Systemic T cell lymphoma presenting as cutis verticis gyrata.

Sir, A 34-year-old man, presented with progressive swelling and serous discharge on his face and scalp for 4 months. General examination revealed pallor and multiple, enlarged, non-tender, discrete, mobile lymph nodes in bilateral cervical, axillary, and inguinal regions, the largest of which was 5 cm × 6 cm in the right axillary region, associated with hepatosplenomegaly. He had a leonine facies. The skin of the scalp was thrown into cerebriform folds with diffuse loss of hair [Figure 1a]. There was generalized xerosis with a few scattered infiltrated nodules [Figure 1b] on the trunk and extremities ranging in size from 1-2 cm and diffuse scaling over the palms and soles. The differentials considered were lymphedema secondary to a lymphatic obstruction, leukemoid/lymphomatous infiltration and sarcoidosis. On hematological examination, he was found to have anemia (10.8 g/ dl) and leukocytosis (63.4 × 109/L) associated with a serum lactate dehydrogenase of 1110 U/L. Serology for Epstein-Barr virus and human T-lymphotrophic virus were negative. Skin biopsies done from the scalp and a nodule on the back showed mild lamellar hyperkeratosis, focal parakeratosis and irregular acanthosis of the epidermis. There was a pan-dermal, moderate to dense infiltrate of medium sized lymphoid cells with vesicular nuclei, irregular nuclear membranes, small nucleoli and scant cytoplasm. Numerous apoptotic bodies were seen [Figure 2a and b]. On immunohistochemistry, these cells were positive for CD3 [Figure 3a], CD4 [Figure 3b], focally positive for CD25 [Figure 3c] and negative for CD5, CD7, CD20, CD34 and terminal deoxynucleotidyl transferase. The MIB-1 proliferation index [Figure 3d] was 85%. Lymph node biopsy from the right axilla was consistent with high grade T-cell lymphoma on histopathology and immunohistochemistry [Figure 4]. Bone marrow biopsy showed myeloid hyperplasia with increased eosinophils and its precursors. T cell receptor re-arrangement studies demonstrated clonality for the alpha-beta type. CD 3 and CD 4 positivity indicated a T cell lymphoma. CD 20 negativity ruled out a B cell lymphoma. CD 7, CD 34 and terminal deoxynucleotidyl transferase negativity ruled out a T cell lymphoblastic leukemia/lymphoma. CD 25 positivity is usually seen in adult T cell lymphoma, which was ruled out in view of the human T lymphotropic virus serology being negative. A high MIB-1 proliferation index indicated a high grade lymphoma. On this basis the diagnosis of high grade T cell non-Hodgkin lymphoma was