytic nevus, primary nodular or metastatic melanoma. Compound variants of CCS, i.e. tumor cells present in both the epidermis and underlying soft tissue have not yet been described. Herein we report such a case, which initially presented as a nodule on the left wrist of a young woman at 19 years of age. The lesion was then interpreted as ‘Spitz nevus, compound type’. Twelve years later the patient noticed an enlarged lymph node in the right axilla. The excised lymph node was nearly completely replaced by malignant tumor cells, which were immunoreactive for S100 protein. They resembled the tumor cells of the wrist lesion. Cytogenetic analysis of the metastatic tumor revealed a t(12;22) translocation. Fluorescence in situ hybridization confirmed Ewing's sarcoma breakpoint region 1 (EWSR1) rearrangement in 70% of the tumor cells, thereby supporting the diagnosis of metastatic CCS. Our case is of interest because it documents that CCS can involve the epidermis. This observation expands the morphological spectrum associated with this tumor.     

A potential contribution of altered cathepsin L expression to the development of dermal fibrosis and vasculopathy in systemic sclerosis

Cathepsin L (CTSL) is a lysosomal proteolytic enzyme involved in inflammation and vascular and extracellular matrix remodelling, which are the three cardinal pathological events associated with systemic sclerosis (SSc). To elucidate the potential role of CTSL in the development of SSc, we here investigated CTSL expression in the lesional skin of patients with SSc and SSc animal models and the clinical correlation of serum CTSL levels. CTSL expression was elevated in dermal small vessels of SSc patients compared with those of healthy controls. Consistently, CTSL mRNA levels were increased in SSc lesional skin samples, but not in cultivated SSc dermal fibroblasts, compared with corresponding control samples from healthy individuals. Serum CTSL levels were significantly higher in SSc patients than in healthy controls and inversely correlated with skin score. Furthermore, the elevation of serum CTSL levels was linked to SSc vasculopathy. Supporting these results, Ctsl mRNA levels were decreased in the skin of bleomycin‐treated mice, an SSc animal model recapitulating its fibrotic aspect, and CTSL expression was enhanced in dermal small vessels of endothelial cell‐specific Fli1 knockout mice, reminiscent of SSc vasculopathy. Importantly, gene silencing of FLI1 induced CTSL mRNA expression and Fli1 occupied the CTSL promoter in human dermal microvascular endothelial cells. Collectively, these results suggest that endothelial CTSL up‐regulation partially due to Fli1 deficiency may contribute to the development of vasculopathy, while the decrease in dermal CTSL expression is likely associated with dermal fibrosis in SSc.     

Quantitative echographic analysis of photochemotherapy on systemic sclerosis skin

Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology, in which development of fibrosis, vascular insufficiency and inflammatory processes are prominent in the skin as well as in other organs. We studied the effect of photochemotherapy with quantitative echography in SSc. Dermal echo intensity and dermal thickness was measured using high-frequency dermal echography before and after therapy. The dermal echo intensity after photochemotherapy (33.51±9.34) significantly increased than that before therapy (21.23±6.00) (P<0.01), while dermal thickness (1.20±0.20) significantly decreased than that before therapy (1.38±0.18) (P<0.05). Photochemotherapy was more likely to improve dermal edema, not fibrosis, because echo intensity after treatment was significantly elevated with that before treatment in patients with edema. Quantitative echographic analysis was concluded to be a reliable method in evaluating the change of skin edema in SSc.     

A hypothesis on the cause of chronic epidermal hyperproliferation in asebia mice

We compared the skin of the mutant mouse asebia (ab/ab) with that of normal BALB/c mice by examining dermal cell counts, epidermal thickness, epidermal cell labelling index and ultrastructure. In asebia we found epidermal hyperproliferation and dermal inflammation, with much cell debris in the dermis. The very numerous macrophages, both intact and disintegrating, contained electron-lucent crystals, apparently composed of lipid. We hypothesize that these crystals cause macrophage lysis followed by dermal inflammation and finally hyperproliferation in the overlying epidermis.