AUTHOR=Shimada Yoko , Ono Hiroto , Tochigi Mei , Taga Fumiaki , Shimizu Akira , Kawano Mitsuhiro , Asahina Masato , Sano Kenji 
  
TITLE=Case Report: Sjögren’s disease diagnosed following generalized anhidrosis
  
JOURNAL=Journal of Cutaneous Immunology and Allergy
  
VOLUME=Volume 9 - 2026
  
YEAR=2026
  
URL=https://www.frontierspartnerships.org/journals/journal-of-cutaneous-immunology-and-allergy/articles/10.3389/jcia.2026.17427
  
DOI=10.3389/jcia.2026.17427
  
ISSN=2574-4593
  
ABSTRACT=Sjögren’s disease typically presents with initial symptoms such as dry mouth, dry eyes, fatigue, and joint pain. This report describes a case of Sjögren’s disease diagnosed following the onset of generalized anhidrosis. The patient had experienced anhidrosis and elevated body temperature for the past 10 yrs. Physical examination revealed no skin rash or cholinergic urticaria. The Minor’s test revealed anhidrosis over 98% of the body surface area. Although idiopathic anhidrosis was initially suspected, a detailed medical history revealed a history of dry eyes, dry mouth, and extensive dental caries. Laboratory tests showed positive antinuclear antibodies and anti-SS-A/Ro antibodies, while serum carcinoembryonic antigen levels and thyroid function were normal. Salivary gland scintigraphy and biopsy confirmed the diagnosis of Sjögren’s disease. Histopathological examination of the anhidrotic areas revealed no obvious inflammatory cell infiltration or glandular atrophy characteristic of Sjögren’s disease, but showed hydropic degeneration of the sweat glands and disruption of the two-cell-layer structure of clear and dark cells. These characteristic pathological changes are observed in acquired idiopathic generalized anhidrosis. However, this patient was a woman in her 50s who lacked typical features of acquired idiopathic generalized anhidrosis, such as elevated serum carcinoembryonic antigen levels and cholinergic urticaria. She did not respond to a half-pulse steroid therapy. Taking these factors into account, it was determined that this case was clinically diagnosed generalized anhidrosis associated with Sjögren’s disease. This case represents a valuable example of a diagnosis of Sjögren’s disease arising from the atypical initial symptom of generalized anhidrosis. In patients with anhidrosis, particularly those suspected of having acquired idiopathic generalized anhidrosis, it is extremely important to conduct a detailed medical history and systematic evaluation to ensure that underlying autoimmune diseases are not overlooked.