Keratin 17 Mutations in Four Families from India with Pachyonychia Congenita.

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Keratin 17 Mutations in Four Families from India with Pachyonychia Congenita.

Indian J Dermatol. 2017 Jul-Aug;62(4):422-426

Authors: Agarwala M, Salphale P, Peter D, Wilson NJ, Pulimood S, Schwartz ME, Smith FJD

Abstract
Pachyonychia congenita (PC) is a rare autosomal dominant genetic skin disorder due to a mutation in any one of the five keratin genes, KRT6A, KRT6B, KRT6C, KRT16, or KRT17. The main features are palmoplantar keratoderma, plantar pain, and nail dystrophy. Cysts of various types, follicular hyperkeratosis, oral leukokeratosis, hyperhidrosis, and natal teeth may also be present. Four unrelated Indian families presented with a clinical diagnosis of PC. This was confirmed by genetic testing; mutations in KRT17 were identified in all affected individuals.

PMID: 28794556 [PubMed]

Recovery of sympathetic nerve function after lumbar sympathectomy is slower in the hind limbs than in the torso.

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Recovery of sympathetic nerve function after lumbar sympathectomy is slower in the hind limbs than in the torso.

Neural Regen Res. 2017 Jul;12(7):1177-1185

Authors: Zheng ZF, Liu YS, Min X, Tang JB, Liu HW, Cheng B

Abstract
Local sympathetic denervation by surgical sympathectomy is used in the treatment of lower limb ulcers and ischemia, but the restoration of cutaneous sympathetic nerve functions is less clear. This study aims to explore the recovery of cutaneous sympathetic functions after bilateral L2-4 sympathectomy. The skin temperature of the left feet, using a point monitoring thermometer, increased intraoperatively after sympathectomy. The cytoplasm of sympathetic neurons contained tyrosine hydroxylase and dopamine β-hydroxylase, visualized by immunofluorescence, indicated the accuracy of sympathectomy. Iodine starch test results suggested that the sweating function of the hind feet plantar skin decreased 2 and 7 weeks after lumbar sympathectomy but had recovered by 3 months. Immunofluorescence and western blot assay results revealed that norepinephrine and dopamine β-hydroxylase expression in the skin from the sacrococcygeal region and hind feet decreased in the sympathectomized group at 2 weeks. Transmission electron microscopy results showed that perinuclear space and axon demyelination in sympathetic cells in the L5 sympathetic trunks were found in the sympathectomized group 3 months after sympathectomy. Although sympathetic denervation occurred in the sacrococcygeal region and hind feet skin 2 weeks after lumbar sympathectomy, the skin functions recovered gradually over 7 weeks to 3 months. In conclusion, sympathetic functional recovery may account for the recurrence of hyperhidrosis after sympathectomy and the normalization of sympathetic nerve trunks after incomplete injury. The recovery of sympathetic nerve function was slower in the limbs than in the torso after bilateral L2-4 sympathectomy.

PMID: 28852403 [PubMed]

Acquired Ulcero-Mutilating Bilateral Acro-Osteopathy (Bureau-Barrière Syndrome).

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Acquired Ulcero-Mutilating Bilateral Acro-Osteopathy (Bureau-Barrière Syndrome).

Open Access Maced J Med Sci. 2017 Jul 25;5(4):558-560

Authors: Tchernev G, Mangarov H, Lozev I, Pidakev I, Lotti T, Wollina U, Gianfaldoni S, Semkova K, Lotti J, França K, Batashki A

Abstract
We present a 35-year-old male patient with Bureau-Barrière syndrome. Bureau-Barrière syndrome is an ulcero-mutilating acropathy almost invariably associated with excessive alcohol intake. It presents with a triad of trophic skin changes with recurrent ulcerations, bone lesions and nerve damage. The clinical presentation includes chronic painless plantar ulcerations with periulcerous hyperkeratosis, hyperhidrosis, livedoid skin colour, nail dystrophy, widening and infiltration of the toes and common interdigital mycoses. Other non-specific skin changes related to the alcohol consumption are commonly observed as well. The condition affects mainly middle-aged men suffering from alcoholism. Often a bilateral location at the lower limb of male alcoholics has been described, as in our patient. Successful treatment of the Bureau-Barrière syndrome requires an interdisciplinary approach. Cessation of alcohol intake and smoking is of paramount importance.

PMID: 28785364 [PubMed]

Tap water iontophoresis in the treatment of pediatric hyperhidrosis.

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Tap water iontophoresis in the treatment of pediatric hyperhidrosis.

J Pediatr Surg. 2017 Feb;52(2):309-312

Authors: Dagash H, McCaffrey S, Mellor K, Roycroft A, Helbling I

Abstract
AIM OF THE STUDY: The treatment options for localized hyperhidrosis include antiperspirants, anticholinergics, iontophoresis, botulinum toxin and surgery. Tap water iontophoresis (TWI) involves immersing the affected area in tap water and passing a small electrical current through the area. Our aim was to assess the success of this therapy in a pediatric cohort.
METHODS: Retrospective case note review of all patients younger than 18years who underwent TWI between 2002 and 2015. Demographic data, number of treatment sessions, side effects and overall success were analyzed. Individuals undergo 7 treatments over 4weeks. A positive outcome was determined as an improvement in symptoms. Pre- and posttreatment hyperhidrosis disease severity scale (HDSS) was measured. Data are presented as mean (range). Statistical analysis was by paired t-test. A P value of <0.05 was regarded as significant.
RESULTS: There were 43 patients (30 females) with a mean age of 15 (8-17) years. Palmar and/or plantar hyperhidrosis (PPH) was present in 39/43 (91%) patients. Axillary hyperhidrosis (AH) was present in 19/43 (44%) patients. All patients (with the exception of one) underwent 7 sessions (5-7). Side effects included paresthesia (88%), pruritus (26%), pain (26%), erythema (14%), dryness (12%) as well as vesicle formation and abrasions in one patient (2%). A positive outcome was found in 84% (36/43) of patients. There was a significant reduction in mean HDSS (pre 3.5 vs. post 2; P=0.0001).
CONCLUSION: TWI is a safe and effective modality of treatment for both PPH and AH in the pediatric population, with minimal side effects. Pediatric surgeons should offer this treatment option before considering more invasive surgical procedures.
LEVEL OF EVIDENCE: IV: Retrospective study.

PMID: 27912978 [PubMed – indexed for MEDLINE]

Sequential Extended Thoracoscopic Sympathicotomy for Palmo-Axillo-Plantar Hyperhidrosis.

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Sequential Extended Thoracoscopic Sympathicotomy for Palmo-Axillo-Plantar Hyperhidrosis.

Ann Thorac Surg. 2017 Oct;104(4):1200-1207

Authors: Elalfy K, Emile S, Elfeki H, Elmetwally A, Farag M, Gado W

Abstract
BACKGROUND: Palmo-axillo-plantar hyperhidrosis (HH) exists in approximately 70% to 100% of patients complaining of HH. Many studies have documented variable effects of thoracoscopic sympathicotomy (TS) on plantar sweating. The present trial evaluated sequential extended thoracoscopic sympathicotomy for the treatment of palmo-axillo-plantar HH regarding its feasibility and outcome on each domain of HH, particularly the plantar domain METHODS: Forty-two patients with severe palmo-axillo-plantar HH underwent sequential extended (T3 to T12) thoracoscopic sympathicotomy. Improvement in HH was assessed using visual analog scale and iodine-starch test, and quality of life was evaluated using the Keller quality of life questionnaire preoperatively and 2 years postoperatively.
RESULTS: Included were 16 men and 26 women with a mean age of 24.3 ± 5.3 years. The average preoperative VAS for the palmar, axillary, and plantar HH was 9 ± 0.66, which declined significantly (p < 0.0001) at 24 months of follow-up to a mean of 0.74 ± 0.4 for the palmar and axillary domains and to 1.26 ± 0.7 for plantar HH. Improvement in quality of life was observed in all patients at 24 months of follow-up as the overall median score decreased from 120.5 to 3.5.
CONCLUSIONS: Sequential extended thoracoscopic sympathicotomy proved to be an effective method for the treatment of combined HH because it achieved satisfactory and sustained improvement of palmar, axillary, and plantar sweating. Although the benefits of sequential extended thoracoscopic sympathicotomy outweigh its drawbacks and technical difficulties, further prospective studies are required to ascertain the effectiveness of this new technique.

PMID: 28728907 [PubMed – indexed for MEDLINE]

Outcome of Limited Video-Assisted Lumbar Sympathetic Block for Plantar Hyperhidrosis Using Clipping Method.

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Outcome of Limited Video-Assisted Lumbar Sympathetic Block for Plantar Hyperhidrosis Using Clipping Method.

J Laparoendosc Adv Surg Tech A. 2017 Jan;27(1):36-42

Authors: Yun SW, Kim YS, Lee Y, Lim HJ, Park SI, Jung JP, Park CR

Abstract
BACKGROUND: There are many ways to treat focal hyperhidrosis, including surgeries for palmar and axillary hyperhidrosis. However, doctors and patients tend to be reluctant to perform surgery for plantar hyperhidrosis due to misconceptions and prejudices about surgical treatment. In addition, few studies have reported the outcome of surgeries for plantar hyperhidrosis. Therefore, the objective of this study was to determine the outcome (early and late postoperative satisfaction, complication, compensatory hyperhidrosis, recurrence rate, and efficiency) of surgical treatment for plantar hyperhidrosis.
MATERIALS AND METHODS: From August 2014 to October 2015, lumbar sympathetic block (LSB) was performed in 82 patients with plantar hyperhidrosis using clipping method. Limited video-assisted LSB was performed using 5 mm ligamax-clip or 3 mm horizontal-clip after identifying L3-4 sympathetic ganglion through finger-touch and endoscopic vision.
RESULTS: Of the 82 patients, 45 were male and 37 were female. Their mean age was 26.38 years (range, 14-51 years). Mean follow-up time was 6.60 ± 3.56 months. Mean early postoperative satisfaction score was 9.6 on the 10th day postoperative evaluation. At more than 1 month later, the mean late postoperative satisfaction score was 9.2. There was no significant difference in early postoperative satisfaction score between clipping level L3 and L4/5. However, late postoperative satisfaction score was significantly better in the L3 group than that in the L4/5 group. Patient’s age and body mass index did not affect the satisfaction score. However, male patients and patients who had history of hyperhidrosis operation showed higher satisfaction score than others.
CONCLUSION: Limited video-assisted LSB using clip provided good results with minimal complications and low compensatory hidrosis, contrary to the prejudice toward it. Therefore, surgical treatment is recommended for plantar hyperhidrosis.

PMID: 27622702 [PubMed – indexed for MEDLINE]

Management of Plantar Hyperhidrosis with Endoscopic Lumbar Sympathectomy.

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Management of Plantar Hyperhidrosis with Endoscopic Lumbar Sympathectomy.

Thorac Surg Clin. 2016 Nov;26(4):465-469

Authors: Rieger R

Abstract
Primary plantar hyperhidrosis is defined as excessive secretion of the sweat glands of the feet and may lead to significant limitations in private and professional lifestyle and reduction of health-related quality of life. Conservative therapy measures usually fail to provide sufficient relieve of symptoms and do not allow long-lasting elimination of hyperhidrosis. Endoscopic lumbar sympathectomy appears to be a safe and effective procedure for eliminating excessive sweating of the feet and improves quality of life of patients with severe plantar hyperhidrosis.

PMID: 27692206 [PubMed – indexed for MEDLINE]

Plantar Hyperhidrosis: An Overview.

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Plantar Hyperhidrosis: An Overview.

Clin Podiatr Med Surg. 2016 Jul;33(3):441-51

Authors: Vlahovic TC

Abstract
Plantar hyperhidrosis, excessive sweating on the soles of feet, can have a significant impact on patients’ quality of life and emotional well-being. Hyperhidrosis is divided into primary and secondary categories, depending on the cause of the sweating, with plantar hyperhidrosis typically being primary and idiopathic. There is an overall increased risk of cutaneous infection in the presence of hyperhidrosis, including fungal, bacterial, and viral infections. This article discusses a range of treatment options including topical aluminum chloride, iontophoresis, injectable botulinum toxin A, glycopyrrolate, oxybutynin, laser, and endoscopic lumbar sympathectomy. Lifestyle changes regarding hygiene, shoe gear, insoles, and socks are also discussed.

PMID: 27215162 [PubMed – indexed for MEDLINE]

A Simple User-made Iontophoresis Device for Palmoplantar Hyperhidrosis.

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A Simple User-made Iontophoresis Device for Palmoplantar Hyperhidrosis.

J Cutan Aesthet Surg. 2016 Jan-Mar;9(1):32-3

Authors: Nagar R, Sengar SS

Abstract
Iontophoresis is defined as passing of an ionized substance through intact skin by application of direct electric current. Tap water iontophoresis is reliable and effective method for treatment of palmar and plantar hyperhydrosis when practiced with appropriate technique and timing. One of the major setback for using iontophoresis is that the apparatus is expensive and is not readily available. A simple user-made Iontophoresis device have been described here, which could be easily constructed and used at home.

PMID: 27081247 [PubMed]

Plantar Sweating as an Indicator of Lower Risk of Compensatory Sweating after Thoracic Sympathectomy.

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Plantar Sweating as an Indicator of Lower Risk of Compensatory Sweating after Thoracic Sympathectomy.

Thorac Cardiovasc Surg. 2017 Sep;65(6):479-483

Authors: Kargi AB

Abstract
Background Hyperhidrosis is a dysfunction of the autonomic nervous system that results in regional excessive sweating, mostly in the hands, armpits, and feet. A permanent and effective treatment of hyperhidrosis can be achieved by interruption of the thoracic sympathetic chain with endoscopic thoracic sympathectomy (ETS). However, some side effects, particularly compensatory sweating (CS), are the limitations of this procedure. The mechanism of CS and the associated risk factors are still controversial. The aim of this retrospective study was to determine the relationship with various parameters associated with CS in patients undergoing ETS. Materials and Methods ETS was performed on a total of 95 patients for palmar hyperhidrosis, axillary hyperhidrosis and facial blushing by the same surgeon. The mean age of the patients was 26.41 (± 7) years, and 54 (56.8%) were males. Palmar hyperhidrosis was present in 54 (56.8%) patients, axillary hyperhidrosis in 33 (34.7%) patients, and facial blushing in 8 (8.5%) patients. Moreover, 38 (40%) patients also had plantar sweating. The severity of CS was rated into three scales as less, moderate, and severe. Results Regarding the severity of CS, 55 (57.9%) patients had no or less CS, 28 (29.5%) had moderate CS, and 12 (12.6%) patients had severe CS. Higher age group had a significant increased risk of severe CS (p = 0.03) (r = 0.262). Patients with body mass index (BMI) > 25 kg/m2 had a statistically significantly increased risk of severe CS (p = 0.016). Facial blushing resulted in severe CS in a significantly higher proportion of patients than by palmar and axillary hyperhidrosis (p = 0.001). The level of surgery was another important risk factor for CS, with the T2 level showing an increased risk of severe CS compared with T3 level (p < 0.001). Furthermore, plantar sweating was inversely and significantly related to the development of CS. Patients with plantar sweating had a significantly decreased incidence of developing CS (p = 0.015). Conclusion CS after thoracic sympathectomy for primary hyperhidrosis is the most displeasing and restrictive side effect. This study demonstrates that older age, operation level, facial blushing, and high BMI are risk factors for CS, as have been shown in several similar studies. An interesting finding of the present study is that there was a decreased incidence of CS among patients with plantar sweating. This situation may help us to distinguish high risk for CS before ETS operation.

PMID: 27043786 [PubMed – indexed for MEDLINE]