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Pyoderma gangrenosum
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis presenting as pustules enlarging and ulcerating with violaceous, necrotic, and undermined borders healing with cribriform scarring. Multiple subtypes exist involving any skin location. PG has several associations ( Table 212.1 ) that should be considered in the workup.
Table 212.1
Systemic associations of PG
| Associated conditions (most to least common) |
|---|
| Hematologic disease (leukemia, lymphoma, myelodysplasia, myelofibrosis, monoclonal gammopathy of undetermined significance, polycythemia vera, IgA gammopathy) |
| IBD (ulcerative colitis, Crohn disease) |
| Other inflammatory diseases and syndromes (rheumatoid arthritis; Sjögren syndrome; systemic lupus erythematosus; granulomatosis with polyangiitis; pyogenic arthritis, hidradenitis suppurativa [PASH]; pyogenic arthritis, pyoderma gangrenosum, acne [PAPA]) |
| Solid organ malignancy |
| Infectious (hepatitis B/C, human immunodeficiency virus) |
| Hidradenitis suppurativa |
| Pregnancy |
| Leukocyte adherence deficiency |
| Endocrine disorders (i.e., metabolic syndrome) |
Management Strategy
Goals of PG treatment include pain control, superinfection prevention, and facilitating healing. Pain is managed with oral acetaminophen, non-steroidal antiinflammatory drugs, opioids, gabapentin, pregabalin, and nortriptyline. Medical and surgical management should be combined with maintaining a moist wound environment with foam or hydrocolloid dressings. Consider conservative debridement, keeping in mind the risk of pathergy.
No treatment is consistently effective for PG . Conservative therapy with wound care and topical corticosteroids or calcineurin inhibitors are reasonable first-line options for small areas of slowly progressive PG. However, if PG is rapidly progressive, systemic therapies like systemic corticosteroids (sCS) or ciclosporin should be initiated early.
sCS are often first-line therapies in conjunction with topical care. Response to sCS is also a diagnostic feature of PG. If PG is associated with a systemic disorder, treating the underlying disorder may improve PG. Suggested investigations for working up PG are listed in Specific Investigations. When active inflammation is evident, therapy should be continued; however, if inflammation subsides, immunomodulatory therapy is discontinued while wound care continues. This is often called ‘Gulliver Sign’ (Landis ET, Taheri A, Jorizzo JL. Gulliver’s sign: a recognizable transition from inflammatory to healing stages of pyoderma gangrenosum. J Dermatolog Treat. 2015;26(2):171–172).
Specific Investigations
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First-Line Therapies
Efficacy of topical calcineurin inhibitors in pyoderma gangrenosum
Abdul-Fattah B, Al-Muriesh M, Huang CZ. Dermatol Ther 2018; 31(5): e12697.
Pyoderma gangrenosum successfully treated with topical tacrolimus
Napolitano M, Megna M, Patrì A, et al. G Ital Dermatol Venereol 2019; 154(3): 361–3.
Case report of female with refractory Crohn disease and parastomal PG with PG responding to topical tacrolimus 0.1% ointment BID.
Caution: a case of systemic absorption and acute nephrotoxicity has been reported in a patient who applied 60 g of tacrolimus ointment (i.e., presumably 60 g of 0.1% ointment) daily to PG .
Pyoderma gangrenosum: a rare cause of cutaneous ulceration and one easily misdiagnosed
Abdelrahman W, Walsh MY, Hoey SE, et al. Case Rep Pediatr 2016; 2016: 5971706.
Dramatic improvement of pediatric PG with clobetasol ointment within 24 hours.
Beclomethasone inhaler used to treat pyoderma gangrenosum
Chriba M, Skellett AM, Levell NJ. Clin Exp Dermatol 2010; 35: 337–8.
A case of peristomal PG healed over 4 weeks using beclomethasone dipropionate 200 mcg, four puffs daily.
Efficacy of systemic dapsone treatment for pyoderma gangrenosum: a retrospective review
Din RS, Tsiaras WG, Li DG, et al. J Drugs Dermatol 2018; 17(10): 1058–60.
The authors reviewed 27 patients with PG treated with dapsone with the most common peak dosage at 50 mg or 100 mg daily. The average time to initial treatment response was 5.3 weeks. The average duration of treatment was 14.3 months.
Low cost and familiarity of dermatologists with dapsone probably contribute to its popularity. Dapsone is often used in combination with other modalities, especially sCS.
Pediatric pyoderma gangrenosum: a retrospective review of clinical features, etiologic associations, and treatment
Schoch JJ, Tolkachjov SN, Cappel JA, et al. Pediatr Dermatol 2017; 34(1): 39–45.
Retrospective review of 13 children with PG. Sulfasalazine or related 5-aminosalicylate drugs were used in 46% of patients, four of whom had complete response (CR) while two had a partial response.
Triamcinolone and pyoderma gangrenosum
Gardner LW, Acker DW. Arch Dermatol 1972; 106: 599–600.
A case of multifocal PG responded to intralesional triamcinolone acetonide 10 mg/mL. Doses ranging from 40 mg to 200 mg were injected at any one time.
The successful use of minocycline in pyoderma gangrenosum – a report of seven cases and review of the literature
Berth-Jones J, Tan SV, Graham-Brown RAC, et al. J Dermatol Treat 1989; 1: 23–5.
Seven cases improved on minocycline dosed at 100 mg BID or 200 mg BID.
Doxycycline and dapsone in chronic pyoderma gangrenosum: revisiting the old therapies
Garg S, Arora D, Singh K, et al. Clin Dermatol Rev 2018; 2: 41–2.
Case report of PG treated with dapsone 100 mg daily and doxycycline 100 mg BID with complete healing.
Successful treatment of pyoderma gangrenosum with topical 0.5% nicotine cream
Patel GK, Rhodes JR, Evans B, et al. J Dermatol Treat 2004; 15: 122–5.
Two cases responded to nicotine in Cetomacrogol cream.
Successful treatment of severe pyoderma gangrenosum with pimecrolimus cream 1%
Bellini V, Simonetti S, Lisi P. J Eur Acad Dermatol Venereol 2008; 22: 113–5.
A patient demonstrated clearance of PG within 8 weeks of using pimecrolimus cream BID.
The treatment of pyoderma gangrenosum with sodium cromoglycate
De Cock KM, Thorne MG. Br J Dermatol 1980; 102: 231–3.
Two cases responded to sodium cromoglycate aqueous solution (2% weight/volume Rynacrom nasal spray).
Safe treatment. Solutions have been applied to lesions in concentrations from 1% to 4%. The solution can be sprayed onto the ulcer or applied on gauze or under occlusion with a hydrocolloid dressing. This drug may act by inhibiting neutrophil migration or cytotoxicity .
Remission of refractory pyoderma gangrenosum, severe acne, and hidradenitis suppurativa (PASH) syndrome using targeted antibiotic therapy in 4 patients
Join-Lambert O, Duchatelet S, Delage M, et al. J Am Acad Dermatol 2015; 73(5 Suppl 1): S66–9.
Four cases of PASH treated with intravenous ceftriaxone and oral metronidazole or intravenous ertapenem for 3–6 weeks, followed by oral therapy combining 2–3 antibiotics (rifampin, moxifloxacin, metronidazole, amoxicillin, and/or linezolid) given in 4–6-week cycles.
Second-Line Therapies ∗
Comparison of the two most commonly used treatments for pyoderma gangrenosum: results of the STOP GAP randomized controlled trial
Ormerod AD, Thomas KS, Craig FE, et al. BMJ 2015; 350: h2956.
In this study with 112 randomized patients, prednisolone 0.75 mg/kg/day was compared with ciclosporin 4 mg/kg/day to a maximum dose of 75 and 400 mg/day, respectively. The treatments did not differ across a range of objective and patient-reported outcomes. At 6 months, 47% of ulcers healed in each group. Infections were more common serious adverse events in the group receiving prednisolone.
Third-Line Therapies
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Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial
Brooklyn TN, Dunnill MG, Shetty A, et al. Gut 2006; 55: 505–9.
Improvement of pyoderma gangrenosum and psoriasis associated with Crohn’s disease with anti-tumor necrosis factor alpha monoclonal antibody
Tan MH, Gordon M, Lebwohl O, et al. Arch Dermatol 2001; 137: 930–3.
Numerous case reports further established response to infliximab.
Treatment of pyoderma gangrenosum with etanercept
McGowan JW 4th, Johnson CA, Lynn A. J Drugs Dermatol 2004; 3: 441–4.
The first of several cases reported to respond to etanercept 25–50 mg twice weekly.
Adalimumab for treatment of pyoderma gangrenosum
Pomerantz RG, Husni ME, Mody E, et al. Br J Dermatol 2007; 157: 1274–5.
Improvement occurred in a case that failed etanercept.
Conventional dosing regimens: 40–80 mg every 2 weeks with and without an initial loading dose of 80 mg.
Pyoderma gangrenosum successfully treated with golimumab: case report and review of the literature
Diotallevi F, Campanati A, Radi G, et al. Dermatol Ther 2019; 32(4): e12928.
Case of ulcerative rectocolitis and PG treated with golimumab, improving both.
Pyoderma gangrenosum. Response to cyclophosphamide therapy
Newell LM, Malkinson FD. Arch Dermatol 1983; 119: 495–7.
A refractory case of PG responded to cyclophosphamide 150 mg daily.
Intravenous cyclophosphamide pulses in the treatment of pyoderma gangrenosum associated with rheumatoid arthritis. Report of 2 cases and review of the literature
Zonana-Nacach A, Jimenez-Balderas FJ, Martinez-Osuna P, et al. J Rheumatol 1994; 21: 1352–6.
Two patients improved on pulsed intravenous cyclophosphamide at doses of 500 mg/m 2 combined with oral corticosteroid. In both cases, CR was subsequently maintained using oral cyclophosphamide 100 mg daily.
Chlorambucil is an effective corticosteroid-sparing agent for recalcitrant pyoderma gangrenosum
Burruss JB, Farmer ER, Callen JP. J Am Acad Dermatol 1996; 35: 720–4.
Chlorambucil, 2–4 mg daily, was successfully used in six cases, both alone and in combination with sCS.
Pyoderma gangrenosum – response to topical nitrogen mustard
Tsele E, Yu RCH, Chu AC. Clin Exp Dermatol 1992; 17: 437–40.
The application of nitrogen mustard 20 mg/100 mL in aqueous solution on gauze swabs proved helpful in a treatment-resistant case associated with immunoglobulin A (IgA) paraproteinemia.
Leukocytapheresis treatment for pyoderma gangrenosum
Fujimoto E, Fujimoto N, Kuroda K, et al. Br J Dermatol 2004; 151: 1090–2.
Efficacy of granulocyte and monocyte adsorption apheresis for three cases of refractory pyoderma gangrenosum
Seishima M, Mizutani Y, Shibuya Y, et al. Ther Apher Dial 2007; 11: 177–82.
Three patients improved after weekly treatments for 10 or 11 weeks.
Intravenous immunoglobulin as adjunct therapy for refractory pyoderma gangrenosum: systematic review of cases and case series
Song H, Lahood N, Mostaghimi A. Br J Dermatol 2018; 178(2): 363–8.
Case series of 49 patients with PG treated with IVIG with CR or partial response (PR) noted in 43 and CR in 26.
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