1. A CASE OF RECURRENT LEG
ULCERS
Dr Rohit K. Singh
2nd yr Resident
(MD Derm, Ven, Leprosy)
Base Hospital, Lucknow
2. Case
• Chandra Bhan Singh, 31 yrs old male, serving soldier
from Etah (U.P)
• DOA - 31St Dec 13
Presenting complaint
- Recurrent ulcers over anterior
aspect of right leg × 16 months
3. History of present illness
• Onset –insidious and progressive
• Started as itching over anterior aspect of right leg
• In a week time patient developed a small ulcer over
the same area
• Ulcer grew rapidly in size and it was associated with
intense pain and pus discharge
• Ulcer healed gradually in about 8 to 10 weeks
duration with scar formation, patient also took some
oral and topical medications for this.
4. History of present illness
• Similar ulcers continued to occur on the right leg and
subsequently healed with scarring.
• Presently patient has developed similar ulcer over
anterior aspect of right leg, 6 weeks back.
5. History of present illness
No h/o
– Oral or topical drugs preceding the ulceration,
fever, joint pain, trauma
– Cough with sputum discharge, photosensitivity
– Recurrent oral ulcers, prolonged standing, pain in
legs
– Symptoms of immunosupression, unprotected
sexual contact or promiscuous behavior
– Irregular bowel movement, blood in stool
– Psychiatric illness
6. History contd..
• Past history
– No h/o hypertension, diabetes mellitus, tuberculosis
• Treatment history
– Oral amoxy-clav
– Oral Rifampicin
– NSAIDS
– Topical antibiotic creams
• Family history – no h/o similar disease in the family
• Personal history
– Married for 9 years with 2 children
– No h/o of alcohol intake
– Non smoker
– Non tobacco chewer
7. General Examination
BMI – 24 kg/msq
Pulse - 76 / min, regular
BP - 120/ 86 mm Hg, T - 98º F
RR - 16 / min
No pallor, icterus, cyanosis, clubbing or
lymphadenopathy
Pedal oedema absent
Peripheral pulses (dorsalis pedis) on both sides are
normally palpable
15. Investigations
• Hb – 13.9 gm%
• TLC – 6900/cumm
• DLC- N 66%, L 25%, M 03%, E 03%
• ESR – 18 mm fall in 1st hr
• Platelet count – 1.8 lacs
• LFT – Serum bilirubin- 0.6 mg/dl
- SGOT – 21 U/l
- SGPT- 36 U/l
Renal functions-
-Blood urea- 34mg/dl
-Serum Creatinine- 0.8 mg/dl
Blood sugar F – 95 mg/dl
PP – 110 mg /dl
Urine RE – NAD
Pus culture and sensitivity – no growth after 48 hrs of incubation
16. Investigations
• PBS - NAD
• HBsAg – negative
• Anti HCV Ab – negative
• ANA – negative
• Rheumatoid factor - negative
• VDRL – non reactor
• Pathergy test - negative
• Doppler study of limbs – NAD
• USG abdomen – normal scan
• Chest X ray - NAD
• G6PD – non reactor
17. Investigations
• Colonoscopy – NAD
• Skin Biopsy – Pyoderma gangrenosum
• Biopsy number – B/27/14 , CH ( CC), LKO.
• Epidermis – mild acanthosis
• Dermis - dense neutrophilic inflammatory cell
infiltrate and fibrinous exudate with mild
perivascular infiltrate
• Tubercular / atypical mycobacterial granulomas not
seen
20. Final diagnosis
Rapid progression of the lesion with pain out of
proportion to the size of ulcer
Exclusion of other causes of ulcer
Presence of cribriform scarring
Appropriate histological findings
Final diagnosis
Pyoderma gangrenosum
34. Pyoderma gangrenosum
• Def: a rare, non – infectious neutrophilic dermatosis
commonly associated with underlying systemic
disease.
– Cutaneous manifestation of systemic disease
– Brocq first described PG in 1916
– Later described by Brunsting et al in 1930
– Females > males
– B/w 40 and 60 years
35. Aetiology and pathogenesis
• Immune – mediated process
(i) Humoral defects
– Autoantibodies againt skin and bowel
– Arthrus / Schwartzmann reactions
– Cross-reactivity b/w bacterial and cut. antigens
with E.coli
– IL-8
36. (ii) Cell – mediated defects
– Cutaneous anergy to candida, streptokinase, PPD
– Altered production of macrophage inhibition factor by
lymphocytes
– Association b/t IBD and arthritides
– Clonal restriction of T- cells and neutrophils
• Vasculitis
– C-ANCA OR P-ANCA
– Drug associated like thiouracils
• Smoking
• Familial associations
– PAPA(pyogenic arthritis, PG, acne)
37. Clinical
manifestations
PROGRESS
Deep painful subcutaneous
nodule/pustule formation
Ulcer formation (may be > 30cm)
•Defined violaceous
borders
•Undermined edges
•Granulation tissue
•Surrounding zone of erythema
•Purulent exudate
Classical /ulcerative variant
38. Other features
Solitary or multiples
Heals with atrophic
cribriform scarring
Most commonly at
legs (extensor aspect)
Other sites
•Upper limbs
•Face
•Trunk
•Mucosal(oral &
genital)
•Sites of cutaneous
trauma(pathergy
phenomenon)
•Site of surgery
39. Clinical manifestations
• Systemic features
– Fever
– Myalgia
– Malaise
– Arthralgia
• Frequent disease
associations
– Inflammatory bowel
disease(IBD)
– Arthritis
– Monoclonal
gammopathy
– Internal malignancy
• Over 70 % have one of
these
44. Diagnosis of PG
1. Typical clinical
features
2. Exclusion of other
cutaneous ulceration
diseases
Criteria for diagnosis of Pyoderma gangrenosum
(classical/ulcerative type)
45. Diagnostic criteria
Major criteria (both required)
1. Rapid (usually > 1 cm/day) progression of painful, necrolytic ulceration with an
irregular, undermined, violaceous border – usually with a preceding papule, pustule or
bulla and pain out of proportion to the size of the ulcerations
2. Exclusion of other causes of ulcerations
Minor criteria ( at least two required)
1. (a) History of pathergy
(b) Presence of cribriform scarring
2. Presence of disease known to associated ( IBD, polyarthritis, myelodysplasia or
leukemia, but monoclonal gammopathy should be added to this list; over all about
50% have an systemic associations
3. Appropiate histological findings
4. Rapid ( at least 50% reduction in size) with oral steroid (1-2mg/day)
46. Differential diagnosis
Stasis ulcer
Stasis ulcer
with visible
varicosity
Cutaneous PAN
Ischemic ulcer
Diabetic ulcer
49. Investigations
• Routine blood investigations including ESR
• Australia antigen, anti HCV antibodies
• Antibody screening : ANA / ANCA
• Rheumatoid factor
• Serum protein electrophoresis
• Swab for culture
• ASO titre
• Mantoux test
• Coagulation screening
• VDRL
• ELISA screening for HIV
50. • Endoscopy ( upper and lower GI)
• Vascular studies
• Bone marrow aspirate examination
• USG abdomen ( including liver/spleen/lymph nodes)
• Chest X ray
• CT scan of thorax, abdomen, brain
• Skin biopsy
51. Treatments: systemic
MEDICATION DOSAGE
PREDNISOLONE 0.5 – 1.5 mg/kg/day PO
METHYLPREDNISOLONE ( PULSE DOSE) 500 mg – 1 gm IV
DAPSONE 50 – 100 mg / day PO
CLOFAZIMINE 200 – 400 mg / day PO
COLCHICINE 0.3 – 1 mg /day PO
MINOCYCLINE 50 – 100 mg bid PO
CYCLOSPORIN 3 – 5 mg/kg/day PO
TACROLIMUS 0.1 0.3 mg/kg PO
MYCOPHENOLATE MOFETIL 500 mg – 1 gm bid PO
INFLIXIMAB 5 mg / kg IV
54. Prognosis
Poor prognostic factors
• PG variant (ulcerative PG)
• Age (> 65 yrs)
• Sex ( male)
• Presence of systemic disease
• Type, dosage and duration of therapy
• PG have a significant risk of relapse, so long term
follow- up is required.
55. Conclusion
– When leg ulcers fail to respond to treatment or
heal in an orderly and timely manner, clinicians
should be prompted to conduct further diagnostic
investigations, refer to specialists as indicated and
ensure a multidisciplinary approach.
56. References
• Rook’s book of dermatology 8th edition.
• Fitzpatrick book of dermatology.
• Indian association of Dermatologist, Venerologist,
and Leprologist textbook of leprosy.
• Lever’ histopathology of the skin.
• IADVL journal.
• JAMA Dermatology journal.