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Pathergy in Periocular Pyoderma Gangrenosum
RS Thampy,B Leatherbarrow,C Lyon
Case report
• 46 y caucasian male
• RE progressive cicatricial ectropion
7 months
Past history
• Antecedent small skin cyst upper cheek
– Treated at Walk-in Centre
Hmm not sure
what that is..
Let’s try a needle
• Fit & well
• Chemical worker
• Smokes 1x pack/day, drinks 1 bottle wine/day
Intense
dramatic
pyogenic
reaction
• Pathergy :Ulceration at site of minor trauma
• Violaceous ulceration with undermined edges
• Swabs - negative
• Biopsy: Non-specific inflammation
» Not malignant
» Not vasculitis
Empirical treatment
• Topical Fucibet
• Oral minocycline
• Reduction in inflammatory component
4 Months post-puncture
5 months later (September 09)
Progressive cribriform cicatrisation inducing further ectropion
Systemic cyclosporin with blood monitoring
Pyoderma Gangrenosum
BMJ Review Article
Brooklyn et al BMJ 2006;333:181-184
• A rare but serious neutrophilic dermatosis
• Commonly missed/ diagnosed late
• Deep ulcer with
– a well defined border,
•
•
•
•
•
usually violet or blue.
edge often undermined
surrounding skin is erythematous and indurated
Most commonly on the legs.
Patients are often systemically unwell with fever & malaise.
Lesions are usually painful and the pain can be severe.
When the lesions heal the scars are often cribriform.
Pathergy occurs in 25-50% of cases—lesions develop at the site of minor
trauma, “so surgery or debridement are
contraindicated.” ??
Subtypes
Sweet’s Syndrome
Halogenoderma
Histopathology
•
Findings:
– depend on:
• age of lesion
• site biopsied
•
Presence (or absence) of vasculitis:
– lymphocytic and/or leukocytoclastic vasculitis at advancing erythematous edge in 73% of cases)
•
Earliest lesion:
– follicular and perifollicular inflammation with intradermal abscess formation
•
Later lesions:
– necrosis of superficial dermis and epidermis:
• forms ulcer: may extend into underlying subcutis
• sometimes giant cells: particularly if associated Crohn's disease
•
Advancing edge:
– lymphocytic vasculitis:
• endothelial swelling
• disputed by some authors
– sometimes leukocytoclastic vasculitis
•
Acanthosis:
– prominent in perilesional erythematous zone
Who gets pyoderma gangrenosum?
50% are associated with underlying systemic conditions
• 30% of cases occur in patients with inflammatory bowel disease.
2% of patients with inflammatory bowel disease will develop pyoderma
PG not related to the activity of the inflammatory bowel disease, often occurs in patients whose bowel disease
is in clinical remission.
• 25% of patients have arthritis, most often seropositive rheumatoid arthritis
Activity of the arthritis is not related to pyoderma.
• Leukaemia is the most frequently reported malignancy, usually AML
PG and the eye
•
Largest series I could find : 4 pts at Moorfields
Rose et al Ophthalmology 2003
– slowly evolving, painful, unilateral blue-grey swellings of the pretarsal tissues
– progressed to frank tissue necrosis and loss of full-thickness eyelid, with patchy sparing of the lid
margin or lashes
– very distinctive preservation of the pretarsal marginal artery across full-thickness eyelid defects
– lid loss characteristically involved lateral one third of the lower eyelid (3 of 4 lids)
–
in one case, extending into the postseptal tissues in the inferotemporal orbit.
–
In 3 patients, the pyoderma, responded well to systemic immunosuppression and eyelid repair
was undertaken during the quiescent phase.
– In a single patient, relapsing disease led to loss of the eye as a result of involvement of the globe
and deep orbital tissues.
•
Other reports scleritis, PUK, orbital- coexistent with Sweet’s
Medical Treatment
• Topical steroid
• Local injection of triamcinolone under ulcer edge
• Topical tacrolimus
• Minocycline 100 mg twice daily
• Prednisolone “is the drug of choice” and is usually started at (60-120 mg)
– (level B evidence)
• IV MePred 1g pulses x 3-5
•
Cyclosporine 3-5 mg/kg
•
serious side effects, including nephrotoxicity, hypertension, and increased risk of cancer have
not been reported for the low doses used to treat pyoderma
• Infliximab + azathioprine
Procianoy et al
Arq Bras Oftalmol. 2009;72(3):384-6
Surgical Management
• Essentially shortage of anterior lamella
• Lateral tarsal strip procedure and scar tissue release with fullthickness skin grafting
• Pretreated with corticosteroid for several months
• Disease was considered quiescent for the previous 6 months under
treatment.
• Prednisolone dose increased from 40 mg/day to 60 mg/day 1 week
before surgery.
• This dose was maintained during the first month post-operatively
and tapered.
A) Preoperative B) 6-month post-operative C) Hypertrophic donor site
A
C
B
Adjacent pustular ulcerated skin over zygoma
Conclusion
• Pyoderma gangrenosum CAN be co-managed
surgically
• Excluding other diagnoses and investigation
underlying associations is essential
• Immunomodulation by experienced dermatologist is
the key to minimisinng surgical excitement
References:
1. Miller J, Yentzer BA, Clark A, et al. Pyoderma gangrenosum: a review and update on new therapies. J Am Acad
Dermatol. 2010;62(4):646-54.
2. Ruocco E, Sangiuliano S, Gravina AG, et al. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol
Venereol 2009;23(9):1008-17.
3.
Callen JP, Jackson JM. Pyoderma gangrenosum: an update. Rheum Dis Clin North Am 2007;33(4):787-802.
4. Lindberg-Larsen R, Fogh K. Traumatic pyoderma gangrenosum of the face: pathergy development after bike
accident. Dermatology 2009;218(3):272-4.
5.
Gulyas K, Kimble FW. Atypical pyoderma gangrenosum after breast reduction. Aesthetic Plast Surg 2003;27:328-331.
6. Rand RP, Olerud JE, Verrier ED. Pyoderma gangrenosum after coronary artery bypass grafting. Ann Thorac Surg
1993;55:1016-1018.
7. Banga F, Schuitemaker N, Meijer P. Pyoderma gangrenosum after caesarean section: a case report. Reprod Health
2006;3:9.
8.
Akhras V, Sarkany R, Walsh S, et al. Superficial granulomatous pyoderma treated preoperatively with infliximab. Clin
Exp Dermatol. 2009;34(5):e183-5.
9. Procianoy F, Barbato MT , Osowski LE et al. Cicatricial ectropion correction in a patient with pyoderma
gangrenosum: case report. Arq Bras Oftalmol. 2009 72(3):384-6