Hidradenitis Suppurativa: Acne Inversa, Velpeau’s Disease…

Hidradenitis Suppurativa (HS) also referred to as Acne Inversa, was first described in 1839 by the French physician Frederick Velpeau and subsequently was referred to as Velpeau’s disease.  In 1854 Aristide Verneuil also described this disorder so in early literature it was also referred to as Verneuil’s disease.  It is believed that Karl Marx had HS and that he wrote about his difficulties coping with his affliction.  HS is a chronic disease characterized by recurrent abscess formation, primarily in the folded regions of the skin–axilla, groin and under the breasts.  These recurrent boil-like nodules and abscesses frequently culminate in pus-like (purulent) discharge, heal poorly, and produce deep scars and fistulas.  It is a disease of apocrine glands which are found in highest concentration in these anatomical regions.  Much like psoriasis, HS is now believed to be an autoimmune skin disorder involving multiple inflammatory pathways.

HURLEY GRADING SYSTEM:

  1. Grade 1–Single or multiple abscesses without sinus tract formation
  2. Grade 2–Recurrent abscesses with one or more sinus tracts and scarring widely separated by normal skin
  3. Grade 3–Diffuse involvement with multiple sinus tracts and no intervening normal skin

HS frequently starts at puberty and is most severe between the ages of 20-40 years.  It is 3 times more common in women than men.

RISK FACTORS & ASSOCIATIONS:

  1.  Obesity and insulin resistance (metabolic syndrome)
  2.  Cigarette smoking
  3.  Inflammatory bowel disease
  4.  Follicular occlusive disorders (acne conglobata, dissecting cellulitis, pilonidal cysts)
  5.  Other family members with HS
  6.  Autosomal dominant forms associated with mutations in gamma-secretase genes NCSTN, PSENEN & PSEN1

TREATMENT:  Established treatment involves a variety of measures.  It is important to remove risk factors, such as smoking and if possible to encourage weight loss, although there are no good studies documenting improvement in the condition if followed.  Oral antibiotics are frequently implemented along with local skin care using topical antibiotics and/or antibacterial soaps…although this disorder is not caused by infection, these treatments appear to help prevent progression and secondary colonization.  Treatment with adalimumab (Humira) to date is the only medication that has been FDA approved for treatment of HS and two excellent studies have been performed studying this disease.  Surgical options are also utilized in the most severe forms of HS with variably success.  A variety of biological agents are being studied.  Apremilast (Otezla) in one study showed promise…to read this abstract click HERE.  To read more about current treatments for HS click HERE. and HERE. To read more about HS click HERE, HERE and HERE.  To visit the HS Foundation, a nonprofit support group, click HERE.