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Year : 2022  |  Volume : 67  |  Issue : 2  |  Page : 175-177
Congenital idiopathic generalized anhidrosis: A rare cause of recurrent fever of unknown origin in children and review of existing literature

1 From the Department of Dermatology, Venereology, and Leprosy, RG Kar Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Consultant Pediatrician and Independent Researcher, Midnapore Medical College and Hospital, Midnapore, West Bengal, India
3 Department of Dermatology, Venereology, and Leprosy, Midnapore Medical College and Hospital, Midnapore, West Bengal, India

Date of Web Publication13-Jul-2022

Correspondence Address:
Abheek Sil
From the Department of Dermatology, Venereology, and Leprosy, RG Kar Medical College and Hospital, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_791_21

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How to cite this article:
Sil A, Ghatak N, Bhanja DB. Congenital idiopathic generalized anhidrosis: A rare cause of recurrent fever of unknown origin in children and review of existing literature. Indian J Dermatol 2022;67:175-7

How to cite this URL:
Sil A, Ghatak N, Bhanja DB. Congenital idiopathic generalized anhidrosis: A rare cause of recurrent fever of unknown origin in children and review of existing literature. Indian J Dermatol [serial online] 2022 [cited 2022 Aug 17];67:175-7. Available from:


Anhidrosis refers to the failure in sweat production in response to physical, thermal, or chemical stimuli. This disorder includes congenital causes (hypohidrotic ectodermal dysplasia (HED), congenital insensitivity to pain and anhidrosis, and Fabry disease) and the acquired variety – idiopathic and secondary causes (neuropathy, endocrinopathy, metabolic disorders, and drug induced).[1] Cases of congenital generalized anhidrosis that occurs without any associated problems have been rarely described in the literature. Here, we report a child with congenital idiopathic generalized anhidrosis (CIGA) and succinctly review the existing literature regarding this condition.

A 3-year-old Indian boy, born at term to nonconsanguineous parentage, presented with high-grade fever for more than 1 month. He used to be irritable during the febrile period and frequented the bathroom to pour cold water over his body. A specific pattern of fever could be elicited – it appeared around 12 noon and resolved spontaneously after sundown. This pattern was noticed only during summer months. Interestingly, after the febrile hours, he used to be fine and playful. Weight gain was appropriate even during febrile period. On further review of history, the informant acknowledged that the child did not sweat even in the hot summer months, right from infancy. On physical examination, the skin was found to be dry (generalized in distribution without skip areas) but without any ichthyotic changes. Other ectodermal structures (hair, teeth, and nails) were unaffected. Family history was noncontributory. On systemic examination, there was no neurodeficit peripheral neuropathy and no murmur on evaluation of the cardiovascular system. Examination of higher mental function and intellectual development was as per age.

Routine workup of fever was not suggestive of any infective, inflammatory, or malignant cause. Considering clinical history and examination, a dermatological consultation was sought for further workup. In the dermatology clinic, colorimetric (starch–iodine) test failed to show any detectable perspiration (blue–black areas) anywhere. [Figure 1] Histopathological examination of skin biopsy, taken from the child's arm, revealed paucity of eccrine apparatus with few rudimentary sweat glands throughout the dermis and hypodermis along with sparse hair follicles. [Figure 2] Based on suggestive history, clinical examination, corroborative thermoregulatory sweat test, and histopathological findings, a diagnosis of CIGA was established. As per the recent Japanese guidelines,[1] he was graded as “severe” (score = 6) and subsequently started on tablet prednisolone (1 mg/day/kg) with gradual tapering. His parents were advised to limit his physical activities. However, no significant clinical improvement was evident even after 8 weeks of treatment. He remains on regular pediatric follow-up. We could not perform genetic study due to institutional limitations.
Figure 1: Starch–iodine test demonstrating complete absence of blue–black areas (some focal blue–black areas seen over the trunk, dorsa of feet, and knees was made by the attendant for proper positioning) (a and b)

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Figure 2: Histopathology (hematoxylin and eosin stain) showing paucity of eccrine structures and hair follicles along with the absence of any inflammatory dermal aggregates (A, 40x); some rudimentary sweat glands in dermis (black arrows) (B, 100x); and normal eccrine coils in the reticular dermis and hypodermis (C, 100x)

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Generalized anhidrosis occurs due to exogenous factors, dermatological diseases (primary), and neuropathic (secondary) causes. HED is one of the most common congenital cause of generalized anhidrosis in a child.[1],[2] Since no ectodermal abnormality was found, the possibility of HED was unlikely. As the informant reported decreased perspiration since infancy, we attributed the diagnosis of generalized anhidrosis to be congenital. Although the exact pathophysiology of CIGA remains unknown, the absence of sweat gland or postsynaptic defect in the cholinergic receptors or secretory cells of eccrine glands may underlie the pathogenesis. Cevoli et al.[3] suggested a congenital ectodermal dysplasia that selectively affected the eccrine apparatus.

Sweat glands play an important role in overseeing the thermoregulatory control of the body. With their heat dissipating mechanisms being hampered, children are particularly more vulnerable because their core body temperature rises faster than adults. This culminates into elevated body temperature and the subsequent febrile response. On reviewing the literature in “PubMed” and “Medline” databases, we came across seven published cases of CIGA till date that we have duly summarized in [Table 1].[3],[4],[5],[6]
Table 1: Clinical characteristics of the eight reported cases of CIGA#

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Systemic corticosteroids have been recommended in patients with acquired idiopathic generalized anhidrosis having severe symptoms that disrupt daily life or work. However, steroid resistance have been noted in patients with prolonged duration of anhidrosis.[1] Corticosteroids proved unsuccessful in our case. Cyclosporine works only in cases where peri-eccrine T-cell infiltrates are observed. Antihistamines may have a role by blocking the inhibitory action of histamine on acetylcholine-induced sweating.[2],[6] As systemic medications provide variable results, emphasis should be on preventive measures. In this regard, care providers should maintain a cool environment (including installation of air-conditioners at home) for the patient and ensure lifestyle modifications like limitation of vigorous exercise and outdoor activities especially in the hot summer months and afternoon time). By adopting these simple measures, the serious complications of anhidrosis (heat cramps, exhaustion, and the dreaded heatstroke) may be averted.

The purpose of detailing this case is to familiarize clinicians with this uncommon condition, add to the literature on this rarely reported entity, and emphasize the need to consider disorders of anhidrosis as a potential cause of fever of unknown origin.

Informed consent

Written informed consent obtained from the guardian.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Ohshima Y, Tamada Y. Classification of systemic and localized sweating disorders. Curr Probl Dermatol 2016;51:7-10.  Back to cited text no. 1
Munetsugu T, Fujimoto T, Oshima Y, Sano K, Murota H, Satoh T, et al. Revised guideline for the diagnosis and treatment of acquired idiopathic generalized anhidrosis in Japan. J Dermatol 2017;44:394-400.  Back to cited text no. 2
Cevoli S, Pierangeli G, Magnifico F, Bonavina G, Barletta G, Candela C, et al. The circadian rhythm of body core temperature (CRT) is normal in patient with congenital generalized anhidrosis. Clin Auton Res 2002;12:170-3.  Back to cited text no. 3
Mahloudji M, Livingston KE. Familial and congenital simple anhidrosis. Am J Dis Child 1967;113:477-9.  Back to cited text no. 4
Frydman M, Cohen HA, Kauschansky A, Matoth Y. Familial simple hypohidrosis with abnormal palmar dermal ridges. Am J Med Genet 1988;31:591-6.  Back to cited text no. 5
Shi G, Zhu CY, Zhou Y, Yang YP, Fan YM. First case of congenital idiopathic hypohidrosis in China. An Bras Dermatol 2015;90:731-3.  Back to cited text no. 6


  [Figure 1], [Figure 2]

  [Table 1]


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