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Possible Compensatory Mechanisms of Segmental and Unilateral Hyperhidrosis

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Possible Compensatory Mechanisms of Segmental and Unilateral Hyperhidrosis Possible compensatory mechanisms of segmental and unilateral hyperhidrosis ● 第 70 回日本自律神経学会総会 / シンポジウム 9 / 分節性／半側性多汗症：臨床的特徴と病態 司会：犬飼洋子・齋藤 博 Possible compensatory mechanisms of segmental and unilateral hyperhidrosis: estimation based on the efferent phase of the physiological mechanism of the skin pressure-sweating reflex Yoko Inukai Kew words: segmental hyperhidrosis, unilateral hyperhidrosis, skin pressure-sweating reflex, compensatory hyperhidro- sis, sweating Abstract: Segmental and unilateral hyperhidrosis are forms of sweating disorder. In some cases, these are accompanied by anhidrosis/hypohidrosis in other skin areas. The pathogenesis of these hyperhidrosis may be compensatory and is likely caused by underlying lesions in anhidrosis/hypohidrosis areas, but the precise mechanism remains unclear. Hyperhidrosis is often located horizontally contralateral same myelomere skin areas as the anhidrosis/hypohidrosis, whereas vertically ipsilateral adjacent to other rostral and caudal my- elomere with anhidrosis/hypohidrosis. The similar efferent phase of the physiological “skin pressure-sweating reflex” might be associated with these mechanisms. This horizontal reflex is primarily due to inhibition of ipsilateral sweating by unilateral skin pressure, secondarily contralateral sweating increases. Microneurog- raphy indicates that this phenomenon occurs because unilateral skin pressure reduces the amplitude of ipsilateral sudomotor nerve activity and increases contralateral activity. Vertically, studies using the ventilated capsule method during heating, show that pressure on the bilateral skin of the back by supination decreases sweating on the upper body and increases sweating on the underbody. Central sudomotor sympathetic outflow (frequency of sweat expulsion) in response to body temperature is simultaneously hyperactivated, indicating that sweating is increased compensatorily to maintain a constant total sweating rate. In conclusion, segmental hyperhidrosis in segments other than those directly affected may be compensatory. （The Autonomic Nervous System, 56: 25 ～ 32, 2019） causes of segmental hyperhidrosis brought by spinal Introduction cord lesions: (1) disinhibition of preganglionic sympathetic Segmental and unilateral hyperhidrosis are the forms neurons due to interruption of the inhibitory descending of sweating disorders. These hyperhidrosis are reported pathway by, for example, Chiari-type malformation18), sporadically and are often uncomfortable for patients. (2) overactivity of relatively intact19) preganglionic The mechanism underlying this condition is unknown. sympathetic neurons due to spinal cord lesions such Previous reports about this condition and its etiology are as syringomyelia with Chiari malformation20), and (3) summarized below. autonomic hyperreflexia due to cutaneous stimulation, such as postural change, or bladder and intestine stimu- Reported segmental hyperhidrosis by overactivity of lation1). It is also reported that segmental hyperhidrosis sympathetic neurons is usually associated with irritation or infiltration of pre- Segmental hyperhidrosis is an uncommon finding. ganglionic sympathetic fibers or the sympathetic chain30) Three mechanisms have been hypothesized as possible by mesothelioma, a thoracic central disc herniation, chickenpox13), herpes zoster30), nonsystemic vasculitic Department of Physiology, Aichi Medical University School neuropathy28), or eccrine angiomatous hamartoma27). of Medicine, 1-1 Yazakokarimata, Nagakute, Aichi 480-1195, Japan （25） 自律神経 56 巻 1 号 2019 年 Reported unilateral hyperhidrosis by excitation due to the lesions of affected body sides Most cases with localized unilateral hyperhidrosis 3UHVV reported were considered to be idiopathic. Secondary localized unilateral hyperhidrosis is usually attributed to neurological factors such as intracranial tumor, cerebral infarction or hematoma, encephalitis, spinal cord injuries including syringomyelia, neuritis, osteoma also of the cervical rib, chickenpox, bronchial carcinoma14) or pleural malignancy7), e.g., mesothelioma29), or trauma to the autonomic nervous system of the affected body side6). Excision of cervical ribs, which may cause hyperhidro- sis by excitation of autonomic efferent fibers abolished this hyperhidrosis26). Thus, direct infiltration of the sympathetic chain is also postulated to cause unilateral hyperhidrosis. The possibility of compensatory hyperhidrosis due to 7DNDJL anhidrotic areas on other area Fig. 1 Sweating the whole body while the left axilla and the In most cases, sweating is localized to the ipsilateral)LJ right iliac crest are pressed (areas of sweat are stained with side as a tumor, although cases of contralateral sweating iodostarch reaction using Minor’s method). Hemihidrosis 8) occurs on the upper body caused by pressure at a have been reported . Since 2007, I and my co-authors contralateral upper-body site, in particular, the side of the reported many cases of segmental and unilateral chest. Hemihidrosis occurs on the underbody due to pressure 2)4) hyperhidrosis accompanied by anhidrosis/hypohidrosis . at a contralateral underbody site, e.g., the side buttocks.24) In some cases of segmental hyperhidrosis, anhidrosic/ hypohidrotic areas occurred ipsilateral to superior or in- ferior adjacent dermatomes. In other cases, anhydrotic/ What is the mechanisms of compensatory segmental or hypohidrotic areas occurred in the same dermatomes unilateral hyperhidrosis?: similar to the efferent phase of contralateral to hyperhidrotic areas. These cases “skin pressure-sweating reflex” included patients with harlequin syndrome, Ross syn- Presumed segmental or unilateral compensatory drome15), cervical disc herniation, and lung cancer (cases hyperhidrosis is similar to phenomena seen in the are described below). Anhidrosis can likely be attributed efferent phase of the physiological “skin pressure- to underlying lesions. We were unable to confirm such a sweating reflex”. In this reflex, unilateral sweating is lesion, however, in some cases. suppressed segmentally primarily by ipsilateral skin The pathogenesis of these sweating disorders may be pressure, and thereby, sweating in the contralateral compensatory but remains unknown. In one case, hyper- same spinal segment is secondarily enhanced (Ogata et hidrosis that presented on the left side of the face and al., 1935)9) (Fig. 1)24). It is suggested that the pressure- anhidrosis that presented on the right hand started at sweating reflex is a somato-sympathetic reflex involving the same time. Hyperhidrosis of the left hand developed spinal segments where afferent impulses produced by 2.5 years later, and thus may have been compensatory5). skin pressure modify central sudomotor volleys. The Ross syndrome is a neurological disorder with progres - contralateral sweating increase is may response to the sive unilateral or bilateral segmental anhidrosis with ipsilateral sweating decrease. It is estimated that this a unilateral segmental compensatory hyperhidrotic latter efferent phase is compensation24). The mechanism band. The most disturbing symptom in these patients for this reflex may help to elucidate the hyperhidrotic is compensatory segmental hyperhidrosis, and often mechanism which is caused by the anhidrosis on the hypohidrosis or anhidrosis is not noticed. Yet, lesions contralateral same spinal segments or ipsilateral different that need to be treated would be exist at the sudomotor segments due to lesions. level in the anhidrotic area. First, cases of segmental or unilateral hyperhidrosis （26） Possible compensatory mechanisms of segmental and unilateral hyperhidrosis that are accompanied by anhidrotic areas will be dis- cussed, along with possible mechanisms for case observa- / 5 tions using results of studies that elucidate mechanisms 5 underlying the skin pressure-sweating reflex. &㻌 Cases Case 1: Unilateral hyperhidrosis A 45-year-old man presented with a chief complaint (CC) of hyperhidrosis since 30 years old. Findings from a thermoregulatory sweat test (TST) are provided in Fig. 2A. Sweat was identified by a change in color to dark purple (by an iodostarch reaction) in the indicator, 6ZHDWLQJDUHD 㻌 which was made up of the liquid mixture of povidone &RPSHQVDWLRQ 0HGLDOVDJLWWDOSODQH $ % iodine solution, absolute ethanol and castor oil painted )LJ Fig. 2 Case 1. A: Findings of the thermoregulatory sweat test. on the skin over the entire body, and the dispersed Sweat was identified by a change in color to dark purple (by potato starch powder (Minor’s method). The patient an iodostarch reaction) in the indicator, which was made up of was exposed at rest to an ambient temperature of the liquid mixture of povidone iodine solution, absolute ethanol 40°C and 50% relative humidity in an artificial climate and castor oil painted on the skin over the entire body, and chamber. Unilateral hyperhidrosis on the left side and the dispersed potato starch powder (Minor’s method). The unilateral anhidrosis in the right side occurred over the photographs and the sketch are shown. The patient was sitting at rest at an ambient temperature of 40°C and 50% whole body. Increased sweating on the left seemed to relative humidity in an artificial climate chamber. Unilateral be compensatory for anhidrosis on the right side, which hyperhidrosis on the left side and contralateral anhidrosis is horizontally contralateral in the same myelomere in occurred over the whole body. The horizontally increase in which hyperhidrosis is observed. sweating on the left side seemed
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