The online version of this article (doi:10.1186/1476-7961-10-6) contains supplementary material, which is available to authorized users.
The author declares that they have no competing interests.
NGD-organized, contributed to manuscript preparation, generated all figures and developed the format;YB organized manuscript; IBM-contributed to molecular and immunological evaluation of these case reports; MO-assisted with manuscript review references; SC-assisted in writing treatment portion; NEF-performed skin biopsies; MRB- contributed to molecular and immunological evaluation of these case reports; BF-performed skin biopsies; RK-organized manuscript; SH-conducted HLA typing; FM-contributed to literature search; MB-assisted with critically revised the manuscript and corrections. All authors read and approved the final manuscript.
Major histocompatibility complex class II deficiency, also referred to as bare lymphocyte syndrome is a rare primary Immunodeficiency disorder characterized by a profondly deficient human leukocyte antigen class II expression and a lack of cellular and humoral immune responses to foreign antigens. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections. The infections begin in the first year of life and involve usually the respiratory system and the gastrointestinal tract. Severe malabsorption with failure to thrive ensues, often leading to death in early childhood. Bone marrow transplantation is the curative treatment.
Here we report two cases with a late outcome MHC class II deficiency. They had a long term history of recurrent bronchopulmonary and gastrointestinal infections. Bone marrow transplantation could not be performed because no compatible donor had been identified. At the age of 12 years, they developed oral papillomatous lesions related to HPV (human papillomavirus). The diagnosis of HPV infection was done by histological examination. HPV typing performed on the tissue obtained at biopsy showed HPV type 6. The lesions were partially removed after two months of laser treatment.
Viral infections are common in patients with MHC class II and remain the main cause of death. Besides warts caused by HPV infection do not exhibit a propensity for malignant transformation; they can cause great psychosocial morbidity.
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- Oral HPV infection and MHC class II deficiency (A study of two cases with atypical outcome)
Imène Ben Mustapha
Nadia El Fekih
Mohamed Ridha Barbouche
- BioMed Central
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