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Quiz: February 2007
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What is your diagnosis for these mildly pruritic papular eruptions

on the axillary areas and areola of a teenager boy?

 

 

 
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Diagnosis: Fox-Fordyce disease

Synonym: Apocrine miliaria 

Fox-Fordyce disease is an uncommon, chronic eruption of apocrine gland-bearing skin caused by blockage and intraepidermal rupture of apocrine ducts. The resulting intensely pruritic papules, which mainly affect the axillae and pubic regions of postbubertal women, are difficult to treat. 

Most cases occur in women between the ages of 13 and 35 years. The disease in only 1/10th one tenth as common in men. It has only rarely been reported before puberty 1,2 or after menopause. Pregnancy invariably has a beneficial influence. 

Clinical manifestations

Begins shortly after puberty and affects mainly the axillary (as in quiz case) and pubic regions, although other apocrine gland-bearing areas, such as areolae(as in quiz case), periumblicus, and perineum, are sometimes involved. Icthing (as in quiz case) is often intense and may precede the formation of typical flesh-colored follicular papules. Paraxysmal bouts of pruritus triggered by emotional stimuli can occur.

Management

  • No treatment has had universal success, but the mainstay of therapy has been oral contraceptives.
  • Topical treatment with 0.1% tretinoin cream. 3,4
  • Intralesional injection of triamcinolone acetonide (5-10 mg/ml),
  • Topical clindamycin solution. 5
  • Systemic isotretinoin.
  • Surgical excision or dermabrasion, electrolysis and CO2 laser intervention.
  • More recently effect of the pimecrolimus also been documented.6

References

 1. Ranalletta M, Rositto A, Drut R. Fox-Fordyce disease in two prepubertal girls: histopathologic demonstration of eccrine sweat gland involvement.
Pediatr Dermatol. 1996 Jul-Aug;13(4):294-7.  

2. Sandhu K, Gupta S, Kanwar AJ. Fox fordyce disease in a prepubertal girl.
Pediatr Dermatol. 2005 Jan-Feb;22(1):89-90. 

3. Giacobetti R, Caro WA, Roenigk HH Jr. Fox-Fordyce disease. Control with tretinoin cream.
Arch Dermatol. 1979 Nov;115(11):1365-6.  

4. Thomas JR 3rd, Doyle JA. The therapeutic uses of topical vitamin A acid.
J Am Acad Dermatol. 1981 May;4(5):505-13. Review.

5. Miller ML, Harford RR, Yeager JK. Fox-Fordyce disease treated with topical clindamycin solution. Arch Dermatol. 1995 Oct;131(10):1112-3.

6. Pock L, Svrckova M, Machackova R, Hercogova J.  Pimecrolimus is effective in Fox-Fordyce disease. Int J Dermatol. 2006 Sep;45(9):1134-5.

Mehrdad Mehravaran, M.D., Dermatologist. / Szeged- Hungary

 

It is a case of Fox-Fordyce disease.

Fox-Fordyce disease is a condition of the axillary apocrine glands and is manifested by pruritic dome-shaped follicular papules.
The itching is exacerbated by increased sweating.
Most common in women, it can also occur in males. 
 It is a chronic blockage of the sweat gland ducts with a secondary, nonbacterial inflammatory response to the secretions and cellular debris in the cysts. Hidradenitis is very similar but tends to have a secondary bacterial infection so that pus-draining sinuses are formed. It is a very devastating skin disease that does not have universally curative treatments.
The mainstay treatment is surgical removal of the skin tissue containing affected sweat glands. Irradiation therapy may also be used and antibiotics are used to reduce the inflammatory response.
No treatment is required for Fordyce granules, except for cosmetic removal of labial lesions. Inflamed glands can be treated topically with clindamycin. When surgically excised, recurrence does not occur. Neoplastic transformation is very rare but has been reported.
 
 
Dr Reza Ghaderi
Associate Professor of Dermatology

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