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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
#
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