Idiopathic pure sudomotor failure

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Idiopathic pure sudomotor failure
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Idiopathic pure sudomotor failure (IPSF) is the most common cause of a rare disorder known as acquired idiopathic generalized anhidrosis (AIGA), a clinical syndrome characterized by generalized decrease or absence of sweating without other autonomic and somatic nervous dysfunctions and without persistent organic cutaneous lesions. [1]

Contents

The term IPSF was first introduced in 1994 after researchers at Saitama Medical School speculated the primary lesion sites in patients were within cholinergic receptors of the sweat glands. The term IPSF represent a distinct subgroup of AIGA without sudomotor neuropathy or sweat gland failure. [2]

Clinical features

Pathology

Intracutaneous injection of pilocarpine (sweat gland stimulant) is known to evoke no sweat response, indicating that lesions are on the post-synaptic side of the nerve-sweat gland junction. [1]

The proposed pathomechanisms of idiopathic pure sudomotor failure include:

Diagnosis

IPSF is a diagnosis made after ruling out other possible causes. It involves a lack of sweating all over the body, excluding certain congenital and acquired conditions. IPSF is characterized by the absence of sweating on the palms and soles, along with cholinergic urticaria. Skin biopsy and specific markers can help distinguish IPSF from other sweat-related conditions. [5]

Management

Treatment of AIGA almost always consists of steroid pulse therapy or high-dose oral steroids and is not consistently effective. Much remains unclear regarding the reasons for recurrent anhidrosis. [6]

Epidemiology

The overwhelming majority of reported AIGA patients are Japanese, but whether AIGA is truly rare in whites or has been simply underreported by Western physicians remains unclear. [1]

AIGA is most prevalent among young men. In a 64 case review of the literature 58 reported cases were males and 6 female, with a mean age of onset at 28 ± 11 years. Cholinergic urticaria or sharp pain over the entire body induced by elevated body temperature was reported in 32 cases (50%). Of 28 cases tested, 12 (43%) displayed elevated serum IgE levels. Skin biopsy was performed in 53 cases, with normal findings in 20 cases (38%), and cellular infiltrates in sweat glands or ducts in 23 cases (43%). [1]

See also

Related Research Articles

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<span class="mw-page-title-main">Poroma</span>

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Acquired idiopathic generalized anhidrosis (AIGA) is characterized by generalized absence of sweating without other autonomic and neurologic dysfunction.

A sweat allergy is the exacerbation of atopic dermatitis associated with an elevated body temperature and resulting increases in the production of sweat. It appears as small reddish welts that become visible in response to increased temperature and resulting production of sweat. It can affect all ages. Sweating can trigger intense itching or cholinergic urticaria. The protein MGL_1304 secreted by mycobiota (fungi) present on the skin such as Malassezia globosa acts as a histamine or antigen. People can be desensitized using their own samples of sweat that have been purified that contains small amounts of the allergen. The allergy is not due to the sweat itself but instead to an allergy-producing protein secreted by bacteria found on the skin.

Electrochemical skin conductance (ESC) is an objective, non-invasive and quantitative electrophysiological measure. It is based on reverse iontophoresis and (multiple) steady chronoamperometry.

References

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  2. Nakazato, Y.; Shimazu, K.; Tamura, N.; Hamaguchi, K. (1994). "Idiopathic pure sudomotor failure". Rinsho Shinkeigaku. 34 (1): 12–15. PMID   8156704.
  3. 1 2 Nakazato, Y.; Tamura, N.; Ohkuma, A.; Yoshimaru, K.; Shimazu, K. (2005). "QSART in idiopathic pure sudomotor failure". Clinical Autonomic Research. 15 (6): 414–416. doi:10.1007/s10286-005-0301-8. PMID   16362546. S2CID   23675423.
  4. Chin, Y. -Y.; Chang, T. C. -C.; Chang, C. -H. (2012). "Idiopathic pure sudomotor failure and cholinergic urticaria in a patient after acute infectious mononucleosis infection". Clinical and Experimental Dermatology. 38 (2): 156–159. doi:10.1111/j.1365-2230.2012.04437.x. PMID   22924754. S2CID   21908635.
  5. Young, Albert T.; Yedidi, Raagini S.; Raffi, Jodie; McCalmont, Timothy H.; North, Jeffrey; Brinker, Alyson; Berger, Timothy G.; Murase, Jenny E. (2020-12-24). "Idiopathic pure sudomotor failure: A review and two cases". International Journal of Women's Dermatology. 7 (3): 276–279. doi:10.1016/j.ijwd.2020.12.011. ISSN   2352-6475. PMC   8243128 . PMID   34222583.
  6. Ohshima, Y.; Yanagishita, T.; Ito, K.; Tamada, Y.; Nishimura, N.; Inukai, Y.; Iwase, S.; Sugenoya, J.; Watanabe, D. (2012). "Treatment of patients with acquired idiopathic generalized anhidrosis". British Journal of Dermatology. 168 (2): 430–432. doi:10.1111/j.1365-2133.2012.11112.x. PMID   22709381. S2CID   29015900.