We report a case of acquired generalized anhidrosis successfully treated with cyclosporine. A skin biopsy showed T cell infiltration around the sweat glands and labial biopsy revealed lymphoplasmacytic infiltration around the minor salivary gland, suggesting an underlying autoimmune disease such as Sjögren's syndrome. Administration of cyclosporine markedly improved the patient's condition and sympathetic skin response; thus cyclosporine may be effective for treating anhidrosis in patients with autoimmune disorders.

Keywords:
Acquired generalized anhidrosis, Cyclosporine, Sympathetic skin response, Sjögren’s syndrome
1.
Greenspan JS, Daniels TE, Talal N, Sylvester RA: The histopathology of Sjögren's syndrome in labial salivary gland biopsies. Oral Surg Oral Med Oral Pathol 1974;37:217-229.
2.
Shoenfeld Y, Shapiro Y, Fisher BK, Dvoretzky I: Sweating in the ‘anhidrotic type' of congenital ectodermal dysplasia. Dermatologica 1975;150:277-282.
3.
Mancini AJ, Lawley LP, Uzel G: X-linked ectodermal dysplasia with immunodeficiency caused by NEMO mutation: early recognition and diagnosis. Arch Dermatol 2008;144:342-346.
4.
Mardy S, Miura Y, Endo F, Matsuda I, Sztriha L, Frossard P, Moosa A, Ismail EA, Macaya A, Andria G, Toscano E, Gibson W, Graham GE, Indo Y: Congenital insensitivity to pain with anhidrosis: novel mutations in the TRKA (NTRK1) gene encoding a high-affinity receptor for nerve growth factor. Am J Hum Genet 1999;64:1570-1579.
5.
Germain DP, Salard D, Fellmann F, Azibi K, Caillaud C, Bernard MC, Poenaru L: Identification of a novel de novo mutation (G373D) in the alpha-galactosidase A gene (GLA) in a patient affected with Fabry disease. Hum Mutat 2001;17:353.
6.
Lao LM, Kumakiri M, Mima H, Kuwahara H, Ishida H, Ishiguro K, Fujita T, Ueda K: The ultrastructural characteristics of eccrine sweat glands in a Fabry disease patient with hypohidrosis. J Dermatol Sci 1998;18:109-117.
7.
Yamamoto K, Sobue G, Iwase S, Kumazawa K, Mitsuma T, Mano T: Possible mechanism of anhidrosis in a symptomatic female carrier of Fabry's disease: an assessment by skin sympathetic nerve activity and sympathetic skin response. Clin Auton Res 1996;6:107-110.
8.
Sulzberger MB, Griffin TB: Induced miliaria, postmiliarial hypohidrosis, and some potential sequelae. Arch Dermatol 1969;99:145-151.
9.
Commens CA, Greaves MW: Anhidrosis following exfoliative dermatitis. Clin Exp Dermatol 1978;3:99-101.
10.
Shelley WB, Horvath PN, Pillsbury DM: Anhidrosis. An etiologic interpretation. Medicine (Baltimore) 1950;29:195-224.
11.
Paquette DL, Falanga V: Cutaneous concerns of scleroderma patients. J Dermatol 2003;30:438-443.
12.
Raveenthiran V: Post-typhoid anhidrosis: a clinical curiosity. Postgrad Med J 1995;71:435-436.
13.
Dubin DB, Hurowitz JC, Brettler D, Bernhard JD, Kadin ME, Wallace JE, Woda B, Pihan G, Kupper TS, Harrist TJ: Adnexotropic T-cell lymphoma presenting with generalized anhidrosis, progressive alopecia, pruritus, and Sjögren's syndrome. J Am Acad Dermatol 1998;38:493-497.
14.
Person JR: Autoimmune anhidrosis. J Am Acad Dermatol 1984;10:117.
15.
Nakazato Y, Tamura N, Ohkuma A, Yoshimaru K, Shimazu K: Idiopathic pure sudomotor failure: anhidrosis due to deficits in cholinergic transmission. Neurology 2004;63:1476-1480.
16.
Dahl MV: Atopic dermatitis: the concept of flare factors. South Med J 1977;70:453-455.
17.
Eishi K, Lee JB, Bae SJ, Takenaka M, Katayama I: Impaired sweating function in adult atopic dermatitis: results of the quantitative sudomotor axon reflex test. Br J Dermatol 2002;147:683-688.
18.
Kitaba S, Matsui S, Iimuro E, Nishioka M, Kijima A, Umegaki N, Murota H, Katayama I: Four cases of atopic dermatitis complicated by Sjögren's syndrome: link between dry skin and autoimmune anhidrosis. Allergol Int 2011;60:387-391.
19.
Gemignani F, Manganelli P, Pavesi G, Marbini A: Polyneuropathy in Sjögren's syndrome. A case of prevalently autonomic neuropathy with tonic pupil and hypohidrosis. Funct Neurol 1988;3:337-348.
20.
Kumazawa K, Sobue G, Yamamoto K, Mitsuma T: Segmental anhidrosis in the spinal dermatomes in Sjögren's syndrome-associated neuropathy. Neurology 1993;43:1820-1823.
21.
Goto H, Matsuo H, Fukudome T, Shibuya N, Ohnishi A, Nakamura H: Chronic autonomic neuropathy in a patient with primary Sjögren's syndrome. J Neurol Neurosurg Psychiatry 2000;69:135.
22.
Mitchell J, Greenspan J, Daniels T, Whitcher JP, Maibach HI: Anhidrosis (hypohidrosis) in Sjögren's syndrome. J Am Acad Dermatol 1987;16:233-235.
23.
Huang CL, Kuo TT, Chan HL: Acquired generalized hypohidrosis/anhidrosis with subclinical Sjögren's syndrome: report of a case with diffuse syringolymphoid hyperplasia and lymphocytic sialadenitis. J Am Acad Dermatol 1996;35:350-352.
24.
Sais G, Admella C, Fantova MJ, Montero JC: Lymphocytic autoimmune hidradenitis, cutaneous leucocytoclastic vasculitis and primary Sjögren's syndrome. Br J Dermatol 1998;139:1073-1076.
25.
Maeda A, Yamanouchi H, Lee JB, Katayama I: Oral prednisolone improved acetylcholine-induced sweating in Sjögren's syndrome-related anhidrosis. Clin Rheumatol 2000;19:396-397.
26.
Tsuji T, Yamamoto T: Acquired generalized anhidrosis. Arch Dermatol 1976;112:1310-1314.
27.
Chung TS, Lee SH: Idiopathic acquired anhidrosis: reversible generalized anhidrosis after sunstroke. J Dermatol 1999;26:615-618.
28.
Sekiguchi T, Ishibashi S, Kubodera T, Fukabori J, Uezato A, Kanbayashi T, Takahashi T, Yokota T, Mizusawa H: Anhidrosis associated with hypothalamic lesions related to anti-aquaporin 4 autoantibody. J Neurol 2011;258:2293-2295.
29.
Saft C, Andrich J, Stücker M, Przuntek H, Müller T: Asymmetric anhidrosis in MSA. Neurology 2003;61:231.
30.
Niimi Y, Ieda T, Hirayama M, Koike Y, Sobue G, Hasegawa Y, Takahashi A: Clinical and physiological characteristics of autonomic failure with Parkinson's disease. Clin Auton Res 1999;9:139-144.
31.
Arcas J, Ferrer T, Roche MC, Martinez-Bermejo A, López-Martin V: Hypohidrosis related to the administration of topiramate to children. Epilepsia 2001;42:1363-1365.
32.
Shimizu T, Yamashita Y, Satoi M, Togo A, Wada N, Matsuishi T, Ohnishi A, Kato H: Heat stroke-like episode in a child caused by zonisamide. Brain Dev 1997;19:366-368.
33.
Fealey RD, Low PA, Thomas JE: Thermoregulatory sweating abnormalities in diabetes mellitus. Mayo Clin Proc 1989;64:617-628.
34.
Goodman JI: Diabetic anhidrosis. Am J Med 1966;41:831-835.
35.
Jensen K, Andersen K, Smith T, Henriksen O, Melgaard B: Sympathetic vasoconstrictor nerve function in alcoholic neuropathy. Clin Physiol 1984;4:253-262.
36.
Katoch K: Autonomic nerve affection in leprosy. Indian J Lepr 1996;68:49-54.
37.
Ghosh PS, Mitra S, Fealey RD: Generalized anhidrosis in a child following presumptive H1N1 influenza. Clin Auton Res 2012;22:109-112.
38.
Bushara KO, Park DM: Botulinum toxin and sweating. J Neurol Neurosurg Psychiatry 1994;57:1437-1438.
39.
Tuck RR, McLeod JG: Autonomic dysfunction in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 1981;44:983-990.
40.
Klein CM, Vernino S, Lennon VA, Sandroni P, Fealey RD, Benrud-Larson L, Sletten D, Low PA: The spectrum of autoimmune autonomic neuropathies. Ann Neurol 2003;53:752-758.
41.
Weller M, Wilhelm H, Sommer N, Dichgans J, Wiethölter H: Tonic pupil, areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature. J Neurol 1992;239:231-234.
42.
Kalapesi FB, Krishnan AV, Kiernan MC: Segmental facial anhidrosis and tonic pupils with preserved deep tendon reflexes: a novel autonomic neuropathy. J Neuroophthalmol 2005;25:5-8.
43.
Arima T, Ando Y, Ando E, Okamura R, Sakashita N, Tanaka Y, Uchino M, Ando M: Secondary amyloidosis with severe autonomic dysfunctions. J Auton Nerv Syst 1995;52:77-81.
44.
Miyazoe S, Matsuo H, Ohnishi A, Tajima F, Fujishita S, Ichinose K, Shibuya N: Acquired idiopathic generalized anhidrosis with isolated sudomotor neuropathy. Ann Neurol 1998;44:378-381.
45.
Nakazato Y, Tamura N, Yamamoto T, Shimazu K: A case of Sjögren's syndrome with heat intolerance induced by generalized anhidrosis as its principal feature (in Japanese). Rinsho Shinkeigaku 2002;42:171-174.
46.
Nakazato Y, Tamura N, Ohkuma A, Yoshimaru K, Shimazu K: QSART in idiopathic pure sudomotor failure. Clin Auton Res 2005;15:414-416.
47.
Strauch JH: Acquired generalized anhidrosis with localized hyperhidrosis. Arch Derm Syphilol 1946;53:70.
48.
Weigand DA, Everett MA: Acquired generalized anhidrosis. Arch Dermatol 1966;93:443-445.
49.
Kay DM, Maibach HI: Pruritus and acquired anhidrosis. Two unusual cases. Arch Dermatol 1969;100:291-293.
50.
Murakami K, Sobue G, Terao S, Mitsuma T: Acquired idiopathic generalized anhidrosis: a distinctive clinical syndrome. J Neurol 1988;235:428-431.
51.
Terui T, Ohkawa Y, Tagami H: Idiopathic acquired generalized anhidrosis: electron-microscopic and immunohistochemical studies and analysis of lectin-binding pattern of the cell membrane. Dermatologica 1989;178:123-125.
52.
Dann EJ, Berkman N: Chronic idiopathic anhydrosis - a rare cause of heat stroke. Postgrad Med J 1992;68:750-752.
53.
Murakami K, Sobue G, Iwase S, Mitsuma T, Mano T: Skin sympathetic nerve activity in acquired idiopathic generalized anhidrosis. Neurology 1993;43:1137-1140.
54.
Ando Y, Fujii S, Sakashita N, Uchino M, Ando M: Acquired idiopathic generalized anhidrosis: clinical manifestations and histochemical studies. J Neurol Sci 1995;132:80-83.
55.
Itakura E, Urabe K, Yasumoto S, Nakayama J, Furue M: Cholinergic urticaria associated with acquired generalized hypohidrosis: report of a case and review of the literature. Br J Dermatol 2000;143:1064-1066.
56.
Kobayashi H, Aiba S, Yamagishi T, Tanita M, Hara M, Saito H, Tagami H: Cholinergic urticaria, a new pathogenic concept: hypohidrosis due to interference with the delivery of sweat to the skin surface. Dermatology 2002;204:173-178.
57.
Thami GP, Kaur S, Kanwar AJ: Acquired idiopathic generalized anhidrosis: a rare cause of heat intolerance. Clin Exp Dermatol 2003;28:262-264.
58.
Ogino J, Saga K, Kagaya M, Kamada A, Kaneko R, Jimbow K: Idiopathic acquired generalized anhidrosis due to occlusion of proximal coiled ducts. Br J Dermatol 2004;150:589-593.
59.
Asahina M, Suzuki A, Tamura N, Yoshida T, Hattori T: Acquired generalized anhidrosis associated with lymphocytic infundibuloneurohypophysitis. Neurology 2004;63:1752.
60.
Palm F, Löser C, Gronau W, Voigtländer V, Grau AJ: Successful treatment of acquired idiopathic generalized anhidrosis. Neurology 2007;68:532-533.
61.
Fukunaga A, Horikawa T, Sato M, Nishigori C: Acquired idiopathic generalized anhidrosis: possible pathogenic role of mast cells. Br J Dermatol 2009;160:1337-1340.
62.
Nakamizo S, Miyachi Y, Kabashima K: A case of cholinergic urticaria associated with acquired generalized hypohidrosis and abnormal neurological findings: association with incomplete Ross syndrome? Br J Dermatol 2010;162:903-905.
63.
Blantley EI, Mutasim DF, Heaton C: Acquired idiopathic generalized anhidrosis: case report. Cutis 2011;87:21-23.
64.
Gumus P, Luquette M, Haymon ML, Valerie E, Morales J, Vargas A: Virilizing para-adrenocortical adenoma associated with idiopathic-acquired generalized anhidrosis in an adolescent girl. J Pediatr Endocrinol Metab 2011;24:233-235.
65.
Sawada Y, Bito T, Kabashima R, Sugita K, Nakamura M, Tokura Y: Epileptic seizures and cholinergic urticaria with generalized hypohidrosis or anhidrosis. Eur J Dermatol 2011;21:99-100.
66.
Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ: Sjögren's syndrome: a clinical, pathological, and serological study of sixty-two cases. Medicine (Baltimore) 1965;44:187-231.
67.
Whaley K, Williamson J, Chisholm DM, Webb J, Mason DK, Buchanan WW: Sjogren's syndrome. I. Sicca components. Q J Med 1973;42:279-304.
68.
Katayama I, Yokozeki H, Nishioka K: Impaired sweating as an exocrine manifestation in Sjögren's syndrome. Br J Dermatol 1995;133:716-720.
69.
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH; European Study Group on Classification Criteria for Sjögren's Syndrome: Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-558.
70.
Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, Schiødt M, Umehara H, Vivino F, Zhao Y, Dong Y, Greenspan D, Heidenreich AM, Helin P, Kirkham B, Kitagawa K, Larkin G, Li M, Lietman T, Lindegaard J, McNamara N, Sack K, Shirlaw P, Sugai S, Vollenweider C, Whitcher J, Wu A, Zhang S, Zhang W, Greenspan J, Daniels T; Sjögren's International Collaborative Clinical Alliance (SICCA) Research Groups: American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken) 2012;64:475-487.
71.
Fujibayashi T, Sugai S, Miyasaka N, Hayashi Y, Tsubota K: Revised Japanese criteria for Sjögren's syndrome (1999): availability and validity. Mod Rheumatol 2004;14:425-434.
72.
Ellman P, Weber F, Goodier TE: A contribution to the pathology of Sjögren's disease. Q J Med 1951;20:33-42.
73.
Tsubota K, Fujita H, Tadano K, Takeuchi T, Murakami T, Saito I, Hayashi Y: Improvement of lacrimal function by topical application of CyA in murine models of Sjögren's syndrome. Invest Ophthalmol Vis Sci 2001;42:101-110.
74.
Vetrugno R, Liquori R, Cortelli P, Montagna P: Sympathetic skin response: basic mechanisms and clinical applications. Clin Auton Res 2003;13:256-270.
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