ABSTRACT
Langerhans cell histiocytosis (LCH) is a disease characterized by proliferation of CD1a+dendritic cells with local or diffuse organ compromise. The identification of recurrent gene mutations has confirmed the hypothesis of LCH as a true neoplasm. Lymphomatoid papulosis (LyP) belongs to the spectrum of CD30+primary cutaneous lymphomas. LCH has been described in association with other lymphoproliferative disorders. However, lesions constituted by Langerhans cells (LC) have been commonly considered reactive, related to cytokines produced by the lymphoma-microenvironment interaction. Some authors designate these lesions as "Langerhans cells-like lesions". We present the case of a 28-years-old woman with multisystem LCH and simultaneous PyL lesions with reactive LC hyperplasia.
Subject(s)
Histiocytosis, Langerhans-Cell/complications , Lymphomatoid Papulosis/complications , Adult , Female , HumansABSTRACT
La papulosis linfomatoide forma parte del espectro de los procesos linfoproliferativos cutáneos primarios de células T CD30+. Es una enfermedad rara de etiopatogenia incierta y compleja. El diagnóstico diferencial puede a veces resultar muy difícil. Se describió el caso de una mujer de 80 años con el diagnóstico, particularmente atípico desde la visión histopatológica, en el cual la correlación anatomoclínica ha sido un importante aspecto que lo hace interesante. El objetivo es comunicar un caso de presentación poco frecuente en la práctica médica (AU).
Lymphomatoid papulosis is part of the primary skin lymph proliferative processes of the T CD30+ cells. It is a rare disease of complex and uncertain etiopathogenesis. The differential diagnosis could be very difficult sometimes. The described case was the one of a female patient, aged 80 years with that diagnosis, particularly atypical from the histopathological point of view, where the anatomoclinical correlation has been an important aspect making it interesting. The objective is to inform a case of infrequent presentation in the medical practice (AU).
Subject(s)
Humans , Female , Adult , Skin Neoplasms/epidemiology , Lymphomatoid Papulosis/epidemiology , Medical Records , Lymphomatoid Papulosis/complications , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/pathology , Diagnosis, Differential , Degloving Injuries/diagnosis , Lymphoma/diagnosisABSTRACT
La papulosis linfomatoide forma parte del espectro de los procesos linfoproliferativos cutáneos primarios de células T CD30+. Es una enfermedad rara de etiopatogenia incierta y compleja. El diagnóstico diferencial puede a veces resultar muy difícil. Se describió el caso de una mujer de 80 años con el diagnóstico, particularmente atípico desde la visión histopatológica, en el cual la correlación anatomoclínica ha sido un importante aspecto que lo hace interesante. El objetivo es comunicar un caso de presentación poco frecuente en la práctica médica (AU).
Lymphomatoid papulosis is part of the primary skin lymph proliferative processes of the T CD30+ cells. It is a rare disease of complex and uncertain etiopathogenesis. The differential diagnosis could be very difficult sometimes. The described case was the one of a female patient, aged 80 years with that diagnosis, particularly atypical from the histopathological point of view, where the anatomoclinical correlation has been an important aspect making it interesting. The objective is to inform a case of infrequent presentation in the medical practice (AU).
Subject(s)
Humans , Adult , Female , Skin Neoplasms/epidemiology , Lymphomatoid Papulosis/epidemiology , Medical Records , Lymphomatoid Papulosis/complications , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/pathology , Diagnosis, Differential , Degloving Injuries/diagnosis , Lymphoma/diagnosisABSTRACT
Comunicamos el caso de una papulosis linfomatoide en una mujer de 38 años, el tipo histológico es B. Efectuamos una revisión de la entidad y sus interrogantes aún no resueltos. Se reivindica a su descubridor y el nombre inicial de su enfermedad: Warren L Macaulay y erupción rítmica paradojal, respectivamente.
A 38 years-old woman with a type B of lymphomatoid papulosis, is reported. A review of this peculiar disease and its unresolved questions is made. We make tribute to the dermatologist who described the disease and the initial name: Warren L. Macaulay and rhythmic paradoxical eruption, respectively.
ABSTRACT
Comunicamos el caso de una papulosis linfomatoide en una mujer de 38 años, el tipo histológico es B. Efectuamos una revisión de la entidad y sus interrogantes aún no resueltos. Se reivindica a su descubridor y el nombre inicial de su enfermedad: Warren L Macaulay y erupción rítmica paradojal, respectivamente.
A 38 years-old woman with a type B of lymphomatoid papulosis, is reported. A review of this peculiar disease and its unresolved questions is made. We make tribute to the dermatologist who described the disease and the initial name: Warren L. Macaulay and rhythmic paradoxical eruption, respectively.
ABSTRACT
Comunicamos el caso de una papulosis linfomatoide en una mujer de 38 años, el tipo histológico es B. Efectuamos una revisión de la entidad y sus interrogantes aún no resueltos. Se reivindica a su descubridor y el nombre inicial de su enfermedad: Warren L Macaulay y erupción rítmica paradojal, respectivamente.(AU)
A 38 years-old woman with a type B of lymphomatoid papulosis, is reported. A review of this peculiar disease and its unresolved questions is made. We make tribute to the dermatologist who described the disease and the initial name: Warren L. Macaulay and rhythmic paradoxical eruption, respectively.(AU)