Vasculitic Disorders












CHAPTER 14
VASCULITIC DISORDERS

 


Acute Hemorrhagic Edema of Infancy (AHE)







































Synonyms Finkelstein disease, Seidlmayer cockade purpura, urticarial vasculitis of infancy, and acute benign cutaneous leukocytoclastic vasculitis of infancy.
Inheritance n/a
Prenatal Diagnosis n/a
Incidence Rare, ~300 cases reported in the literature.
Age at Presentation Four months to 2 years of age.
Pathogenesis Unknown. Thought to be an immune-mediated process where immune complexes form, leading to the clinical and histologic features. Infections, medications, and immunizations have also been reported to be associated with the development of acute hemorrhagic edema of infancy.
Key Features Triad of clinical findings is often described:

  • Purpura: Target or “cockade-like” (medallion or rosette shaped).
  • Edema: Hands and feet initially; can extend up to the arms and legs; scrotal edema has also been reported.
  • Fever: Low grade (37.5-38.3 °C/99.5-101 °F).

Systemic: Rarely may involve the joints, kidneys, and gastrointestinal tract. Might be a variant of Henoch-Schönlein purpura (HSP) without systemic involvement.

Differential Diagnosis HSP, meningococcemia, erythema multiforme, Sweet syndrome, neonatal lupus, urticaria, urticarial vasculitis, and Kawasaki disease.
Laboratory Data Routine lab tests are often normal. There may be some degree of evaluation if the diagnosis is not suspected to rule out other conditions.

  • Biopsy: Leukocytoclastic vasculitis; direct immunofluorescence of adjacent normal skin shows deposition of various immunoreactants, immunoglobulin A (IgA) most often seen though in no more than a third of cases. This finding may help to exclude HSP (IgA positive in most patients).
Management

No treatment has been shown to alter the course of the disease.


Supportive therapy may alleviate any symptoms.

Prognosis

Resolves spontaneously over 1 to 3 weeks. Complete recovery is typical.


Recurrence has been reported but it is rare.

image

14.1. Acute hemorrhagic edema of infancy. Courtesy of Mark Crowe, M.D.


image PEARL/WHAT PARENTS ASK


Parents often are concerned about this being a life-threatening condition. Convincing parents that no treatment is necessary is always a challenge. It is also important to emphasize that systemic involvement is rare and usually not persistent.











Skin | Associated Findings
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Kawasaki Disease






































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Aug 17, 2025 | Posted by in PEDIATRICS | Comments Off on Vasculitic Disorders

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Synonyms Acute febrile mucocutaneous lymph node syndrome and mucocutaneous lymph node syndrome.
Inheritance n/a
Prenatal Diagnosis n/a
Incidence In the US, 19 cases per 100,000 children under the age of 5 years (higher in those of Asian ancestry); in Japan, 265 cases per 100,000 children under the age of 5 years; male-to-female ratio ~1.5:1; siblings of the affected children have 10 to 20 times higher risk compared with the general population; Japanese children whose parents had the disease often have a more severe course and may have recurrent episodes. A similar condition (multisystem inflammatory syndrome in children [MIS-C]) has been described as associated with COVID-19 infection in children.
Age at Presentation Eighty percent to ninety percent of cases occur in children under the age of 5 years; 95% of cases are in children under the age of 10 years.
Pathogenesis No single etiology has been identified; pathogenesis is likely multifactorial; infectious agent is presumed to be the trigger as the disease can present in epidemics every 2 to 3 years; autoimmune and genetic factors may also play a part. Generalized vasculitis involves small- to medium-sized arteries and is particularly pronounced in coronary vessels; edema in endothelial cells and vascular media in the early stages. Over time, internal elastic lamina becomes fragmented; resultant vascular damage leads to characteristic, potentially fatal coronary artery damage.
Key Features Kawasaki disease (KD) is the most common cause of vasculitis and acquired heart disease in children.

  • Diagnostic criteria: Fever for 5 days (102-104 °F/39-40 °C), plus 4 out of 5 of the following clinical findings:

    1. Edema and erythema of hands and feet with desquamation.
    2. Polymorphous exanthem (urticarial, morbilliform, scarlatiniform, targetoid, without vesicles or crusts); monomorphous in a given patient, perineal erythema and desquamation; accentuation in genital and perianal areas.
    3. Bilateral, nonpurulent conjunctivitis.
    4. Lip and oral mucosa changes (strawberry tongue).
    5. Nonpurulent unilateral cervical lymphadenopathy (>1.5 cm).


  • “CRASH and Burn” mnemonic:

    • Conjunctival injection
    • Rash, polymorphous
    • Adenopathy, cervical
    • Strawberry tongue
    • Hand and feet desquamation
    • Burn = fever

Phases of KD



  • Acute (onset of fever to its resolution):

    • Abrupt onset of high fever lasting 1 to 2 weeks (102-104 °F/39-40 °C), unresponsive to treatment (antipyretics or antibiotics).
    • Typically resolves within 48 hours after treatment with intravenous immunoglobulin.
    • Irritability.
    • Diagnostic clinical findings appear.
    • Other systemic manifestations: Hepatic, renal, and gastrointestinal dysfunction (including hydrops of the gall bladder, diarrhea, ileus, hepatitis, and pancreatitis); myocarditis and pericarditis.



  • Convalescent

    • Occurs within 3 months of presentation.
    • Resolution of clinical signs.
    • Erythrocyte sedimentation (ESR) and C-reactive protein (CRP) return to normal.
    • Beau’s lines on nails can be seen (not diagnostic for KD but is a sign of recent significant physical illness) 1 to 2 months after fever.
    • Sixty percent of small cardiac aneurysms will resolve.


  • Chronic phase

    • In patients with cardiac complications.
    • Lifetime follow-up to monitor status of any aneurysms that developed in childhood.
Differential Diagnosis Rheumatic fever, Measles (rubeola), adenovirus, parvovirus B19 infection, Epstein-Barr virus, enterovirus, scarlet fever, staphylococcal scalded skin syndrome, toxic shock syndrome, Rocky Mountain spotted fever, cervical lymphadenitis, Lyme disease, Stevens-Johnson syndrome, morbilliform drug reactions, systemic lupus erythematosus, systemic juvenile rheumatoid arthritis, COVID-19 associated MIS-C.
Laboratory Data No specific diagnostic laboratory test; diagnosis is based on maintaining a high index of suspicion in patients with at-risk symptoms; ESR and CRP can be used to follow the course of the disease and will gradually return to baseline after 6 to 10 weeks.
Management