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Advances in dermatology and venereology Acta Dermato-Venereologica
Skin Ultrastructural Findings in Acquired Generalized Hypohidrosis / Anhidrosis in a Patient with Subclinical Sjögren Syndrome
Yu-Jen HUANG 1 , Pei-Ling CHIH 1 , Tzu-Hung HUANG 1 , Hsin-Su YU 1 – 3 , Yu-Lin HSIEH 4 , 5 # * and Sebastian YU 1 , 2 ,# *
1
Department of Dermatology , 5 Department of Medical Research , Kaohsiung Medical University Hospital , Kaohsiung Medical University , Kaohsiung 80708 , 2 Graduate Institute of Clinical Medicine , College of Medicine , 4 Department of Anatomy , College of Medicine , Kaohsiung Medical University , Kaohsiung , 3 National Institute of Environmental Health Sciences , National Health Research Institutes , Miaoli County , Taiwan . * E-mail : epor324 @ gmail . com and ylhsieh @ kmu . edu . tw
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These authors contributed equally . Accepted Apr 27 , 2017 ; Epub ahead of print Apr 27 , 2017
Sjögren ’ s syndrome ( SS ) is an autoimmune disorder characterized by exocrine dysfunction , such as xerophthalmia or xerostomia , focal lymphocytic sialoadenitis , and a high titre of autoantibodies inclusive of anti-Ro and anti-La . SS is also associated with both organic dermatological disorders and neuropathy . Dry skin is a complication in 23 – 67 % of patients with SS , especially those under 50 years of age ( 1 ). Two cases of acquired generalized hypohidrosis / anhidrosis ( AGHA ) associated with subclinical SS and absence of dryness of the eyes and mouth have been described previously ( 2 , 3 ). In addition , peripheral neuropathy , the spectrum of which includes autonomic neuropathy , is the most common neurological complication of SS , with a prevalence ranging from less than 2 % to greater than 60 % ( 4 ). Autonomic neuropathy may cause symptoms such as orthostatic hypotension , bowel dysfunction and anhidrosis . We report here a case of subclinical SS-associated AGHA .
CASE REPORT
A 35-year-old man experienced an episode of dry , hot sensation and fever up to 39 ° C after cycling . No chills , muscle soreness , dizziness , skin rash , itch or infections were noted at that time . Thereafter , persistently generalized decreased sweating was noted for 1 month . The patient had previously been healthy and his family history was unremarkable . He visited our outpatient department and hypohidrosis / anhidrosis was confirmed with a starch-iodine test , showing decreased or absent sweating on the face , trunk and extremities . In addition , intradermal injection of 1 % pilocarpine did not elicit sweating . Laboratory findings revealed elevated anti-Ro (> 240 EliAU / ml , normal < 7 EliAU / ml ), anti-La ( 190 EliAU / ml , normal < 7 EliAU / ml ), antinuclear antibody ( 1:40 ), anti-extractable nuclear antigen ( ENA > 32 , normal < 0.7 ), antimicrosomal antibody ( 331 IU / ml , normal < 35 IU / ml ), carcinoembryonic antigen ( CEA , 310 ng / ml , normal : 0 ~ 5 ng / ml ), serum IgG ( 1,960 mg / dl , normal : 700 ~ 1,600 mg / dl ) and IgE ( 153.0 IU / ml , normal < 87 IU / ml ). Serum complement C3 was reduced ( 72.5 mg / dl , normal : 90 ~ 180 mg / dl ). Anti-RNP , anti-Sm , anti-dsDNA , anti-Scl 70 antibodies , antiphospholipid antibodies ( APL ) IgG / IgM , anticardiolipin ( aCL ) IgG / IgM , rheumatoid factor , creatine phosphokinase ( CPK ), anti-mitochondrial antibody , HBs antigen , anti-HCV IgG and HIV test were negative . A complete blood cell count , renal function , alkaline phosphatase ( ALP ), bilirubin level , urinalysis , stool routine , were within normal limits , except for a slight elevation of aspartate aminotransferase ( AST ) and alanine transaminase ( ALT ), which might be due to certain inflammatory process . Salivary scintigraphy uncovered mild sialoadenitis and reduced salivary excretory function . Schirmer ’ s test was unremarkable . Brain magnetic resonance imaging did not reveal any notable
abnormality . Electromyography revealed abnormal sudomotor sympathetic function . A nerve conduction velocity test disclosed mild bilateral median motor neuropathy and mild right ulnar sensory neuropathy . Incisional biopsy of anhidrotic skin showed lymphocytic infiltration in the vessels of the upper dermis and the eccrine glands ( Fig . 1 ). No obvious structural abnormality was found in the epidermis , dermis and appendages , including sweat glands . Direct immunofluorescence was unremarkable . electron microscopy ( EM ) study of the sweat gland showed macrophage phagocytosed unmyelinated nerve bundle ( Remak bundle , Fig . 2A ) and the profiles of collagen pockets on Remak bundle ( Fig . 2B ), indicating the degeneration of sympathetic efferents to eccrine glands . The patient declined a labial biopsy .
It was considered that the patient ’ s anhidrosis might be associated with subclinical SS , in that no apparent dryness of the mouth or eyes could fully conform to diagnostic criteria . Sweating increased after intravenous steroid pulse therapy ( methylprednisolone 250 mg every 12 h for 3 days ), and remained stable for the following 8 months . The CEA level decreased significantly after treatment , from 310 ng / ml to 72 ng / ml at 3 weeks , and to 46 ng / ml at 8 months .
DISCUSSION
It has been suggested that a possible mechanism behind generalized anhidrosis associated with SS is an acquired anomaly of the sweat glands ( 3 ). In our patient , no remarkable structural abnormality was noticed , except for prominent lymphocytic infiltration around the vessels
Fig . 1 . Histopathology of anhidrotic skin . Biopsy showing eccrine glands ( EG ) and vessels ( Ve ) infiltrated by lymphocytes ( L ), indicting that skin inflammation underlies the anhidrosis observed in a patient with subclinical Sjögren ’ s syndrome . ( Haematoxylin and eosin staining . Bar : 100 µ m ).
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2690 Acta Derm Venereol 2017 ; 97 : 981 – 983