Low rates of neurological abnormalities in patients with pigmentary mosaicism: A retrospective cohort study from a tertiary dermatology center.

BACKGROUND: Pigmentary mosaicism (PM) is a descriptive term encompassing a range of hyper- and hypo-pigmented phenotypes in various patterns. Information from the neurology literature initially noted neurological abnormalities (NA) in up to 90% of children with PM. The dermatology literature suggests lower associated rates (15%-30%) of NA. Variations in terminology, inclusion criteria, and small population sizes makes interpreting existing PM literature complicated. We aimed to assess rates of NA in children presenting to dermatology with PM. METHODS: We included patients <19 years, diagnosed with PM, nevus depigmentosus and/or segmental café au lait macules (CALM) seen in our dermatology department between 1 January 2006 and 31 December 2020. Patients with neurofibromatosis, McCune-Albright syndrome, and non-segmental CALM were excluded. Data collected included pigmentation, pattern, site(s) affected, presence of seizures, developmental delay, and microcephaly. RESULTS: One hundred fifty patients were included (49.3% female), with a mean age at diagnosis of 4.27 years. Patterns of mosaicism were ascertained for 149 patients and included blaschkolinear (60/149, 40.3%), blocklike (79/149, 53.0%), or a combination of both patterns (10/149, 6.7%). Patients with a combination of patterns were more likely to have NA (p < .01). Overall, 22/149 (14.8%) had NA. Nine out of twenty-two patients with NA had hypopigmented blaschkolinear lesions (40.9%). Patients with ≥4 body sites affected were more likely to have NA (p < .01). DISCUSSION: Overall, our population had low rates of NA in PM patients. A combination of blaschkolinear and blocklike patterns, or ≥4 body sites involved were associated with higher rates of NA.

Development and Implementation of a Web-Based International Registry Dedicated to Atypical Pigmented Skin Lesions of the Face: Teledermatologic Investigation on Epidemiology and Risk Factors.

Background: Atypical pigmented facial lesions (aPFLs) often display clinical and dermoscopic equivocal and/or overlapping features, thus causing a challenging and delayed diagnosis and/or inappropriate excisions. No specific registry dedicated to aPFL paired with clinical data is available to date. Methods: The dataset is hosted on a specifically designed web platform. Each complete case was composed of the following data: (1) one dermoscopic picture; (2) one clinical picture; (3) two lesion data, that is, maximum diameter and facial location (e.g., orbital area/forehead/nose/cheek/chin/mouth); (4) patient's demographics: family history of melanoma, history of sunburns in childhood, phototype, pheomelanine, eyes/hair color, multiple nevi/dysplastic nevi on the body; and (5) acquisition device (videodermatoscope/camera-based/smartphone-based system). Results: A total of 11 dermatologic centers contributed to a final teledermoscopy database of 1,197 aPFL with a distribution of 353 lentigo maligna (LM), 146 lentigo maligna melanoma (LMM), 231 pigmented actinic keratoses, 266 solar lentigo, 125 atypical nevi, 48 seborrheic keratosis, and 28 seborrheic-lichenoid keratoses. The cheek site was involved in half of aPFL cases (50%). Compared with those with the other aPFL cases, patients with LM/LMM were predominantly men, older (69.32 ± 12.9 years on average vs. 62.69 ± 14.51), exhibited larger lesions (11.88 ± 7.74 mm average maximum diameter vs. 9.33 ± 6.46 mm), and reported a positive history of sunburn in childhood. Conclusions: The iDScore facial dataset currently represents a precious source of data suitable for the design of diagnostic support tools based on risk scoring classifiers to help dermatologists in recognizing LM/LMM among challenging aPFL in clinical practice.

Segmental Pigmentation Disorder: Clinical Manifestations and Epidemiological Features in 144 patients, a Retrospective Case-control Study.

Segmental pigmentation disorder (SPD) is characterized by hypo- or hyper-pigmented patches segmentally distributed, present in infancy, more prominently in darker-skinned children. The aim of this study was to define the demographic and clinical characteristics of SPD in a large series of patients. This was a retrospective case-control study at 2 paediatric dermatology centres in Israel. Data were collected through a telephone questionnaire and medical records. The study group consisted of 144 individuals with SPD and 144 individuals visiting the same institutions matched for age and sex. Median age of onset of SPD was near birth; 51% of patients were Sephardic Jews, and patients were followed up for a median period of 27 years. The patches were located on the torso (43%), mostly hypopigmented (52%), and remained of the same intensity and size in 55% and 41% of cases, accordingly. No differences in extracutaneous morbidities were found between SPD and control patients. This study delineates the demographic and clinical characteristics of SPD, confirms that cutaneous findings in SPD are more prominent in darker skin, tends not to expand in size or accentuate throughout the years, nor to be associated with extracutaneous morbidities.

Cutaneous sequelae in neonatal lupus: A retrospective cohort study.

BACKGROUND: Cutaneous eruptions in neonatal lupus erythematosus (NLE) are thought to be self-resolving. Limited literature suggests cutaneous changes may persist. OBJECTIVE: To characterize cutaneous residua in NLE and identify predictors for their development. METHODS: A retrospective cohort study of patients with cutaneous NLE born between January 1980 and May 2017 was performed. Primary outcome was the proportion of patients with cutaneous residua. Secondary outcomes included associations/predictors of sequelae. RESULTS: At the last follow-up, at a mean age of 4 years (range, 0.5-18.7 years), 34% of 106 patients had cutaneous sequelae, 13% had telangiectasia, 17% had dyspigmentation, and 9% had atrophic scarring. Scarring at the last follow-up was significantly associated with the presence of skin lesions at birth (P < .001). LIMITATIONS: This study was limited by the retrospective design, short follow-up duration in a subset of patients, and small sample size. CONCLUSION: Cutaneous NLE can exhibit long-term cutaneous residua. These findings underlie the importance of accurate diagnosis, long-term monitoring, and appropriate counseling.

Clinicopathological evaluation of patients with rippled pattern pigmentation of the skin: A single-center study.

The aim of the present study was to investigate the clinical association between rippled pattern pigmentation and the positivity of histopathological analysis for amyloid. A total of 50 patients (90% women) with rippled pattern pigmentation referring to Dermatology Clinic of Shahid Faghihi Hospital, Shiraz, Iran, in 2015 participated in this study. Two biopsies were performed for all the cases from the most frequently affected sites. The specimens were evaluated for amyloid deposits with hematoxylin-eosin (H&E), Congo red (CR), and crystal violet (CV) stains. The upper back was the most frequently affected area in the patients. Family history (28%), atopy (14%), pruritus as a common finding (86%), and history of friction (54%) were positive. The prevalence of disease was higher in patients with skin photo Type 3. Amyloid deposit was not detected in most patients by these stains. No statistically significant difference was found between the amyloid positive cases stained with H&E, CR, and CV (p-value > 0.05). Only the difference in positive results between biopsy number 2 and the total biopsy (1 and 2) was significant (p-value < .05). In conclusion, it seems that it is useful to increase the number of biopsies and other more sensitive staining methods to detect small focal amyloid deposits.

Microneedling Treatment of Striae Distensae in Light and Dark Skin With Long-Term Follow-Up.

BACKGROUND: Striae distensae have notoriously been difficult to treat due to their extensive involvement of nonfacial skin. Microneedling with its lack of thermal injury during microneedling treatment renders it a viable treatment option in darker skin tones and nonfacial regions due to the reduced risk of postinflammatory hyperpigmentation. OBJECTIVE: To describe the clinical results and side effects of microneedling in a series of 25 individuals with striae distensae. MATERIALS AND METHODS: Twenty-five consecutive adults (SPT I-V) with striae distensae involving the trunk and extremities were treated using a microneedling device. No additional treatments (topical or intralesional) were applied. Two assessors blinded to treatment protocol rated clinical improvement of striae on a 5-point scale. Side effects were monitored and tabulated. RESULTS: Patients received 1 to 3 consecutive monthly treatments. All striae improved at least 50% after an average of 1.8 treatments, and 28% of patients demonstrated more than 75% clinical improvement. Striae in thicker skin regions (e.g., buttocks/thighs) showed comparable clinical improvement than those in thinner skin areas (e.g., breasts) and did not require additional treatment sessions. Side effects were limited to transient erythema in all skin phototypes. No infections or dyspigmentation were observed. CONCLUSION: The clinical results obtained in this study support the safe and effective treatment of striae distensae with microneedling in light and dark skin tones in various body locations. Standardization of treatment protocols are anticipated with further (ongoing) studies.

Prevalence of Skin Disease in Urban Haiti: A Cross-Sectional Study.

The prevalence of skin disease varies with geographic location and may be affected by cultural factors. In Haiti, the combination of poor health care access, infrastructure lack, and natural disasters may affect the prevalence of skin conditions. Unfortunately, little epidemiological information has been obtained on the prevalence of skin diseases in Haiti. The objective of this study is to describe data on the prevalence of dermatological conditions in urban areas of Haiti. Univariate, bivariate, and χ2 analyses were used to evaluate associations. During February 2016, we documented 137 patients. The mean age was 27.7 years; most of the subjects were women (61.3%) in a cohort mostly adult (67.1%). The infectious and inflammatory dermatoses were the most prevalent (74.2%). Infectious skin diseases were more prevalent in men and in adults in general (p < 0.01). Inflammatory conditions were also more common in adults (p < 0.05). Additionally, alopecia and dyschromia were limited to women (p < 0.01). Infectious skin diseases, in particular fungal infections, were amongst the most common diagnoses in the urban area of Haiti. Therefore, improved access to antimicrobial and anti-inflammatory medications would likely benefit Haitian patients with dermatological conditions. These findings may help local public health officials and future dermatological expeditions better serve this population.

[Cutaneous pigmentation related to intravenous iron extravasation: Analysis from the French pharmacovigilance database]. / Pigmentation cutanée liée à l'extravasation de fer injectable. Analyse de la base française de pharmacovigilance de 2000 à 2016.

INTRODUCTION: Intravenous iron infusion may be complicated by extravasation and lead to cutaneous pigmentation. METHODS: We queried the French pharmacovigilance database to assess the spontaneously reported cases over the 2000-2016 period. RESULTS: Fifty-one cases of cutaneous pigmentation related to intravenous iron extravasation were retrieved, none was associated to necrosis. Most of patients were women aged 20 to 49 years old. The pigmentation was mostly a brown coloration, persisting over one month in 19 cases (37.2%) and over 6 months in 9 cases (17.6%). The management of extravasation and pigmentation was heterogeneous and was rarely followed by a decrease of the coloration. CONCLUSION: Cutaneous pigmentation after intravenous iron extravasation can persist over time and create an aesthetic prejudice, particularly in young women. Standardized extravasation and iron-induced pigmentation management procedures appear necessary.

Pigmentary changes in patients treated with targeted anticancer agents: A systematic review and meta-analysis.

BACKGROUND: The discovery of signaling networks that drive oncogenic processes has led to the development of targeted anticancer agents. The burden of pigmentary adverse events from these drugs is unknown. OBJECTIVE: To conduct a systematic review and meta-analysis of published clinical trials and determine the incidence and risk of development of targeted therapy-induced pigmentary changes. METHODS: A comprehensive search was conducted to identify studies reporting targeted therapy-induced pigmentary changes. The incidence and relative risk were calculated. Case reports and series were reviewed to understand clinical characteristics. RESULTS: A total of 8052 patients from 36 clinical trials were included. The calculated overall incidences of targeted cancer therapy-induced all-grade pigmentary changes in the skin and hair were 17.7% (95% confidence interval [CI], 11.9-25.4) and 21.5% (95% CI, 14.9-30.1), respectively. The relative risk of all-grade pigmentary changes of skin and hair were 93.7 (95% CI, 5.86-1497.164) and 20.1 (95% CI, 8.35-48.248). Across 53 case reports/series (N = 75 patients), epidermal growth factor receptor and breakpoint cluster region-abelson inhibitors were the most common offending agents. LIMITATIONS: Potential under-reporting and variability in oncologists reporting these events. CONCLUSION: There is a significant risk of development of pigmentary changes during treatment with targeted anticancer therapies. Appropriate counseling and management are critical to minimize psychosocial impairment and deterioration in quality of life.

Effect of socio-demographic characteristics and clinical findings on the quality of life of patients with chronic venous insufficiency.

Objective This study was planned and implemented to evaluate the effect of socio-demographic characteristics and clinical findings on the quality of life of patients with chronic venous insufficiency. Methods The sample of this cross-sectional study consisted of 163 patients that presented with the diagnosis of chronic venous insufficiency to the cardiovascular surgery clinic of an education and research hospital in the west of Turkey. The data were collected during face-to-face interviews using a personal information form, clinical, etiology, anatomy, pathophysiology classification, venous insufficiency epidemiological and economic study-quality of life/symptoms scales and the Short Form-36. Descriptive statistics as well as univariate and multivariate analyses were used to analyze the data. Results The chronic venous insufficiency patients were found to have a low quality of life. Advanced age, higher body mass index, longer working times, being on regular medication, hypertension and presence of pigmentation according to the clinical, etiology, anatomy, pathophysiology classification were found to be associated with a reduced physical score in SF-36. Furthermore, longer weekly working hours and presence of pigmentation reduced the Venous Insufficiency Epidemiological and Economic Study-Quality of Life/Symptoms scores. Undertaking physical exercise at twice and more than twice a week increased the overall physical scores in SF-36. Conclusions The results of the study showed that the quality of life of chronic venous insufficiency patients are affected by not only physical characteristics, working hours and physical activity but also presence of edema and pigmentation.

Risk Factors for Premature Hair Graying in Young Turkish Adults.

BACKGROUND: Premature hair graying (PHG) is a common condition resulting in loss of self-esteem. Studies investigating PHG risk factors for both sexes with a large number of patients are scarce. We sought to investigate the socioclinical risk factors for PHG in young Turkish men and women and the differences between the sexes. METHODS: A cross-sectional study was conducted in 1,119 participants who answered a survey about PHG and some socioclinical characteristics between February and July 2015. The number of gray hairs, onset age of hair graying, and family history of PHG were asked about, as well as demographic characteristics, anthropometric measures, body mass index, smoking, alcohol consumption, sports life, diet, medical history, educational status, occupation, marital status, monthly income, and Fitzpatrick skin type. RESULTS: Of 1,119 participants, 315 (28.1%) had PHG and 804 did not. Maternal and paternal PHG, alcohol consumption, presence of chronic disease, educational status, hair loss, perceived stress scale (PSS) score, age, and height were significantly higher in subjects with PHG. Rates of maternal and paternal PHG were high in women with PHG, and the rate of paternal PHG was high in men with PHG. According to the multivariate ordinal regression analysis, PSS score, age, hair loss, and family history of PHG were correlated with the severity of PHG. CONCLUSION: PHG is closely related to factors causing oxidative stress, such as emotional stress, alcohol consumption, and chronic diseases in genetically predisposed men and women.

Acro-Dermato-Ungual-Lacrimal-Tooth Syndrome: An Uncommon Member of the Ectodermal Dysplasias.

Acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome is a rare form of autosomal dominant ectodermal dysplasia due to mutations in the TP63 gene, a locus that has also been implicated in other syndromic forms of ectodermal dysplasia. It shares many phenotypic characteristics with other TP63 gene mutation syndromes, often making an accurate diagnosis difficult. Long-term management and follow-up of the various sequelae of ectodermal dysplasia require an accurate diagnosis. We report a familial case of ADULT syndrome in a daughter, mother, and son and provide a brief review of the clinical characteristics of this syndrome.

Association of premature hair graying with family history, smoking, and obesity: a cross-sectional study.

BACKGROUND: Many researchers have been concerned about the association of hair graying with systemic diseases. However, the common factors associated with hair graying and systemic diseases have not been elucidated. OBJECTIVE: This study aimed to identify risk factors for premature hair graying (PHG) in young men. METHODS: We conducted a cross-sectional study using questionnaires in young men. After a pilot study that included 1069 men, we surveyed 6390 men younger than 30 years about their gray hair status and various socioclinical characteristics. RESULTS: The age of participants in the main survey was 20.2 ± 1.3 years (mean ± SD). Of the 6390 participants, 1618 (25.3%) presented with PHG. Family history of PHG (odds ratio [OR], 12.82), obesity (OR, 2.61), and >5 pack-years history of smoking (OR, 1.61) were significantly associated with PHG. In the multivariate analysis, family history of PHG (OR, 2.63) and obesity (OR, 2.22) correlated with the severity of PHG. LIMITATIONS: Owing to the use of questionnaires, the possibility of recall bias exists. Women were not evaluated in this study. CONCLUSION: Smoking, family history of PHG, and obesity are important factors associated with PHG.

Skin diseases in pediatric patients attending a tertiary dermatology hospital in Northern Tanzania: a cross-sectional study.

BACKGROUND: Skin diseases affect 21-87 % of children in developing countries in Africa. However, the spectrum of the skin diseases varies from region to region due to several factors such as genetics, socioeconomic and environmental. The aim of this study was to determine the spectrum of childhood skin diseases in Tanzania. METHODS: We conducted a prospective hospital- based cross-sectional study between September 2012 and August 2013 at a tertiary referral dermatology clinic. Children younger than 14 years presenting with new skin conditions were recruited. Diagnosis was mainly done clinically, but if the diagnosis was not clinically clear, further investigations were undertaken accordingly. RESULTS: A total of 340 patients were recruited of which 56 (16.5 %) had more than one skin condition. Both genders were equally affected. Infections and infestations accounted for the majority (43.5 %, n = 177) of the skin conditions followed by eczematous dermatitis (28.5 %, n = 116) and pigmentary disorders (7.4 %, n = 30). Among the 152 infectious skin diseases, fungal infections predominated (50.7 %, n = 77) in the infectious group followed by bacterial (29.6 %, n = 45), and viral (19.7 %, n = 30). CONCLUSIONS: Skin infections are still the main cause of dermatological consultations in children although with a reduced prevalence. Inflammatory skin conditions are increasing and can be attributed to improved socioeconomic status and HIV pandemic.

A clinical analysis of 58 chinese cases of pigmented fungiform papillae of the tongue.

BACKGROUND: Pigmented fungiform papillae of the tongue (PFPT) is a disorder in which the fungiform papillae of the tongue have abnormal coloration. However, Chinese-specific clinical data for PFPT are lacking. OBJECTIVE: To determine the prevalence and characteristics of PFPT among the Chinese population. METHODS: A survey was carried out using a clinical examination and a questionnaire on 14,346 first-time outpatients in our dermatology department, and 58 cases of PFPT were subsequently diagnosed. RESULTS: The prevalence of PFPT was 0.4% among dermatological outpatients. All patients had pin-sized, brownish fungiform papillae on the tip, lateral or dorsal parts of the tongue. Of the three subtypes, type I was the most common (87.93%). PFPT generally coexisted with Hori's nevus (48.28%), melasma (20.69%), hysteromyoma (24.14%) and breast cystic hyperplasia (20.69%). CONCLUSION: Our study confirms that PFPT is a relatively common disorder among Chinese outpatients, and it was presumed to be closely coupled with Hori's nevus, melasma and other disorders.

Hypopigmented mycosis fungoides in Egyptian patients.

Hypopigmented mycosis fungoides (HMF) is uncommon clinical variant that was commonly observed in dark-skinned individuals. We described the clinical characteristics, pathological features, immunohistochemical profile and prognosis of HMF in Egyptian patients. During the period from January 2004 to December 2011, we were able to diagnose and follow up 27 patients with HMF. The study included 18 males (66.7%) and 9 females (33.3%) with a mean age of 35.39 ±13.13 years. The duration ranged from 1 to 6 years with a mean of 3.26 ±1.7 years. The majority of patients were skin type IV (63%) and presented with multiple (88.9%), asymptomatic (74.1%), ill-defined (70.4%) and non-scaly (77.8%) lesions distributed on the trunk (81.5%). Histologically, epidermotropic lymphocytes were observed in 100%, basal alignment of lymphocytes in 81.5%, Pautrier's microabscesses in 29% and folliculotropism in 18.5%. Immunostaining showed predominance of epidermal CD8+ cells in 51.9% while in 29.6% CD4+ cells were predominant. Phototherapy was effective in 86.7% of patients with success rate 66.7% of narrow band (NB) ultraviolet-B and 80% of psoralen ultraviolet-A. HMF among Egyptians could be classified as non-aggressive epidermotropic cytotoxic CD8+ variant. It is common among middle age males with skin type IV and mostly well respond to phototherapy.

Pigmented purpura dermatosis and viral hepatitis: a case-control study.

Pigmented purpuric dermatosis (PPD) is characterized by petechial and pigmented macules on the lower limbs. The aetiology of PPD remains obscure. Some reports have suggested an association between PPD and hepatitis B or C infection. This prospective case-control study was designed to investigate the association of positive hepatitis B or C serology with PPD. A total of 60 PPD patients and 230 randomly selected controls were enrolled. Sera from all patients and controls were tested for liver function tests (LFT), hepatitis B surface antigen (HBS Ag), and hepatitis C virus antibody (HCV Ab). The prevalence of HBS Ag in patients with PPD and the controls was 3 per cent (5/60) and 4.3 per cent (10/230), respectively. The prevalence of HCV Ab was 1.7 per cent (1/60) and 1.3 per cent (3/230) among patients and controls, respectively. No statistically significant difference was noted in the prevalence of positive hepatitis B or C serology (P-values 0.73 and 0.58, respectively). No statistically significant difference in LFT was observed between the two groups. Therefore, the authors believe it is unlikely that HBV or HCV are directly involved in the pathogenesis of PPD.