Classification of Systemic and Localized Sweating Disorders.

Hyperhidrosis can be subdivided into generalized hyperhidrosis, with increased sweating over the entire body, and focal hyperhidrosis, in which the excessive sweating is restricted to specific parts of the body. Generalized hyperhidrosis may be either primary (idiopathic) or secondary. Secondary generalized hyperhidrosis may be caused by infections such as tuberculosis, hyperthyroidism, endocrine and metabolic disturbances such as pheochromocytoma, neurological disorders, or drugs. Focal hyperhidrosis may also be primary (idiopathic) or secondary. Frey's syndrome is one form of secondary focal hyperhidrosis that occurs during eating together with reddening of the area in front of the ear following parotid gland surgery or injury. Primary focal hyperhidrosis is particularly common on the palms and soles of the feet, in the axilla, and on the head. Anhidrosis may be either congenital/genetic or acquired. Some of the most typical forms of congenital/genetic anhidrosis include hypohidrotic ectodermal dysplasia, congenital insensitivity to pain and anhidrosis, and Fabry disease. Acquired anhidrosis is classified as secondary anhidrosis, which may be due to an underlying disorder such as a neurological disorder, an endocrine or metabolic disturbance, or the effect of drugs, or idiopathic anhidrosis for which the pathology, cause, and mechanism are unknown. Idiopathic anhidrosis is classified into acquired idiopathic generalized anhidrosis (AIGA), idiopathic segmental anhidrosis, and Ross syndrome. AIGA is divided into three categories according to differences in the site of disturbance: (1) sudomotor neuropathy, (2) idiopathic pure sudomotor failure, and (3) sweat gland failure.

Identification of subgroups of acquired idiopathic generalized anhidrosis.

BACKGROUND: Acquired idiopathic generalized anhidrosis (AIGA) is a rarely encountered clinical syndrome. Sixty-five cases have been reported and 62 of them are Japanese. AIGA was further classified into 3 subgroups with idiopathic pure sudomotor failure being the most common. However, it is burdensome to diagnose AIGA and identify its subgroups. Some of the tools used to diagnose AIGA such as the quantitative sudomotor axon reflex test and microneurography are not generally available. CASE SUMMARY: We report the first Chinese patient with AIGA and review the literature to develop a flowchart for the diagnosis and identification of subgroups of AIGA. CONCLUSION: We conclude that skin biopsy is crucial for the identification of subgroups of AIGA.

[Idiopathic segmental anhidrosis].

We described two cases of idiopathic segmental anhidrosis. Case 1 was a 47-year-old man, who noticed anhidrosis on the right side of face and chest during body heating. Case 2 was a 54-year-old woman, who complained of anhidrosis on the left side of face and upper chest during exercise. Both cases had neither somatic neurological deficit nor autonomic failure except for anhidrosis pupil size and deep tendon reflexes were normal. Reflex sweating to pilocarpine was exaggerated in the anhidrotic areas in both cases, suggesting lesions in the preganglionic sudomotor nerves. Abnormal laboratory finding was elevated serum rheumatoid factor level in only case 2. Segmental anhidrosis was static for 1.5 year and one year, respectively. The previous literature contains similar four cases. Idiopathic segmental anhidrosis may be an abortive form of Ross' syndrome (tonic pupil, hyporeflexia and segmental anhidrosis).