Infantile Systemic Lupus Erythematosus with Cerebral Calcification and Delayed Development

Excerpt

To the Editor : Systemic lupus erythematosus (SLE) occurring in the first year of life is rare. We present a 2.5-y-old female, product of non-consanguineous marriage whose symptoms of itchy red, raised plaques over face, scalp and trunk started at 6 mo of age. Few of them progressed to form angiomatous papules, while others healed with pigmentation and scarring. New lesions kept on erupting over 2 y, aggravating on photo-exposure. On examination, multiple erythematous atrophic scaly plaques with telangiectasias and few angiomatous papules were evident (Figs. 1, 2, and 3). Histopathology from angiomatous papule revealed multiple dilated thin walled congested blood vessels in the papillary dermis (consistent with angiokeratoma) (Fig. 4). Direct immunofluorescence from non-lesional, non sun-exposed skin showed positive C3 (1+, granular), fibrinogen (2+, continuous), IgG and IgM at the dermo-epidermal junction. These unusual cutaneous features of atrophic telangiectatic plaques and angiomatous papules with histopathology resembling angiokeratoma have been classically described in neonatal lupus erythematosus (NLE) but not in SLE [1].

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