81 - Hyperhidrosis and Anhidrosis

Question 1

Eccrine sweat glands are innervated by _____ nerve fibers

Answer 1

Sympathetic

Question 2

Main neurotransmitter on eccrine glands

Answer 2

Acetylcholine

Question 3

Excessive sweating of the palms and soles, axillae, craniofacial region, groin, or a combination of body sites
Typically begins in childhood or during puberty, and continues to persist into adulthood

Answer 3

Primary focal hyperhidrosis

Question 4

Hyperhidrosis has been reported to predispose the patient to increased risk of

Answer 4

Cutaneous infection, such as pitted keratolysis, dermatophytosis, and verruca vulgaris or plantaris

Question 5

To establish a diagnosis of primary focal hyperhidrosis, symptoms should be present for at least _____ without apparent cause

Answer 5

6 months

Question 6

To establish a diagnosis of primary focal hyperhidrosis, at least the following characteristics should be included

Answer 6

Bilateral and relatively symmetric
Age of onset typically before age of 25 years
Cessation of sweating from the focal areas during sleep
Frequency of at least once per week
Positive family history
Sweating impairs daily activities

Question 7

Helpful to identify the involved areas of hyperhidrosis at baseline, but does not provide any information on severity of the disease

Answer 7

Minor starch-iodine test

Question 8

Objective measurement of the amount of sweat production, but is not practical for routine clinical use

Answer 8

Gravimetric (weight-based) assessment

Question 9

First-line therapy for hyperhidrosis

Answer 9

Over-the-counter antiperspirants
Aluminum chloride hexahydrate (10% to 35%)
Topical glycopyrrolate (0.5%-2%

Question 10

Aluminum chloride hexahydrate works best when applied to dry skin at _____ and washed off after 6 to 8 hours

Answer 10

Bedtime

Question 11

If the patient fails to respond to topical therapy, _____ may be administered to areas of extensive sweating

Answer 11

Intradermal injection of botulinum toxin

Question 12

Microwave energy is readily absorbed by water molecules and as a result can easily target tissues with high water content, such as the eccrine glands

Answer 12

Electromagnetic energy thermolysis

Question 13

Y/N: Eccrine glands do not regenerate

Answer 13

Yes

Question 14

Use the passage of a direct electrical current onto the skin

Answer 14

Tap water iontophoresis therapy

Question 15

Severe cases of palmar hyperhidrosis that failed conservative management may be considered for

Answer 15

Endoscopic thoracic sympathectomy

Question 16

Systemic medications that are commonly used for hyperhidrosis

Answer 16

Anticholinergics (glycopyrrolate and oxybutynin)

Clonidine

Question 17

Anticholinergic agents are contraindicated in those with

Answer 17

Myasthenia gravis
Paralytic ileus
Pyloric stenosis

Question 18

Focal, regional, or generalized sweating that often persists during sleep
Tends to present in adulthood

Answer 18

Secondary hyperhidrosis

Question 19

Usually asymmetric gustatory sweating in the distribution of the auriculotemporal nerve after injury or surgery in the region of the parotid gland

Answer 19

Frey syndrome

Question 20

Frey syndrome can also be seen in infants and children, often after

Answer 20

Birth trauma with forceps delivery

Question 21

Daytime paroxysmal hyperhidrosis affecting the head, neck and upper trunk can occasionally affect

Answer 21

Older postmenopausal women

Question 22

Y/N: Hot flashes are typically associated with paroxysmal localized hyperhidrosis, and hormonal replacement therapy is usually effective

Answer 22

No - not associated; ineffective

Question 23

Excessive sweating has been reported in association with certain cutaneous disorders

Answer 23

Blue rubber bleb nevus syndrome
Perilesional skin of a glomus tumor 
Tufted angioma
Eccrine angiomatous hamartoma or eccrine nevus
Grierson-Gopalan disease 
Pachydermoperiostosis
Pretibial myxedema

Question 24

Rare skin hamartoma histologically defined as focal hyperplasia or hypertrophy of eccrine glands

Answer 24

Eccrine nevus

Question 25

Hyperhidrotic isolated patch of skin with no epidermal changes Frequently located on the forearm

Answer 25

Eccrine nevus

Question 26

Secondary hyperhidrosis has been previously reported in association with

Answer 26

``` Diabetes mellitus Hypoglycemia Thyrotoxicosis Carcinoid syndrome Hyperpituitarism (acromegaly) Congestive heart failure Dumping syndrome Menopause Malignancy Drug withdrawal Exogenous bacterial pyrogens: tuberculosis, malaria, brucellosis, subacute bacterial endocarditis Hodgkin lymphoma ```

Question 27

Also known as Riley-Day syndrome

Answer 27

Familial dysautonomia

Question 28

Secondary hyperhidrosis has been previously reported in association with

Answer 28

``` Diabetes mellitus Hypoglycemia Thyrotoxicosis Carcinoid syndrome Hyperpituitarism (acromegaly) Congestive heart failure Dumping syndrome Menopause Malignancy Drug withdrawal Exogenous bacterial pyrogens (tuberculosis, malaria, brucellosis, subacute bacterial endocarditis) Hodgkin lymphoma ```

Question 29

Riley-Day syndrome is also known

Answer 29

Familial dysautonomia

Question 30

Most intensively studied of the hereditary sensory-autonomic neuropathies

Answer 30

Riley-Day syndrome

Question 31

Riley-Day syndrome is caused by mutations in the

Answer 31

IKAP gene

Question 32

Characterized by pronounced autonomic dysregulation with profuse sweating and salivation, diminished production of tears, red blotching of the skin, absence of fungiform papillae of the tongue, episodic orthostatic hypotension, arterial hypertension, reduced deep tendon reflexes, and behavioral abnormalities

Answer 32

Riley-Day syndrome

Question 33

Hyperhidrosis can be associated with the following medications

Answer 33

Serotonin (5-hydroxytryptamine) reuptake inhibitors Opioids Prostaglandin inhibitors (naproxen) Tricyclic antidepressants Cholinergic agonists (pilocarpine, bethanechol, pyridostigmine)

Question 34

Most common form of the ectodermal dysplasias

Answer 34

X-linked hypohidrotic ectodermal dysplasia

Question 35

Mutations in the _____ gene encoding for _____ result in X-linked hypohidrotic ectodermal dysplasia

Answer 35

ED-1 | Ectodysplasin

Question 36

Rare autosomal recessive disorder characterized by the congenital insensitivity to noxious stimuli, anhidrosis, recurrent hyperpyrexia, mental retardation, and self-mutilating behavior

Answer 36

Congenital insensitivity to pain with anhidrosis or | HSAN type IV

Question 37

CIPA is reported to occur because of loss-of-function variants in _____ gene

Answer 37

Neurotrophic tyrosinase repector kinase 1 (NTRK1)

Question 38

Inherited X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A Early manifestations include hypohidrosis, telangiectasia, angiokeratoma, acroparestheisa, and gastrointestinal symptoms

Answer 38

Fabry disease

Question 39

Second most common symptom of Fabry disease

Answer 39

Hypohidrosis

Question 40

Rare cause of anhidrosis that can result from idiopathic pure sudomotor failure, sudomotor neuropathy, or eccrine gland failure

Answer 40

Acquired idiopathic generalized anhidrosis

Question 41

Three types of clinically distinctive miliaria based on the level of obstruction

Answer 41

Miliaria crystallina Miliaria rubra Miliaria profunda

Question 42

To identify possible sweat gland abnormalities, a _____ should be obtained in patients with anhidrosis

Answer 42

Biopsy specimen from the affected area