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Pyoderma gangrenosum

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 Review Article

Pyoderma Gangrenosum
Renu Saigal***, Yadvinder Singh**, Manoop Mittal**, Amit Kansal**, Hari Ram Maharia**
Abstract
Pyoderma gangrenosum (PG) is an idiopathic, ulcerative, noninfective chronic inflammatory skin disorder
of unknown etiology. It is associated with systemic medical illness in 50% of cases like inflammatory bowel
disease, systemic arthritis, haematological diseases and malignancies. Characteristic lesions begin as pustule or
vesiculopustule and progresses to an ulcer or deep erosion with violaceous overhanging or undermined borders.
Diagnosis of pyoderma gangrenosum is clinical and depends on exclusion of other causes of cutaneous
ulceration. The management of PG is treatment of underlying systemic medical illness and judicious use of
immunosuppressants. Association of PG with these medical illnesses and treatment with immunosuppressants
make the clinical utility for internists, gastroenterologists, haematologists and rheumatologists.

Introduction most commonly presents typically as a tender pustule of

essentially non-infectious aetiology that evolves to an enlarging
P yoderma gangrenosum (PG) was first described in 1930 by
Brusting and colleagues.1 PG is an idiopathic, ulcerative,
chronic inflammatory skin disease of uncertain etiology. PG is
necrotic suppurative ulcer after gangrenous changes into the
overlying skin. The lesions tend to endure, lasting months to
years, and heal with cribriform scarring.
more frequent in adults but children may be affected on rare
occasion.2 Cutaneous lesions are characteristic as PG begin as Diagnosis is clinical and depends on the exclusion of other
pustule or vesiculopustule and progresses to an ulcer or deep causes of cutaneous ulceration. No specific pathologic or
erosion with violaceous overhanging or undermined borders.3 laboratory findings exist. Concurrent systemic disease occurs in
Lesions are most commonly found on the lower legs, but they 50% of affected patients (Table 1). 4 Remaining cases are thought
may occur on the abdomen, thighs, buttocks, chest, head, neck, to be idiopathic or autoimmune.5 The management of this
and anywhere on the skin. disorder begins with treatment of underlying disease and local
or systemic glucocorticoids and immunomodulators.
“Pyoderma gangrenosum” term was given because of
characteristic appearance of the lesion. The cutaneous lesion Actually PG is generally associated with other medical
illnesses like rheumatoid arthritis, inflammatory bowel
Table 1 : Associated systemic diseases disease, leukaemia etc. Therefore, early diagnosis of PG is
1. Inflammatory bowel disease 15% mandatory because this condition is usually misdiagnosed and
2. Rheumatoid arthritis and systemic lupus erythematosus (37%) underdiagnosed by physicians as well as surgeons as these cases
3. Immune abnormalities: are also referred to them as non-healing ulcers.
Humoral- Moreover, surgical intervention may lead to deterioration of
Congenital and acquired hypogammaglobulinemia
natural history of PG because of pathergy reaction. Cases of PG
Selective and complete hyperimmunoglobulin E syndrome
Cell Mediated- are erroneously referred to surgeons but surgical intervention
Immunodeficient/ Immunosuppressed leads to more harm due to pathergy phenomenon and this entity
Congenital deficiency in leukocyte adherence glycoprotein should be treated by immunosuppressants.
Defective neutrophil function
4. Hematologic Diseases- Pathophysiology
Acute and chronic myeloid leukaemia
Multiple myeloma The pathophysiology of PG is poorly understood, but
Monoclonal gammopathy dysregulation of immune system, especially altered neutrophil
Waldenstrom’s macroglobulinemia chemotaxis is the primary process.6 The complete connection
Lymphoma-Hodgkin's lymphoma, non-Hodgkin’s lymphoma between neutrophil and tumor necrosis factor (TNF) has yet
Polycythemia Vera to be established, however, evidence of enhanced neutrophil
Large granular Lymphocytic leukaemia activation due to TNF-α has been uncovered.7 TNF-α increases
Myelofibrosis neutrophilic infiltration by-
5. Liver Diseases:
Chronic active hepatitis i. Upregulating expression of adhesion molecules namely
Cryoglobulin and hepatitis C intracellular adhesion molecule-1 (ICAM-1), vascular cell
Primary biliary cirrhosis adhesion molecule-1 (VCAM-1) and E-selectin.
6 Solid tumours like colon, bladder, prostate, breast, bronchus, ii. Increasing levels of interleukin-8.
ovary, adrenocortical carcinoma
iii. Increases synthesis of granulocyte colony stimulating factor.
7. Drugs – Alpha 2-b Interferon .
8. Miscellaneous –Thyroid diseases, sarcoid, diabetes mellitus, HIV, Pathergy phenomenon i.e. development of skin lesion at the
COPD site of injury, is a common feature of PG and occurs in 30% of
patients.
Incharge Rheumatology Clinic, Prof. & Unit Head, **Resident,
***
Clinical Variants: To aid understanding of clinical variants
Department of Medicine, Sawai Man Singh Medical College and
Hospital, Jaipur, India. of PG, lesions have been classified into 4 categories:9
Received: 16.01.2009; Revised: 18.06.2009; Accepted: 20.06.2009 A. Ulcerative B. Pustular

378 © JAPI • june 2010 • VOL. 58

C. Bullous D. Vegetative
A. Ulcerative (Typical or Classic) PG
This is the typical form of PG. Lesions are most commonly
located on lower limbs but may occur anywhere. Lesions
often begin as pustules, but can also be pathergic in
etiology. A large ulcer with a well defined, undermined
border (Fig. 1) surrounded by a halo of erythema is usually
formed within a few days at the site of minor trauma.1
There is associated pain often severe and out of proportion
to size of lesion. Ulcerative PG is often associated with
arthritis, inflammatory bowel disease and monoclonal
gammopathies.9 It has been suggested that the ulcerations
in patients who have classic PG associated with arthritis are
more refractory to treatment than in patients who have PG
without associated arthritis.10
B. Pustular PG
Pustular lesions are initiating feature of ulcerative PG as
discussed above. Although not all pustules progress to
ulceration and in some patients multiple painful eruptive
inflammatory cutaneous pustules develop without
subsequent ulceration. This distinctive eruption has been
reported almost exclusively in the setting of acute IBD, which
often clear with control of bowel disease. Pyostomatitis
vegetans, a 2-3mm pustule which may ulcerate is found
on oral mucosa, is a pustular variant of PG. Oedematous
mucosa makes deep folds called “snail track” appearance.9
Fig. 1 : Ulcerative pyoderma gangrenosum
(a) (b)
Fig. 3 : Post surgical pyoderma gangrenosum (a) Before Treatment (b) After treatment with oral prednisolone and mesh-graft transplantation.39
© JAPI • june 2010 • VOL. 58 379
C. Bullous (Atypical) PG
Characteristic feature of atypical PG is that they begin as
bullae and are superficial. Patients with this type of PG
develop painful rapidly enlarging bullous lesion which
becomes superficially erosive and then ulcerative, especially
on the dorsum of the hands (Fig. 2). This variant is also
termed as “Neutrophilic dermatoses of dorsal hands”.
It is associated with hematological diseases especially
myelogenous leukemia, myelodysplastic disorders,
refractory anemia and IgA paraproteinemia.11 The presence
of lesions is an indicator that the patient with leukemia has
a poor prognosis. This condition may be confused with
sweet’s disease, acute febrile neutrophilic dermatoses. It
has been proposed that atypical PG and sweet’s disease are
points on a continuum.12
D. Vegetative PG
Vegetative PG, also known as superficial granulomatous
pyoderma, presents as chronic, nonprogressive, superficial
ulcer that is not painful and often lacks a violaceous
undermined border.9 It is often a solitary lesion, especially
on trunk, that is uncommonly associated with systemic
diseases. Histologically histiocytes are prominent within the
neutrophilic infiltrates. This variant has a good prognosis.
It responds to simple modes of treatment.
E. Other variants
Peristomal PG
This variant characterized by ulcerations resembling classic
PG in a peristomal (ileostomy or colostomy) location.13 It
occurs most often in patients who have IBD particularly
Fig. 2 : Bullous pyoderma gangrenosum
 Table 2 : Differential diagnosis of PG
1. Infections Herpes simplex, impetigo, ecthyma
gangrenosum, cutaneous tuberculosis,
deep fungal infections
2. Vasculitis Polyarteritis nodosa, Wegener’s
granulomatosis, mixed cryoglobulinemia
3. Thrombophilic states Livedoid vasculitis, antiphospholipid
syndrome, factor V Leiden mutation
4. Venous insufficiency
5. Malignancies Squamous cell carcinoma, cutaneous
lymphoma, metastatic carcinoma
6. Ext. of underlying IBD
7. Factitious
8. Insect bite
9. Drugs Isotretinoin, granulocyte colony
stimulating factor, iodine and bromide
overdosage, alpha 2b-interferon17
Table 3 : Criteria (including clinical characteristics) for
diagnosis of Pyoderma Gangrenosum36
Major criteria
1. Rapida progression of a painfulb necrolytic cutaneous ulcerc with
an irregular, violaceous, and undermined border
2. Exclusion of other causes of cutaneous ulceration
Minor criteria
1. History suggestive of pathergyd or clinical finding of cribriform
scarring
2. Systemic diseases associated with PGe
3. Histopathologic findings (sterile dermal neutrophilia ± mixed
inflammation ± lymphocytic vasculitis)
4. Treatment response (rapid response to systemic glucocorticoid
treatment)f
a
Characteristic margin expansion of 1 to 2 cm/d, or a 50% increase in
ulcer size within 1 month; bPain is usually out of proportion to the size
of the ulceration; cTypically preceded by a papule, pustule, or bulla;
d
Ulcer development at sites of minor cutaneous injury; eInflammatory
bowel disease, polyarthritis, myelocytic leukemia, or preleukemia;
f
Generally responds to a dosage of 1 to 2 mg/kg/d, with a 50% decrease
in size within 1 month
Crohn’s disease.
Post surgical PG (PSPG):
PSPG represents a specific entity which shows particular
clinical presentation and evolution.14 The onset of PSPG
follows a sequence. After an apparently normal evolution of
scar formation following surgical procedure such as breast
surgery, the scar presents with many small dehiscences,
which will progressively coalesce to form larger areas
of wound ulceration, with no visible granulation tissue
(Fig. 3).
Genital
Typical lesions of PG occurs on vulva, penis and scrotum.
Behcet’s disease is a close mimicker.
Extracutaneous Disease
Extracutaneous neutrophilic infiltration may complicate
the course of PG. These lesions do not contain pathogenic
microorganisms and thus have been termed sterile
neutrophilic abscesses. The most common extracutaneous
involvement is in the lungs,15 where it can present as culture
negative pulmonary infiltrates. Other sites of involvement
include bones (Multifocal sterile recurrent osteomyelitis),16
cornea, liver, spleen, heart, skeletal muscle and the central
nervous system. All of these manifestations are rare.
380 © JAPI • june 2010 • VOL. 58
Table 4 : Evaluation of Possible PG
1. Detailed history including drugs, trauma, insect bite, detailed
systemic review*
2. General physical examination*
3. PG lesion – location, type, size, outline, depth*
4. Blood tests – CBC, ESR*
Renal*/ liver profile including hepatitis markers*/ bone profile*
Urine analysis*
Blood cultures*
Serum urine and protein electrophoresis**, cryoglobulins**
Rheumatoid factor**, Antinuclear antibody**
Antineutrophilic cytoplasmic antibody**,
Syphilis serology**
Antiphospholipid antibody**
5. Skin biopsy (histology with stain for organisms + culture)*
Ulcer cultures*
Chest X-ray*
6. Special investigations – CT scan** (if deep or multiorgan
neutrophilic abscesses suspected)
Endoscopies (flexible sigmoidoscopy and/or colonoscopy)**
Bone marrow aspiration and biopsy**
Angiographic or Doppler studies**
*
Mandatory in all patients of pyoderma gangrenosum; **Investigations
required as dictated by type of ulcer (see clinical variants) and other
systemic symptomatology suggestive of associated disease.
Timing and Course with Associated
Diseases
Cases associated with an internal malignancy do not
demonstrate unique histopathologic findings compared
with other cases of pyoderma gangrenosum. Treatment of an
associated malignancy may or may not lead to improvement of
pyoderma gangrenosum. there are no reliable clinical features
to predict which cases have a paraneoplastic association.
The mean duration of chronic ulcerative colitis before the
appearance of PG is 10 years.37 The lesions generally appear
during the course of active bowel disease, but they also occur in
inactive colitis or less severe disease and may not appear until
after colectomy. Pyoderma resolved without intestinal resection
in two thirds of patients. Healing after intestinal resection is
unpredictable regarding both timing and extent of resection.38
Differential Diagnosis
The diagnosis of PG involves the exclusion of other diseases
that cause erosive or ulcerative skin lesions. Differential
diagnosis of PG includes: (Table 2)
Evaluation of Possible PG
The diagnosis of PG is established by consideration of clinical
features, review of histopathologic findings and with the benefit
of negative culture results and other investigations (Table 4),
undertaken as appropriate in the individual patients. Blood and
other investigations are carried out as indicated by the history
and examination. Investigations are done to diagnose or exclude
the presence of an associated systemic disease.
As described above, the diagnosis of PG is clinical and
depends on exclusion of other causes of cutaneous ulcerations.
Various diagnostic criteria have been proposed for PG, one of
them given by Su WPD, Davis MD et al36 is shown in Table
3. It includes characteristic clinical features of pyoderma
gangrenosum, helpful in diagnosis. It has been recommended
to help guide clinical evaluation and avoid misdiagnosis.
Table 4 shows systematic workup of a patient that comes
 Table 5 : Multistep approach to therapy for pyoderma with cutaneous ulceration having characteristic features of PG.
gangrenosum After detailed history, general physical examination and local
examination, a biopsy of the affected area (Elliptical incisional
Therapy
biopsy preferable to punch biopsy) for routine processing and
Initial therapy Corticosteroids
special stains for microorganisms should be performed in almost
Incomplete response to
all patients. Laboratory evaluation should include a complete
corticosteroids
I. First choice Corticosteroids + cyclosporine A
blood count, chemistry profile, hepatitis panel and urinalysis.
Corticosteroids + azathioprine Patients who have peristomal PG should be evaluated
II. Second choice Mycofenolate mofetil carefully for active bowel disease or malignancy if there is a
Dapsone previous history of gastrointestinal malignancy. Serum and/
Chlorambucil or urine protein electrophoresis, peripheral smear, and bone
Sulfasalazine marrow aspiration should be performed if indicated to evaluate
Minocycline for hematologic malignancies. Colonoscopy or other tests to
III. For recalcitrant PG Cyclophosphamide exclude associated inflammatory bowel disease or ulcerative
Infliximab colitis may be useful in patients with symptoms. The evaluation
Thalidomide
of patients with pyoderma gangrenosum and no symptoms of
Tacrolimus
Table 6 : Systemic therapeutic options for pyoderma gangrenosum
Drug Dose schedule Facts
Oral steroids Prednisone 0.5-1 mg/kg/d • Agent of choice
• High doses until lesion is healed,
followed by low-dose maintenance along
with steroid sparing agents to prevent
recurrence
• Long-term risk for infections, insulin
resistance, HTN, glaucoma, and adrenal
suppression
Intravenous pulsed steroids Methyl prednisone 1 g daily in 5% dextrose for • Fewer side effects than oral
1-5 days • Can cause cardiac arrhythmias, sudden
death and anaphylaxis
• Refractory cases only
Cyclosporine 4–5 mg/kg/d • Most effective steroid sparing agent
• Risk of Hypertension, renal toxicity,
hypertriglyceridemia, hypertrichosis
Azathioprine 1-2 mg/kg/d (50-200 mg) • Check thiopurine methyltransferase levels
to prevent toxicity in patients lacking the
enzyme
• Slow onset of action, Bone marrow
suppression
Cyclophosphamide 1.5–3.0 mg/kg/d (oral) • Hemorrhagic cystitis(Good hydration
IV Pulse-500 mg /2 wks or 1000 mg/month before and during therapy), azoospermia
Dapsone 50–200 mg/d • Slow onset of action
• Risk of methemoglobinemia, anemia,
neuropathy
Methotrexate 10-30 mg/wk • Risk of hepatotoxicity, bone marrow
suppression
Mycophenolate mofetil 2-3 g/d • Steroid sparing agent, slow onset of action
Thalidomide 50-200 mg/d • Steroid sparing agent. Used in penile PG.
• Risk of birth defects, neuropathy,
coagulopathy
Etanercept 50-100 mg SC qwk • Soluble p75 TNF receptor fusion protein
(sTNFR-Ig). Inhibits TNF binding to cell
surface receptors
• Risk of pathergy reaction, infections at
injection site pain, rare cases of lupus like
symptoms and heart failure
Infliximab 3 mg/kg IV infusion • For recalcitrant PG
Adalimumab 40 mg SC q wk • For recalcitrant PG
Tacrolimus 0.15 mg/kg twice daily • Macrolide antibiotic with
immunosuppressant property
• Toxicity- Nephrotoxic, Diabetes Mellitus &
Neurotoxicity
Plasmapheresis • Effective in isolated cases, used alone or in
combination with other modalities
IVIG, hyperbaric O2, radiation, electron beam • Effective in isolated cases
irradiation

© JAPI • june 2010 • VOL. 58 381

bowel disease is still uncertain.
Histological Findings
Histopathological findings in PG are not specific. However
a biopsy is suggested in all instances to exclude other
diseases. Biopsy should not be deferred because of concerns
of pathergy. Microscopic features include extensive sterile
dermal neutrophilia. In early cases mixed cell infiltrations may
be present. Although vascular inflammation in lesions of PG
is not uncommon, the histology is not that of true vasculitis
(leukocytoclastic vasculitis).9 Granuloma formation is generally
believed to be incompatible with the diagnosis of PG.3
Management
Management of PG is divided into local wound care, topical
therapy and systemic therapy.18,19
Local Wound Care
Local care includes moist dressings (such as hydrocolloid,
allograft, Vaseline gauze) to relieve pain, promote re-
epithelialization and prevent trauma.9 Bioengineered skin
dressings may be beneficial in covering ulcers and preventing
the need for skin grafts.20
Topical Therapy
Topical therapy is helpful in some cases and successful
use of cromolyn sodium, potent topical glucocorticoids, 21
tacrolimus,22,23 pimecrolimus, nicotine24 or hyperbaric oxygen
has been used in individual patients. Intralesional injections of
glucocoticoids, cyclosporine or tacrolimus are alternative local
therapies, although most useful in early ulcers or there are small
isolated ulcers.
Systemic Therapy
Systemic treatment may be required for more severe cases or
those refractory to local treatment (Tables 5, 6). Systemic therapies
for PG have include glucocorticoids, azathioprine, cyclosporine,25
tacrolimus, cyclophosphamide, 26 mycophenolate mofetil, 27
methotrexate,28 thalidomide,29 and dapsone, minocycline or
sulfapyridine.
Among these systemic modalities, high dose corticosteroids
are usually first line therapy. Steroid sparing agents should
be considered early in the course of PG,18 because of various
toxicities (Table 6). Cyclosporine is considered as most
effective steroid sparing agent. In steroid resistant cases of
PG, combination of corticosteroids with cyclosporine or
azathioprine are considered as first choice therapy on the
basis of clinical experiences though there are currently no
general recommendations for the management of PG. Another
alternatives are mycophenolate mofetil, dapsone, sulfasalazine,
cyclophosphamide and biologics. Pulse methylprednisolone,
pulse cyclophosphamide and intravenous immunoglobulin30
have shown benefits in some patients. Doses and salient features
of these therapies are explained in Table 6.
Biologics
TNF-α inhibitors (infliximab, adalimumab and etanercept)
are showing promise in the treatment of PG.31-35 Infliximab is
preferred TNF-α inhibitor because of risk of pathergy along with
subcutaneous injection of etanercept and adalimumab.
Infliximab is administered as 3-5 mg/kg iv infusion every
1-3 weekly. Important considerations and side effects regarding
infliximab therapy are reactivation of latent tuberculosis and
other infections, demyelinating diseases, lymphomas, congestive
382 © JAPI • june 2010 • VOL. 58
heart failure and lupus like illness. The pain and swelling
decreased within first 4-5 days and complete healing was
achieved within 1-3 months.
Surgical Intervention
Surgical intervention may be harmful because there is risk of
pathergy. When extensive necrosis of the skin is present or vital
tissues, such as tendons and ligaments, are exposed at the ulcer
bed, debridement and skin grafting may be necessary. In such
cases, concomitant systemic therapy with steroids is required to
halt the inflammatory process. Cultured keratinocyte autografts
are shown to be effective.
Course & Prognosis
The prognosis of PG for most patients is good. Many patients
who have PG develop a single episode i.e. uniphasic resolves
with a short course of therapy. Patients having chronic or
relapsing form of PG often require long term therapy and are
associated with chronic diseases. They may require combination
therapy and regular follow up for close monitoring for toxicities
from therapy. A recent series of 41 patients demonstrated that
50% of patients required long-term therapy to avoid recurrence.4
Some patients demonstrate pathergy, and, in such instances,
protection of skin from trauma may prevent a recurrence.
Patients who present with atypical PG need regular follow-up
to evaluate for the development of myeloproliferative disorders.
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