DOCSLIB.ORG

Acquired Idiopathic Generalized Anhidrosis: Case Report

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Acquired Idiopathic Generalized Anhidrosis: Case Report Acquired Idiopathic Generalized Anhidrosis: Case Report Elise I. Brantley, MD; Diya F. Mutasim, MD; Charles Heaton, MD We report a case of acquired idiopathic gener- abnormalities. Her medical history included hyper- alized anhidrosis (AIGA) in a 56-year-old white lipidemia and hypertension. At the time of onset, woman. Acquired idiopathic generalized anhi- she was not taking any medications or supplements. drosis is an exceedingly rare group of heteroge- Her current medications included lisinopril, con- neous disorders that has been almost exclusively jugated estrogens, and atorvastatin. The result of a reported in young Japanese males. Our case is full skin examination revealed no abnormalities. A unique in that AIGA may be underrecognized in starch-iodine test revealed minimal axillary hidrosis this patient population. and complete absence of sweating elsewhere. Her Cutis. 2011;87:21-23. workup included a complete blood cell count as well as a renal, liver, and metabolic panel; all results were normal. An electromyogram was normal. Magnetic cquired idiopathic generalized anhi- resonance imaging of the spine revealed multi-level drosis (AIGA) was first described in 1917 by cervical spondylosis with mild midcervical compres- A Lutembacher.1 It is anCUTIS exceedingly rare het- sion. The result of a complete neurologic evaluation, erogeneous group of disorders characterized by an epi- however, was negative, and it was determined that sodic burning sensation and erythema associated with the compression was not related to her symptoms. decreased or absent sweating. Most cases of AIGA Skin biopsy revealed a slight decrease in the number have been described in young Japanese males.2-6 We of eccrine coils with a few adjacent lymphocytes. report a case of AIGA in a 56-year-old white woman. Comment CaseDo Report NotAcquired Copy idiopathic generalized anhidrosis is an A 56-year-old white woman presented with an acute exceedingly rare group of heterogeneous disorders cutaneous burning sensation upon heat exposure of that clinically manifest as diffuse progressive loss of 10 years’ duration that was associated with decreased sweating. The typical patient is a young and other- sweating, elevated body temperature, and diffuse wise healthy male, occurring almost exclusively in erythema. The phenomenon initially involved her Japanese males.2-5,7 In general, heat exposure produces extremities and then progressed to involve her trunk an acute elevation of body temperature as well as a and face. Symptoms markedly improved with cool burning stinging sensation of the skin associated with showers and decreased body temperatures. Gaba- bright erythema and a lack of or marked decrease in pentin 300 mg every 8 hours and a 4-week course sweating. Many patients also have associated cho- of prednisone 40 mg daily provided no relief, while linergic urticaria and increased IgE levels.2,3,5,6 Few oral triamcinolone caused an acute increase in symp- cases have occurred immediately after heat stroke.8,9 toms. A complete review of systems revealed no A minority of the patients may have a progressive insidious onset of symptoms.6 Several histopathologic findings have been observed in AIGA, further contributing to the het- From the Department of Dermatology, University of Cincinnati, erogeneous nature of the disorder. The majority of Ohio. Dr. Mutasim also is from the Department of Pathology and cases demonstrate with normal-appearing eccrine Laboratory Medicine. glands; some cases have eccrine gland atrophy, periec- The authors report no conflict of interest. Correspondence: Diya F. Mutasim, MD, University of Cincinnati, crine lymphocytic or mast cell infiltrates, irregular Dermatology Department, Medical Science Building, 234 Goodman eccrine arrangements within the dermis and subcuta- 3-7,10,11 Ave, Cincinnati, OH 45267 ([email protected]). neous tissues, or rarely poral occlusion. WWW.CUTIS.COM VOLUME 87, JANUARY 2011 21 Copyright Cutis 2011. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Acquired Idiopathic Generalized Anhidrosis In cases reported following heat stroke, elec- exclusively in young Japanese males. We report a tron microscopy often revealed vacuolar changes white woman with AIGA. in the cytoplasm of eccrine secretory cells, which was sometimes appreciated on light microscopy.6,8,9 REFERENCES In some cases, immunohistochemistry studies have 1. Lutembacher R. Annales de Dermatologie et de shown changes in membrane glycoproteins, such as Syphilographie. 1917;6:470. Cited by: Dann EJ, Berkman decreased lectin binding.12 The significance of this N. Chronic idiopathic anhydrosis—a rare cause of heat observation is not known. stroke. Postgrad Med J. 1992;68:750-752. The role of neurotransmitters has been evaluated. 2. Itakura E, Urabe K, Yasumoto S, et al. Cholinergic urticaria Eccrine glands primarily are innervated by postgan- associated with acquired generalized hypohidrosis: report glionic cholinergic sympathetic nerves. Although of a case and review of the literature. Br J Dermatol. few patients with AIGA have been found to have 2000;143:1064-1066. a decrease in terminal synapse densities, most have 3. Nakazato Y, Tamura N, Ohkuma A, et al. Idiopathic pure normal neural density and activity.3,13 However, sev- sudomotor failure: anhidrosis due to deficits in cholinergic eral case studies have demonstrated a lack of response transmission. Neurology. 2004;63:1476-1480. to cholinesterase agonists such as pilocarpine, sug- 4. Murakami K, Sobue G, Terao S, et al. Acquired idiopathic gesting that the postsynaptic muscarinic receptors generalized anhidrosis: a distinctive clinical syndrome. J may be at fault.2,3,14 Neurol. 1988;235:428-431. Given the heterogeneous pathobiologic, clini- 5. Murakami K, Sobue G, Terao S, et al. A case of acquired cal, and histopathologic findings in AIGA, attempts generalized anhidrosis. Rinsho Shinkeigaku. 1987;27:511-517. have been made to further classify this disorder into 6. Thami GP, Kaur S, Kanwar AJ. Acquired idiopathic 3 subgroups: idiopathic pure sudomotor failure, sudo- generalized anhidrosis: a rare cause of heat intolerance. motor neuropathy, and sweat gland failure. Idiopathic Clin Exp Dermatol. 2003;28:262-264. pure sudomotor failure represents the vast majority of 7. Kobayashi H, Aiba S, Yamagishi T, et al. Cholinergic cases and includes patients thought to have cholin- urticaria, a new pathogenic concept: hypohidrosis due to ergic transmission and reception deficits. Sudomotor interference with the delivery of sweat to the skin surface. neuropathy reflects postganglionicCUTIS cholinergic dys- Dermatology. 2002;204:173-178. function, and sweat gland failure refers to the small 8. Chung TS, Lee SH. Idiopathic acquired anhidrosis: population of patients with inflammation and degen- reversible generalized anhidrosis after sunstroke. J eration of sweat glands on histopathology.15,16 Dermatol. 1999;26:615-618. Acquired idiopathic generalized anhidrosis often 9. Tsuji T, Yamamoto T. Acquired generalized anhidrosis. is resistant to treatment. Some patients respond to Arch Dermatol. 1976;112:1310-1314. short courses of prednisone, while some cases sponta- 10. Ando Y, Fujii S, Sakashita N, et al. Acquired idiopathic neouslyDo resolve; however, mostNot cases are chronic.10,17 generalizedCopy anhidrosis: clinical manifestations and Treatment is aimed at symptomatic relief and decreas- histochemical studies. J Neurol Sci. 1995;132:80-83. ing the core body temperature with the application 11. Ogino J, Saga K, Kagaya M, et al. Idiopathic acquired of cool water and avoidance of overheating.6 Our generalized anhidrosis due to occlusion of proximal coiled patient adapted to her environment by avoiding high ducts. Br J Dermatol. 2004;150:589-593. ambient temperatures and spraying her skin with cold 12. Terui T, Ohkawa Y, Tagami H. Idiopathic acquired water to maintain a normal core body temperature. generalized anhidrosis: electron-microscopic and Acquired anhidrosis has occurred in association immunohistochemical studies and analysis of lectin- or conjunction with several conditions including binding pattern of the cell membrane. Dermatologica. hereditary metabolic disorders such as Fabry disease, 1989;178:123-125. diabetes mellitus, and Hodgkin lymphoma; as part of 13. Miyazoe S, Matsuo H, Ohnishi A, et al. Acquired paraneoplastic syndromes, Sjögren disease, and other idiopathic generalized anhidrosis with isolated sudomotor connective tissue diseases; lymphocytic infundibulo- neuropathy. Ann Neurol. 1998;44:378-381. neurohypophysitis, an autoimmune disorder resulting 14. Murakami K, Sobue G, Iwase S, et al. Skin sympathetic in neurohypophyseal dysfunction; drugs; and other nerve activity in acquired idiopathic generalized causes of neuropathy.18-25 Therefore, a complete his- anhidrosis. Neurology. 1993;43:1137-1140. tory and workup should be done to rule out other 15. Chen YC, Wu CS, Chen GS, et al. Identification of conditions before diagnosing AIGA. subgroups of acquired idiopathic generalized anhidrosis. Neurologist. 2008;14:318-320. Conclusion 16. Palm F, Löser C, Gronau W, et al. Successful treatment Acquired idiopathic generalized anhidrosis is a rare of acquired idiopathic generalized anhidrosis. Neurology. group of heterogeneous disorders reported almost 2007;68:532-533. 22 CUTIS® WWW.CUTIS.COM Copyright Cutis 2011. No part of this publication may be reproduced, stored, or transmitted without the prior written permission
Load more

Recommended publications
  • Diagnosis and Treatment of Multiple System Atrophy: an Update
    ReviewSection Article Diagnosis and Treatment of Multiple System Atrophy: an Update Abstract the common parkinsonian variant (MSA-P) from PD. In his review provides an update on the diagnosis a clinicopathologic study1, primary neurologists (who Tand therapy of multiple system atrophy (MSA), a followed up the patients clinically) identified only 25% of sporadic neurodegenerative disorder characterised MSA patients at the first visit (42 months after disease clinically by any combination of parkinsonian, auto- onset) and even at their last neurological follow-up (74 nomic, cerebellar or pyramidal symptoms and signs months after disease onset), half of the patients were still and pathologically by cell loss, gliosis and glial cyto- misdiagnosed with the correct diagnosis in the other half plasmic inclusions in several brain and spinal cord being established on average 4 years after disease onset. structures. The term MSA was introduced in 1969 Mean rater sensitivity for movement disorder specialists although prior to this cases of MSA were reported was higher but still suboptimal at the first (56%) and last Gregor Wenning obtained an MD at the under the rubrics of striatonigral degeneration, olivo- (69%) visit. In 1998 an International Consensus University of Münster pontocerebellar atrophy, Shy-Drager syndrome and Conference promoted by the American Academy of (Germany) in 1991 and idiopathic orthostatic hypotension. In the late Neurology was convened to develop new and optimised a PhD at the University nineties, |-synuclein immunostaining was recognised criteria for a clinical diagnosis of MSA2, which are now of London in 1996. He received his neurology as the most sensitive marker of inclusion pathology in widely used by neurologists.
    [Show full text]
  • Post-Typhoid Anhidrosis: a Clinical Curiosity
    Post-typhoid anhidrosis 435 Postgrad Med J: first published as 10.1136/pgmj.71.837.435 on 1 July 1995. Downloaded from Post-typhoid anhidrosis: a clinical curiosity V Raveenthiran Summary family physician. Shortly after convalescence A 19-year-old girl developed generalised she felt vague discomfort and later recognised anhidrosis following typhoid fever. Elab- that she was not sweating as before. In the past orate investigations disclosed nothing seven years she never noticed sweating in any abnormal. A skin biopsy revealed the part ofher body. During the summer and after presence of atrophic as well as normal physical exercise she was disabled by an eccrine glands. This appears to be the episodic rise of body temperature (41.4°C was third case of its kind in the English recorded once). Such episodes were associated literature. It is postulated that typhoid with general malaise, headache, palpitations, fever might have damaged the efferent dyspnoea, chest pain, sore throat, dry mouth, pathway of sweating. muscular cramps, dizziness, syncope, inability to concentrate, and leucorrhoea. She attained Keywords: anhidrosis, hypohidrosis, sweat gland, menarche at the age of 12 and her menstrual typhoid fever cycles were normal. Hypothalamic functions such as hunger, thirst, emotions, libido, and sleep were normal. Two years before admission Anhidrosis is defined as the inability of the she had been investigated at another centre. A body to produce and/or deliver sweat to the skin biopsy performed there reported normal skin surface in the presence of an appropriate eccrine sweat glands. stimulus and environment' and has many forms An elaborate physical examination ofgeneral (box 1).
    [Show full text]
  • What Is the Autonomic Nervous System?
    J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.74.suppl_3.iii31 on 21 August 2003. Downloaded from AUTONOMIC DISEASES: CLINICAL FEATURES AND LABORATORY EVALUATION *iii31 Christopher J Mathias J Neurol Neurosurg Psychiatry 2003;74(Suppl III):iii31–iii41 he autonomic nervous system has a craniosacral parasympathetic and a thoracolumbar sym- pathetic pathway (fig 1) and supplies every organ in the body. It influences localised organ Tfunction and also integrated processes that control vital functions such as arterial blood pres- sure and body temperature. There are specific neurotransmitters in each system that influence ganglionic and post-ganglionic function (fig 2). The symptoms and signs of autonomic disease cover a wide spectrum (table 1) that vary depending upon the aetiology (tables 2 and 3). In some they are localised (table 4). Autonomic dis- ease can result in underactivity or overactivity. Sympathetic adrenergic failure causes orthostatic (postural) hypotension and in the male ejaculatory failure, while sympathetic cholinergic failure results in anhidrosis; parasympathetic failure causes dilated pupils, a fixed heart rate, a sluggish urinary bladder, an atonic large bowel and, in the male, erectile failure. With autonomic hyperac- tivity, the reverse occurs. In some disorders, particularly in neurally mediated syncope, there may be a combination of effects, with bradycardia caused by parasympathetic activity and hypotension resulting from withdrawal of sympathetic activity. The history is of particular importance in the consideration and recognition of autonomic disease, and in separating dysfunction that may result from non-autonomic disorders. CLINICAL FEATURES c copyright. General aspects Autonomic disease may present at any age group; at birth in familial dysautonomia (Riley-Day syndrome), in teenage years in vasovagal syncope, and between the ages of 30–50 years in familial amyloid polyneuropathy (FAP).
    [Show full text]
  • Evaluating Patients' Unmet Needs in Hidradenitis Suppurativa
    Evaluating patients’ unmet needs in hidradenitis suppurativa: Results from the Global Survey Of Impact and Healthcare Needs (VOICE) Project Amit Garg, Erica Neuren, Denny Cha, Joslyn Kirby, John Ingram, Gregor B.E. Jemec, Solveig Esmann, Linnea Thorlacius, Bente Villumsen, Véronique Del Marmol, et al. To cite this version: Amit Garg, Erica Neuren, Denny Cha, Joslyn Kirby, John Ingram, et al.. Evaluating patients’ unmet needs in hidradenitis suppurativa: Results from the Global Survey Of Impact and Healthcare Needs (VOICE) Project. Journal of The American Academy of Dermatology, Elsevier, 2020, 82 (2), pp.366- 376. 10.1016/j.jaad.2019.06.1301. pasteur-02547249 HAL Id: pasteur-02547249 https://hal-pasteur.archives-ouvertes.fr/pasteur-02547249 Submitted on 19 Apr 2020 HAL is a multi-disciplinary open access L’archive ouverte pluridisciplinaire HAL, est archive for the deposit and dissemination of sci- destinée au dépôt et à la diffusion de documents entific research documents, whether they are pub- scientifiques de niveau recherche, publiés ou non, lished or not. The documents may come from émanant des établissements d’enseignement et de teaching and research institutions in France or recherche français ou étrangers, des laboratoires abroad, or from public or private research centers. publics ou privés. LETTERS TO THE EDITOR A TP63 Mutation Causes Prominent Alopecia with Mild Ectodermal Dysplasia Journal of Investigative Dermatology (2019) -, -e-; doi:10.1016/j.jid.2019.06.154 TO THE EDITOR synechiae (IV.5). Altogether, these mi- ectodermal, orofacial, and limb devel- TP63 mutations are the primary source nor ectodermal abnormalities sug- opment (Rinne et al., 2007). The use of of several autosomal dominant ecto- gested an unclassified form of different transcription initiation sites dermal dysplasias, which are charac- ectodermal dysplasias.
    [Show full text]
  • Keratitis-Ichthyosis-Deafness Syndrome in Association With
    Genes and skin Eur J Dermatol 2005; 15 (5): 347-52 Laura MAINTZ1 Regina C. BETZ2 Keratitis-ichthyosis-deafness syndrome Jean-Pierre ALLAM1 in association with follicular occlusion triad Jörg WENZEL1 Axel JAKSCHE3 Nicolaus FRIEDRICHS4 Thomas BIEBER1 Keratitis-Ichthyosis-Deafness syndrome is a rare congenital disorder of Natalija NOVAK1 the ectoderm caused by mutations in the connexin-26 gene (GJB2) on 1 chromosome 13q11-q12, giving rise to keratitis, erythrokeratoderma Department of Dermatology, University of and neurosensory deafness. We report the case of a 31-year-old black Bonn, Sigmund-Freud-Str. 25, 53105 Bonn, Germany male diagnosed as having KID syndrome. Sequencing analysis showed 2 Institute of Human Genetics, University of a heterozygous missense mutation D50N (148G > A) in the GJB2 gene. Bonn, Wilhelmstr. 31, 53115 Bonn, In addition to the classical features of vascularizing keratitis, erythro- Germany 3 Department of Ophthalmology, University keratoderma and congenital deafness, our patient presented a follicular of Bonn, Sigmund-Freud-Str. 25, 53105 occlusion triad with hidradenitis suppurativa (HS, alias acne inversa), Bonn, Germany acne conglobata and dissecting cellulitis of the scalp, leading to cicatri- 4 Institute of Pathology, University of Bonn, Sigmund-Freud-Str. 25, 53105 Bonn, cial alopecia and disfiguring, inflammatory vegetations of his scalp. Germany Conservative therapy such as a keratolytic, rehydrating and antiseptic external therapy, antibiotic, antimycotic and retinoids were only of Reprints: N. Novak moderate benefit, so we finally chose the curative possibility of surgery Fax: (+49) 228 287 4883 <[email protected]> therapy of the axillar papillomas and of the scalp. The inflammatory papillomatous regions of the axillae and of the scalp were radically debrided.
    [Show full text]
  • Pili Torti: a Feature of Numerous Congenital and Acquired Conditions
    Journal of Clinical Medicine Review Pili Torti: A Feature of Numerous Congenital and Acquired Conditions Aleksandra Hoffmann 1 , Anna Wa´skiel-Burnat 1,*, Jakub Z˙ ółkiewicz 1 , Leszek Blicharz 1, Adriana Rakowska 1, Mohamad Goldust 2 , Małgorzata Olszewska 1 and Lidia Rudnicka 1 1 Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland; [email protected] (A.H.); [email protected] (J.Z.);˙ [email protected] (L.B.); [email protected] (A.R.); [email protected] (M.O.); [email protected] (L.R.) 2 Department of Dermatology, University Medical Center of the Johannes Gutenberg University, 55122 Mainz, Germany; [email protected] * Correspondence: [email protected]; Tel.: +48-22-5021-324; Fax: +48-22-824-2200 Abstract: Pili torti is a rare condition characterized by the presence of the hair shaft, which is flattened at irregular intervals and twisted 180◦ along its long axis. It is a form of hair shaft disorder with increased fragility. The condition is classified into inherited and acquired. Inherited forms may be either isolated or associated with numerous genetic diseases or syndromes (e.g., Menkes disease, Björnstad syndrome, Netherton syndrome, and Bazex-Dupré-Christol syndrome). Moreover, pili torti may be a feature of various ectodermal dysplasias (such as Rapp-Hodgkin syndrome and Ankyloblepharon-ectodermal defects-cleft lip/palate syndrome). Acquired pili torti was described in numerous forms of alopecia (e.g., lichen planopilaris, discoid lupus erythematosus, dissecting Citation: Hoffmann, A.; cellulitis, folliculitis decalvans, alopecia areata) as well as neoplastic and systemic diseases (such Wa´skiel-Burnat,A.; Zółkiewicz,˙ J.; as cutaneous T-cell lymphoma, scalp metastasis of breast cancer, anorexia nervosa, malnutrition, Blicharz, L.; Rakowska, A.; Goldust, M.; Olszewska, M.; Rudnicka, L.
    [Show full text]
  • 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.
    [Show full text]
  • Drug-Induced Hyperhidrosis and Hypohidrosis Incidence, Prevention and Management
    Drug Safety 2008; 31 (2): 109-126 REVIEW ARTICLE 0114-5916/08/0002-0109/$48.00/0 © 2008 Adis Data Information BV. All rights reserved. Drug-Induced Hyperhidrosis and Hypohidrosis Incidence, Prevention and Management William P. Cheshire Jr1 and Robert D. Fealey2 1 Department of Neurology, Autonomic Reflex Laboratory, Mayo Clinic, Jacksonville, Florida, USA 2 Department of Neurology, Thermoregulatory Sweating Laboratory, Mayo Clinic, Rochester, Minnesota, USA Contents Abstract ....................................................................................109 1. Clinical Importance ......................................................................110 1.1 Hyperhidrosis ........................................................................110 1.2 Hypohidrosis .........................................................................111 2. Sites of Drug Action ......................................................................112 3. Drug-Induced Hyperhidrosis ...............................................................114 3.1 Anticholinesterases ..................................................................114 3.2 Antidepressants......................................................................114 3.3 Antiglaucoma Agents ................................................................115 3.4 Bladder Stimulants ...................................................................115 3.5 Drugs for Dementia ..................................................................115 3.6 Opioids .............................................................................115
    [Show full text]
  • Neurological Aspects of Anhidrosis: Differential Diagnoses and Diagnostic Tools
    REVIEW ARTICLE Ann Clin Neurophysiol 2019;21(1):1-6 https://doi.org/10.14253/acn.2019.21.1.1 ANNALS OF CLINICAL NEUROPHYSIOLOGY Neurological aspects of anhidrosis: differential diagnoses and diagnostic tools Kee Hong Park1 and Ki-Jong Park2,3 1Department of Neurology, Gyeongsang National University Hospital, Jinju, Korea 2Department of Neurology, Gyeongsang National University Changwon Hospital, Changwon, Korea 3Department of Neurology, Gyeongsang National University School of Medicine, Jinju, Korea Received: June 21, 2018 Revised: August 7, 2018 Anhidrosis refers to the condition in which the body does not respond appropriately to Accepted: August 13, 2018 thermal stimuli by sweating. Sweating plays an important role in maintaining the body tem- perature, and its absence should not be overlooked since an elevated body temperature can cause various symptoms, even leading to death when uncontrolled. The various neurological disorders that can induce anhidrosis make a detailed neurological evaluation essential. The medication history of the patient should also be checked because anhidrosis can be caused Correspondence to by various drugs. The tests available for evaluating sweating include the quantitative sudo- Ki-Jong Park motor axon reflex sweat test, thermoregulatory sweat test, sympathetic skin response, and Department of Neurology, Gyeongsang electrochemical skin conductance. Pathological findings can also be checked directly in a skin National University School of Medicine, 79 Gangnam-ro, Jinju 52727, Korea biopsy. This review discusses the differential diagnosis and evaluation of anhidrosis. Tel: +82-55-214-3810 Fax: +82-55-214-3255 Key words: Anhidrosis; Sweating; Autonomic nervous system E-mail: [email protected] ORCID Kee Hong Park http://orcid.org/0000-0001-5724-7432 I NTRODUCTION Ki-Jong Park http://orcid.org/0000-0003-4391-6265 Sweating is controlled by the autonomic nervous system and performs an important function in keeping the body temperature constant.
    [Show full text]
  • C. Pyoderma Gangrenosum
    Volume 19 Number 6 June 2013 Review Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis Noah Scheinfeld MD Dermatology Online Journal 19 (6): 2 150 West 55th Street NYC NY 100128 Correspondence: Noah Scheinfeld MD: [email protected] Abstract Hidradenitis suppurativa (HS), a pathologic follicular disease, impacts patients' lives profoundly and usually occurs in isolation. The diseases with the strongest association are obesity, depression, and pain. HS is associated with many diseases including acne conglobata (AC), dissecting cellulitis, pilonidal cysts, and obesity. Pyoderma fistulans sinifica (fox den disease) appears to be the same entity as Hurley Stage 2 of 3 HS. The rate of acne vulgaris in HS patients mirrors unaffected controls. The most common, albeit still uncommon, association is with seronegative, haplotype unlinked arthritis (most importantly B27), in particular spondolyarthritis. Crohn disease and HS occur together at a rate that varies from 0.6% to 38% in retrospective cases series. Ulcerative colitis occurred with HS in 14% of patients in one series. The next most common association is with pyoderma gangrenosum, but this association is likely under-reported. Synovitis-Acne-Pustulosis Hyperostosis-Osteitis (SAPHO) syndrome, which is rare, has more than 10 reports linking it to HS. Nine case reports have linked Dowling-Degos disease (DDD) to HS and two reports related HS to Fox-Fordyce disease (FF), but because both occur in the axilla this might be a mere coincidence. HS is rarely associated with ophthalmic pathology. Specifically, more than 5 reports link it to Keratitis-Ichthyosis-Deafness syndrome (KID); greater than10 cases link it to interstitial keratitis and 2 cases are linked to Behçet’s disease.
    [Show full text]
  • Progression and Prognosis in Pure Autonomic Failure (PAF
    947 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2004.049023 on 16 June 2005. Downloaded from PAPER Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy N Mabuchi, M Hirayama, Y Koike, H Watanabe, H Ito, R Kobayashi, K Hamada, G Sobue ............................................................................................................................... J Neurol Neurosurg Psychiatry 2005;76:947–952. doi: 10.1136/jnnp.2004.049023 Objective: To clarify the progression of autonomic symptoms and functional deterioration in pure autonomic failure (PAF), particularly in comparison with multiple system atrophy (MSA). See end of article for Methods: The investigation involved eight patients with PAF (M/F = 7/1; mean age at onset, 57 years) and authors’ affiliations ....................... 22 with probable MSA matched for age at onset (M/F = 14/8; onset 56 years). Subjects were followed up for neurological symptoms, activities of daily living, and autonomic function for more than seven years. Correspondence to: Autonomic functional tests were carried out. Dr Gen Sobue, Department of Neurology, Results: In PAF, fainting or sudomotor dysfunction occurred first, followed by constipation and syncope. Nagoya University Urinary dysfunction developed late, and respiratory dysfunction was not evident. This clinical course Graduate School of contrasted sharply with that in MSA, where early urinary dysfunction usually proceeded to sudomotor Medicine, Nagoya 466– 8550, Japan; sobueg@ dysfunction or orthostatic hypotension (p = 0.004), followed by respiratory dysfunction (p = 0.0004). med.nagoya-u.ac.jp Results of pharmacological tests also distinguished PAF from MSA. Progression and prognosis in patients with PAF did not worsen, unlike the steady progressive autonomic dysfunction in MSA (p,0.0001, Received 6 July 2004 p,0.0001, p = 0.0009, and p = 0.003, for progression to modified Rankin scale grade III, IV, V, and In revised form 15 October 2004 death, respectively).
    [Show full text]
  • Sjogren Syndrome: Neurologic Complications Veronica P Cipriani MD MS ( Dr
    Sjogren syndrome: neurologic complications Veronica P Cipriani MD MS ( Dr. Cipriani of the University of Chicago Medical Center received consulting fees from Genetech as an advisory board participant ) Francesc Graus MD PhD, editor. ( Dr. Graus of the University of Barcelona has no relevant financial relationships to disclose.) Originally released June 28, 2006; last updated June 30, 2018; expires June 30, 2021 Introduction This article includes discussion of the neurologic complications of Sjögren syndrome, Gougerot-Sjögren syndrome, and Sicca complex. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations. Overview Neurologic manifestations occur in 20% to 27% of patients with Sjögren syndrome, often preceding the diagnosis of this systemic autoimmune disease. The peripheral nervous system, skeletal muscles, and central nervous system may be involved. Sjögren syndrome has been associated with neuromyelitis optica with positive serum aquaporin autoantibody. The symptoms of neurologic Sjögren syndrome may also mimic multiple sclerosis. HTLV-1 infection and vitamin B12 deficiency can complicate Sjögren myeloneuropathies. In this article, the author reviews the clinical presentations and postulated pathogenesis of these complications and offers current treatment recommendations. Key points • Sjögren syndrome is a common autoimmune disease, particularly among postmenopausal women, and it is manifested by dry mouth, dry eyes, fatigue, and arthralgias. • Neurologic symptoms occur in 20% to 27% of patients with Sjögren syndrome due to involvement of cranial nerves (Bell palsy, trigeminal neuralgia, diplopia), peripheral nerves (sensorimotor neuropathies), skeletal muscles (fibromyalgia, polymyositis), and the central nervous system. • Sjögren syndrome can mimic the symptoms and radiographic features of multiple sclerosis. • Sjögren syndrome is closely associated with neuromyelitis optica, a demyelinating disease of the central nervous system caused by antibodies to aquaporin-4 (NMO IgG).
    [Show full text]