83: Fox-Fordyce Disease

Question 1

What is the typical demographic affected by Fox-Fordyce Disease?

Answer 1

Females between the ages of 13 and 35 years comprise more than 90% of cases, with no known racial predilection for the disease.

Question 2

What are the common cutaneous findings in Fox-Fordyce Disease?

Answer 2

The disease manifests as grouped, symmetrically distributed, monomorphic, dome-shaped papules (1 to 3 mm) that are typically follicular based, flesh-colored to mildly erythematous, and intermittently pruritic. Commonly affected areas include the axillae, pubic, perineal, areolar, umbilicus, and sternal areas.

Question 3

What factors can exacerbate pruritus in Fox-Fordyce Disease?

Answer 3

Pruritus is intermittent and intense, worsening with sympathetic stimulation such as sweating, emotional stress, excitement, and warm weather.

Question 4

What complications can arise from Fox-Fordyce Disease?

Answer 4

Complications include localized superinfections secondary to repeated scratching, which can be managed with antihistamines and standard antibiotic therapy. There is no evidence that Fox-Fordyce disease progresses to hidradenitis suppurativa despite some observations.

Question 5

What is known about the etiology and pathogenesis of Fox-Fordyce Disease?

Answer 5

The etiology is unclear, but the tendency to present at puberty and remit after menopause suggests a hormonal component. Improvements have been reported during pregnancy and with the use of oral contraceptives. It is rarely observed in men, preadolescent girls, and postmenopausal women.

Question 6

What demographic is most affected by Fox-Fordyce disease and what is the typical age of onset?

Answer 6

Fox-Fordyce disease predominantly affects females between the ages of 13 and 35 years, with the most common onset occurring after puberty.

Question 7

What are the typical cutaneous findings associated with Fox-Fordyce disease?

Answer 7

The cutaneous findings of Fox-Fordyce disease typically manifest as:

Characteristic | Description |
|—————-|————-|
| Papules | Grouped, symmetrically distributed, monomorphic, dome-shaped (1 to 3 mm) |
| Color | Flesh-colored to mildly erythematous |
| Pruritus | Intermittently pruritic |
| Affected Areas | Axillae, pubic, perineal, areolar, umbilicus, sternal areas |
| Other Findings | Excoriations and lichenification due to scratching; diminished sweating production in affected areas |

Question 8

What are the common sites affected by Fox-Fordyce disease?

Answer 8

Common sites include the axillae, pubic, perineal, areolar, umbilicus, and sternal areas.

Question 9

What are the clinical features of Fox-Fordyce disease that distinguish it from other pruritic conditions?

Answer 9

Fox-Fordyce disease is characterized by grouped, symmetrically distributed, monomorphic, dome-shaped papules that are follicular-based, flesh-colored to mildly erythematous, and intermittently pruritic.

Question 10

What is the significance of diminished sweating in Fox-Fordyce disease?

Answer 10

Diminished sweating in affected areas is a common finding in Fox-Fordyce disease and is associated with the obstruction of apocrine gland ducts.

Question 11

What are the secondary skin changes observed in Fox-Fordyce disease?

Answer 11

Secondary skin changes include excoriations and lichenification, often due to scratching.

Question 12

What is the role of oral contraceptives in Fox-Fordyce disease?

Answer 12

Oral contraceptives have been reported to improve symptoms of Fox-Fordyce disease, suggesting a hormonal component to the disease.

Question 13

What are the potential triggers for Fox-Fordyce disease flares?

Answer 13

Potential triggers include sympathetic stimulation such as sweating, emotional stress, excitement, and warm weather.

Question 14

What is the primary pathophysiologic event believed to cause Fox-Fordyce disease?

Answer 14

Hyperkeratotic obstruction of the follicular infundibulum at the apocrine gland duct insertion site is believed to be the primary pathophysiologic event.

Question 15

What are the clinical manifestations of Fox-Fordyce disease?

Answer 15

Fox-Fordyce disease manifests clinically as intensely pruritic, dome-shaped, perifollicular papules due to the inflammatory response from glandular contents expelled into the dermis.

Question 16

How is Fox-Fordyce disease diagnosed?

Answer 16

Fox-Fordyce disease is diagnosed clinically based on careful history and cutaneous findings; histopathologic findings can assist but are variable and nonspecific.

Question 17

What histopathologic findings are commonly associated with Fox-Fordyce disease?

Answer 17

Common histopathologic findings include hyperkeratosis of the infundibular epithelium, dilation of the follicular infundibulum, and the presence of perifollicular and periductal xanthomatosis cells.

Question 18

What is the clinical course and prognosis of Fox-Fordyce disease?

Answer 18

Fox-Fordyce disease is chronic, difficult to treat, and has no definitive cure; however, remission has been observed in some cases after menopause.

Question 19

What are some management strategies for Fox-Fordyce disease?

Answer 19

Management strategies include symptomatic therapies like oral antihistamines, topical treatments such as short-term steroids and calcineurin inhibitors, and procedural therapies like intralesional steroid injection and surgical excision.

Question 20

What is the significance of perifollicular adventitial fibrosis in Fox-Fordyce disease?

Answer 20

Perifollicular adventitial fibrosis is a histopathologic finding commonly observed in Fox-Fordyce disease.

Question 21

What is the significance of lymphohistiocytic infiltrate in Fox-Fordyce disease?

Answer 21

Lymphohistiocytic infiltrate is a histopathologic finding commonly observed in Fox-Fordyce disease.

Question 22

What is the significance of xanthomatosis cells in Fox-Fordyce disease?

Answer 22

Perifollicular and periductal xanthomatosis cells are histopathologic findings commonly observed in Fox-Fordyce disease.

Question 23

What histopathologic findings are commonly observed in Fox-Fordyce disease?

Answer 23

Histopathologic findings include hyperkeratosis of the infundibular epithelium, dilation of the follicular infundibulum, perifollicular and periductal xanthomatosis cells, focal spongiosis of the upper infundibulum, perifollicular adventitial fibrosis, and lymphohistiocytic infiltrate.

Question 24

What are the differential diagnoses for Fox-Fordyce disease, and how is it clinically distinguished?

Answer 24

Fox-Fordyce disease is clinically distinguished by its characteristic grouped, dome-shaped, perifollicular papules and pruritus that worsens with sympathetic stimulation.

Question 25

What are the limitations of imaging and laboratory testing in diagnosing Fox-Fordyce disease?

Answer 25

Imaging and laboratory testing are not useful in diagnosing Fox-Fordyce disease.

Question 26

What conservative measures can help minimize pruritus in patients?

Answer 26

- Stress reduction and heat avoidance may help minimize pruritus. - First-generation oral antihistamines can be used for itch, especially during the night. - Low doses of doxepin (<10 mg), up to 3 times a day, can be used as an alternative to antihistamines. - Avoid thick creams and lotions that can exacerbate follicular obstruction.

Question 27

What are the first-line topical therapies for alleviating itching?

Answer 27

- Topical steroid creams are first-line therapies and can temporarily relieve itching. - Intradermal triamcinolone may help alleviate itching during acute flares. - Topical calcineurin inhibitors (1% pimecrolimus cream, 0.1% tacrolimus) can be used as alternatives to steroids.

Question 28

What are some alternative medical therapies for Fox-Fordyce Disease?

Answer 28

- Topical tretinoin can be effective; 0.1% adapalene gel is better tolerated than tretinoin. - Other medical therapies include diethylstilbestrol, oral contraceptives, testosterone, corticotropin, ultraviolet light therapy, X-ray therapy, and oral isotretinoin. - A one-time injection of botulinum toxin type A may also be considered.

Question 29

What procedural therapies are available for Fox-Fordyce Disease?

Answer 29

- Electrocautery - Excision-liposuction with curettage - Microwave thermal ablation - Laser-based excisions These procedures can be curative but are generally considered late-line options due to concerns about infection, hypertrophic scarring, or disfigurement.

Question 30

What is isotretinoin?

Answer 30

Isotretinoin is one of the medical therapies for Fox-Fordyce disease, but it is not a first-line treatment.

Question 31

What procedural therapies are available for Fox-Fordyce Disease?

Answer 31

Procedural therapies include electrocautery, excision-liposuction with curettage, microwave thermal ablation, and laser-based excisions. ## Footnote These therapies can be curative but are generally considered late-line options due to concerns about infection, hypertrophic scarring, or disfigurement.

Question 32

What conservative measures can be taken to minimize pruritus in patients with Fox-Fordyce Disease?

Answer 32

Conservative measures include stress reduction and heat avoidance to minimize pruritus. First-generation oral antihistamines can also be used for itch relief, especially at night.

Question 33

What are the first-line topical and medical therapies for Fox-Fordyce Disease?

Answer 33

First-line topical and medical therapies include topical steroid creams for temporary relief of itching, intradermal triamcinolone to alleviate itching during acute flares, and topical calcineurin inhibitors as alternatives to steroids. ## Footnote Other therapies include topical tretinoin and 0.1% adapalene gel, which is better tolerated than tretinoin.

Question 34

What are the risks associated with thick creams and lotions in Fox-Fordyce disease?

Answer 34

Thick creams and lotions can exacerbate follicular obstruction and worsen symptoms of Fox-Fordyce disease.

Question 35

What is the role of ultraviolet light therapy in Fox-Fordyce disease?

Answer 35

Ultraviolet light therapy is one of the medical therapies for Fox-Fordyce disease, though it is not a first-line treatment.

Question 36

What is the role of diethylstilbestrol in managing Fox-Fordyce disease?

Answer 36

Diethylstilbestrol is one of the medical therapies for Fox-Fordyce disease, though it is not a first-line treatment.

Question 37

What is the role of intradermal triamcinolone in managing Fox-Fordyce disease?

Answer 37

Intradermal triamcinolone may help alleviate itching during acute flares of Fox-Fordyce disease.

Question 38

What is the role of corticotropin in managing Fox-Fordyce disease?

Answer 38

Corticotropin is one of the medical therapies for Fox-Fordyce disease, though it is not a first-line treatment.

Question 39

What is the role of stress reduction in managing Fox-Fordyce disease?

Answer 39

Stress reduction can help minimize pruritus in Fox-Fordyce disease.

Question 40

What are the first-line topical therapies for managing Fox-Fordyce disease?

Answer 40

First-line topical therapies include topical steroid creams, which temporarily relieve itching, and intradermal triamcinolone to alleviate itching during acute flares.

Question 41

What conservative measures can help minimize pruritus in Fox-Fordyce disease?

Answer 41

Conservative measures include stress reduction and heat avoidance. First-generation oral antihistamines can also be used for itch, especially during the night.

Question 42

What are the options for topical calcineurin inhibitors in Fox-Fordyce disease?

Answer 42

Topical calcineurin inhibitors such as 1% pimecrolimus cream and 0.1% tacrolimus can be used as alternatives to steroids for managing Fox-Fordyce disease.

Question 43

What should a patient know about oral isotretinoin for Fox-Fordyce disease?

Answer 43

Oral isotretinoin is one of the medical therapies for Fox-Fordyce disease, but it is not a first-line treatment.

Question 44

What is the role of botulinum toxin type A in managing Fox-Fordyce disease?

Answer 44

A one-time injection of botulinum toxin type A is one of the medical therapies for Fox-Fordyce disease.

Question 45

What is the role of topical tretinoin in managing Fox-Fordyce disease?

Answer 45

Topical tretinoin can be effective in managing Fox-Fordyce disease. Topical 0.1% adapalene gel, which is better tolerated than tretinoin, is also an option.