A 5 year old boy with Cutaneous Lupus

Cutaneous lupus is rare in childhood. A 5-yr old Asian boy presented to the ENT department with a skin lesions involving his left ear. This was initially diagnosed as cellulitis and treated with intravenous antibiotics with no significant improvement. He presented with recurrence of skin lesions. Examination revealed several non tender erythematous, polymorphous skin lesions involving his face, right external ear, scalp, nose and soles. The lesions in the right ear showed areas of necrosis and crusting without discharge. Multiple annular erythematous palpable lesions were present over postero lateral aspects of both soles. The trunk, limbs, genitalia and oral mucosa were spared. Systemic examination was unremarkable. He was growing along his previous weight and height centiles

His immunological profile showed grossly elevated IgG levels with normal compliment level. The autoimmune screen showed positive for ANA, anti-Ro and anti-La and negative ANCA. Histopathology of the skin lesion revealed marked perivascular lymphocytic infiltrates, red blood cell extravasations and arteriole wall inflammation in the dermis without fibrinoid necrosis. His immunological profile and histopathology confirmed the diagnosis of cutaneous lupus.

The skin lesions were treated with topical and oral steroids and subsequently with Hydroxychloroquine. The skin lesions rapidly resolved with the introduction of Hydroxychloroquine therapy. He has been followed up regularly in our Paediatric Rheumatology department and he remains clinically stable with no systemic features of Lupus.

Literature review reveals that Cutaneous Lupus is an uncommon clinical condition in this age group. Sub-acute presentation is uncommon and the response to Hydroxy Chloroquine is well documented. This boy never had any systemic symptoms. There was no history of Lupus in his mother. The initial presentation and the delay in diagnosis highlights the need for clinicians managing paediatric patients to be aware of this uncommon clinical condition.