Pyoderma Gangrenosum

History Taking

Chief Complaint:

• The patient may present with a painful skin ulcer or multiple ulcers.

• Complaints of rapidly expanding skin sores.

History of Presenting Complaint:

• Initially may have noticed a pustule or a small bump, that quickly became a painful ulcer.

• The ulcer has undermined borders and is deep, with a purple-blue color around the edges.

• There may be a history of minor trauma before the appearance of the ulcer, known as pathergy.

• The sores may have started small but expanded rapidly over a few days to a few weeks.

• Ulcers may have a bloody or purulent discharge.

• The patient may describe the ulcer as very painful.

System Review:

• May report symptoms suggestive of systemic diseases that are associated with PG, such as abdominal pain or bloody diarrhea (if Inflammatory Bowel Disease is present), joint pain or stiffness (if Rheumatoid Arthritis is present), or fatigue and malaise.

• Fever or general malaise.

Past Medical History:

• History of auto-immune or inflammatory disorders such as Inflammatory Bowel Disease (Crohn’s disease or Ulcerative Colitis), Arthritis (Rheumatoid Arthritis), or Hematological diseases (Leukemia, Myeloma).

• History of unexplained skin ulcers or skin disorders.

Drug History:

• Recent use of medications, such as biologics used for treating autoimmune conditions, that could potentially trigger PG.

• History of prior or ongoing treatment for PG such as corticosteroids, cyclosporine, or other immunosuppressants.

Family History:

• A positive family history of autoimmune or inflammatory disorders could be present but is not a defining feature of the disease.

Social History:

• No specific social history elements are typically related to PG, but the impact of the disease on the patient's lifestyle, work, and social interactions could be noted.

Personal History:

• Stressful events or trauma, as these have been known to possibly trigger PG in predisposed individuals.

Significant psychological impact due to disease chronicity, recurrent painful ulcers, or disfigurement.

Physical Examination

General Examination:

• General appearance may show signs of discomfort or distress due to pain.

• Skin examination may reveal one or multiple deep, necrotic ulcers with a violaceous border, most commonly found on the legs but could be anywhere on the body.

• Pathergy might be visible, where trauma sites display new ulcers.

• Inspection of the hands, arms, face, and neck may reveal lesions if present there.

Examination of the Eyes:

• Uveitis or episcleritis may be present in association with underlying systemic disease like Inflammatory Bowel Disease.

Examination of the Mouth:

• Aphthous ulcers could be present, especially if there's an underlying condition like Inflammatory Bowel Disease.

Cardiac Examination:

• Not typically relevant unless patient has a co-existing condition that impacts the heart.

Respiratory Examination:

• Not typically relevant unless patient has a co-existing condition that impacts the lungs.

Abdominal Examination:

• Examination for signs of underlying systemic diseases such as Inflammatory Bowel Disease (e.g. tenderness, distension, peristaltic rushes) or liver disease (e.g. hepatosplenomegaly, ascites).

Neurological Examination:

• Not typically relevant unless the patient has a co-existing condition that impacts the nervous system.

Additional Examination:

• Joint examination could be relevant if there's a co-existing arthritic condition such as Rheumatoid Arthritis. Look for signs of joint swelling, deformity, tenderness, or reduced range of motion.

• Any signs of Septicemia if the ulcer becomes secondarily infected (e.g. fever, tachycardia).

Investigations

Laboratory Investigations:

• Full Blood Count: To check for signs of anemia or raised white cell count indicating inflammation or infection.

• Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP): Can be elevated in inflammation and infection.

• Liver and renal function tests: Baseline investigation and also to rule out any associated systemic diseases.

• Autoimmune screen: Including Rheumatoid factor (RF), Anti-Cyclic Citrullinated Peptide (Anti-CCP) for rheumatoid arthritis, and Anti-Neutrophil Cytoplasmic Antibodies (ANCA) if vasculitis is suspected.

• Fecal calprotectin: If Inflammatory Bowel Disease (IBD) is suspected.

• Blood cultures: If systemic infection or sepsis is suspected.

Imaging Studies:

• X-ray of affected areas: To rule out osteomyelitis if ulcers are deep and overlying bony areas.

• Chest X-ray: If there are respiratory symptoms or signs of systemic infection.

Invasive Investigations:

• Skin biopsy: Important to confirm the diagnosis and to exclude other causes of ulcers such as vasculitis, malignancy, or infection.

Other Tests:

• Direct immunofluorescence: Rarely used but can help differentiate PG from other causes of ulceration.

• Colonoscopy or Gastroscopy: If IBD is suspected.

Management

General Management:

• Education: Explain the nature of the condition to the patient.

• Pain Management: Analgesics to manage the significant pain often associated with the ulcers.

• Wound Care: Regular cleaning and dressing of the ulcers to prevent secondary infection.

Medical Management:

• Topical corticosteroids: Initial therapy for mild, localized disease.

• Systemic corticosteroids: If disease is extensive or rapidly progressive.

• Cyclosporine: Used in combination with corticosteroids in severe cases.

• Infliximab or Adalimumab: Can be used if other treatments are ineffective, particularly in patients with associated IBD.

• Other immunosuppressive agents: Such as Azathioprine, Methotrexate, or Mycophenolate mofetil may be used if initial treatments are ineffective or contraindicated.

• Antibiotics: If secondary bacterial infection is present.

• Treat underlying disease: If associated with systemic disease like IBD or Rheumatoid Arthritis, specific treatment for that condition is necessary.

Surgical Management:

• Debridement: To remove necrotic tissue, but it is avoided if possible due to risk of pathergy.

• Skin grafting or Flaps: In case of large, non-healing ulcers, or after the disease is controlled.

• Surgical intervention: To treat underlying diseases such as resection in case of IBD.

Other Management:

• Psychological Support: Due to the chronic nature of the disease and impact on the quality of life.

• Multidisciplinary Team: Involvement including Dermatologists, Rheumatologists, Surgeons, and Wound Care Nurses.