WILD syndrome: A rare presentation of primary lymphedema, generalized warts and immune deficiency diagnosed in Bangladesh

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Brief description: WILD syndrome is a rare disease characterized by warts, impaired cell mediated immunity, primary lymphedema and anogenital dysplasia. Our patient who is a 13 year old boy presented with swelling of right lower limb since birth. The swelling gradually involved both upper limbs and right side of his face. The swelling over the right lower limb grew to such an extent that he has been unable to do his daily activities for the last 3 years and warty skin lesions over his whole body for the last 5 years. At the same time he has swelling of his external genitalia and warty lesion around the anus. He has recurrent painless swelling of his abdomen with scanty micturition. On examination, he was mildly anemic, non-icteric, non-pitting odema of right leg, normal vital parameters, right sided pleural effusion, pericardial effusion and ascites, no organomegaly. Swelling and distortion of right lower limb with nodular lesions over the right leg, generalized hyper and hypopigmented verrucous papules and paques over whole body, swelling of the glans and flat warts around the anus.  His investigations revealed leucopenia, chylous pleural effusion, lymphedema of right lower limb with venous incompetency and normal arterial supply, reduced CD4 and CD8 T cell count, normal immunoglobulin levels, negative ICT for filarial, histology of skin lesion revealed elephanthiasis verrucosa nostra, and anogenital dysplasia with a large number of koilocytes.

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