Papulosis linfomatoide. Presentación de un caso / Lymphomatoid papulosis. Report of a case

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).

Frequency and Risk Factors for Associated Lymphomas in Patients With Lymphomatoid Papulosis.

BACKGROUND: Lymphomatoid papulosis (LyP) is classified as an indolent cutaneous lymphoma, but outcome dramatically worsens if LyP is associated with lymphoma. The frequency of this association remains unclear in the literature. Here, we assess the frequency and risk factors of association between LyP and another lymphoma in an 11-year retrospective study conducted in 8 dermatology departments belonging to the French Study Group on Cutaneous Lymphoma (FSGCL). PATIENTS AND METHODS: Patients with LyP were identified and data extracted from the FSGCL registry between 1991 and 2006. Patients were followed up to January 2014. Age, sex, number of skin lesions, histologic subtype, and genotype were recorded at baseline. Risk factors were determined using univariate and multivariate analysis. Cumulative probability of association was calculated using the Kaplan-Meier method. RESULTS: We observed 52 cases of lymphomas (cutaneous, n = 38; systemic, n = 14) in 44 of 106 patients (41%). Lymphoma diagnosis was concomitant with or prior to LyP diagnosis in 31 cases and occurred during the course of LyP in 21 cases (cutaneous, n = 14; systemic, n = 7; median delay: 5 years; interquartile range: 1.5-7 years). In multivariate analysis, main prognostic factors for association between LyP and another lymphoma were older age (odds ratio [OR]: 1.05 per year; 95% confidence interval [CI]: 1.01-1.08; p = .011) and presence of a T-cell clone in LyP lesions (OR: 7.55; 95% CI: 2.18-26.18; p = .001). CONCLUSION: Older age and presence of a T-cell clone in LyP lesions are risk factors for associated lymphomas in patients with LyP. These findings should help to identify patients who require close management in clinical practice. IMPLICATIONS FOR PRACTICE: The management of lymphomatoid papulosis (LyP) is that of an indolent cutaneous lymphoma, based on its excellent prognosis. However, this good prognosis is altered if LyP is associated with lymphoma. Furthermore, risk factors for and frequency of this association remain unclear in the literature. The results presented here demonstrate a high rate of association between LyP and other lymphomas (41%) as well as a long median delay of occurrence (5 years), which emphasizes the need for prolonged follow-up of patients with LyP. Moreover, two main risk factors (i.e., older age and presence of a T-cell clone in LyP lesions) are highlighted, which should help clinical practitioners to identify patients who require close management.

Lymphomatoid papulosis in a patient with Waldenström's macroglobulinemia.

Lymphomatoid papulosis (LyP) is a rare skin disease with a benign course but a malignant histologic appearance based on atypical lymphocytes. Waldenström's macroglobulinemia (WM) is a diffuse infiltration of the bone marrow by cells that synthesize IgM immunoglobulins. A 46-year-old female presented with a two year history of weakness, fatigue, anemia, and recurring multiple erythematous papules and nodules in the skin. The skin biopsy showed pleomorphic lymphoid cells with atypical mitoses permeated by a diffuse dermal infiltrate of normal appearing lymphocytes. The neoplastic cells were positive for CD30, CD3, and CD5 but negative for CD20 and EMA. The bone marrow was hypercellular due to a diffuse infiltration by lymphocytes, plasma cells, and plasmacytoid lymphocytes. LyP is a lymphoproliferative disorder of CD30-positive T cells that may be associated with other lymphoid malignancies, particularly Hodgkin's disease, mycosis fungoides, and anaplastic T cell lymphomas. To our knowledge, this is the first report of a LyP appearing in a patient with WM.

Lymphomatoid papulosis

Lymphomatoid papulosis is a chronic disease of cutaneous lymphoid infiltration characterized clinically by involuting and recurring papules, plaques, and nodules. The intriguing combination of a usually benign clinical course, a cytologically malignant lymphoid infiltrate on histologic examination, and a clear, but sporadic association with extracutaneous lymphomas has stimulated significant investigation. Application of recent technical advances to research in lymphomatoid papulosis prompts this review.