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Objectives: Skin changes in acromegaly are often the first sign of the disease. The aim of this study was to describe the cutaneous findings in patients with acromegaly. In addition, a secondary aim was to investigate the possible association of these findings with remission status and concomitant endocrinopathies., Design, Patients, and Measurements: In this prospective multicenter study, 278 patients over the age of 18 years with acromegaly who were followed up in 14 different tertiary healthcare institutions were included. These patients, who were followed up by the Endocrinology Department, were then referred to a dermatologist for dermatological examination. The frequency of skin lesions was investigated by detailed dermatologic examination. Dermatological diagnosis is reached by clinical, dermatological and/or dermoscopic examination, and rarely skin punch biopsy examinations in suspicious cases. The possible association of the skin findings between remitted and nonremitted patients and with concomitant endocrinopathies were evaluated., Results: The most common skin findings in patients with acromegaly in our study were skin tags (52.5%), cherry angiomas (47.4%), seborrhoea (37%), varicose veins (33%), acneiform lesions (28.8%), hyperhidrosis (26.9%) and hypertrichosis (18.3%). Hypertrichosis was significantly more prevalent in patients nonremitted (p: .001), while xerosis cutis was significantly more prevalent in patients remitted (p: .001). The frequency of diabetes mellitus and hypothyroidism was significantly higher in patients with varicose veins and seborrhoeic keratosis than those without. Additionally, the coexistence of hypothyroidism, hyperthyroidism and galactorrhea was significantly higher in patients with Cherry angioma than in those without Cherry angioma (p-values: .024, .034 and .027, respectively). The frequency of hypogonadism in those with xerosis cutis was significantly higher than in those without (p: .035)., Conclusions: Cutaneous androgenization findings such as skin tag, seborrhoea, acne and acanthosis nigricans are common in patients with acromegaly. Clinicians should be aware that skin findings associated with insulin resistance may develop in these patients. It can be said that the remission state in acromegaly has no curative effect on cutaneous findings. Only patients in remission were less likely to have hypertrichosis. This may allow earlier review of the follow-up and treatment of acromegaly patients presenting with complaints of hypertrichosis. Additionally, it can be said that patients with skin findings such as cherry angioma may be predisposed to a second endocrinopathy, especially hypothyroidism. Including dermatology in a multidisciplinary perspective in acromegaly patient management would be beneficial to detect cutaneous findings earlier., (© 2024 John Wiley & Sons Ltd.)

Objectives: Shapiro's syndrome (SS) is a rare condition characterized by spontaneous periodic hypothermia. The underlying pathophysiological mechanisms and etiology of this syndrome remain controversial, and fewer than 100 cases have been reported to date. The objective of this case report is to present a unique iatrogenic case of SS and contribute additional insights into the underlying etiology of this rare disorder., Methods: We conducted an analysis of existing medical literature and described a clinical case of SS secondary to a neurosurgical procedure., Results: To our knowledge, we present the first iatrogenic case of SS in a 53-year-old woman who underwent a partial right parieto-occipital lobectomy in 2003 as a treatment for refractory epilepsy. Several years after the surgical procedure, she began experiencing recurrent episodes of hypothermia. Brain magnetic resonance imaging (MRI) revealed the absence of the splenium of the corpus callosum (CC) and pituitary hyperplasia. After ruling out other potential causes of hypothermia, a diagnosis of SS was made., Discussion: The most plausible mechanism to explain the recurrent hypothermia associated with SS in our patient is a probable disruption of the pathways involved in thermoregulation through the CC as a consequence of the surgical procedure. This case report provides further insights into the etiology of this rare disorder., (© 2023. Fondazione Società Italiana di Neurologia.)

Background: Aquagenic wrinkling of the palms (AWP) is an excessive and early palmar wrinkling occurring after brief immersion to water (BIW), and has been reported as a frequent finding among Cystic Fibrosis (CF) patients., Objectives: To investigate any associations of CF patients presenting AWP with other disease characteristics and explore the pathomechanism of AWP phenomenon., Methods: We evaluated AWP in CF patients and assessed the AWP parameters of palmar wrinkling, oedema, papules, pruritus and pain at 3, 7 and 11 min after a BIW test with other disease characteristics. Statistical analyses explored the associations of AWP with genotype, lung function, pancreatic insufficiency, hyperhidrosis, personal and family history of atopy and sweat chloride levels., Results: One hundred CF patients (mean age 10.4 years) were included in the analysis. The genotypic distribution was ΔF508/ΔF508: 47%, ΔF508/other: 41% and other/other: 12%. Statistically significant associations of Kaplan-Meier curves of the AWP parameters with various disease characteristics and personal/family history were detected. Wrinkling was associated with history of atopy, hyperhidrosis and levels of sweat chloride test. The time to presentation of oedema and the appearance of papules were associated with history of hyperhidrosis and age at diagnosis. Finally, time to appearance of pruritus was related to history of atopy and of hyperhidrosis. Regarding TEWL regression analysis showed significant associations with age at diagnosis (p = 0.024), sweat chloride test levels (p = 0.005), history of hyperhidrosis (p = 0.033), history of atopy (p = 0.002) and hepatic-pancreatic involvement (p = 0.027)., Conclusions: The existence of a statistically significant association between AWP and the history of hyperhidrosis, atopy, sweat chloride levels and hepatic-pancreatic function in CF patients was detected. A strong association between AWP and CF was detected. AWP after BIW could be elicited easily and possibly can be used as an initial screening tool to diagnose an individual with symptoms and signs that raise the likelihood of CF., (© 2023 European Academy of Dermatology and Venereology.)

Purpose: The burden of different skin diseases may vary leading individuals to have different sensitivity to stress. Therefore, we compared the health-related quality of life (HRQoL) and stress before and during the universal stress from the severe acute respiratory syndrome coronavirus-2-pandemic in individuals with and without hyperhidrosis, hidradenitis suppurativa, or psoriasis., Methods: The study cohort was the Danish Blood Donor Study. Overall, 12,798 participants completed a baseline questionnaire before the pandemic, in 2018-2019, and a follow-up questionnaire during the pandemic, in 2020. Regression determined the association between the skin diseases and outcomes. Outcomes were the physical and mental component summary (MCS, PCS, respectively), which assess the mental and physical HRQoL, and the perceived stress scale, which assesses stress in the past four weeks., Results: Overall, 1168 (9.1%) participants had hyperhidrosis, 363 (2.8%) had hidradenitis suppurativa, and 402 (3.1%) had psoriasis. At follow-up, the participants with hyperhidrosis had worse MCS (coefficient -0.59 [95% confidence interval (CI) -1.05, -0.13]) and higher odds of moderate-to-severe stress (odds ratio 1.37 [95% CI 1.13, 1.65]) and the participants with hidradenitis suppurativa worse PCS (coefficient -0.74 [95% CI -1.21, -0.27]) than the control groups. The associations were independent of baseline HRQoL, stress, the Connor-Davidson Resilience scale, and other covariables. Psoriasis was not associated with the outcomes., Conclusion: Individuals with hyperhidrosis or hidradenitis suppurativa experienced worse mental or physical well-being and individuals with hyperhidrosis also had higher stress during the pandemic compared to healthy individuals. This suggests that individuals with these skin diseases are particularly susceptible to external stress., (© 2023. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Introduction: Hyperhidrosis, causing excessive sweat, can be treated with Botulinum neurotoxin injection. Botulinum toxin, an effective and safe treatment for hyperhidrosis, unfortunately involves significant pain due to multiple injections. This study aims to propose a more efficient and less painful approach to nerve blocks for relief, by identifying optimal injection points to block the median nerve, thereby enhancing palmar hyperhidrosis treatment., Methods: This study, involving 52 Korean cadaver arms (mean age 73.5 years), measured the location of the median nerve relative to the transverse line at the pisiform level to establish better nerve block injection sites., Results: In between the extensor carpi radialis and palmaris longus, the median nerve was located at an average distance of 47.39 ± 6.43 mm and 29.39 ± 6.43 mm from the transverse line at the pisiform level., Discussion: To minimize discomfort preceding the botulinum neurotoxin injection, we recommend the optimal injection site for local anesthesia to be located 4 cm distal to the transverse line of the pisiform, within the tendons of the palmaris longus and flexor carpi radialis muscles., (© 2023. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Abstract: Hemosiderosis consists of an iron deposit in tissues, which does not cause organic damage to them. However, in the case of the skin, being an organ exposed to sight, siderosis can produce a brownish coloration that is aesthetically discomforting for the individual. Most cutaneous sideroses are because of venous insufficiency with hemorrhagic extravasation. There is also a group secondary to iron extravasation in the injection site of the transfusion. However, there are very few cases in which an intravenous injection of an iron preparation has produced diffuse siderosis in extensive areas of the skin. We present the case of a 31-year-old woman with hyperhidrosis, who was transfused as a result of postpartum hemorrhage and, shortly after receiving an intravenous iron infusion, developed extensive hyperpigmentation in both axillae., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Unilateral hyperhidrosis is an uncommon manifestation, and the majority of cases have been attributed to neurological diseases. There are few cases of unilateral hyperhidrosis associated with thoracic malignant tumors. We herein report a 74-year-old Japanese man with squamous cell carcinoma of the lung who presented with unilateral hyperhidrosis in the right thoracic area as one of the first clinical manifestations. We should consider the possibility of pleural diseases, including metastatic lung cancer, when encountering patients presenting with unilateral thoracic hyperhidrosis.

Ross Syndrome is a rare disorder characterized by tonic pupils, hyporeflexia, and abnormal segmental sweating. The pathophysiology of the disease remains unclear, with either hypohidrosis or hyperhidrosis reported in individual patients. We present the case of a man, aged 57 years, who presented with hyperhidrosis in his right extremities, anhidrosis in the left extremities, and changes in his pupils. The disease was not associated with markers of autoimmune disease, which supports recent research findings on the role of neurodegeneration. The patient's son was exhibiting similar symptoms, which implicates genetic inheritance in the process. A multidisciplinary approach is crucial for the diagnosis and ultimate management of patients with Ross Syndrome., Competing Interests: Disclosure: Informed consent was obtained from the patient to publish this case report. The authors have declared they have no conflicts of interest or financial support for this work., (Copyright © 2023 Marshfield Clinic Health System.)

Background: Harlequin syndrome is a rare disorder caused by autonomic nervous system dysfunction. It manifests as asymmetric facial flashing and sweating with contralateral anhidrosis. It may be primary (idiopathic) with a benign course or can occur secondary to structural abnormalities or iatrogenic factors. To our knowledge, there has been no report of idiopathic Harlequin syndrome published from Ethiopia. We are reporting this case since it signifies the existence of idiopathic Harlequin syndrome in our setting and the need to properly diagnose this condition., Case Presentation: We are reporting a 29-year-old female from Addis Ababa, Ethiopia, who presented with a complaint of left hemifacial hyperhidrosis of 8 years which worsen after routine household activities and exercise. Physical examination revealed left hemifacial hyperhidrosis with right-side anhidrosis. She was diagnosed with idiopathic Harlequin syndrome after an appropriate investigation revealed a nonremarkable finding. Symptomatic treatment showed no significant improvement and the patient was also counseled on the disease entity., Conclusions: The patient described here signifies an idiopathic Harlequin syndrome in an Ethiopian woman. This case highlights the existence of idiopathic Harlequin syndrome within our setting and the need to properly diagnose this condition., (© 2023 Dereje Melka, et al.)

Background: Drug repurposing refers to the application of existing drugs to new therapeutic indications. As phenotypic indicators of human drug response, drug side effects may provide direct signals and unique opportunities for drug repurposing., Objectives: We aimed to identify drugs frequently associated with hypohidrosis or anhidrosis adverse reactions (that is, the opposite condition of hyperhidrosis) from the pharmacovigilance database, which could be potential candidates as anti-hyperhidrosis treatment agents., Methods: In this observational, retrospective, pharmacovigilance study, adverse event reports of hypohidrosis or anhidrosis in the US Food and Drug Administration (FDA) Adverse Event Reporting System (FAERS) were assessed between January 2004 and December 2021 using reporting odds ratio (ROR) estimates and categorized by the World Health Organization Anatomical Therapeutic Chemical (ATC) classification code. The onset time of drug-associated hypohidrosis or anhidrosis was also examined., Results: There were 540 reports of 192 drugs with suspected drug-associated hypohidrosis or anhidrosis in the FAERS database, of which 39 drugs were found to have statistically significant signals. Nervous system drugs were most frequently reported (187 cases, 55.82%), followed by alimentary tract and metabolism drugs (35 cases, 10.45%), genitourinary system and sex hormones (28 cases, 8.36%), and dermatologicals (22 cases, 6.57%). The top 3 drug subclasses were antiepileptics, drugs for urinary frequency and incontinence, and antidepressants. Taking disproportionality signals, pharmacological characteristics of drugs and appropriate onset time into consideration, the main putative drugs for hyperhidrosis were glycopyrronium, solifenacin, oxybutynin, and botulinum toxin type A. Other drugs, such as topiramate, zonisamide, agalsidase beta, finasteride, metformin, lamotrigine, citalopram, ciprofloxacin, bupropion, duloxetine, aripiprazole, prednisolone, and risperidone need more investigation., Conclusions: Several candidate agents among hypohidrosis or anhidrosis-related drugs were identified that may be redirected for diminishing sweat production. There are affirmative data for some candidate drugs, and the remaining proposed candidate drugs without already known sweat reduction mechanisms of action should be further explored., (© 2023. The Author(s).)

Hyperhidrosis is characterized by excessive sweating beyond thermoregulatory needs that affects patients' quality of life. It results from an excessive stimulation of eccrine sweat glands in the skin by the sympathetic nervous system. Hyperhidrosis may be primary or secondary to an underlying cause. Nocturnal hyperhidrosis is associated with different sleep disorders, such as obstructive sleep apnea, insomnia, restless legs syndrome/periodic limb movement during sleep and narcolepsy. The major cause of the hyperhidrosis is sympathetic overactivity and, in the case of narcolepsy type 1, orexin deficiency may also contribute. In this narrative review, we will provide an outline of the possible mechanisms underlying sudomotor dysfunction and the resulting nocturnal hyperhidrosis in these different sleep disorders and explore its clinical relevance., (© 2022 European Sleep Research Society.)

Purpose: To evaluate the prevalence of sleep disorders in Brazilian preschool children and its associations with parental report of dental pain and discomfort., Methods: This cross-sectional study involved 604 Brazilian preschoolers (4-5 years old). Sleep disorders (SD) and the parental report of dental pain and discomfort (DPD) were evaluated using the Brazilian versions of the Sleep Disturbance Scale for Children (SDSC) and the Dental Discomfort Questionnaire (DDQ-B), respectively. Bivariate and multivariate Poisson regression analyses with robust variance were performed to analyze the association between SDSC and DP., Results: Prevalence of SD ranged from 7 to 21%. 7.9% of the children had DPD indicating the need for more invasive dental procedures (DDQ-B ≥ 5). Significant associations were found between DPD and the following SDSC domains: sleep hyperhidrosis (p = 0.024; PR a  = 1.38; 95% CI: 1.04-1.83), disorders of initiating and maintaining sleep (p < 0.001; PR a  = 1.41; 95% CI: 1.15-1.73), parasomnias (p < 0.001; PR a  = 1.82; 95% CI: 1.39-2.37), and sleep-wake transition disorders (p = 0.018; PR a  = 1.28; 95% CI: 1.04-1.58). Children with higher prevalence of DPD presented 20% higher prevalence of SD than children lower prevalence of DPD (p = 0.039; PR a  = 1.20; 95% CI: 1.01-1.44)., Conclusion: Preschool children with higher prevalence of DPD are more likely to have SD, such as hyperhidrosis, disorders of initiating and maintaining sleep, parasomnias, and sleep-wake transition., (© 2022. The Author(s), under exclusive licence to European Academy of Paediatric Dentistry.)

Objective: Excessive growth hormone and insulin-like growth factor 1 contribute to cutaneous changes in acromegaly. We investigated the sub-macroscopic skin manifestation of acromegaly patients and explored its reversibility upon hormone reduction after pituitary adenoma surgery., Design: Prospectively cohort study., Methods: We enrolled 26 patients with acromegaly and 26 patients with non-functioning pituitary adenomas undergoing pituitary adenomectomy at Peking Union Medical College Hospital from July 2021 to March 2022. Skin presentations were evaluated by dermatoscopy and ultra-high-frequency ultrasound before and after surgery., Results: Skin thickening, follicular plugs, perifollicular pigmentations, perifollicular orange haloes, red structureless areas, increased hair shafts, honeycomb-like pigmentations, widened dermatoglyphics, dilated appendage openings, excessive seborrhea, hyperhidrosis, enlarged pores, and acne-like lesions were commonly occurring in acromegaly patients, and their incidences were higher than the controls (P<0.05). At 3-month follow-up after surgery, the thickness of skin reduced (4.0 ± 0.4 to 3.7 ± 0.4, P=0.007), the incidences of hyperhidrosis (92.3% to 69.2%, P=0.035) and acne-like lesions (53.8% to 26.9%, P=0.048) declined, and the severity of multiple cutaneous lesions improved. Patients with surgical endocrine remission (53.8%) had greater declines in the thickness of skin than those without remission. Patients with improvement of >1 skin lesions were younger (P=0.028) and had higher baseline GH levels (P=0.021) than those with improvement of ≤1 skin lesion., Conclusions: Dermatoscopy and ultra-high-frequency ultrasound provided augmented visual examination of the cutaneous changes in acromegaly. Some of the skin lesions could improve or reverse after pituitary surgery. Baseline GH levels, age, and endocrine remission were correlated with skin improvement at 3-month follow-up., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Guo, Wang, Yao, Bao, Duan, Zhu, Xing and Liu.)

Competing Interests: Conflicts of interest None disclosed.

Background: Angelman syndrome (AS) is a neurogenetic disorder that causes severe intellectual disability, expressive language deficits, motor impairment, ataxia, sleep problems, epileptic seizures and a happy disposition. People with AS frequently experience gastrointestinal (GI) symptoms., Method: This study used data from the Global Angelman Syndrome Registry to explore the relationship between early and current GI symptoms and co-morbidity in children and adolescents with AS (n = 173). Two groups that experienced a high (n = 91) and a low (n = 82) frequency of GI symptoms were examined in relation to feeding and GI history in infancy, sleep and toileting problems, levels of language and communication and challenging behaviours. Predictors of GI symptoms were then investigated using a series of logistic regressions., Results: This analysis found that constipation and gastroesophageal reflux affected 84% and 64%, of the sample, respectively. The high frequency of GI symptoms were significantly associated with: 'refusal to nurse', 'vomiting', 'arching', 'difficulty gaining weight', gastroesophageal reflux, 'solid food transition', frequency of night-time urinary continence and sleep hyperhidrosis during infancy. GI symptoms were not significantly associated with sleep, toileting, language or challenging behaviours. Significant predictors of high frequency GI symptoms were gastroesophageal reflux and sleep hyperhidrosis., Conclusions: Future research needs to investigate the association between AS and GI co-morbidity in adults with AS., (© 2022 The Authors. Journal of Intellectual Disability Research published by MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.)

Introduction: Geriatric symptoms are common in dementia cases, while few studies have focused on these symptoms in Lewy body dementia (LBD). The purpose of this study is to investigate the distributions of Apolipoprotein E (APOE) ε4 and geriatric symptoms, and explore their associaitons in Dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD)., Methods: A retrospective study with 185 mild-moderate probable DLB (n = 93) and PDD (n = 92) patients was assigned. Demographic and clinical characteristics, neuropsychological assessments, and APOE genotypes were recorded. Description, correlation and logistic regression models were used to analyze the presence of geriatric symptom complaints and their associations with APOE ε4., Results: DLB patients displayed more frequency of fluctuating cognition, visual hallucination, rapid eye movement sleep behavior disorder, delusion, depression, anxiety, apathy, and loss of appetite, whereas the PDD cases had constipation, fear of falling, and insomnia more frequently. The APOE ε4 allele was more common in DLB than PDD (29.9% vs. 7.0%, p < 0.001), and the patients with DLB + APOE ε4 (+) were presented more delusions (p = 0.005) and apathy (p = 0.007) than patients with PDD + APOE ε4 (+). We also found that the APOE ε4 allele was significantly associated with hyperhidrosis (OR = 3.472, 95%CI: 1.082-11.144, p = 0.036) and depression (OR = 3.002, 95%CI: 1.079-8.353, p = 0.035) in DLB patients, while there were no significant associations between APOE ε4 allele and the age at visit, the age at onset, scores of MDS-UPDRS III, H&Y stage, ADL, MMSE, MOCA and NPI, as well as the presences of fluctuating cognition, VH, parkinsonism and RBD in both groups., Conclusion: The presence and co-incidence of geriatric symptoms are common in patients with mild-moderate LBD. The presence of APOE ε4 allele is associated with hyperhidrosis and depression, but not global cognition, activitives of daily life, motor function and other neuropsychitric symptoms in DLB. These findings improve the awareness of geriatric symptoms, and contribute to the healthcare management of mild-moderate DLB and PDD., (© 2022. The Author(s).)

Background: Although hyperhidrosis is a common symptom in patients with Parkinson's disease (PD), no study has yet examined it longitudinally., Objectives: We conducted a 3-year prospective cohort study to investigate the development, evolution and correlates of hyperhidrosis in patients with PD., Methods: A total of 224 patients with early-stage PD were enrolled at baseline and followed up annually for three consecutive years. Hyperhidrosis was assessed using hyperhidrosis question (item 30) of the Non-Motor Symptoms Scale (NMSS). The generalized estimating equations model was applied to investigate the correlates of both presence and severity of hyperhidrosis., Results: The frequency of hyperhidrosis in PD had an overall increasing tendency from 24.1% at baseline to 34.4% after 3 years, although hyperhidrosis was not always persistent in all patients over the 3-year study period. The presence of hyperhidrosis was found to be associated with dyskinesia (OR 2.27 [1.02-5.04], P = 0.045), the sexual function domain subscore of the NMSS (OR 1.04 [1.01-1.07], P = 0.016), the Hamilton Anxiety Rating Scale (HARS) score (OR 1.08 [1.03-1.13], P = 0.001) and the Unified Parkinson's Disease Rating Scale part III score (OR 1.02 [1.00-1.04], P = 0.036). Only the HARS score was associated with the severity of hyperhidrosis (B 0.08 [0.03-0.12], P = 0.001)., Conclusions: Hyperhidrosis is common in PD, and its frequency increases along with disease duration. Hyperhidrosis in PD is associated not only with motor severity and motor complication such as dyskinesia, but also with non-motor symptoms such as sexual dysfunction and anxiety., (© 2022 European Academy of Dermatology and Venereology.)

Background This study aimed to investigate the presence of sleep disturbances in children with Prader-Willi syndrome (PWS) using the Sleep Disturbance Scale for Children (SDSC). Methods The SDSC, which was designed to identify the presence and severity of different sleep disorders, was applied to 50 patients with PWS and 112 controls. Results Patients with PWS achieved worse scores in the sleep-disordered breathing and disorders in initiating and maintaining sleep in the SDSC questionnaire as compared with controls. We also observed that patients with PWS were more prone to having hyperhidrosis. We did not observe significant differences in the presence of other types of sleep disorders (such as hypersomnolence) between the PWS and control groups. Conclusions The results obtained with the SDSC questionnaire showed that children with PWS have more sleep breathing disorders and disorders in initiating and maintaining sleep as compared to controls. Additionally, we demonstrated that patients with PWS associates significantly with the presence of hyperhidrosis during sleep. However, SDSC was not reliable to identify the excessive daytime somnolence in patients with PWS, as previously reported in the literature.

Purpose of Review: This article reviews disorders of sweating, including hyperhidrosis and anhidrosis due to central or peripheral autonomic nervous system causes., Recent Findings: Disorders of thermoregulation and sweating may manifest with hyperhidrosis or hypohidrosis/anhidrosis. Primary disorders of hyperhidrosis may significantly impact quality of life yet tend to be benign. Many sweating disorders present with compensatory hyperhidrosis due to areas of anhidrosis. Anhidrosis may occur due to either central or peripheral damage to the autonomic nervous system. The thermoregulatory control of sweating involves central pathways from the hypothalamus to the brainstem and then spinal cord as well as projections to peripheral structures, including the sympathetic chain ganglia, peripheral nerves, and eccrine sweat glands. Disruption at any point of this pathway may lead to impaired sweating. Characterization of sweating dysfunction helps localize different autonomic disorders to guide diagnosis and may allow for evaluation of treatment effect., Summary: Sweating dysfunction manifests in myriad ways, including essential hyperhidrosis, complete anhidrosis with heat intolerance, and compensatory hyperhidrosis due to anhidrosis, and often indicates involvement of underlying central or peripheral autonomic dysfunction.

Background: The efficacy of botulinum toxin A (BTX-A) therapy in axillary hyperhidrosis has been documented; however, there are a few studies reporting the efficacy of BTX-A in treating axillary bromhidrosis. The histological changes occurring in sweat glands after BTX-A treatment are also unknown., Objective: The authors report on the efficacy and safety of BTX-A in the treatment of axillary bromhidrosis and on the histological changes in sweat glands after BTX-A treatment., Materials and Methods: Nineteen patients were included in this study. The patients were administered BTX-A injection in one axilla and sterile normal saline as placebo in the other axilla. The degree of malodor was evaluated subjectively by the patients before and 3 months after treatment. Sweat secretion was quantified by the gravimetric method. All patients underwent standard apocrinectomy in both axillary regions., Results: The mean degree of malodor and mean sweat production in the BTX-A-treated axilla were significantly lower than those in the control axilla (2.42 vs 8.00; p < .0001 and 13.33 vs 33.75 mg/min; p = .0028, respectively) at 3 months after therapy. The histological studies showed apocrine sweat glands with atrophic changes and hypoplasia in treated axilla., Conclusion: BTX-A injection is an easy, fast, noninvasive method of treating axillary bromhidrosis.

Hyperhidrosis can seriously impair patients' quality of life. Medical history, including heredity and hyperhidrosis during youth, as well as current age and time elapsed since menopause, is important to consider when distinguishing between postmenopausal hyperhidrosis and vasomotor symptoms to enable adequate treatment. This report concerns a subgroup of eight postmenopausal patients participating in a randomized controlled trial regarding botulinum toxin (Btx) type B treatment in craniofacial hyperhidrosis. Even though the sample size is small and the enrolment is not yet completed, the promising data collected hitherto are interesting to present in advance because this subtype of craniofacial hyperhidrosis is often underrecognized and challenging to treat. Patients were randomized to receive Btx type B or placebo. Measurements were performed before treatment and 3 ± 1 weeks after. The Dermatology Life Quality Index (DLQI) score was improved for all patients after Btx type B treatment (n = 3) with a median decrease of 9 points (90% median improvement). The placebo group (n = 5) had a median increase of 2 points (-18% median decline). When the same group (n = 5) received Btx type B (open) the DLQI score decreased with a median of 7 points compared with baseline (91% median improvement). Treatment-related adverse events were temporary and did not prevent improvement of life quality. Furthermore, background data evaluation uncovered interesting findings regarding vasomotor symptoms in relation to postmenopausal hyperhidrosis. In conclusion, the results indicated that Btx type B seems to be a safe and effective treatment in postmenopausal craniofacial hyperhidrosis. Further research is encouraged., (© 2019 Japanese Dermatological Association.)

Background: Ingrown nail is a condition frequently seen in children and adolescents, the pain from which can affect their daily living activities and school performances. The purpose of this study was to determine the clinical and sociodemographic characteristics of ingrown nails in children., Methods: The clinical and sociodemographic characteristics of patients aged 0 to 18 years presenting with ingrown nail were evaluated retrospectively from clinic records., Results: Sixty-two patients aged 3 to 18 years (mean age, 15 years; male to female ratio, 1.06) were enrolled. A total of 175 ingrown nails were evaluated (all of them were in the halluces, 54.3% of them were on the lateral margin). A positive family history of ingrown nail was present in 15.7%. High prevalences of incorrect nail cutting (72.1%), trauma (36.1%), poorly fitting shoes (29%), hyperhidrosis (12.9%), obesity (9.7%), and accompanying nail disorders (9.7%) were determined among the patients., Conclusions: This study revealed the clinical and sociodemographic characteristics of ingrown nails in children. These data will be useful in preventing the occurrence of ingrown nail by revealing and then eliminating predisposing factors.

Competing Interests: Competing interests: None declared.

Background: The pinch and turn-over technique was developed to minimize the incision in the manual subdermal excision of apocrine glands in axillary osmidrosis for the best cure and fewest complications., Methods: Through a 2-cm-long incision, peripheral subdermal tissue was excised by the technique under direct vision using intravenous anesthesia and tumescent technique. Ninety-nine axillae in 50 patients including seven secondary cases were operated on from 2009 to 2018. Long-term follow-up (average 30.3 months) could be done on 40 patients with questionnaires consisting of four-point ordinal-scale questions regarding osmidrosis grade, hyperhidrosis grade and satisfaction. For systematic review regarding incision length, 220 articles from 1962 to 2018 were reviewed with the search terms "osmidrosis" or "bromhidrosis.", Results: Thirty-five and 30 of 40 respondents (87.5% and 75.0%) had excellent or good postoperative results in osmidrosis and hyperhidrosis grade. Postoperative improvement of osmidrosis grade (from 2.53 to 0.80) and hyperhidrosis grade (from 1.88 to 0.95) was statistically significant (p < 0.01). Thirty-four patients (85.0%) reported very satisfactory or satisfactory. Hematoma (2.0%), hyperpigmentation (5.0%), infection (5.0%) and noticeable scar (2.5%) were rare. Necrosis of flap margins was frequent (27.3%) but necessitated revision in two cases (2.0%). Among eleven articles found to describe incision length in pure manual subdermal excision, this technique provided the shortest incision compared with eight direct subdermal excision methods with an average incision length of 5.0 cm., Conclusions: This technique offers an open, direct and selective approach with a short incision. It could maintain the best efficiency while neutralizing negative side effects of conventional manual subdermal excision for axillary osmidrosis., Level of Evidence Iv: This journal requires that authors assign a level of evidence to each article. For a full description of these evidence-based medicine ratings, please refer to the table of contents or the online instructions to authors www.springer.com/00266 .

Pourfour du Petit syndrome is an uncommon cause of eyelid retraction, associated with unilateral mydriasis and hemifacial hyperhidrosis. This syndrome is caused by hyperactivity of the ipsilateral oculosympathetic pathway and needs to be recognized because it has an opposite clinical presentation but the same topographic and diagnostic value as Horner syndrome. The authors report a rare case of Pourfour du Petit syndrome associated with cluster headache and discuss pathophysiological hypotheses, clinical presentation, complementary exams, pharmacologic testing, treatment options, and prognosis. Early detection of these symptoms may lead to swift diagnosis and treatment.

Sweating plays a vital role for humans. However, excessive sweating, also called hyperhidrosis, is a condition resulting in sweating beyond what is physiologically necessary. The increased rate of sweating is not caused by external stimuli or temperature fluctuations, as with an individual without hyperhidrosis. Hyperhidrosis affects approximately 4.8% of Americans. Primary hyperhidrosis, a specific classification of the disease, primarily affects younger adults aged 18 to 39 years, and it often has a genetic component. Living with hyperhidrosis presents many challenges and impacts numerous aspects of daily life. Patients with hyperhidrosis are impacted in their social and professional lifestyles, as well as their mental and emotional health. These negative effects, which have been studied, lead to a lower quality of life (QOL) in this population. Additionally, constant moisture from sweating can lead to skin maceration. This increases the risk of skin conditions such as athlete's foot and more severe conditions such as bacterial infections or pitted keratolysis. Study results report a nearly 30% greater risk of skin infections in patients with hyperhidrosis compared with healthy controls. This section of the continuing education supplement will examine the pathophysiology and clinical severity of hyperhidrosis and identify the comorbidities and QOL challenges associated with this condition.

Background: Hyperhidrosis is a condition where the amount of sweat released to skin surface increases due to the over-active eccrine sweat glands. Hyperhidrosis causes considerable psychosocial distress in affected people. It affects the quality of life and leads to social anxiety disorders., Aims: No study has been conducted in our country to investigate the epidemiological, clinical, and laboratory data of patients with hyperhidrosis. In this study, we aimed to retrospectively investigate the clinical and demographic characteristics, causes of sweating and laboratory findings in patients treated for hyperhidrosis at our outpatient clinic and to compare these data with the literature data., Materials and Methods: A retrospective review was conducted on medical records of patients diagnosed with and treated for hyperhidrosis at outpatient clinic between 2014 and 2017. Adults aged over 18 years were included in study. Age and gender of patients, type and localization of sweating, duration of disease, age of onset of sweating, presence of stress, fever, joint pain and comorbidity, family history, medication use, and examination results were recorded., Results: Records of a total of 70 patients consisting of 30 men and 40 women with hyperhidrosis were examined. Overall mean age was 37.1 years. Mean age was 41 years in women and 32 years in men. Most frequent forms were palmoplantar and axillary hyperhidrosis for primary hyperhidrosis (primary HH), and head-neck and generalized hyperhidrosis for secondary hyperhidrosis (secondary HH). Most common comorbidities were diabetes mellitus, thyroid disease, non-specific joint and bone pain, cardiovascular disease, and neuropsychiatric disease. Cases with secondary HH had a history of drug use (antithyroid drugs, nonsteroidal anti-inflammatory drugs, antidiabetic agents, antidepressants, and antihypertensives)., Conclusion: This is the first study that investigated the characteristics of patients with primary and secondary HH in our country. These characteristics can help determine the cause and apply treatment for hyperhidrosis by an appropriate examination and approach.

Introduction: Shapiro syndrome (SS) is characterized by spontaneous recurrent episodes of hypothermia, hyperhidrosis and corpus callosum (CC) agenesis. Less than 60 cases have been reported to date and the pathogenic mechanism as well as the prognosis of this syndrome are still debated. We describe the clinical features and long-term follow-up of a pediatric cohort of SS patients., Methods: We collected 13 (10 novel) pediatric cases of SS and report their long-term follow-up and neurological outcome., Results: All patients experienced recurring hypothermia, with body temperature below 35 °C during the episodes, often accompanied by hyperidrosis. CC agenesis was an inconstant structural feature in the present series (2/13 patients). Seven patients received antiepileptic drugs (AEDs) or other drug therapy for a mean period of 12 months. At long-term follow-up (mean = 61 months, range: 60-96), all individuals were free from episodes of paroxysmal hypothermia independently from previous AED use or other drug therapy., Conclusion: Paroxysmal hypothermia, the core symptom of SS, behaved as a age-dependent feature in our cohort, supporting a good long-term prognosis for SS. A prompt diagnosis of SS is crucial to avoid unnecessary diagnostic investigations., (Copyright © 2018 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Background: Hyperhidrosis (excessive sweating) affects 1%-3% of the population. Primary focal hyperhidrosis most commonly affects the axilla, palms, and soles. There are few case reports of hyperhidrosis of the genital region, typically described as inguinal hyperhidrosis or Hexsel's hyperhidrosis., Case: A 17-year-old girl presented with 3 years of copious, clear "vaginal" discharge causing significant emotional distress. After extensive gynecologic and urologic workup was negative, further review of her history was notable for excessive axillary sweating. Inguinal hyperhidrosis was suspected and she was treated with topical aluminum chloride hexahydrate with complete resolution of her symptoms., Summary and Conclusions: Inguinal hyperhidrosis, compared with other sites, is not widely described in the literature. Awareness of inguinal hyperhidrosis is important because it causes significant social embarrassment but is a treatable condition., (Copyright © 2018 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Fibrous hamartoma of infancy (FHI) is a rare entity with a benign nature. The typical clinical features are a single, slowly growing, painless mass on the trunk that appears within the first 2 years of life. We report a 13-month-old boy who presented with a plaque on the lower back since 4 months of age. The plaque had gradually become larger and firm, and hyperhidrosis and hypertrichosis were noticed. No visible connection between the spinal cord and the lesion was found in radiologic studies, indicating a disease other than spinal dysraphism. Histopathological findings showed well-defined fibrous trabeculae, mature adipose tissue, and primitive mesenchymal cells, all consistent with FHI. This is the first case of FHI presenting with hyperhidrosis and hypertrichosis reported in Korea., Competing Interests: The authors have no potential conflicts of interest to disclose., (© 2018 The Korean Academy of Medical Sciences.)

Klippel-Trenaunay syndrome (KTS) is a rare, clinically variable congenital disorder involving capillary malformations, soft tissue or bone hypertrophy, and venous malformations or varicose veins. We report a 28-year-old man who presented with a hypertrophic right arm as well as markedly increased ipsilateral axillary hyperhidrosis and erythematous patches on the back, chest, and arm. This case of KTS is unusual because our patient presented with a markedly increased unilateral axillary hyperhidrosis ipsilateral to the hypertrophic limb.

Objective: To evaluate the value of radial and ulnar arteries hemodynamic changes by ultrasound in patients with primary palmar hyperhidrosis after thoracic sympathetic block. Methods: This was a prospective study, from January 2016 to September 2016, 23 patients with primary palmar hyperhidrosis were admitted to the First Hospital of Jiaxing City, Zhejiang Province, 92 arteries of these patients who underwent thoracic sympathetic block were enrolled into this study. Diameters, peak systolic velocity (PSV), end diastolic velocity (EDV) and resistive index (RI) of the radial and ulnar arteries were examined one day before and after the operation by color Doppler ultrasound. Spectral waveforms of these arteries were observed, and palms temperature (T), oxygen saturation (SpO(2)), perfusion index (PI) were also measured. Diameters, PSV, EDV, RI, T, SpO(2) and PI of the patients with PH were compared before and after the surgery. The effect of operation was also evaluated. Results: Before surgery, diameters and EDV of the radial and ulnar arteries of the patients with PH were(2.12±0.36) mm, (1.50±0.32) mm, (4.90±1.84) cm/s, (4.71±1.65) cm/s, respectively, after surgery, the diameter of the RA, UA and EDV were (2.45±0.54) mm, (1.87±0.44) mm, (9.37±1.69) cm/s and (9.12±1.54) cm/s, which were significantly increased.Before surgery, RI of the RA and UA of the patients were (0.85±0.05), (0.97±0.07) , respectively, after surgery, RI of the RA and UA were (0.57±0.04), (0.64±0.09), respectively, which were significantly decreased after surgery. The difference was statistically significant ( t =-5.23, -2.33, -19.80, -14.68, 3.31, 3.48, all P <0.01). Before surgery, PSV of the RA and UA of the patients were (46.38±15.12) cm/s, (45.60±14.88) cm/s , respectively, after surgery, PSV of the RA and UA were (46.93±16.02) cm/s, (46.19±15.30) cm/s , respectively. The difference was not statistically significant ( t =-0.23, -1.39, all P >0.05). Before surgery, T, SpO(2) and PI of the patients were(29.7±1.04) ℃, (93.24±2.23) %, (1.11±0.13) % , respectively, after surgery, T, SpO(2) and PI of the patients were (35.09±1.21) ℃, (98.10±1.34) %, (4.77±1.22)% , respectively. The difference was statistically significant ( t =-20.174, -1.140, -23.601, all P <0.05). The symptoms of hyperhidrosis of the patients were disappeared immediately after the surgery in both hands. All of the patients were cured within 3 months and there was no severe complications. Conclusion: Thoracic sympathetic block increase Diameters and EDV but decrease RI of the radial and ulnar arteries of the patients with PH and increase the palms temperature. Artery diameters, EDV, and RI measured by ultrasound can be used as parameters to evaluate the effect of thoracic sympathetic block in patients with PH. It's objective, non-invasive and convenient.

Background & Objectives: Hyperhidrosis is a pathologic condition of excessive sweating in amounts greater than physiologic needs. Endoscopic thoracic sympathectomy (ETS) is a globally accepted treatment modality for primary palmar and axillary hyperhidrosis. ETS also has distinctive effects on the heart, circulatory and respiratory systems. In this study early effects of ETS on electrocardiographic (ECG) parameters of hyperhidrosis patients were evaluated., Methods: Twelve-lead ECGs were performed on 72 patients who were free from cardiovascular, metabolic, neurological and pulmonary diseases and underwent planned ETS because of hyperhidrosis, before and after the procedure within the first 24 h. Heart rate (HR), PR, QT, corrected QT (QTc), QTc/Tpeak-Tend (TpTe) intervals, P-wave and QTc/TpTe dispersions were compared by ECG., Results: A total of 72 patients (24.1±6.0 yr, 17 female) were included in the study. The pre-operative HR of patients was significantly higher than post-operative HR of patients (73.8±12.8 vs. 68.1±12.6 beats/ min; P<0.001). The QTc dispersion (QTcd) durations of pre-operative patients were significantly longer than those of post-operative patients (51.5±6.3 vs. 44.9±5.6 msec; P<0.01). The TpTe dispersion value of pre-operative patients was significantly (P<0.001) higher than that of post-operative patients., Interpretation & Conclusions: Our study showed that ETS through clipping procedure had positive effects on the mechanisms of arrhythmia by reducing HR, QTcd, TpTe and TpTe dispersion parameters of ECG in early periods in hyperhidrosis patients.

Onychomycosis is a fungal infection of the fingernails and/or toenails caused by dermatophytes, yeasts and non-dermatophyte moulds. The epidemiology of onychomycosis in Serbia is yet to be fully established. This epidemiological study was aimed at evaluating the epidemiology of onychomycosis in a sample of the Serbian patients at risk of onychomycosis, to determine the fungal aetiological agents and to identify the possible risk factors. The study population included 374 patients from six centres in Serbia with suspected onychomycosis. Demographic data, data about comorbidities, lifestyle, clinical aspects of onychomycosis, trauma, excessive perspiration and personal and family history of previous onychomycosis were studied. Laboratory confirmation of diagnosis was done by direct microscopy, fungal culture and PCR. Diagnosis of onychomycosis was confirmed in 50.8% of patients, who tested positive to at least one laboratory test (direct microscopy, fungal culture or PCR). Trichophyton rubrum was predominant both on toenails (85.98%) and on fingernails (38.46%). Independent risk factors for onychomycosis were: old age (OR = 2.285; P < 0.001), family history of previous onychomycosis and/or tinea pedis (OR = 2.452; P = 0.005), excessive perspiration (OR = 2.165; P = 0.002) and higher degree of hyperkeratosis (OR = 1.755; P = 0.020). This is a first epidemiological study of onychomycosis from Serbia., (© 2016 Blackwell Verlag GmbH.)

Postural orthostatic tachycardia syndrome is characterized by orthostatic intolerance. Orthostasis (or other mild physical stress) triggers a cascade of inappropriate tachycardia, lightheadedness, palpitations, and often fainting. The underlying defect is sympathetic dysregulation of the heart, which receives its sympathetic tone from the cervical and upper thoracic sympathetic ganglia. Primary hyperhidrosis is also thought to be the result of sympathetic dysregulation. We present the case of a patient treated with CT-guided, percutaneous T2 EtOH sympatholysis for craniofacial hyperhidrosis. The patient also suffered from postural orthostatic tachycardia syndrome for many years and was unresponsive to treatment. Immediately after sympatholysis, the patient experienced resolution of both craniofacial hyperhidrosis and postural orthostatic tachycardia syndrome.

Objective:: to compare the intensity of reflex sweating with the degree of anxiety and its interference in the quality of life of patients undergoing Thoracoscopic (VATS) sympathectomy in the pre- and postoperative period., Methods:: we evaluated 54 patients with a mean age of 26 years (16-49 years) undergoing sympathectomy in the R3-R4 level. We applied two questionnaires at three different times: "Quality of life in patients with primary hyperhidrosis" and "Scale for anxiety and depression"., Results:: of the patients studied, 93% showed significant improvement in quality of life 30 days after surgery, the effects remaining after six months. There were no postoperative complications. The patient's level of anxiety is highly correlated with the intensity of reflex sweating after 30 and 180 days., Conclusion:: Thoracoscopic sympathectomy improves quality of life of patients with primary hyperhidrosis, even with the emergence of reflex sweating. Anxiety directly relates to the intensity of reflex sweating, without compromising the degree of patient satisfaction., Objetivo:: avaliar a intensidade de sudorese reflexa com o grau de ansiedade e sua interferência na qualidade de vida de indivíduos submetidos à simpatectomia por videotoracoscopia nos períodos pré e pós-operatório., Métodos:: foram avaliados 54 pacientes com média de idade de 26 anos (16 a 49 anos), submetidos à simpatectomia em nível R3-R4. Dois questionários foram aplicados em três momentos diferentes: "Qualidade de vida em pacientes com hiperidrose primária e "Escala para ansiedade e depressão"., Resultados:: dos pacientes estudados, 93% mostrou melhora significativa na qualidade de vida após 30 dias da cirurgia, com os efeitos remanescentes após seis meses. Não houve complicações pós-operatórias. A análise mostrou que o nível de ansiedade do paciente é altamente correlacionado com a intensidade da sudorese reflexa após 30 e 180 dias., Conclusão:: a simpatectomia torácica por videotoracoscopia melhora a qualidade de vida de pacientes com hiperidrose primária, mesmo com o surgimento de sudorese reflexa. A ansiedade está diretamente relacionada com a intensidade da sudorese reflexa, sem comprometer o grau de satisfação do paciente.

Crisponi syndrome (CS)/cold-induced sweating syndrome type 1 (CISS1) is a very rare autosomal-recessive disorder characterized by a complex phenotype with high neonatal lethality, associated with the following main clinical features: hyperthermia and feeding difficulties in the neonatal period, scoliosis, and paradoxical sweating induced by cold since early childhood. CS/CISS1 can be caused by mutations in cytokine receptor-like factor 1 (CRLF1). However, the physiopathological role of CRLF1 is still poorly understood. A subset of CS/CISS1 cases remain yet genetically unexplained after CRLF1 sequencing. In five of them, exome sequencing and targeted Sanger sequencing identified four homozygous disease-causing mutations in kelch-like family member 7 (KLHL7), affecting the Kelch domains of the protein. KLHL7 encodes a BTB-Kelch-related protein involved in the ubiquitination of target proteins for proteasome-mediated degradation. Mono-allelic substitutions in other domains of KLHL7 have been reported in three families affected by a late-onset form of autosomal-dominant retinitis pigmentosa. Retinitis pigmentosa was also present in two surviving children reported here carrying bi-allelic KLHL7 mutations. KLHL7 mutations are thus associated with a more severe phenotype in recessive than in dominant cases. Although these data further support the pathogenic role of KLHL7 mutations in a CS/CISS1-like phenotype, they do not explain all their clinical manifestations and highlight the high phenotypic heterogeneity associated with mutations in KLHL7., (Copyright © 2016 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Anomalies of the corpus callosum are the most frequent malformations of the central nervous system. The triad of spontaneous periodic hypothermia and hyperhydrosis with the agenesis of corpus callosum is described as Shapiro syndrome. Shapiro syndrome is a very rare condition and it can occur in every age group. The presence of agenesis of corpus callosum is not a strict criteria of the syndrome; the most important presenting symptom is paroxysmal hypothermia. Although the definite cause of recurrent hypothermia is unknown, dysfunction of the hypothalamus is suspected. From therapeutic aspects, only supportive therapy is available. In this report the authors present the first Shapiro syndrome case diagnosed in Hungary. The main symptoms of the 21-year-old male patient were recurrent hyperhydrosis with hypothermia resulting in severe general malaise. The skull magnetic resonance imaging demonstrated agenesis of corpus callosum. The patient was treated with clonidine resulting in significant improvement of symptoms.