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Case 16 F 56 Polycythaemia rubra vera with myelofibrosis Commenced on bromide (pipobroman) Nodular, pustular eruptions involving face, upper chest, upper back, limbs Personality change and headaches Auditory hallucinations, acute psychosis Incision biopsy from left posterior shoulder

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Page 1: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Case 16

• F 56

• Polycythaemia rubra vera with myelofibrosis

• Commenced on bromide (pipobroman)

• Nodular, pustular eruptions involving face, upper chest, upper back, limbs

• Personality change and headaches• Auditory hallucinations, acute psychosis

• Incision biopsy from left posterior shoulder

Page 2: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Case 16

• Pyoderma gangrenosum

• Erythema elevatum diutinum

• Bromoderma

• Papular urticaria

Page 3: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving
Page 4: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving
Page 5: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving
Page 6: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving
Page 7: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Case 16

• Pyoderma gangrenosum

• Erythema elevatum diutinum

• Bromoderma

• Papular urticaria

Page 8: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Neutrophilic dermatosis - bromoderma

• Rare pustular dermatosis

• Classically affects face, upper trunk, lower limbs

• Can persist for weeks after drug withdrawal

Page 9: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Clinical differential diagnosis

• ? Sweet’s Syndrome

• ? Drug – induced

• ? Infection

Page 10: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Neutrophilic dermatosis - bromoderma

• Pipobroman discontinued:• 1 week, no further skin lesions

• 4 weeks, significant improvement in skin eruption

• 8 weeks, complete resolution of skin eruption and normal mental state

Page 11: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Pipobroman

System SE

Rash Acneiform, pustular,

granulomatous, ulcers, bullae,

panniculitis, TEN

Psychological Personality changes, psychosis

GI Nausea, Vomiting, diarrhoea

Haem Myelosuppression, Leukaemia

General Headache, restlessness,

anorexia, fatigue

Page 12: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Neutrophilic dermatosis - bromoderma

• Histological differential diagnosis• Infection• Sweet’s syndrome• Pyoderma gangrenosum• Arthropod bite reaction• Bowel associated dermatosis• Rheumatoid neutrophilic dermatosis• Urticaria• Vasculitis• Erythema elevatum diutinum• Immunobullous disorders

Page 13: Case 16 - Virtual Pathology 2015 16.pdf · Case 16 •F 56 •Polycythaemia rubra vera with myelofibrosis •Commenced on bromide (pipobroman) •Nodular, pustular eruptions involving

Neutrophilic dermatosis - bromodermaPyoderma gangrenosum Erythema elevatum diutinum Papular urticaria

Necrotic ulcers with pustular

erythema, violaceous, undermined

edges

Painful, persistent, pathergy

Lower legs + trunk, face, arms

Multiple systemic associations

Ulceration, abscesses, epidermal

involvement and pustules

Localised chronic vasculitis

Nodules on extensor surfaces, fingers,

toes, hands, ankles, knees, elbows

Systemic associations – paraprotein,

haematological malignancy, IBD

LCV, neutrophils, histiocytes,

lymphocytes, eosinophils, fibrosis,

storiform scarring

Hypersensitivity reaction usually to

insect bites

Usually localised but may be multiple

areas involved

Highly itchy papules, wheals and

plaques with blisters and excoriations

Reactive epidermal changes,

superficial and deep inflammation -

lymphocytes, eosinophils

Neutrophilic urticaria – clinically

typical urticaria, histology of dermal

oedema with perivascular and

interstitial neutrophils