Hidradenitis Suppurativa

Hidradeni(s Suppura(va
A comprehensive review

Prepared By Yoon K. Cohen

Hidradeni(s Suppura(va
  HS is a chronic, recurrent, inﬂammatory disease
presen(ng as painful subcutaneous nodules
  Double comedones, deep sinus tracts, and abscesses are
characteris(c for HS
  A diagnosis of HS is made clinically without the use of
laboratory tests

Alikhan A, Lynch PJ, Eisen DB. Suppurativa Suppurativa: A comprehensive review. J Am Acad Dermatol 2009;60:539-61.

Demographics
  HS tends to begin in the second or third decades of life
  Females are more likely to develop HS
  Recent studies suggest a prevalence of about 1%


Clinical Presenta(on
  HS mostly typically occurs in the axillary, inguinal,
perianal, perineal, mammary, and inframammary regions
  The distribu(on paKern corresponds with the “milk line”
distribu(on of apocrine related mammary (ssue in
mammals
  The most commonly aﬀected site is the axilla
  Perianal HS is associated with more debilita(ng
outcomes than axillary HS


Clinical Presenta(on


Clinical staging
  Hurley’s staging system is simple and relies on the
subjec(ve extent of diseased (ssue
  The Sartorius system is more sophis(cated then Hurley’s
and is likely to supplant it as a means for conduc(ng
clinical trials


Hurley Staging System
  Stage I  abscess formation (single or multiple) without sinus
tracts and cicatrization

  Stage II  one or more widely separated recurrent abscesses
with tract formation and scars

  Stage III  multiple interconnected tracts and abscesses
throughout an entire area


Differen(al Diagnosis
  The differen(al diagnosis for HS is extensive, but can
easily be differen(ated from other diseases by the age of
onset and characteris(c loca(ons of the lesions


Differen(al Diagnosis
Early lesions   Late lesions
Acne Ac(nomycosis
Carbuncles Anal fistula
Celluli(s Cat scratch disease
Cutaneous blastomycosis Crohn disease
Dermoid cyst Granuloma inguinale
Erysipelas Ischiorectal abscess
Furuncles Lymphogranuloma venereum
Inflamed epidermoid cysts Nocardia infec(on
Lymphadenopathy Noduloulcera(ve syphilis
Perirectal abscess Pilonidal disease
Pilonidal cyst Tuberculous abscess
   Tularemia

E(opathogenesis
  Long thought to be a disorder of apocrine origin, it is now thought
to be caused by follicular occlusion
  Families with autosomal dominance type inheritance have been
reported
  Hyperandrogenism likely does not play a role in HS
  Bacteria are probably secondary colonizers, which may exacerbate
HS, but they are not the primary e(ologic agents
  Poor hygiene does not cause HS
  Some inves(gators postulate that there may be an underlying
immunologic aberra(on involved in the pathogenesis of HS; more
work needs to be done in this ﬁeld before any conclusions can be
made
  Smoking and obesity are not primary causes of HS but are strongly
associated with the disease and may exacerbate it


E(opathogenesis
  Follicular and glandular factors
  Gene(c factor
  Endocrinologic factor
  Bacterial factors
  Environmental factors
  Host defense factors
  Pharmacologic factors
  Smoking
  Obesity

Typical Histologic Changes in HS


Psychological and physiologic
morbidity
  HS pa(ents experience a quality of life worse than
those with alopecia, mild to moderate psoriasis, and
several other dermatologic condi(ons
  HS results in economic and psychological disability in
addi(on to the physical problems


Disease associa(ons
  HS is associated with other diseases of the follicular
occlusion tetrad, acne vulgaris, KID syndrome, IK,
DDD, SAPHO syndrome, CD, Jackson–Lawler type
pachyonychia congenital, reﬂex sympathe(c
dystrophy, and FFD, among others


Disease associa(ons
Common associa1ons Rare associa1ons
Acne conglobata Acanthosis nigricans
Acne vulgaris Bazex‐Dupre‐Christol syndrome
Crohn disease Dowling–Degos disease
Dissec(ng celluli(s of the scalp Fox–Fordyce disease
Obesity Inters((al kera((s
Pilonidal disease Kera((s‐ichthyosis‐deafness syndrome
Smoking Pachyonychia congenita
   PAPA syndrome
   Pyoderma gangrenosum
   Reﬂex sympathe(c dystrophy
   SAPHO syndrome
   Scrotal elephan(asis

Prognosis and disease complica(ons
  The risk of SCC and other malignancies may be
increased in those with HS
  Contractures, strictures, serious infec(on, and
anemia are also poten(al sequelae


Complica(ons
  Anal, urethral, and rectal strictures and ﬁstulas
  Anemia
  Contractures and limb mobility limita(ons
  Cutaneous squamous cell carcinoma
  Increased risk of other malignancy
  Lumbosacral epidural abscess
  Sacral bacterial osteomyeli(s


Treatment
  There is no uniformly eﬀec(ve single therapy for HS;
therefore, clinicians will likely have to try an array of
diﬀerent treatment modali(es depending on the
pa(ent's disease
  For pa(ents with extensive disease, wide excision can
drama(cally improve the pa(ents' quality of life
  Most of the listed therapies are ones that dermatologists
possess in(mate knowledge of and are thus in the best
posi(on to treat this debilita(ng disorder.


Treatment
  An(bio(cs
  Re(noids
  Hormones
  Immunosuppressive and an(inﬂammatory agents
  Neurotoxins
  Radiotherapy
  Light, radiofrequency, and other procedures
  Surgery


Treatment ‐Surgery


Approach to treatment
Hurley stage I disease or ﬁrst‐line therapy
  An(bio(cs, either topical or systmic (A)
  Hormonal therapy (A)
  Re(noids (A)
  Zinc (A)
  Cryotherapy (B)
  Botox (C)
  Radiofrequency treatment (C)
  Short‐term cor(costeropids (C)


Hurley stage II disease or second‐line therapy
  CO2 laser abla(on (A)
  Immunosuppressive therapies (A)
  Limited excisions (A)
  Radia(on therapy (A)
  Radiofrequency treatment (C)


Hurley stage III disease or third‐line therapy
  Radia(on therapy (A)
  Wide excision (A)
  Laying open of sinus tracts (C)


General Treatment Sugges(ons
  Avoidance of (ght‐ﬁeng clothing
  Nonnarco(c analgesics
  Reassurance
  Smoking cessa(on
  Stress management
  Support group referral
  Weight loss


Conclusion
  The prevalence of HS may equal or even surpass that of
psoriasis, yet it receives only a frac(on of the aKen(on and
scholarly contribu(ons. Though no overwhelmingly
eﬀec(ve treatments have been described, novel therapies
are emerging
  Many—if not all—of these therapies are well known to
dermatologists; therefore, we should play a lead role in
managing this debilita(ng disorder. As our understanding of
this disorder grows, so will our treatment op(ons


Thank You

Yoon K. Cohen

Hidradenitis Suppurativa

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