[Chronic prurigo revealing an angioimmunoblastic T cell lymphoma]. / Prurigo chronique révélant un lymphome T angio- immunoblastique.

BACKGROUND: Cutaneous manifestations in angio-immunoblastic T cell lymphoma (AITL) can be seen in almost 50% of patients. They are especially represented by maculo-papular pseudotoxic exanthema. The other manifestations, like nodular prurigo are rarely observed. We report a case of AITL diagnosed after an etiologic screening for chronic prurigo. AIM: The objective of our work is to stress on the possibility to diagnose a hemopathy in etiologic screening of chronic pruritus. CASE REPORT: A 45-year-old patient presented a chronic pruritus of 18 months associated with general manifestations (fever, night sweating and weight loss). Physical examination showed diffuse adenomegalies. On histology, the lymph nodes were composed of polymorphous lymphoid proliferation made of middle to large sized cells with clusters of epithelioid cells and post-capillary veinules hyperplasia. Immunohistochemical study showed T cell phenotype (CD3+). Large cells were CD30+. Latent Protein of EBV virus was not expressed. Molecular biological analysis of a lymph node showed a T cell clonal proliferation. Cutaneous biopsy showed a little dermic perivascular lymphocytic inflammatory infiltrate. The diagnosis of angio-immunoblastic T cell lymphoma was made. The abdomino-pelvian CT scanner showed multiple inter-aortico-cave lymph nodes and a splenomegaly. A polychemotherapy was initiated. CONCLUSION: In front of chronic prurigo with general manifestations, a careful etiologic screening should be done to detect internal disorders especially malignant hemopathies.

[Brooke-Spiegler syndrome]. / Syndrome de brooke-Spiegler.

Brooke Spiegler syndrome is a rare genodermatosis characterized by an association of multiple trichoepitheliomas and cylindromas, sometimes accompanied by other adnexal tumors (spiradenoma, mila), we report two family cases of Brooke Spiegler syndrome. In the first case: a 31 years-old woman, with multiple trichoepitheliomas involving in the naso-genal areas, with multiple cylindromas of the scalp. The second case regarding the brother: 37 years-old, presented with multiple trichoepitheliomas of the face, associated with milia. No neoplastic tumor was identified. A co-existence of adnexal tumors was found in the other members of the family. Brooke Spiegler syndrome is an autosomial dominant disease with variable penetrance, it's characterized by a family history of trichoepitheliomas, with other adnexal tumors. The course of the disease is characterized by a multiplication of lesions, a malignant transformation was reported, but remains rare. Brooke Spiegler syndrome is usually a benign disease, but patients with this syndrome should be explored for malignancy. A family study is indicated.