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Dermatological manifestations of Down's syndrome.

Down's syndrome (DS) is associated with rare dermatological disorders and increased frequency of some common dermatoses. Owing to advances in medical care and changes in attitude, the median age of death in this population has increased to 49 years, and the life expectancy of a 1-year-old person with DS today is more than 60 years and is likely to improve. With the increase in the number of individuals with DS in the population and an increased life span, dermatologists are more likely to encounter the wide spectrum of dermatological disorders that occurs in these patients. Furthermore, new reports of possible associations are frequent in the literature. The purpose of this article is to discuss the various dermatological conditions that affect DS individuals. A brief overview is given of the new information on genetics and the immunology of DS. We also discuss the molecular mechanisms of premature ageing, to which DS individuals are prone. We review the literature and discuss the known dermatological manifestations, concentrating on recent reports.

Adolescent↗

Dermatologic radiotheraphy: selection of radiation qualities and treatment techniques.

The penetration of x-rays should be correlated the depth of the pathological process. The introduction of the "half-value depth" (D1/2) as a guidline in the selection of radiation qualities has greatly simplified cutaneous radiotherapy. Modern dermatologic x-ray units with beryllium windows operated between 10 and 50 (or 100 kV, offer the widest spectrum of radiation qualities suitable for dermatologic therapy, although older types of radiation equipment are still useful for most dermatology indications. Previous risks in dermatologic radiation therapy have been reduced to a large degree by the introduction of new sophisticated safety devices and by a deliberate reduction of unnecessary variations in the selection of physical factors. Beryllium window machine can be operated without changing milliamperage, target-skin distance or dose rate for different indications. The recommended standardization of physical factors offers a safer and easier alternative to previous techniques.

Humans↗

Preliminary, dermatologic first step criteria for lupus erythematosus and second step criteria for systemic lupus erythematosus.

BACKGROUND: Comparisons of cases of systemic lupus erythematosus (SLE) with cases of rheumatoid arthritis and other rheumatologic disorders affords the basis of the 1982 revised criteria of the American Rheumatism Association (ARA) for classifying SLE cases. We address three questions: Do comparisons of LE cases with non-LE cases that have suggestive skin lesions yield criteria for use in dermatology clinics for primary classification of cases with photo distributions of skin lesions? Do comparisons of SLE with cutaneous LE cases yield the same or similar criteria to the revised ARA criteria for SLE? How should subacute cutaneous LE cases be evaluated for signs of significant systemic involvement? METHODS: Discriminant analyses on 168 cases with skin lesions suggestive of LE were performed using data based on the ARA criteria for SLE and study factors for cutaneous LE suggested by the European Academy of Dermatology and Venereology. RESULTS: These yielded two sets of criteria: (1) The 11 preliminary, dermatologic first step criteria (10 plus 1 for discoid lesions and histology) serve to classify cases as LE or non-LE. (2) The 11 preliminary, dermatologic second step criteria classify LE cases as cutaneous LE or systemic LE. Interestingly, 5 of 11 of these second step criteria differ from the 11 ARA criteria for systemic LE. These second step criteria afford a useful means of distinguishing between subacute cutaneous LE cases with or without significant systemic involvement. CONCLUSIONS: The study factors included in both the first and the second step criteria fall into three groups, notably clinical criteria, laboratory criteria, and "added study factors." The latter factors distinguish between the groups compared (LE vs. non-LE and cutaneous vs. systemic LE) but not as well as the study factors included as "criteria."

Humans↗

Skin cancer and quality of life: assessment with the Dermatology Life Quality Index.

BACKGROUND: Quality of life (QOL) has been identified as an important outcome in cancer research, yet the most common malignancy among humans, nonmelanoma skin cancer (NMSC), has been poorly studied in this regard. OBJECTIVE: To determine whether change occurred in the QOL of NMSC patients after surgery using a general, validated dermatology QOL instrument: the Dermatology Life Quality Index (DLQI). METHODS: A prospective study was conducted on 121 consecutive patients referred to a dermatologic Mohs surgery clinic with NMSC of the head and neck. QOL assessment was performed using the DLQI before (n=121) and after surgical treatment at 4 months (n=101). RESULTS: QOL scores demonstrated little handicap at initial diagnosis. The total DLQI scores showed little change over time, but an item analysis revealed that 2 of the 10 items demonstrated statistically significant change over time, with QOL improving after treatment-decreased painfulness/itchiness/soreness and less necessity to use concealing clothing. CONCLUSIONS: General dermatology QOL instruments demonstrated minimal handicap at initial diagnosis and little change after treatment of NMSC. Although the associations were modest, improvement in some aspects of well-being after treatment of NMSC was demonstrated. A more disease-specific instrument may be necessary to study this disease process further.

Aged↗

A comparison of the concordance of digital images, live examinations, and skin biopsies for the diagnosis of hospitalized dermatology consultation patients.

OBJECTIVE: The goal of this study was to compare live and digital image examination of hospitalized dermatology consultation patients. DESIGN: A medical student digitally imaged patients with a Nikon Coolpix 950 digital camera (Nikon Corp., Tokyo, Japan). A resident reviewed these images and the student recorded the differential diagnosis. Digital examination was followed by live dermatologic examination by the dermatology resident and then by a supervising board certified dermatologist. The diagnoses in 29 of these cases were compared with definitive diagnostic tests (e.g., skin biopsy). SETTING: Montefiore Hospital, Bronx, NY Patients. In March, 2000, 48 of 58 consecutive hospitalized patients for whom dermatology consultations were requested and from whom consent for photographs could be obtained. MAIN OUTCOME MEASURES: The accuracy and consistency of the examiners top (up to three) digital diagnoses, (up to three) live diagnoses, and histopathologic diagnoses were compared. RESULTS: Eighty-one percent (38/47) of resident digital consult diagnoses agreed with live resident diagnoses. Live resident diagnosis agreement with live supervising board certified dermatologists diagnosis was 90% (18/20). Resident digital image diagnosis correlation with definitive diagnostic test results was 76% (22/29). Live supervising board certified dermatologist diagnosis correlation with definitive diagnostic test results was 73% (14/19). CONCLUSIONS: Digital image review results in reliable and accurate diagnosis compared to live consultations. For inpatient consultations, three-dimensional lesions are more amenable to digital diagnosis than flat, generalized rashes.

Biopsy↗

Dermatological manifestations in primary biliary cirrhosis patients: a case control study.

OBJECTIVES: Primary biliary cirrhosis (PBC), a disease of probable autoimmune etiology that affects the small intrahepatic bile ducts of mainly middle-aged women is commonly associated with pruritus, xanthomatous lesions, and melanosis. We conducted a prospective study to systematically describe the skin disorders of a group of PBC patients. METHODS: A prospective evaluation and analysis of dermatological manifestations including oral and genital lesions was carried out, in 49 PBC patients (45 females and 4 males). Median age 63 yr (range 35-87 yr). They were compared with 45 age and sex matched controls, selected among persons attending the dermatologic outpatient clinic. RESULTS: A total of 330 skin disorders were found in the 49 PBC patients versus 76 in the 45 controls; 31.5% of all lesions were skin fungal infections. Of all lesions analyzed with the Bonferroni rule of multiple comparisons significantly more common in PBC patients were plantar mycoses, onychomycoses, and interdigital mycoses. Pruritus was found in 69.3% of patients versus 22.2% of controls, xerosis in 69.3%versus 2.2%, dermographism in 57.1%versus 4.4%, and melanosis in 46.9%versus 0%. In 38.7% of the PBC patients the dermatologic lesion was the presenting symptom. CONCLUSIONS: PBC patients present with a wide variety of cutaneous manifestations varying in severity. Multiple skin fungal infections have been found even in the early stages. Since in more than one third of our PBC patients the dermatologic lesion was the presenting sign or symptom leading to diagnosis we believe that physicians should be aware so that a prompt and early diagnosis may be achieved.

Adult↗

Multicentre randomised control trial comparing real time teledermatology with conventional outpatient dermatological care: societal cost-benefit analysis.

OBJECTIVES: Comparison of real time teledermatology with outpatient dermatology in terms of clinical outcomes, cost-benefits, and patient reattendance. DESIGN: Randomised controlled trial with a minimum follow up of three months. SETTING: Four health centres (two urban, two rural) and two regional hospitals. SUBJECTS: 204 general practice patients requiring referral to dermatology services; 102 were randomised to teledermatology consultation and 102 to traditional outpatient consultation. MAIN OUTCOME MEASURES: Reported clinical outcome of initial consultation, primary care and outpatient reattendance data, and cost-benefit analysis of both methods of delivering care. RESULTS: No major differences were found in the reported clinical outcomes of teledermatology and conventional dermatology. Of patients randomised to teledermatology, 55 (54%) were managed within primary care and 47 (46%) required at least one hospital appointment. Of patients randomised to the conventional hospital outpatient consultation, 46 (45%) required at least one further hospital appointment, 15 (15%) required general practice review, and 40 (39%) no follow up visits. Clinical records showed that 42 (41%) patients seen by teledermatology attended subsequent hospital appointments compared with 41 (40%) patients seen conventionally. The net societal cost of the initial consultation was pound132.10 per patient for teledermatology and pound48.73 for conventional consultation. Sensitivity analysis revealed that if each health centre had allocated one morning session a week to teledermatology and the average round trip to hospital had been 78 km instead of 26 km, the costs of the two methods of care would have been equal. CONCLUSIONS: Real time teledermatology was clinically feasible but not cost effective compared with conventional dermatological outpatient care. However, if the equipment were purchased at current prices and the travelling distances greater, teledermatology would be a cost effective alternative to conventional care.

Adolescent↗

Survey of the prevalence, diagnosis and treatment of dermatological conditions in small animals in general practice.

A survey was made of the prevalence, diagnosis and treatment of dermatological conditions in small animals in general practice in the UK. Out of 3707 small animal consultations in general practice that were observed and recorded, 795 (21.4 per cent) involved animals that had a dermatological problem. In dogs and exotic species, pruritus was the most common presenting sign, accounting for 30 to 40 per cent of the dermatological consultations. In cats, cutaneous swellings were the most common presentation (36 per cent). A diagnosis or recommendation for treatment was made on the basis of the presenting clinical signs and physical examination alone in 576 (72 per cent) of the cases, and various diagnostic tests were performed in the other cases. In dogs, parasitic infestations, bacterial infections and neoplasia accounted for the majority of the diagnoses. In cats, parasites and bacterial infections were the most common. In exotic species, parasites accounted for over 80 per cent of the dermatological diagnoses. In dogs, the most common final diagnoses were otitis, pyoderma, anal sac impaction, flea infestation and atopic dermatitis. In cats, abscesses, flea infestation, and otitis were the most common diagnoses. In exotic species, the most common diagnosis was an unspecified mite infestation. Systemic antibiotics were prescribed in 196 cases (25 per cent), systemic glucocorticoids were prescribed in 162 cases (20 per cent) and treatment with an ectoparasiticide was prescribed in 167 cases (21 per cent).

Animals↗

Dermatological and ophthalmological sequels in toxic epidermal necrolysis.

BACKGROUND: Toxic epidermal necrolysis (TEN) is a rare, drug-induced disease characterized by epidermal detachment and mucosal involvement. After an acute period, potentially disabling cutaneous and ocular sequels may appear. Although long-term complications are not rare, only few outcome studies are published. OBJECTIVE: To evaluate the incidence of dermatological and ophthalmological sequels following TEN, to describe its clinical aspects and correlation with acute involvement. PATIENTS AND METHODS: Eight patients surviving to TEN were submitted to dermatological and ophthalmological observation ranging from 0.5 to 8 years after hospitalization. Cutaneous and ocular involvement, during the acute phase, was retrospectively analysed. RESULTS: Dermatological sequels were observed in 6 patients (75%) corresponding to those with more extensive skin involvement in the acute phase. The most frequent complications were cutaneous dyschromia (62.5%) and nail dystrophies (37.5%). Six patients (75%) had ocular complications with tarsal conjunctiva keratinization in 5 (62.5%) and keratoconjunctivitis sicca in 4 of them (50%). Trichiasis, corneal neovascularization and symblepharon were observed in 1 case. There was no correlation between the severity of acute ocular involvement and long-term complications. CONCLUSION: Following TEN, most patients have dermatological and ophthalmological sequels that persist for several years.

Adolescent↗

How many GP referrals to dermatology outpatients are really necessary?

In a survey of patients referred to the dermatology outpatients department of a British teaching hospital, 26% of referrals were considered unnecessary by a senior house officer with three months practical dermatological experience. We conclude that better undergraduate and postgraduate education in dermatology is essential. A period spent in dermatology should be included in all vocational training schemes for general practice.

Ambulatory Care↗

Psychiatric symptoms in dermatology patients.

The 30-item General Health Questionnaire (GHQ) (Goldberg, 1972) was administered to 196 consecutive new dermatology out-patients and 40 consecutive admissions to dermatology beds. Thirty per cent of the out-patients and 60 per cent of the in-patients obtained high scores, while half the high scorers in each group scored high on the Wakefield Self-Assessment Depression Scale (Snaith et al, 1971). These findings suggest that dermatology out-patients have a higher prevalence of psychiatric disorder than the general population, and dermatology in-patients a higher prevalence than general medical in-patients. High GHQ scores were associated with (a) diagnoses of acne, eczema, psoriasis or alopecia; with (b) extensive lesions on exposed parts of the body; and with (c) the use of high potency topical steroid. We indicate other areas that might be profitably explored in a full-scale study.

Adolescent↗

The present status of anti-inflammatory agents in dermatology.

Many classes of drugs exert anti-inflammatory activity through mechanisms which affect all or part of the inflammatory process. Some of these agents are beneficial in the practice of dermatology, while others, such as penicillamine, mast cell blockers and serotonin antagonists, find little or no application. Corticosteroids, for example, are nonspecific in their anti-inflammatory effects and remain a mainstay of therapy, despite their side effect profile. Other drugs, such as the non-steroidal anti-inflammatory agents or gold, can be used in the treatment of diseases associated with rheumatic or autoimmune states. Moreover, antihistamines play an important role in the control of itching, but are mainly indicated in controlling non-dermatological allergic sequelae. Interestingly, chloroquine and dapsone, which were originally developed for use in malaria prophylaxis and leprosy, respectively, have value in treating a wide range of dermatological conditions via mechanisms which include the inhibition of P-450 isoenzymes. In diseases characterised by disturbed cornification (e.g. psoriasis pustulosa), retinoids are of particular value. These drugs are thought to act by inhibition of collagenases, proteases and granulocyte migration. Undoubtedly, further investigation of drug classes such as oxygen radical controllers and immunomodulators will clarify their mechanisms and establish their therapeutic usefulness among the anti-inflammatory agents now available for dermatological use.

Administration, Topical↗

Dermatologic side effects associated with gefitinib therapy: clinical experience and management.

Gefitinib (ZD1839, Iressa ), a selective inhibitor of the epidermal growth factor receptor-tyrosine kinase, is currently in clinical trials to treat a variety of solid tumors. Similar side effects were seen in numerous clinical trials of gefitinib, including recent phase II trials (Iressa Dose Evaluation in Advanced Lung Cancer [IDEAL]-1 and IDEAL-2) in patients with advanced, previously treated non-small-cell lung cancer (NSCLC). The most frequent drug-related adverse effects reported in these trials were diarrhea, dry skin, acneiform rash, and nausea and vomiting. Recently, IDEAL-2 investigators and oncology nurses participated in a panel discussion on the management of dermatologic adverse effects associated with gefitinib treatment. These dermatologic effects were related to the mechanism of action of gefitinib and were not caused by a drug-related allergic reaction. In IDEAL-2, investigators managed the symptoms of dry skin and rash with a variety of treatments, including topical clindamycin, antibiotics, and corticosteroids. Most patients had a high level of tolerance for the dermatologic effects associated with gefitinib, which were milder than toxicities associated with chemotherapy. The severity of skin effects cycled over time during treatment but typically subsided after several weeks of gefitinib treatment. In general, dermatologic effects of gefitinib were easily managed, reversible, and well tolerated by these patients with advanced NSCLC.

Journal Article↗

Dermatology precautions and slower titration yield low incidence of lamotrigine treatment-emergent rash.

OBJECTIVE: To assess treatment-emergent rash incidence when using dermatology precautions (limited antigen exposure) and slower titration during lamotrigine initiation. METHOD: We assessed rash incidence in 100 patients with DSM-IV bipolar disorder instructed, for their first 3 months taking lamotrigine, to avoid other new medicines and new foods, cosmetics, conditioners, deodorants, detergents, and fabric softeners, as well as sunburn and exposure to poison ivy/oak. Lamotrigine was not started within 2 weeks of a rash, viral syndrome, or vaccination. In addition, lamotrigine was titrated more slowly than in the prescribing information. Patients were monitored for rash and clinical phenomena using the Systematic Treatment Enhancement Program for Bipolar Disorder Clinical Monitoring Form. Descriptive statistics were compiled. RESULTS: No patient had serious rash. Benign rash occurred in 5 patients (5%) and resolved uneventfully in 3 patients discontinuing and 2 patients continuing lamotrigine. Two patients with rash were found to be not adherent to dermatology precautions. Therefore, among the remaining patients, only 3/98 (3.1%) had benign rashes. CONCLUSION: The observed rate of benign rash was lower than the 10% incidence in other clinical studies. The design of this study confounds efforts to determine the relative contributions of slower titration versus dermatology precautions to the low rate of rash. Systematic studies are needed to confirm these preliminary findings, which suggest that adhering to dermatology precautions with slower titration may yield a low incidence of rash with lamotrigine.

Adult↗

The effect of dermatologic precautions on the incidence of rash with addition of lamotrigine in the treatment of bipolar I disorder: a randomized trial.

OBJECTIVE: Prescribing recommendations specify that lamotrigine should ordinarily be discontinued at the first sign of rash, regardless of its type and severity, unless the rash is clearly not drug related. This practice helps to ensure that lamotrigine is discontinued in instances of serious rash (an event occurring in up to 0.13% of cases in bipolar clinical trials) but may lead to unnecessary discontinuation of lamotrigine for cases of nonserious rash arising from nondrug causes. Measures aimed at reducing overall occurrence of dermatologic reactions might reduce the incidence of nonserious rash leading to premature lamotrigine discontinuation. This study assessed the impact of specific instructions designed to decrease risk of dermatologic reactions, including nonserious rash, during initiation of and early treatment with lamotrigine in patients with bipolar I disorder. METHOD: Outpatients with DSM-IV-diagnosed bipolar I disorder >/= 13 years of age at 188 sites were randomly assigned to receive Usual Care Precautions (UCP; precautions from the patient instructions in the prescribing information for reducing risk of rash including nonserious rash) or Dermatologic Precautions (DP; precautions as above [UCP] plus additional precautions intended to decrease risk of any dermatologic reaction including nonserious rash) during 12 weeks of adding open-label lamotrigine to concomitant medications. Patients with comorbid medical and psychiatric problems were not excluded unless, in the opinion of the investigators, these problems were sufficiently severe to preclude participation. Investigators and patients were blinded to which precaution group patients were randomly assigned. The primary outcome measure was the rate of rash during the treatment period. Secondary outcome measures included clinical response to lamotrigine, assessed with the investigator- and self-rated Clinical Global Impressions-Bipolar version (CGI-BP) and the Clinical Global Impressions-Efficacy Index (CGI-EI). Data were collected from August 2003 to August 2004. RESULTS: 867 (74%) of 1175 patients completed the study. Only 182 (15%) of 1175 patients had an adverse event leading to discontinuation of study medication or withdrawal, including 62 (5.3%) of 1175 due to non-serious rash. No serious rashes were reported during the study in either group. The incidence of nonserious rash was similarly low in patients with UCP and DP (8.8% and 8.6%, respectively). CGI-BP-Severity and -Improvement scores indicated mood improvement when lamotrigine was added to existing therapy, and CGI-EI scores at weeks 5 and 12 reflected a favorable balance between control of mood symptoms and tolerability. At both weeks 5 and 12, investigators reported that therapeutic effects of additional lamotrigine outweighed side effects in 74% of subjects. CONCLUSION: UCP and DP yielded low, similar non-serious rash rates, which were marginally lower than nonserious rash rates in prior clinical trials that did not utilize DP but marginally higher than that in a prior open case series using DP. Nevertheless, the results are encouraging: in this large study reflecting real-world use, lamotrigine was well tolerated with no serious rash and low incidences of nonserious rash and discontinuation due to rash, and lamotrigine therapy was associated with clinical improvement in a heterogeneous cohort of patients with bipolar I disorder.

Adolescent↗

Neoprene splinting: dermatological issues.

Occupational therapists are expanding their use of custom and commercial soft splints fabricated from neoprene (polychloroprene), but little has been written regarding dermatological issues associated with this material. Skin contact with neoprene poses two dermatological risks: allergic contact dermatitis (ACD) and miliaria rubra (i.e., prickly heat). Allergic reaction to neoprene is generally ascribed to the accelerants used to manufacture the man-made rubber, specifically thiourea compounds and mercaptobenzothiazole (MBT). Symptoms of neoprene-related ACD include itching, skin eruptions, swelling, and hemorrhages into the skin. Miliaria rubra creates small, red, elevated, inflammatory papules and a tingling, burning sensation. Although neoprene hypersensitivity is rare, its incidence may grow as neoprene becomes a more commonly used material. It is recommended that therapists screen patients for a history of dermatological reactions to neoprene or other materials containing thiourea compounds or MBT and educate patients to discontinue splint use if dermatological symptoms develop. Therapists are also encouraged to notify splint manufacturers regarding all ACD reactions.

Adult↗

Dermatologic surgery into the next millennium: Part I.

Dermatologic cosmetic surgery is a subspecialty of dermatology that has recently been developed. In this four-part series, we describe many of the procedures performed in our ambulatory surgery setting. We also highlight the significant changes in the techniques and treatment environments for these procedures over the past three decades. In part one, we focus on liposuction and face lift operations. Patients increasingly expect less invasive procedures, decreased "down time," and superior results. These expectations create a greater demand for the dermatologic surgeon. The trends and prospects for dermatologic cosmetic surgery in the next millennium are illustrated.

Esthetics↗

Ultrasound in dermatology. Part II. Ultrasound of regional lymph node basins and subcutaneous tumours.

Ultrasound in dermatology is a non-invasive tool for the detection and verification of tumours in skin, subcutaneous tissues and in lymph node basins. Since the introduction of ultrasound examination in the routine practice of dermatology in the late seventies [1], ultrasound has evolved to become a standard diagnostic method, at least in Europe. Subcutaneous or regional nodal metastases or benign tumours or tissue accumulation may not be palpable due to small size, distance from the skin surface or location in an area of postoperative or radiation fibrosis, which renders a physical examination on its own difficult. Palpation even by an experienced physician is also known to be an inaccurate technique for the assessment of lesions in head and neck cancer [2] as well as in scar tissue. Ultrasound, on the contrary is able to give additional and reliable information about the exact position of a tumour, surrounding anatomical structures, the dimension of the lesion in two perpendicular diameters in mm, its echo pattern as well as the distance from the skin surface. High resolution ultrasound technique is not only able to detect small targets (< 3 mm), but also to predict difficulties in the surgical search for metastases due to a deep subcutaneous or intramuscular position, or a localization near structures (e.g. vessels) which could easily be traumatized. Several invasive and non-invasive staging tools have been developed for the detection of regional disease or tumours in the soft tissue to support the often ineffective physical examination on its own. Among these techniques, ultrasound has been proven to have superior sensitivity to physical examination in the detection of regional metastases in melanoma patients [3-6] as well as in hematology and oncology patients [7] and it even facilitates the surgical management of such patients [8-10]. Based on technical equipment and clinical requirements, ultrasound used in dermatology is performed using frequencies between 7.5 and 15 MHz. Additionally duplex and colour flow analysis may complement grey-scale ultrasound by demonstrating tumour vascularity and characterising masses [11, 12]. In this part of the two CME articles on ultrasound we want to illustrate the most important technical details, procedural steps and the clinical use of high-frequency ultrasound in dermatology.

Humans↗