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An introduction to skin and skin disease
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
The eccrine sweat glands consist of a coiled secretory portion deep in the dermis next to the subcutaneous fat and a long, straight, tubular duct whose final portion is coiled and penetrates the epidermis to drain at the sweat pore on the surface. The gland and its duct are lined by a single layer of secretory cells and surrounded by myoepithelial cells. The secretion of eccrine sweat glands is basically an aqueous solution of electrolytes. The disturbance in basic pathophysiology of sweat formation and secretion leads to hyperhidrosis and hypohidrosis.
Fabry disease
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
It is the appearance of the skin lesions in adolescence or later that usually leads to the diagnosis [5, 21]. These lesions are dark red punctate macules that do not blanch with pressure (Figures 87.2–87.4). They occur in clusters and may be mistaken for petechiae. With time, some become papular and may feel rough to touch. There is some tendency for bilaterally symmetric distribution. The areas of most common involvement are the scrotum and buttocks, but they are also seen on the hips, back, and thighs in a bathing-trunks distribution. The oral mucosa may also be involved. Microscopically, the skin lesions are angiectatic lesions in the dermis with keratotic build-up superficially. These angiokeratomas are not usually symptomatic, but occasionally large lesions on the scrotum may bleed. The hands or feet or just the tips of the toes or fingers may be bright red, and sensitive to touch. Lymphedema may also be seen in the legs. Hypohidrosis or even absence of sweating is another dermatologic manifestation of the disease. Sweat pores may appear reduced. Patients are intolerant of heat and flush with exercise. One of our patients responded to hot weather by filling rubber boots with water and sloshing around in them, as well as soaking his head in cold water.
Autonomic dysfunction
Published in Jeremy Playfer, John Hindle, Andrew Lees, Parkinson's Disease in the Older Patient, 2018
Whilst hyperhidrosis or excessive sweating is frequently cited as a feature of autonomic disturbance in PD, it is related to ‘off’ periods with tremor and is felt by many authors to represent an appropriate thermoregulatory response to increased muscle activity. Patients with true autonomic failure exhibit hypohidrosis and anhidrosis with defective temperature regulation.49
Ross Syndrome
Published in Neuro-Ophthalmology, 2020
Manikanta Damagatla, Pratyusha Ganne, Rakesh Upparakadiyala, Prabhakaran N
Her general condition was normal and her vital signs were stable. Cutaneous examination showed decreased sweating over the face, neck, upper back and arms (patchy areas of hypohidrosis and anhidrosis) on both sides. An iodine-starch test (thermoregulatory sweat test) confirmed this observation (Figure 2). There was no sensory disturbance or enlarged peripheral nerves. All of the deep tendon reflexes in the arms and legs were absent bilaterally. There was no response to Jendrassik manoeuvre. Examination of other organ systems was normal. Orthostatic hypotension was absent and the cardiovascular reflexes (heart rate and blood pressure responses to Valsalva manoeuvre and analysis of heart rate variability on electrocardiogram) were normal. Laboratory investigations including complete blood count, thyroid function tests, blood gluocose and MRI of the brain and spine were normal.
Understanding and managing autonomic dysfunction in persons with multiple sclerosis
Published in Expert Review of Neurotherapeutics, 2021
Ivan Adamec, Magdalena Krbot Skorić, Mario Habek
The main goal of treatment of sweating dysfunction is to ensure that the patient is not overheating in warm environments [74]. Physical activity should be avoided during warm weather, the patients should reside in air-conditioned environment and damp clothing may be used when being outside. Precooling therapies have shown to improve the functional capacity in pwMS and should be considered prior to physical rehabilitation [75]. Medications that may cause hypohidrosis should be avoided including anticholinergics, tricyclic antidepressants and carbonic anhydrase inhibitors. This is especially important in pwMS as anticholinergics are frequently prescribed for bladder dysfunction in this population.
Correlation of neurological level and sweating level of injury in persons with spinal cord injury
Published in The Journal of Spinal Cord Medicine, 2021
Michelle Trbovich, Ashley Ford, Yubo Wu, Wouter Koek, Jill Wecht, Dean Kellogg
While the potential for this thermoregulatory dysfunction is well known, predicting which persons with SCI, based on their neurological level of injury (NLOI), are most susceptible to HRI is not well defined. Motor and sensory deficits are defined by the International Standards for Neurological Classification after Spinal Cord Injury (ISNCSCI) exam. On the other hand a validated classification system that predicts function and neurological recovery, defining autonomic nervous system (ANS) deficits after SCI is more complicated, as the anatomy of the ANS neurological pathways is more intricate and involves multiple organ systems.5–7 The International Standards for Autonomic Function after SCI (ISAFSCI) was recently developed,8–10 but this exam is: (1) not as effective at predicting ANS function or recovery; (2) requires subjective symptom reporting; and (3) is only used by about 50% of clinicians.11 Assessing thermal regulation using the ISAFSCI is rudimentary and consists of only 2 questions: (1) “temperature regulations” (box to check for history of hyper or hypothermia) and (2) “autonomic control of sweating” (box to check for reported hyperhidrosis above the NLOI and hyper or hypohidrosis below the NLOI). In its current form, the validity of this portion of the ISAFSCI is based on the patient’s accuracy as a historian and does not objectively quantify the severity of dysfunction. Furthermore, there is no assessment of hypohidrosis above the LOI – as has been reported in small numbers of persons with tetraplegia (n = 1–6).12–14 In summary, more comprehensive knowledge of how NLOI predicts individuals at the greatest risk of HRI would directly improve clinical care, education and outcomes in the SCI population.