Hydroa Vacciniforme Lymphoproliferative Disorder in a Young Spanish Woman: An Infrequent Case With Fatal Outcome.

ABSTRACT: Hydroa vacciniforme (HV) lymphoproliferative disorder is a rare NK/T-cell lymphoma mainly affecting children and with a clinical resemblance to HV, which is mostly reported in Latin American and some Asian countries. Overall, the mature T cell and NK-cell neoplasms are now grouped into 9 families based on diverse concepts: cell of origin/differentiation state, clinical scenario, disease localization, and cytomorphology. HV lymphoproliferative disorder is listed within the group of Ebstein Barr Virus-positive T-cell and NK-cell lymphoid proliferations and lymphomas of childhood according to the fifth edition of the World Health Organization Classification of mature lymphoid neoplasms. We report the extraordinary case of a 22-year-old white woman, native of Spain, first presented in 2016 when she started suffering from recurrent facial edema. Four years later, the disease progressed with lymph node spreading and a fatal outcome. Here, we describe the clinical and histological presentation of the lymphoma throughout its evolution. Cases like this can be difficult to classify posing a real challenge to clinicians and pathologists. So, it is vital to be aware of the rare presentation of this disease to be able to identify the clinical and histological picture to make a correct diagnosis and establish an early treatment.

[Apocrine hidrocystoma associated with gouty tophi of the pinna]. / Hidrocistoma apocrino asociado con tofo gotoso en pabellón auricular.

BACKGROUND: Apocrine hidrocystomas, also known as apocrine cystadenomas, are bening cystic tumours derived from the secretory portion of apocrine sweat glands. CLINICAL CASE: A 78-year old female was referred to our division for assesment an asymptomatic translucent, well-defined cystic lesion located on the upper helix. The histological features were consistent with apocrine hidrocystoma coexisting with gouty tophi. CONCLUSIONS: We report the second case of apocrine hydrocystoma located in the pinna, outside of ear canal and the first case of its association with gouty tophi described. It is difficult to know which lesion was first established and if the tophi may lead to ductal obstruction and subsequent cystic retention.

Introducción: los hidrocistomas apocrinos, también conocidos como cistoadenomas apocrinos, son lesiones quísticas benignas derivadas de la porción secretora de las glándulas apocrinas. Caso clínico: remiten al servicio de Dermatología a una mujer de 78 años para valorar una lesión asintomática translúcida, bien definida, localizada en la porción superior del hélix. Los hallazgos histológicos fueron compatibles con un hidrocistoma apocrino asociado a tofo gotoso. Conclusiones: reportamos el segundo caso de hidrocistoma apocrino localizado en el pabellón auricular externo y el primer caso asociado a tofo gotoso. Es difícil de conocer qué lesión se estableció primero, y si el tofo gotoso pudo provocar una obstrucción ductal con la subsiguiente retención quística.