15 - 81 - HYPERHIDROSIS AND ANHIDROSIS Flashcards
describe Primary focal (essential) hyperhidrosis
- 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.
describe Secondary hyperhidrosis
- 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
Eccrine sweat glands are innervated by sympathetic nerve fibers. What is the main neurotransmitter?
acetylcholine
hyperhydrosis may predispose the patient to what disorders?
- pitted keratolysis,
- dermatophytosis, and
- verruca vulgaris or plantaris
To establish a diagnosis of primary focal hyperhidrosis, what symptoms should be present?
helpful to identify the involved area of hyperhidrosis at baseline, but it does not provide any information on severity of the disease
Minor starch-iodine test
objective measurement of the amount of sweat production
Gravimetric (weightbased) assessment
commonly performed in clinical research studies, but it is not practical for routine clinical use
clinical course and prognosis of primary focal hyperhidrosis
- 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
First-line therapy for primary focal hyperhidrosis
- over-the-counter (OTC) antiperspirants or aluminum chloride hexahydrate (10% to 35%).
- Topical glycopyrrolate (0.5%–2%)
how do you apply aluminum chloride hexahydrate
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
Hyperhidrosis Disease Severity Scale
Intolerable; always interferes
4
Hyperhidrosis Disease Severity Scale
Tolerable; sometimes interferes
2
Hyperhidrosis Disease Severity Scale
Never noticeable; never interferes
1
Hyperhidrosis Disease Severity Scale
Barely tolerable; frequently interferes
3
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
onabotulinum toxin A (onabotA)
median duration of effect of toxin injection on hyperhidrosis
4 to 5 months
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?
4 to 12 months
where do you inject toxin on the skin?
dermal subcutaneous junction where the eccrine glands reside
- 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
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
Electromagnetic energy thermolysis cleared by the FDA in 2011 for adults with primary axillary hyperhidrosis
miraDry
uses the passage of a direct electrical current onto the skin to control of palmar-plantar hyperhidrosis
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.
Treatment Options for Primary Focal Hyperhidrosis
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
endoscopic thoracic sympathectomy (ETS)
oral agents that are commonly used as treatment for hyperhidrosis
anticholinergics **(glycopyrrolate and oxybutynin) **or clonidine, a centrally acting α2-adrenergic agonist.
- 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.
localized sweating on the lips, forehead, scalp, and nose while eating hot and spicy foods as a physiologic response via trigeminovascular reflex
GUSTATORY SWEATING
- 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.
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
Frey syndrome
- 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
hyperhidrosis affecting the head, neck and upper trunk can occasionally affect older postmenopausal women and less commonly, men
PAROXYSMAL LOCALIZED HYPERHIDROSIS
give examples of conditions which may present with excessive sweating
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
rare skin hamartoma histologically defined as focal hyperplasia or hypertrophy of eccrine glands
Eccrine nevus
Clinically, it presents as hyperhidrotic isolated patch of skin with no epidermal changes and is frequently located on the forearm
Clinically, it presents as hyperhidrotic isolated patch of skin with no epidermal changes and is frequently located on the forearm
Eccrine nevus
Isolated case reports have shown successful response to botulinum toxin injections and topical glycopyrrolate
some of the infectious etiologies that could present with generalized hyperhidrosis
Tuberculosis, malaria, brucellosis, and subacute bacterial endocarditis
With regard to malignancy, excessive production of what interleukin by Hodgkin lymphoma cells has been shown to result in fever and subsequent night sweating?
IL-6
Riley-Day Syndrome is caused by what mutations
IKAP gene, located on chromosome 9
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
Familial dysautonomia (FD), also known as Riley-Day syndrome
most intensively studied of the hereditary sensoryautonomic neuropathies (designated as hereditary sensory and autonomic neuropathy [HSAN] type III)
Generalized anhidrosis or anhidrosis with large areas of involvement usual manifestations
- heat exhaustion, inability to tolerate increased physical activity in a hot environment, or dizziness upon exposure to heat
Patients with focal or segmental anhidrosis may become aware of their disease because of compensatory hyperhidrosis in other regions
mutation of X-linked hypohidrotic ectodermal dysplasia
most common form of the ectodermal dysplasias
ED-1 gene encoding for ectodysplasin
rare autosomal recessive disorder characterized by the congenital insensitivity to noxious stimuli, anhidrosis, recurrent hyperpyrexia, mental retardation, and self-mutilating behavior
Congenital insensitivity to pain with anhidrosis (CIPA), also known as HSAN type IV
- 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
Fabry disease is an inherited X-linked lysosomal storage disorder caused by deficient activity of what enzyme?
- 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
α-galactosidase A
second most common symptom of Fabry disease
Hypohidrosis
pathogenesis is unclear but could be the result of accumulation of glycosphingolipids in eccrine sweat glands and secondary to autonomic peripheral neuropathy
rare cause of anhidrosis that can result from idiopathic pure sudomotor failure, sudomotor neuropathy, or eccrine sweat gland failure
Acquired idiopathic generalized anhidrosis (AIGA)
- 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.
Miliaria results from obstruction of what structures?
eccrine sweat ducts and occurs in conditions of increased heat and humidity
In miliaria crystallina, ductal obstruction occurs at what structure?
stratum corneum
- 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.
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
miliaria rubra
Sterile pustules may also develop and are termed miliaria pustulosa.
- 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
miliaria profunda
The topical application of this medication has resulted in dramatic improvement in patients with miliaria profunda
anhydrous lanolin
