Prognosis after steroid pulse therapy and seasonal effect in acquired idiopathic generalized anhidrosis

Iida, Tadatsune; Nakamura, Michiko; Inazawa, Minako; Munetsugu, Takichi; Nishida, Masaki; Fujimoto, Tomoko; Sasaki, Yuya; Ohshima, Yuichiro; Nakazato, Yoichi; Namiki, Takeshi; Yokozeki, Hiroo

doi:10.1111/1346-8138.15666

Cited by 12 publications

(12 citation statements)

References 22 publications

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“…Based on the autoimmune mechanism, corticosteroid therapy, and in particular corticosteroid pulse therapy, is recommended as the primary treatment for AIGA [1], although it does not improve all cases. It has been reported that younger age and delay from the onset to the treatment are negative factors for the effectiveness of AIGA treatment [4]. Seasonal factors have a significant effect on both treatment and recurrence for AIGA in Japan [4].…”

Section: Discussionmentioning

confidence: 99%

“…It has been reported that younger age and delay from the onset to the treatment are negative factors for the effectiveness of AIGA treatment [4]. Seasonal factors have a significant effect on both treatment and recurrence for AIGA in Japan [4]. The effectiveness of oral administration of cyclosporine and Chinese herbal medicine has been reported in a limited number of the patients [5].…”

Section: Discussionmentioning

confidence: 99%

“…Systemic administration of corticosteroids is recommended for the treatment of AIGA, and there is a report that other immunosuppressive agents such as cyclosporine are also effective [1,4,5]. Case reports that have been recently accumulated indicate that patients with AIGA have various complications or comorbidities, including autoimmune disorders.…”

Section: Introductionmentioning

confidence: 99%

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Acquired Idiopathic Generalized Anhidrosis (AIGA) and Its Complications: Implications for AIGA as an Autoimmune Disease

Kageyama

Honda

Tokura³

2021

IJMS

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Acquired idiopathic generalized anhidrosis (AIGA) is a rare disorder in which systemic anhidrosis/hypohidrosis occurs without causative dermatological, metabolic or neurological disorder. Most cases of AIGA have been reported in Asia, especially in Japan, but there have been only a few reports in Europe and the United States. Severe AIGA may result in heatstroke and can reduce quality of life due to restriction of exercise and outdoor works. AIGA is often accompanied by cholinergic urticaria (CholU), and it is thought that AIGA and CholU with anhidrosis/hypohidrosis belong to the same spectrum of the disease. However, the pathophysiology of AIGA has not yet been clarified. Decreased expression of cholinergic receptor M3 on the epithelial cells of eccrine sweat glands is often accompanied by T cell infiltration around eccrine apparatus, suggesting an immunological mechanism of disordered perspiration. AIGA is occasionally associated with various complications indicative of autoimmune disorders. The association of autoimmune complications further suggests that AIGA is an autoimmune disorder. Studies on complications may lead to a better understanding of the pathophysiology of AIGA.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Acquired Idiopathic Generalized Anhidrosis (AIGA) and Its Complications: Implications for AIGA as an Autoimmune Disease

Kageyama

Honda

Tokura³

2021

IJMS

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“…A recent report stated that the treatment response rate of steroid pulse therapy in 57 patients with AIGA was 73%, and the recurrence rate was 48%. Furthermore, it is interesting that the effectiveness was reduced when the steroid pulse therapy was given in the autumn before it became cold [ 82 ]. A trial of oral immunosuppressants is worthwhile in patients who do not respond to steroid pulse therapy, although only one case is reported in Japan [ 30 ].…”

Section: Management Of and Therapeutic Approach To Cholumentioning

confidence: 99%

Cholinergic Urticaria: Subtype Classification and Clinical Approach

et al. 2022

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Cholinergic urticaria (CholU) is a subtype of chronic inducible urticaria with a chief complaint of itching and/or stinging, painful papular wheals that develop simultaneously with sweating. This review specifically focuses on several subtypes of CholU and specifically investigates the relationship between CholU and anhidrosis. We review recent publications and update the evidence around CholU, including the epidemiology, clinical features, diagnostic approaches, physiopathology, subtype classification, and therapeutic approaches. Multiple mechanisms contribute in a complex manner to the development of CholU, including histamine, sweat allergy, cholinergic-related substances, poral occlusion, and hypohidrosis/anhidrosis. A new schematic of the currently known pathological conditions has been created. Specific methods for diagnosing CholU, a provocation test, and evaluation methods for disease severity/activity and disease burden of CholU are summarized. The characteristics of the diseases that should be differentiated from CholU and examination methods are also summarized. The primary finding of this review is that CholU should be categorized based on the etiology and clinical characteristics of each subtype to properly manage and treat the disease. This categorization leads to improvement of therapeutic resistance status of this disease. In particular, a sweating abnormality should be given more attention when examining patients with CholU. Because CholU is not a homogeneous disease, its subtype classification is important for selection of the most suitable therapeutic method. Further elucidation of the pathophysiology of each subtype is expected.

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“…Although the main symptom of AGAH is heat retention due to decreased perspiration, AIGA is characteristically accompanied by cholinergic urticaria (CholU)‐like rash, stinging dermal pain, or both, triggered by sweating stimuli 1 . Anhidrosis is effectively treated by steroid pulse therapy; 2,3 however, the pain can be intractable in some cases of AIGA 4,5 . The site affected by the rash and pain is not necessarily the same as that of anhidrosis 6 .…”

Section: Introductionmentioning

confidence: 99%

Anhidrosis accompanied by cholinergic urticaria‐like rash and dermal pain in a patient with Sjögren's syndrome

Yanagida

Takahagi

Aoyama

et al. 2022

The Journal of Dermatology

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Cholinergic urticaria (CholU)‐like rash and dermal pain on sweating occur in patients with acquired idiopathic generalized anhidrosis (AIGA). However, it is unclear whether these are symptoms specific to AIGA among the various types of acquired generalized anhidrosis/hypohidrosis (AGAH). Moreover, the pathogenesis underlying CholU‐like rash and dermal pain observed with anhidrosis remains to be clarified. A 20‐year‐old Japanese man with Sjögren's syndrome (SS) presented with anhidrosis. Transient stinging pain on the skin and pinpoint wheals were observed when his body temperature increased. Thermoregulatory sweat testing revealed anhidrotic areas covering 69% of the body surface area with a symmetrical distribution. A high concentration of histamine was detected (506 ng/mL) in the sweat. A skin biopsy specimen from the anhidrotic area showed the inflamed secretory portion of eccrine glands. This suggested inflammation‐mediated damage to sweat glands, consistent with AGAH related to SS. Furthermore, immunohistochemical analysis revealed an ectopic distribution of dermcidin, a sweat‐specific peptide, in the dermal tissue surrounding the secretory portion of eccrine glands. The expression of claudin‐3, a tight junction (TJ) component of sweat glands, decreased or distributed in a mottled manner in the secretory portion. No decreased expression of muscarinic cholinergic receptor M3 was detected. These results suggested that sweat had leaked into the dermis in association with impaired TJ in the secretory portion, along with the damage to inflamed sweat glands related to SS. Collectively, CholU‐like rash and dermal pain on sweating were observed in an AGAH patient with SS. The sweat leakage into the dermis may contribute to the development of the rash and pain.

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Prognosis after steroid pulse therapy and seasonal effect in acquired idiopathic generalized anhidrosis

Cited by 12 publications

References 22 publications

Acquired Idiopathic Generalized Anhidrosis (AIGA) and Its Complications: Implications for AIGA as an Autoimmune Disease

Acquired Idiopathic Generalized Anhidrosis (AIGA) and Its Complications: Implications for AIGA as an Autoimmune Disease

Cholinergic Urticaria: Subtype Classification and Clinical Approach

Anhidrosis accompanied by cholinergic urticaria‐like rash and dermal pain in a patient with Sjögren's syndrome

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