WILD syndrome: A rare presentation lymphedema, generalized warts and deficiency diagnosed in Bangladesh | [Journal of Pakistan Association of Dermatologists • 2019]

Author(s):

1. Sabrina Yesmin: Department of Medicine, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

2. AKM Ruhul Amin: Department of Surgery, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

3. Towhida Noor: Department of Dermatology & Venereology, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

4. SM Khodeza Nahar Begum: Department of Pathology, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

5. Tareq M Bhuiyan: Department of Gastroenterology, Bangladesh Institute of Research and Rehabilitation in Diabetes, Endocrine and Metabolic Disorders, Dhaka, India

6. Mehtab Al-Wadud Khan: Department of Anesthesiology, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

7. Rozina Akhter: Department of Paediatrics, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

8. Md. Selim Mahmod: Department of Cardiology, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

9. Jahid Hasan: Department of Medicine, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

10. MA Baqui: Department of Surgery, Dr. Sirajul Islam Medical College & Hospital, Dhaka, India

Abstract:

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.

Page(s): 125-129

DOI: DOI not available

Published: Journal: Journal of Pakistan Association of Dermatologists, Volume: 29, Issue: 1, Year: 2019

Keywords:

Generalized warts , primary lymphedema , impaired cell mediated immunity , anogenital dysplasia , koilocytes

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