Presenting History
In children presenting with suspected IgA Vasculitis (Henoch-Schönlein Purpura), elicit the distribution and character of purpuric lesions, abdominal pain, joint swelling, and urinary abnormalities following a recent infection.
- Palpable Purpura (The Mandatory Hallmark):
- Onset & Site: Did the red-purple spots begin around the ankles and shins, spreading upwards to the calves, thighs, and buttocks?
- Palpability: Are the lesions raised and felt as small bumps/nodules (palpable purpura)?
- Non-Blanching: Do the spots remain dark red/purple when pressed (extravasated blood)?
- Spared Areas: Are the trunk, face, palms, and soles completely spared?
- Gastrointestinal Symptoms:
- Is there severe, colicky, cramping periumbilical abdominal pain causing the child to double over?
- Any vomiting, blood in stools (hematochezia / melena), or visible abdominal swelling?
- Joint Symptoms:
- Is there acute pain, swelling, and periarticular puffiness around the ankles and knees?
- Is the child limping or refusing to bear weight?
- Urinary Symptoms (HSP Nephritis):
- Has the urine appeared red, tea-colored, smoky, or frothy?
- Is there morning facial puffiness or swelling of feet?
Negative History (3C 1D Framework)
| Category | Pertinent Negative Question | Rationale / Significance |
|---|---|---|
| Causes | Upper Respiratory Precursor: Note history of sore throat, rhinorrhea, or low fever 1-3 weeks prior. Drugs: No intake of new antibiotics or NSAIDs prior to purpura. | Group A Streptococcus and respiratory viruses commonly trigger IgA1 immune complexes. Rules out drug-induced leukocytoclastic vasculitis. |
| Complaints (Differentiating) | Thrombocytopenia: No flat petechiae on trunk/face, no epistaxis, gum bleeding, or mucosal wet purpura. Acute Abdomen: No bilious vomiting or continuous focal right iliac fossa guarding. | Differentiates non-thrombocytopenic HSP from Immune Thrombocytopenia (ITP). Differentiates uncomplicated bowel vasculitis from intussusception or appendicitis. |
| Complications | Intussusception: No episodic screaming with leg pulling, currant-jelly stools, or palpable mass. Severe Nephritis / AKI: No oliguria, headache, vomiting, visual blurring, or convulsions. | Ileo-ileal intussusception is the commonest surgical emergency in childhood HSP. Severe crescentic HSP nephritis causes hypertension and acute renal failure. |
| Differentials | Meningococcemia: No high fever, prostration, shock, or necrotic purpura fulminans. PSGN: PSGN lacks palpable purpura and GI vasculitis. | Differentiates benign IgA vasculitis from fulminant meningococcal sepsis. Differentiates isolated post-streptococcal nephritis. |
Other Relevant History
- Family History & Pedigree: Trace atopy and renal diseases.
- Scrotal Symptoms (in Boys): Check for acute scrotal swelling or testicular pain (scrotal vasculitis).
History Summary
"Master/Miss `Patient Name`, a `Age` old `male/female` child, `Birth Order` born of a `consanguineous/non-consanguineous` marriage from `City, State`, presented with a `Duration in days` history of symmetrical palpable purpura over the lower limbs and buttocks, colicky periumbilical abdominal pain, `blood in stools`, ankle periarticular swelling, and `smoky urine`, preceded by an upper respiratory infection, in the absence of mucosal bleeding or thrombocytopenia.
In view of the mandatory palpable purpura with lower limb predominance, abdominal pain, arthritis, and urinary sediment, I would like to consider a provisional diagnosis of IgA Vasculitis (Henoch-Schönlein Purpura), presenting with active gastrointestinal involvement and HSP Nephritis, without acute intussusception or renal failure."
General & Head-to-Toe Examination
- Vitals:
- Blood Pressure: Mandatory measurement with appropriate cuff (screen for hypertension from HSP nephritis).
- Pulse rate, respiratory rate, temperature.
- Dermatological Examination (Palpable Purpura):
- Inspect distribution: Confirm concentration over extensor surfaces of lower limbs and buttocks; verify sparing of trunk and face.
- Palpate lesions: Confirm raised, non-blanching purpuric papules and plaques (2 to 10 mm).
- Assess for dependent subcutaneous non-pitting edema (dorsum of feet, periorbital).
- Musculoskeletal Examination:
- Examine bilateral ankles and knees: Assess periarticular swelling and tenderness; confirm absence of true intra-articular effusion or erythema.
- Genitourinary Examination (Boys):
- Inspect and palpate scrotum and testes (rule out scrotal vasculitis and testicular torsion).
Systemic Examination
Abdomen
- Palpate gently: Assess periumbilical tenderness; check strictly for localized guarding, rebound tenderness, or a palpable sausage-shaped mass (intussusception).
- Perform digital rectal examination: Check for occult or gross blood.
Cardiovascular, Respiratory & Nervous Systems
- Clinically normal; fundoscopy to exclude hypertensive retinopathy if blood pressure is elevated.
Final Summary & Diagnosis
"A `Age` old `male/female` child presenting with symmetrical non-thrombocytopenic palpable purpura over lower extremities and buttocks, colicky abdominal pain, ankle arthritis, and elevated blood pressure (`BP in mmHg`) with hematuria and proteinuria.
My final diagnosis is IgA Vasculitis (Henoch-Schönlein Purpura) fulfilling EULAR/PRINTO/PRES consensus criteria, presenting with active gastrointestinal vasculitis and active HSP Nephritis, without ileo-ileal intussusception, acute kidney injury, or scrotal vasculitis."