Acquired Generalized Anhidrosis: Review of the Literature and Report of a Case with Lymphocytic Hidradenitis and Sialadenitis Successfully Treated with Cyclosporine

Abstract

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.

Degranulation and shrinkage of dark cells in eccrine glands and elevated serum carcinoembryonic antigen in patients with acquired idiopathic generalized anhidrosis

“…1,5,6 Skin biopsy usually shows no significant pathological alterations in eccrine glands, although some patients show a small degree of T-cell and/or mast-cell infiltration, atrophic eccrine glands or a reduced number of eccrine glands. 1,5,7 The pathognomonic changes are still unknown, but good responses to steroid therapy suggest that an autoimmune mechanism underlies AIGA aetiology. Pathological roles of various cells and molecules have been implied so far, such as mast cells, 8 T cells, 5 acetylcholine esterase been established as having definite pathological effects.…”

Prognosis of acquired idiopathic generalized anhidrosis

“…Nur einer unserer Patienten wurde mit Kortikosteroiden behandelt und das Ansprechen hielt nicht über den aktuellen Behandlungszeitraum hinaus an. Zu anderen therapeutischen Optionen, wie Cyclosporin oder IVIG, existieren nur wenige Daten [3,8,9]. In einem Fallbericht zu zwei japanischen Patienten wurde vermutet, dass IVIG hinsichtlich der Vermeidung von Rezidiven wirksam sein könnte [9].…”

British Association of Dermatologists guidelines for the safe and effective prescribing of oral ciclosporin in dermatology 2018

“…382 There is a marked interpatient variation regarding the onset of nephrotoxicity and the speed at which it progresses. Older 126 3 Actinic reticuloid 127, 128 3 a Acute generalized exanthematous pustulosis [129][130][131] 3 Amicrobial pustulosis 132 3 Anhidrosis (autoimmune) 133 3 Annular elastolytic giant cell granuloma 134 3 Antiphospholipid syndrome (ulceration) 135 3 b Annular erythema of Sjogren syndrome 136 3 Atopic dermatitis [137][138][139][140][141][142][143][144] 1+ Atopic keratoconjunctivitis 110, 145 3 Behc ßet disease [146][147][148][149][150] 1+ Bullous erythema multiforme 151 3 Bullous pemphigoid [152][153][154] 3 Capillaritis 155 3 Chronic actinic dermatitis [156][157][158] 3 Chronic spontaneous (idiopathic) urticaria [159][160][161][162][163][164] 1+ Cold urticaria 165 3 Contact allergic dermatitis [166][167][168] 3 Crohn disease (cutaneous) 169 3 Dari...…”