A histo-immunopathologic and prognostic study of erythrodermic cutaneous T-cell lymphoma.

BACKGROUND: Sézary syndrome (SS) and erythrodermic mycosis fungoides (E-MF) represent two expressions of erythrodermic cutaneous T-cell lymphoma (E-CTCL). METHODS: Histopathologic features were compared on skin specimens from 41 patients with SS and 70 patients with E-MF. Immunopathologic findings were compared on 42 SS and 79 E-MF specimens. RESULTS: Specimens of SS usually showed band-like dermal infiltrates with intermediate-sized lymphoid cells and few plasma cells; on the other hand E-MF more often had a perivascular infiltrative pattern, predominance of small/mixed lymphoid cells and eosinophils. SS also had lower numbers of CD8+ cells and higher numbers of CD62L+ cells compared to E-MF. For E-MF patients, the presence of large Pautrier collections, infiltrates with intermediate-sized cells, increased number of mitotic figures and ≥50% CD62L+ cells in the dermal infiltrate correlated with a relatively poor disease-specific survival. However, only the presence of mitotic figures retained prognostic significance with clinical stage as a covariate. CONCLUSIONS: Clinical stage provides the most important prognostic information for patients with E-CTCL. However, mitotic activity for E-MF and CD8+ cells <20% for SS have additional value. We hypothesize that observed differences in plasma cell and eosinophil numbers may reflect the influence of CD62L+ central memory T-cells in the microenvironment.

Sézary syndrome coexisting with B-cell chronic lymphocytic leukemia: case report and review of the literature.

INTRODUCTION: The simultaneous presentation of chronic B-cell lymphocytic leukemia (B-CLL) and cutaneous T-cell lymphoma (CTCL) is extremely rare. CASE REPORT: We describe a patient with B-CLL and Sézary syndrome (SS), an erythrodermic and leukemic variant of CTCL. Despite treatment, the SS progressed to involve internal organs and eventual death of the patient from sepsis. This is the first reported case of SS coexisting with chronic lymphocytic leukemia in which an anti-V beta 13.6 antibody was used to serially track changes in circulating neoplastic T cells vis-à-vis neoplastic B cells and to detect neoplastic T cells in ascitic fluid near the end of the patient's life. DISCUSSION: We speculate that the coexistence of B-CLL and CTCL is the result of an initiating genetic or epigenetic defect at the level of the common lymphoid stem cell that predisposes both B-cell and T-cell lineages to additional oncogenic changes at a more advanced stage of differentiation.

Epidermotropic metastatic prostate carcinoma presenting as an umbilical nodule-Sister Mary Joseph nodule.

Carcinoma of the prostate accounts for fewer than 1% of all skin metastases. Cutaneous metastases from prostate carcinoma most often involve the penis, the anterior aspect of the thighs, the suprapubic area, and the perineum, but they also have been reported in the scalp, the chest, the back, and even the face. We report an unusual case of metastatic prostate adenocarcinoma that presented as an umbilical nodule (Sister Mary Joseph nodule) and demonstrated significant epidermotropism histologically. A review of the literature has found only one documented case of prostatic carcinoma metastasizing to the umbilicus, and one other documented case of epidermotropic metastatic prostate carcinoma.

Granular parakeratotic acanthoma.

Granular parakeratosis was originally described as a dermatosis confined to the axillae that histopathologically shows characteristic findings in the cornified layer consisting of parakeratotic corneocytes housing keratohyaline granules. Since the original description of this entity, non-intertriginous examples and even incidental foci of granular parakeratotic corneocytes in association with other dermatoses have been recognized. This report details the findings of a previously unreported entity that we are naming granular parakeratotic acanthoma, which is fundamentally akin to acantholytic dyskeratotic acanthoma and epidermolytic acanthoma (ie, a solitary keratosis that histopathologically displays features indicated in its name).

Dermatophyte-related granular parakeratosis.

A patient presented with a two-month history of an annular eruption on her back. Biopsies of this process revealed dermatophytosis and concurrent granular parakeratosis. This patient's eruption resolved completely with antifungal therapy suggesting that the fungal organisms caused the granular parakeratosis.