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Image Number 415 (Systemic lupus erythematosus in childhood)

 
Morphology:
Plaque

Diagnosis: Systemic lupus erythematosus in childhood

Site: Face

Sex: F

Age: 6

Type: Clinical

Description: Malar rash across cheeks and nose

Submitted by: Shahbaz Janjua

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Differential Diagnosis

History:

Childhood systemic lupus erythematosus is fundamentally the same disease as in adults with similar etiology, pathogenesis, clinical manifestations, and laboratory findings.  Childhood SLE affects girls more often than boys.  Although it can occur at any age, SLE becomes more frequent after five years of age and is increasingly prevalent after the first decade of life.  Clinical features in children are generally similar to those described in adults but the disease tends to be more severe. Renal involvement is more common. Lymphadenopathy is alsoseen more commonly in children.  Glucocorticoids are the mainstay of therapy and the doses depend on the disease severity.

Neonatal lupus usually occurs in babies of mothers with anti-Ro (SSA) and anti-La (SSB) antibodies. Mothers may be asymptomatic. It may present as a transient syndrome with cutaneous lesions like annular lesions, malar erythema and discoid lesions either immediately after birth or within  the first few months of life. The cutaneous lesions may clear with the disappearance of maternal antibodies. Treatment is usually not required. Thrombocytopenia, haemolytic anaemia and leucopenia also can occur. Complete heart block can also occur which needs early delivery and pacing in 50% of cases. Nephritis does not seem to occur.



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