15 - 84 - HIDRADENITIS SUPPURATIVA Flashcards
disproportionately affected population in HS
Reproductive-age women
consistent histologic finding of early disease and is thought to be the primary pathophysiologic event of hidradenitis suppurativa
Hyperkeratotic plugging of the terminal hair follicle
(Keratinous occlusion of the terminal hair follicle)
describe the clinical manifestation of HS
- Lesions may begin as tenderness or pruritus that progresses to a tender papule or deep-rooted nodule (Fig. 84-1).
- Nodules can become quite large and painful.
- They may resolve slowly without drainage or progress to an abscess-like lesion that eventually ruptures and drains purulent material before involuting (Fig. 84-2).
- Involution may take 7 to 10 days, but in some patients, healing may be delayed, resulting in persistent open wounds with variable amounts of granulation tissue (Fig. 84-3).
- The process then reoccurs in adjacent and/or other intertriginous sites. With repeated episodes, epithelial strands may develop from ruptured follicular epithelium, leading to sinus tract formation and intermittent drainage of foul-smelling serosanguinous and/or purulent material
infrequent complication of longstanding chronically inflamed lesions
Squamous cell carcinoma (SCC)
HS-associated anogenital SCC is often associated with high-risk human papillomavirus strains, most commonly what HPV serotype?
HPV 16
In animal models, the deficiency of this enzyme leads to sebaceous gland atrophy, as well as epidermal cyst formation.
γ-secretase deficiency
proinflammatory cytokine markedly elevated in HS lesions and in the serum of patients with HS
tumor necrosis factor (TNF)-α
Interleukin (IL)-1β
- The proinflammatory cytokine, tumor necrosis factor (TNF)-α, is markedly elevated in HS lesions and in the serum of patients with HS compared to healthy controls.
- Interleukin (IL)-1β, another potent proinflammatory cytokine, is also strikingly elevated in lesional and perilesional skin (34- to 115-fold higher in lesional compared to healthy skin)
most frequently isolated organisms in HS lesions
Coagulase-negative staphylococcus and anaerobic bacteria
tetrad of HS
- acne conglobata,
- dissecting cellulitis of the scalp,
- pilonidal cysts
- HS
PASH
pyoderma gangrenosum, acne, and HS
PAPASH
pyogenic arthritis, pyoderma gangrenosum, acne, and HS
PsAPASH
pyoderma gangrenosum, acne, and HS
Mutations in what genes have been observed in PASH?
NCSTN and PSTPIP1 (proline-serine-threonine phosphatase interacting protein 1)
diagnostic criteria of HS
- Typical lesion (1 or more) - deep-seated painful nodules, abscesses, draining sinuses, double-open comedones (Fig. 84-9), and bridged scars
- Typical distribution - with 1 or more typical lesions in the axillae, groin, buttocks, perineal, or inframammary region
- **Chronicity and recurrence of lesions **- 2 recurrences over a 6-month period
Hurley Staging system
* Stage I - recurrent abscesses **without scarring or sinus tracts; **
* Stage II - recurrent abscesses with scarring and sinus tract(s), separated by normal skin
* Stage III -recurrent abscesses with diffuse scarring and interconnected sinus tracts with minimal to no normal skin between lesions
Clinical phenotypes/subtypes of HS
**1. classic “axillary-mammary” subtype **- breast and axillary involvement with hypertrophic scarring
2. “follicular” subtype - predilection for follicular lesions (eg, epidermal cysts, pilonidal sinus, comedones, severe acne) and atypical topography involving the ears, chest, back, or legs
3. “gluteal” subtype - follicular papules, folliculitis, and gluteal involvement
nonspecific but universal histopathologic finding in HS irrespective of disease duration
Follicular occlusion
histopath findings of early lesions of HS
- follicular hyperkeratosis of the terminal hair follicles, hyperplasia of the follicular infundibulum, and perifolliculitis
- Epidermal psoriasiform hyperplasia (lacking parakeratosis) and subepidermal perifollicular collections of lymphocytes
histopath findings of mature lesions of HS
- noncaseating granulomas, abscesses, epidermal cysts, sinus tracts, granulation tissue, and dermal fibrosis
- Subcutaneous inflammation, fibrosis, and fat necrosis also can be observed
mainstays of management of mild-to-moderate HS
Topical and oral antibiotic therapy
cornerstone for managing chronic HS
Excisional surgical interventions
first-line therapy for HS
Clindamycin (topical)
Clindamycin/rifampicin (oral)
Adalimumab (subcutaneous)
Tetracycline (oral)
