Approach to Purpura

Definition:
•Purpura is an erythematous macule due to extravasation of RBCs into
the dermis.
•It is non-blanchable - if a glass slide is pressed onto the lesion
(diascopy), erythema persists
 Types:
Petechiae: lesions <=0.5 cm
Ecchymosis: lesions>0.5cm
Palpable purpura
Retiform : stellate or branching lesions with angular or
geometric borders
Inflammatory
Non-inflammatory
Petechiae: Pinpoint reddish-purple haemorrhages
Ecchymosis: large confluent lesions commonly referred to as
‘bruises’. They may be tender or raised.
Palpable
Retiform purpura
Classification of purpura based on clinical presentation:
Purpura
Retiform/non-
inflammatory
petechiae
•Platelet occlusion
Palpable/inflam •Systemic coagulopaties
•Platelet functon disorder •Emboli
•Thrombocytopenia matory •reticulocytes

ecchymosis •Leukocytoclastic
vasculitis- henoch Retiform/inflammation
schonlein, urticarial
•Procoagulant defects- vasculitis, rheumatic
anticoagulants, liver disease, vasculitis
•Rheumatic vasculitis, polyarteritis
vit k deficiency, DIC •ANCA- wegener’s nodosa, ANCA vasculitis
•Decreased dermal support granulomatosis, churg
or minor trauma- stauss syndrome
corticosteroids, scurvy,
systemic amyloidosis
 CAUSES OF PURPURA
Purpura

Disruption in Abnormalities in
Abnormalities in
Vascular secondary
primary hemostasis
Integrity hemostasis

Platelet number

Platelet function
 Eitiological classification of Purpura

Platelet Coagulation Vascular

others
defects defects defect

Thrombocytopenia Inherited defects- Senile purpura

hemophilia, Vasculitis (HSP)
CT disorder Topical or
DIC christmas Infectious- mcc corticosteroid
Hemolytic anemia CT disorder septicemia therapy
Hypersplenism
DIC Drugs- aspirin
Giant hemangiomas
Scurvy
Acquired defects- Painful bruising
BM damage syndrome Lichen sclerosis
liver
Drugs-
penicillin,sulphonamides, disease,0anticoagul Systemic
salicylates ant therapy, vit k amyloidosis
deficiency
1.Defect in platelets
•Number
•function
Defect in number
 Immune Thrombocytopenic Purpura
(ITP)
• Immune mediated destruction
• Sudden development of petechiae and
ecchymosis in an otherwise healthy child
• Epistaxis (10-20%) or more serious bleeding
• Most common in children 1 – 5 years of age
Diagnosis based on:
Isolated thrombocytopenia with no clinically
apparent associated conditions.
 Thrombotic thrombocytopenic
purpura
• PENTAD of thrombosis, thrombocytopenia,
purpura, fever and neurological abnormalities

• Deficiency of ADAMTS-13 protease (which is

responsible for cleaving vWF multimers)
Circulation of multimers
Microvascular platelet thrombosis
 Haemolytic Uremic Syndrome (HUS)
• Most common in young children
• TRIAD of thrombocytopenic, microangiopathic
anaemia, acute renal failure
• Typical – follows a prodromal diarrhoea /
respiratory infection
 Others
•DIC
•Bone marrow infiltration / failure
•Inherited thrombocytopenias
•Sequestration, splenomegaly
Funtional defect of platelets:
•Inherited
•Aqcuired
 Inherited platelet function
abnormalities
• (Uncommon)
• Most severe –
– GLANZMANN THROMBASTHENIA – deficiency of
GP IIb/IIIa complex
– BERNARD SOULIER SYNDROME – deficiency of
GPIb/IX/V complex
 Acquired abnormalities in platelet
function
• Drugs – Aspirin, NSAIDs (Ibuprofen)
Also, antihistamines, phenothiazines,
valproate
• Uremia
 2.Coagulation Factor Deficiencies
•vWD
•Hemophilia
•Other congenital factor deficiencies
•Vitamin K deficiency
•Liver disease
3.Defect vascular integrity
 Trauma
Frequently located over shins, knees, elbows
forehead (accidental trauma)
Face, back, upper arms, buttocks (intentional
trauma)
Appearance –
Red/blue/purple – recent lesions
Yellow/brown/green – older lesions
 Infection
Purpura fulminans – an acute, life-threatening sequala
of Neisseria meningitidis (also others - varicella, GAS,
Str. Pneumoniae)

Pathology - Microvascular thrombosis

Tissue necrosis, skin infarction and haemorrhage

Appearance - Central areas of black haemorrhagic

necrosis with a surrounding erythematous border
Patients also are acutely ill, with fever, hypotension and
bleeding, often in DIC
 Vasculitis
• IgA vasculitis - Henoch-Schonlein purpura [HSP]
• The most common cause of vasculitis in children
Pathology - Deposition of IgA1 in blood vessel walls
Appearance – Palpable purpuric lesions, 2-10mm in
diameter that often coalesce; concentrated in buttocks
and lower extremities
Patients also have arthritis, abdominal pain +/- GI
bleeding, nephritis

Others – Polyarteritis Nodosa, Cryoglobulinemic

vasculitis, etc.
 Drug induced vasculitis
• Most common – Sulfonamides, Penicillins,
Chloral hydrate, Phenytoin, Propylthiouracil,
Hydralazine, NSAIDs, Corticosteroids
• Develops within 7 – 21 days of starting the
drug
 Other, less common causes
•Neonatal purpura fulminans

•Ehlers-Danlos syndrome

•Vitamin C deficiency – scurvy

 Evaluation of Purpura
HISTORY
Age
Sex
Onset of symptoms
Location and type of purpura
Prior bleeding history
Family history of bleeding
Drug history
Dietary history
Past Medical History
Examination
•Size, type and distribution of purpura
•Whether the purpura is palpable or not
•Joint examination
•Lymph nodes
•hepatomegaly/splenomegaly
 Are the lesions palpable?

palpable
macular
Retiform/branc
<=5 mm : petechiae Round: hing
classic
Do platelet count >5mm: Sorrounding
Ecchymosis erythema?
Low:<
1,50,000
ITP,TTP,DIC
Normal: Yes
-drug
induced >1,50,000
No
HSP
Cong/aqcuired Liver
platelet funtion disease Cryoglobuli ANCA
nemia Micro-
defect Vit K def valculitis
occlusive
Pigmented Amyloid Rheumatic Polyarterit process
purpuric vasculitis is nodosa from
DIC
dermatoses Wegener’s Rheumati diseases
Steroid
IV local pressure abuse c like
Churg purpura
or trauma scurvy strauss vasculitis
fulminans
 References:
• Kabir Sardana textbook of Dermatology
• Khannah
• Up-to-date