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Exciting Stuff! Pathergy in Periocular Pyoderma Gangrenosum RS Thampy,B Leatherbarrow,C Lyon

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Case report 46 y caucasian male RE progressive cicatricial ectropion 7 months

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Past history Antecedent small skin cyst upper cheek Treated at Walk-in Centre Fit & well Chemical worker Smokes 1x pack/day, drinks 1 bottle wine/day Hmm not sure what that is.. Lets try a needle

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pyogenic reaction dramaticIntense

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Pathergy :Ulceration at site of minor trauma Violaceous ulceration with undermined edges Swabs - negative Biopsy: Non-specific inflammation Not malignant Not vasculitis

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Empirical treatment Topical Fucibet Oral minocycline Reduction in inflammatory component

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4 Months post-puncture

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Progressive cribriform cicatrisation inducing further ectropion Systemic cyclosporin with blood monitoring 5 months later (September 09)

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Pyoderma Gangrenosum

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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 caseslesions develop at the site of minor trauma, so surgery or debridement are contraindicated. ??

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Subtypes

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Halogenoderma Sweets Syndrome

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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

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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

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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 Sweets

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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

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Procianoy et al Arq Bras Oftalmol. 2009;72(3):384-6

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Surgical Management Essentially shortage of anterior lamella Lateral tarsal strip procedure and scar tissue release with full- thickness 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.

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A) Preoperative B) 6-month post-operative C) Hypertrophic donor site A B C

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Adjacent pustular ulcerated skin over zygoma

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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

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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