Contents
Uncommon, ulcerative cutaneous condition of uncertain etiology
- #2 M/C cutaneous manifestation of IBD (1–3%)
Aetiology
Associated conditions:
- Inflammatory bowel disease (M/C cause):
- Ulcerative colitis
- Crohn’s disease
- Arthritides:
- Rheumatoid arthritis
- Seronegative arthritis
- Hematological disease:
- Myelocytic leukaemia
- Hairy cell leukaemia
- Myelofibrosis
- Myeloid metaplasia
- Monoclonal gammopathy
- Autoinflammatory disease:
- Pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome)
Clinical features
Primary types:
- Typical (ulcerative) form
- Mainly in the legs
- Atypical form
- More superficial and occurs in the hands and other parts of the body
Other variations:
- Peristomal pyoderma gangrenosum (15% cases)
- Bullous pyoderma gangrenosum
- Pustular pyoderma gangrenosum
- Vegetative pyoderma gangrenosum
Diagnosis
Diagnostic criteria
Biopsy demonstrating a neutrophilic infiltrate + ≥ 4 minor criteria:.
- Histology: Exclusion of infection (including histologically indicated stains and tissue cultures)
- Pathergy (ulcer occurring at sites of trauma, with ulcer extending past area of trauma)
- Personal history of inflammatory bowel disease or inflammatory arthritis
- History of papule, pustule, or vesicle that rapidly ulcerated
- Clinical examination (or photographic evidence) of peripheral erythema, undermining border, and tenderness at site of ulceration
- Multiple ulcerations (at least 1 occurring on an anterior lower leg)
- Cribriform or “wrinkled paper” scar(s) at sites of healed ulcers
- Decrease in ulcer size within 1 mo of initiating immunosuppressive medication(s)
Management
First-line therapy:
- Systemic treatment: Corticosteroids + ciclosporin
- Topical treatment: Clobetasol/mupirocin/gentamicin + tacrolimus
If ineffective, alternative therapeutic procedures:
- Systemic treatment:
- Corticosteroids + mycophenolate mofetil
- Mycophenolate mofetil + ciclosporin
- Tacrolimus
- Thalidomide
- Infliximab
- Plasmapheresis