15 - 81 - HYPERHIDROSIS AND ANHIDROSIS

Question 1

describe Primary focal (essential) hyperhidrosis

Answer 1

• Idiopathic and symmetric and can affect the palms, soles, axillae, craniofacial region, groin, other areas, or combination of body sites
• It affects males and females equally, typically begins in childhood (palmarplantar) or during puberty (axillary), and continues to persist into adulthood, with rare reports of spontaneous improvement.

Question 2

describe Secondary hyperhidrosis

Answer 2

• Can occur from underlying systemic illnesses, medications, or both (obtaining a detailed medical history is crucial).
• The pattern of sweating is classically generalized, but sometimes can be** focal or regional **if caused by neurologic disease or trauma, or a primary dermatologic cause (eg, eccrine nevus)
• often persists during sleep
• tends to present in adulthood, especially if it is caused by an underlying acquired disease

Question 3

Eccrine sweat glands are innervated by sympathetic nerve fibers. What is the main neurotransmitter?

Answer 3

acetylcholine

Question 4

hyperhydrosis may predispose the patient to what disorders?

Answer 4

• pitted keratolysis,
• dermatophytosis, and
• verruca vulgaris or plantaris

Question 5

To establish a diagnosis of primary focal hyperhidrosis, what symptoms should be present?

Answer 5

Question 6

helpful to identify the involved area of hyperhidrosis at baseline, but it does not provide any information on severity of the disease

Answer 6

Minor starch-iodine test

Question 7

objective measurement of the amount of sweat production

Answer 7

Gravimetric (weightbased) assessment

commonly performed in clinical research studies, but it is not practical for routine clinical use

Question 8

clinical course and prognosis of primary focal hyperhidrosis

Answer 8

• chronic disorder, and it does not affect life expectancy
• have a very negative impact on the quality of life, affecting all domains of social life, sense of well-being, and mental health

Question 9

First-line therapy for primary focal hyperhidrosis

Answer 9

• over-the-counter (OTC) antiperspirants or aluminum chloride hexahydrate (10% to 35%).
• Topical glycopyrrolate (0.5%–2%)

Question 10

how do you apply aluminum chloride hexahydrate

Answer 10

works best when applied to dry skin at bedtime and washed off after 6 to 8 hours.

• Patients should be counseled carefully on the proper application to reduce the risk of irritation.
• If moisture is present on the skin when aluminum chloride hexahydrate is applied, this can result in formation of a weak hydrochloric acid, which can cause symptoms of burning, irritation, or desquamation.
• If irritation occurs, it can usually be minimized by decreasing application to every other night or few times per week

Question 11

Question 12

Hyperhidrosis Disease Severity Scale

Intolerable; always interferes

Answer 12

4

Question 13

Hyperhidrosis Disease Severity Scale

Tolerable; sometimes interferes

Answer 13

2

Question 14

Hyperhidrosis Disease Severity Scale

Never noticeable; never interferes

Answer 14

1

Question 15

Hyperhidrosis Disease Severity Scale

Barely tolerable; frequently interferes

Answer 15

3

Question 16

approved by the U.S. Food and Drug Administration (FDA) in 2004 for the treatment of severe primary axillary hyperhidrosis in adults 18 years and older

Answer 16

onabotulinum toxin A (onabotA)

Question 17

median duration of effect of toxin injection on hyperhidrosis

Answer 17

4 to 5 months

Question 18

Botulinum toxin injections are also used off-label for treatment of other focal areas, such as the face or scalp, palms, soles, and inframammary and inguinal folds, with average efficacy duration of approximately how many months?

Answer 18

4 to 12 months

Question 19

where do you inject toxin on the skin?

Answer 19

dermal subcutaneous junction where the eccrine glands reside

Question 20

• non-invasive procedure that can provide long-lasting reduction in axillary sweating
• 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
• Eccrine glands do not regenerate, and their destruction theoretically reduces sweating in the treated area permanently

Answer 20

Electromagnetic energy thermolysis

• Side effects are generally minor and include edema, erythema, bruising from device vacuum suction, axillary tenderness or pain, paresthesia in the axilla or upper arm, and less commonly, blisters or burns at the treatment site, scar tissue formation, and patchy axillary alopecia (permanent).
• This technology cannot be applied to nonaxillary body

Question 21

Electromagnetic energy thermolysis cleared by the FDA in 2011 for adults with primary axillary hyperhidrosis

Answer 21

miraDry

Question 22

uses the passage of a direct electrical current onto the skin to control of palmar-plantar hyperhidrosis

Answer 22

tap water iontophoresis therapy

• An anticholinergic, such as glycopyrrolate, can be crushed and mixed with the water to enhance sweat reduction.
• Side effects are typically minor (erythema, mild pain or discomfort, and paresthesia in the treatment zone) and related to higher amperage.

Question 23

Treatment Options for Primary Focal Hyperhidrosis

Answer 23

Question 24

This surgical procedure carries an increased risk of creating minor to severe compensatory sweating in body segments below the treated area, as well as less common complications such as Horner syndrome, bradycardia, pneumothorax requiring chest tube drainage, pleural effusion, acute bleeding or delayed hemothorax, chylothorax, and persistent intercostal neuralgia

Answer 24

endoscopic thoracic sympathectomy (ETS)

Question 25

oral agents that are commonly used as treatment for hyperhidrosis

Answer 25

anticholinergics **(glycopyrrolate and oxybutynin) **or **clonidine**, a centrally acting α2-adrenergic agonist. ## Footnote * Anticholinergic agents are contraindicated in those with myasthenia gravis, paralytic ileus, and pyloric stenosis and should be used with caution in patients with closed-angle glaucoma, bladder outflow obstruction, gastroesophageal reflux disease, and cardiac insufficiency.

Question 26

localized sweating on the lips, forehead, scalp, and nose while eating hot and spicy foods as a physiologic response via **trigeminovascular reflex**

Answer 26

GUSTATORY SWEATING ## Footnote * may also occur after upper thoracic and cervical sympathectomy, facial herpes zoster, or chorda tympani injury and has been described in association with cluster headache and diabetes mellitus * Treatment with topical glycopyrrolate, aluminum chloride, or botulinum toxin injection can be effective; rarely, intracranial section of the glossopharyngeal nerve or tympanic neurectomy is needed.

Question 27

**pathologic gustatory sweating** is usually **asymmetric** and **intense** and can occur in the distribution of the **auriculotemporal nerve** after injury or surgery in the region of the parotid gland This syndrome is known as

Answer 27

**Frey syndrome** ## Footnote * thought to be caused by **aberrant regeneration of autonomic fibers** after local trauma to the auriculotemporal nerve * can also be seen in infants and children, often after birth trauma with forceps delivery, but cases of familial, bilateral Frey syndrome without birth trauma have been reported

Question 28

hyperhidrosis affecting the head, neck and upper trunk can occasionally affect older postmenopausal women and less commonly, men

Answer 28

PAROXYSMAL LOCALIZED HYPERHIDROSIS

Question 29

give examples of conditions which may present with excessive sweating

Answer 29

blue rubber bleb nevus syndrome, perilesional skin of a glomus tumor, tufted angioma, eccrine angiomatous hamartoma or eccrine nevus, Grierson-Gopalan disease, pachydermoperiostosis, and pretibial myxedema

Question 30

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

Answer 30

Eccrine nevus ## Footnote Clinically, it presents as hyperhidrotic isolated patch of skin with no epidermal changes and is frequently located on the forearm

Question 31

Clinically, it presents as **hyperhidrotic isolated patch of skin** with n**o epidermal changes** and is frequently located on the forearm

Answer 31

Eccrine nevus ## Footnote Isolated case reports have shown successful response to botulinum toxin injections and topical glycopyrrolate

Question 32

some of the infectious etiologies that could present with generalized hyperhidrosis

Answer 32

Tuberculosis, malaria, brucellosis, and subacute bacterial endocarditis

Question 33

With regard to malignancy, excessive production of what interleukin by Hodgkin lymphoma cells has been shown to result in fever and subsequent night sweating?

Answer 33

IL-6

Question 34

Riley-Day Syndrome is caused by what mutations

Answer 34

IKAP gene, located on chromosome 9

Question 35

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 35

Familial dysautonomia (FD), also known as Riley-Day syndrome ## Footnote most intensively studied of the hereditary sensoryautonomic neuropathies (designated as hereditary sensory and autonomic neuropathy [HSAN] type III)

Question 36

Generalized anhidrosis or anhidrosis with large areas of involvement usual manifestations

Answer 36

* heat exhaustion, inability to tolerate increased physical activity in a hot environment, or dizziness upon exposure to heat ## Footnote Patients with focal or segmental anhidrosis may become aware of their disease because of compensatory hyperhidrosis in other regions

Question 37

mutation of X-linked hypohidrotic ectodermal dysplasia ## Footnote most common form of the ectodermal dysplasias

Answer 37

**ED-1 gene** encoding for ectodysplasin

Question 38

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

Answer 38

Congenital insensitivity to pain with anhidrosis (CIPA), also known as HSAN type IV ## Footnote * reported to occur because of lossof-function variants in neurotrophic tyrosine receptor kinase 1** (NTRK1) gene** * Nearly all of the associated symptoms can be attributed to the inability of NTRK1 signaling pathways to regulate the development of nociceptive, sympathetic, and central cholinergic neurons

Question 39

Fabry disease is an inherited X-linked lysosomal storage disorder caused by deficient activity of what enzyme? ## Footnote * presents during childhood in both male and female patients * Early manifestations include **hypohidrosis, telangiectasia, angiokeratoma, acroparesthesia, and gastrointestinal symptoms** * Later in adolescence or adulthood, manifestations can include renal, cardiac, and central nervous system dysfunction

Answer 39

α-galactosidase A

Question 40

second most common symptom of Fabry disease

Answer 40

Hypohidrosis ## Footnote pathogenesis is unclear but could be the result of accumulation of glycosphingolipids in eccrine sweat glands and secondary to autonomic peripheral neuropathy

Question 41

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

Answer 41

Acquired idiopathic generalized anhidrosis (AIGA) ## Footnote * has a heterogenous clinical presentation and can include the following clinical features: acute onset, cholinergic urticaria, elevated serum immunoglobulin E levels, absence of other autonomic dysfunction, and marked response to glucocorticoids.

Question 42

Miliaria results from obstruction of what structures?

Answer 42

**eccrine sweat ducts** and occurs in conditions of **increased heat and humidity**

Question 43

In miliaria crystallina, ductal obstruction occurs at what structure?

Answer 43

stratum corneum ## Footnote * It presents as small, 1-mm, clear, fragile, vesicles that rupture easily * They are commonly seen on the **face and upper trunk **in infants and on the trunk in adults.

Question 44

obstruction occurs deeper **within the epidermis **and results in pruritic, 1- to 3-mm, nonfollicular, erythematous macules and papules on the upper trunk and neck

Answer 44

miliaria rubra ## Footnote Sterile pustules may also develop and are termed miliaria pustulosa.

Question 45

* When miliaria rubra becomes **chronic** or recurrent, occlusion of eccrine sweat ducts extends to a **deeper level** * ductal obstruction occurs at the **dermal–epidermal junction** and produces **asymptomatic**, 1- to 3-mm **white papules**

Answer 45

miliaria profunda

Question 46

The topical application of this medication has resulted in dramatic improvement in patients with miliaria profunda

Answer 46

anhydrous lanolin