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Hunting for a Diagnosis

WD – 14 / 15

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Introduction.

Mr. H, 94 year old farmer from Thalgampala. Father of eight children. Never had Diabetes, Hypertension and Asthma in the

past. No history of prolonged previous hospital admissions. Non smoker. Has consumed alcohol occasionally in the past.

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Reason for admission,

Painful blackening of bilateral finger tips. Onset – insidious Progressive over a three weeks. Involved bilateral 2nd, 3rd, 5th finger tips on arrival.

bilateral toes not involved. No history suggestive of Raynaud's phenomenon No history of rashes elsewhere.

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Review of systems

No history of intermittent claudication No history of fever at any point of illness No Cough, shortness of breath No haematuria No history of abdominal pain, especially after meals There was no fatigue, recurrent epistaxis, sinus pain,

hearing loss, and arthralgias. No history of longstanding joint pain, morning stiffness,

or joint deformities No history of snake bite

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Contd,

No history of previous blood transfusions. No history suggestive MI, TIA in the past. No recent weight loss, good appetite. No history of prolonged consumption of

medications

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Examination Findings Bilateral symmetrical peripheral gangrene involving finger

tips, feet spared. Peripheral pulses - palpable BP – 130/80 mmHg in both arms. No murmurs were audible. No rashes No pallor, icterus, lymphadenopathy or organomegaly. No abdominal masses. No joint deformities or nodules. No peripheral or CNS involvement.` Fundus : no retinal infarction or thrombosis.

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• Problem list:

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Problem list:

Bilateral symmetrical peripheral gangrene involving finger tips, sparing feet at extreme of age in a previously healthy man.

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• Differential diagnosis?

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Differential diagnosis:

Possibilities include,1. Vasculitis

2. Recurrent small thromboembolic disease3. Infective endocarditis4. Manifestation of a Paraneoplastic syndrome5. Hyperviscosity syndrome

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Investigations:

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Inflammatory markers

ESR – 110 mm 1st hour.

CRP – 30 mg/dl

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Urine ward test for albumin – negative

UFR – No RBC or casts.

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FBC

Hb – 11.4 mg/dl

WBC – 8400 Cumm3

RBC – 3.37 x 105

Plt - 157000 Cumm3

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Blood Picture

Normocytic normochromic anaemia WBC – no left shift or toxic granules Platelet within normal range. No fragmented RBC

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Direct and indirect Coomb’s test negative

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Blood cultures were repeatedly normal.

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Rheumatoid factor – positive

ANA - negative

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VDRL – Negative.

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Mantoux test negative

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Cryoglobulin assay

Not detected after 72 hrs.

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Hep. B surface antigen – negative

Hep. C antibody - negative

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Coagulation profile

Normal.

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Urine BJP – negative.

Skeletal survey – no lytic lesions detected.

Serum protein electrophoresis – no monoclonal band observed.

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C-ANCA - negative

P-ANCA - negative

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Complement assay.

C3, C4 levels - normal

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2D – Echo – No vegetations, repeatedly.

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USS – Abdomen No Para-aortic lymadenopathy No hepatosplenomegaly

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Duplex scan of bilateral lower limbs. No evidence of DVT

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Contrast CT abdomen, pelvis and Thorax.

No abnormality detected

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How we managed him,

Multidisciplinary approach

Rheumatology dept. Radiology dept. Blood bank Vascular surgical team

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Desperate measure,

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Biopsy Gangrene following vasculitis is the favored

microscopic diagnosis. No granuloma formation observed.

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This scenario fits the ever-widening etiological spectrum of SPG.

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Symmetrical peripheral gangrene (SPG)

rare clinical syndrome characterized by bilateral distal ischemic damage leading to

gangrene in the absence of major vascular occlusive disease

Peripheral pulses are usually palpable as a result of sparing of larger vessels

The mechanism of vascular occlusion is poorly understood A wide array of infective and non infective etiological factors Could be associated with sepsis, low-flow states, vasospastic

conditions, myeloproliferative disorders, and hyperviscosity syndromes

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Thank you!