HIDRADENITIS

SUPPURATIVA
BY DR. SAMAN MUBASHAR
PGR DERMATOLOGY SZH
DEFINITION
“ Hidradenitis suppurativa is a chronic, inflammatory, recurrent,
debilitating, follicular disease that usually presents after puberty.
There are painful, deep‐seated inflamed lesions in the apocrine
gland‐bearing areas of the body, most commonly the axillary,
inguinal and ano‐genital regions. “
CRITERIA TO BE MET:

 The following three criteria must be met for the diagnosis to be

made:
1. Typical lesions: that is deep‐seated, painful nodules: ‘blind boils’ in
early lesions; abscesses, draining sinuses, bridged scars and paired
or multiheaded open pseudocomedones in secondary lesions.
2 .Typical topography: axillae, groin, perineal and perianal region,
buttocks and infra‐ and intermammary folds.
3. Chronicity and recurrence of lesions.
SYNONYMS

 ACNE INVERSA
 VERNEUIL DISEASE
 VERPEAU DISEASE
 PYODERMA FISTULANS SIGNIFICA
 ECTOPIC ACNE
EPIDEMIOLOGY

 AGE : Second and third decade of life , rarely beyond fifth decade

 SEX: Women more affected than males (2.7:1)

 Topographical distribution:
perianal and gluteal disease _____ Males
genito-femoral and sub-mammary___ Females

 ETHINICITY: no ethnic variation

ASSOCIATED DISEASES
FOLLICULAR OCCLUSION
TRIAD
• Acne conglobata
• Dissecting cellulitis of
the scalp
• Pilonidal sinus
SYSTEMIC INFLAMMATORY
DISEASES GENODERMATOSES
• CROHNS DISEASE
• Keratosis ichthyosis
• SAPHO (synovitis, acne,
pustulosis,hyperostosis and
deafness syndrome
osteitis) • Pachyonychia congenita
• PAPA (pyogenic arthritis,
• Steatocystoma multiplex
pyoderma gangrenosum and
acne) • Dowling–Degos disease
• PASH (pyoderma gangrenosum,
acneconglobata and
suppurative hidradenitis)
• PAPASH (pyogenic arthritis,
pyoderma gangrenosum, acne
and suppurative hidradenitis)
FOLLICULAR OCCLUSION TRIAD
PATHOPHYSIOLOGY

 Follicular involvement is central to pathogenesis.

The following sequence of events has been suggested:

1. infundibular hyperkeratosis
2. follicular dilatation/cyst formation,
3. follicular rupture with subsequent inflammation
4. fistula formation by epidermal strands .
PREDISPOSING FACTORS

 OBESITY
 SMOKING
 HORMONAL INFLUENCES : documented and supported
by a female preponderance, observed perimenstrual
disease flares and improvement during pregnancy .
 MEDICATION: Lithium and sirolimus therapy
HOST DEFENCE

 Alterations of the innate immune system are thought to underlie disease

pathogenesis.
 enhanced production of free oxygen radicals by stimulated neutrophil granulocytes
 an impaired secretion of tumour necrosis factor a (TNF‐α) and interleukin 6
 diminished percentage of natural killer cells in the blood
 Highly up‐regulated cytokines include IL‐1β, TNF‐α and IL‐10.
 IL‐2,IL‐4, IL‐5 and interferon γ (INF‐γ) are hardly detectable in HS lesions
 The IL‐23/Th17 pathway seems to be activated, reflected by enhanced expression of
IL‐17A, IL‐12 and IL‐23 in HS skin
 decreased expression of Toll‐like receptors .
 reduced mRNA expression levels of regulators and inducers ofantimicrobial peptides
(AMPs) such as IL‐22 and IL‐20 .
PATHOLOGY

• EARLY LESION : sparse lymphocytic infiltrate of the terminal

follicular unit and sebaceous gland atrophy.
• DEVELOPED LESION:
o Follicular hyperplasia,
o perifollicular lymphocytic inflammatory infiltration,
o interfollicular psoriasiform hyperplasia
o dilatation of the follicular lumen
o Cysts lined by stratified squamous epithelium containing
lammellated keratin
o free hair shafts appear
 FLARES:
 abscess formation
 ruptured follicular units
 associated with a dense, dermal,mixed, inflammatory infiltrate
including histiocytes and giant cells that extends to interfollicular
apocrine and eccrine structures and deep into the subcutis.
 Sinus tract formation and fibrosis follows
HS with sinus tracts
CAUSATIVE ORGANISM

Primary inflammatory disorder without a defined infectious

trigger.
The most common bacterial isolates
 Staphylococcus epidermidis
 and Staphylococcus aureus
 Peptostreptococcus species
 Propionibacterium acnes
GENETICS

 Loss‐of‐function mutations in the γ‐secretase genes

Nicastrin, Presenilin‐1 and Presenilin enhancer‐2 .
 Gamma‐secretase regulates notch signalling, which
plays a role in :
 epidermal and terminal hair follicle differentiation,
immune cell development and immune functions.
ENVIRONMENT FACTORS

Mechanical irritation

 Shear forces
CLINICAL PRESENTATION:

 HISTORY
• A history of typical lesions at typical sites with recurrence and chronicity is required for
diagnosis
• Painful subcutaneous nodules or abscesses for a mean duration of 7–15 days.
• Followed by spontaneous regression, partial regression (to form non‐
inflammatory,asymptomatic nodules)
• Or progression to abscess formation with the rupture and release of purulent
malodorous discharges.
• Recurrences are common
• Periods of remission (characterized by normal skin or persistent non‐inflammatory
nodules) may last for weeks to months.
 PRESENTATION:
 INDEX LESION: inflamed and non‐inflamed dermal and subcutaneous nodules
 rounded (as opposed to ‘pointing’) abscesses
 draining or non‐draining sinus tracts
 Scarring: bridged or ‘rope‐like’, hypertrophic or atrophic producing depressions especially on
the buttocks, and maybe associated with contractures
 Pseudo- (secondary) comedones seen, typically paired, polyporous and grouped
 Closed comedones and regional lymphadenopathy not seen.
SITES

• inverse (flexural) area:

• the axillae and inguinal region
• ano‐genital regions including external genitalia and the perineal, perianal and gluteal
skin
• Sub‐ and intermammary skin
• less commonly, retroauricular, preauricular and occipital skin.
• Truncal variants also reported.
ASSOCIATED LESIONS:

Follicular papules and pustules, pyogenic granulomas at
sinus tract openings and indurated plaques.
 Epidermoid cysts are seen in some patients on external
genitalia, the face and the thorax.
CLINICAL VARIANTS

CLASSIC VARIANT SECOND VARIANT THIRD VARIANT

• Characterized by the
• Characterized by • Characterized by
additional involvement
scarring lesions in the gluteal involvement,
of the ears, chest, back
axillae and under the papules and folliculitis
or legs,
breasts • may be more common
• with pilonidal sinuses
• occurs most often in in men.
women • comedones,severe acne
• often includes lesions • and a family history of
in the ano‐genital area. HS which may be a part
of the follicular
occlusion triad
D/D:

 PRIMARY CUTANEOUS BACTERIAL INFECTION

 SECONDARY INFECTION OF CYSTIC STRUCTURES
 CROHNS DISEASE
 TUBERCULOSIS
 SPOROTRICHOSIS
 ACTINOMYCOSIS
 LYMPHOGRANULOMA VENERUM
 STEATOCYSTOMA MULTIPLEX
 LANGERHAN CELL HISTOCYTOSIS
SEVERITY SCORES
HSS (MODIFIED SARTORIUS SCORE)
ASSESS SEVERITY AND TREATMENT RESPONSE
COMPLICATIONS:

 LYMPTHAIC OBSTRUCTION (LYMPHEDEMA)

 SCROTAL ELEPHANTIASIS
 FISTULAS (CHRONS DISEASE SHOULD BE RULED OUT)
 ANEMIA
 HYPOALBUMINEMIA
 HYPERGAMMAGLOBULINEMIA
 AMYLOIDOSIS
 DEPRESSION IS FREQUENT
DISEASE COURSE:

 CHRONICITY IS HALLMARK
 HURLEYS STAGE 1 : EFFECTS TWO THIRD OF PATIENTS
 HURLEYS STAGE II : EFFECTS ONE QUARTER OF PATIENTS
 HURLEYS STAGE III : EFFECTS ONE FIFTH OF PATIENTS
 CHANCES OF NATURAL REMISSION INCREASE WITH INCREASING AGE
ESPECIALLY AFTER 50 YEARS OF AGE .
INVESTIGATIONS:

• MICROBIOLOGY (swabs, purulent exudate and tissue) and

HISTOPATHOLOGY : for refractory or atypical cases.
• IMAGING (both USG and MRI) : subclinical extension, complications of
severe disease and informs preoperative planning.
• BLOOD LABS in severe disease ( HURLEYS STAGES III ):
o CBC ( anemia )
o SERUM ALBUMIN LEVEL (reduced)
o POLYGONAL HYPERGAMMAGLOBULINEMIA
o C REACTIVE PROTEINS (elevated)
MANAGEMENT:

GENERAL MEASUREMENTS
• AVOIDANCE OF TIGHT FITTING CLOTHING
• WEIGHT LOSS
• SMOKING CESSATION
• ANALGESICS (NSAIDS and paracetamol)
• REASSURANCE
• STRESS MANAGEMENT
TOPICAL AGENTS:

• Topical CLINDAMYCIN lotion 0.1% :for control of milder lesions

(Hurley Stage I)
• Topical RESORCINOL 15% in a suitable ointment :
localized,recalcitrant, established lesions (Hurley stage II)
• The benefits of antiseptics such as chlorhexidine washes or
benzoyl peroxide remain unproven.
SYSTEMIC ANTIBIOTICS

 MILD DISEASE : Oral TETRACYCLINES 500mg B.D for 3

months
 ADVANCED CASES : combination CLINDAMYCIN 300mg
B.D and RIFAMPACIN 300mg B.D for 10 weeks
 Others : Combination of RIFAMPICIN, METRONIDAZOLE
and MOXIFLOXACIN effective in small series of cases.
ANTI INFLAMMATORY TREATMENT

 For single lesions : intralesional triamcinolone (3–5 mg)

 For more severe disease:
• short‐term systemic PREDNISOLONE (0.5–1.0 mg/kg body
weight)or
• CICLOSPORIN(3–5 mg/kg body weight), or
• longer term treatment using DAPSONE (100 mg daily).
OTHERS

 BIOLOGICAL AGENTS :
o Adalimumab ( 160mg at 0 week , 80mg at 2 week foolowed at 4 weeks by 40mg every
week for a total of 12 weeks.)
 RETINOIDS :
o Acitretin (50mg or more) effective in some cases.
 EXPERIMENTAL THERAPIES :
o Zinc gluconate (90mg/day)
o IM Immunoglobulins
o Metformin
o Botulinum toxin
SURGERY

 INCISION AND DRAINAGE

 DEROOFING
 LOCALIZED SURGERY
 EXTENSIVE SURGERY for extensive disease with multiple interconnecting sinus tracts.
 CO2 LASER EVAPORATION
 Nd:YAG AND INTENSE PULSED LASER (IPL) – monthly
 RADIOTHERAPY
THANKYOU