Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos con compromiso facial / Indolent CD8-positive t-cell lymphoid proliferation: on purpose three cases of facial commitment

Resumen La proliferación linfoide indolente cutánea CD8 positiva es una variante recientemente descrita de linfoma T cutáneo que se caracteriza por un nódulo, pápula o placa eritematosa de crecimiento lento que puede afectar la región facial o extrafacial. En el estudio de patología se caracteriza por un infiltrado monomorfo de linfocitosTalo largo de la dermis con presencia de zona de Grenz y ausencia de epidermotropismo. El infiltrado es característicamente CD8+ así como CD3+, TIA-1+, CD4-, CD56- CD30-, PD-1-, Granzima B- y EBER negativo. El índice de proliferación Ki-67 es inferior al 10% y se observan reordenamientos clonales de los genes del receptor de antígeno de la célula T, TCR. El seguimiento clínico es favorable y no se ha observado compromiso sistémico. Se presentan tres casos con compromiso facial (dos casos en pabellón auricular y un caso con compromiso nasal), con presentación clínica y hallaz gos histopatológicos típicos (curiosamente un caso con cambio de célula clara), y además se realizaron estudios de clonalidad.

Abstract Primary cutaneous indolent CD8-positive lymphoid proliferation is a recent variant of cutaneous T lymphoma that is characterized by nodule, papule or plaque erythematous with slow growth that can affect the facial or extrafacial region. In the histopathology study it is characterized by an infiltration of monomorphic T lymphocytes throughout the dermis with presence of Grenz zone and absence of epidermotropism. The infiltrate is characteristically CD 8+ and CD3+ TIA-1+ CD4-, CD56- CD30, PD-1, Granzyme B- and negative EBER. Ki-67 Proliferación linfoide indolente cutánea CD8 positiva a propósito de tres casos proliferation index is less than 10% and clonal T-cell receptor gene rearrangements. Clinical follow-up is favorable and has not been observed systemic involvement. We present three cases with facial involvement (two cases in ear and one case with nasal commitment) with typical clinical presentation, histopathological findings (curiously a case with clear cell change) and clonality studies.

Metastatic Malignant Pilomatrixoma in an 8-Year-Old Girl Misdiagnosed as a Recurrent Pilomatrixoma.

Malignant pilomatrixoma or pilomatrix carcinoma is a rare, locally aggressive malignant neoplasm, derived from the hair follicle with a high propensity for local recurrence. Only a few cases of metastatic pilomatrixoma have been described in the literature. Till date, only 17 cases have been reported in the English-language medical literature, most commonly occurring associated with local recurrence in adults. We report the first case in children of a metastatic malignant pilomatrixoma in an 8-year-old girl presenting with a recurrent pilomatrix carcinoma of the forehead with metastases in cervical and parotid lymph nodes.

Cutaneous Collision Tumor Associated With Porocarcinomatous and Angiosarcomatous Components: A Potential Diagnostic Pitfall.

Sarcomatoid eccrine porocarcinoma (SEP) is a very rare malignancy including epithelial and mesenchymal components exhibiting pleomorphic cells, nuclear hyperchromasia, and high mitotic activity in both elements. To date, only 6 cases of this uncommon neoplasm have been reported, corresponding to women over 70 years of age with ulcerated skin lesions. The authors describe the first sarcomatoid eccrine porocarcinoma in a 75-year-old male patient with a right hallux lesion, presenting a collision tumor with a mixed population of epithelial cells and a spindle cell angiosarcomatous mesenchymal component each expressing distinct and nonoverlapping morphologic and immunohistochemical features of epithelial and mesenchymal differentiation.