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Background: Angiocentric cutaneous T-cell lymphomas of childhood (ACTCLC) are an unusual type of T-cell lymphomas that present with a vesiculopapular eruption mimicking hydroa vacciniforme. Most patients have been children from Asia and Latin America., Objective: The purpose of this study was to describe four cases of ACTCLC; to discuss its clinical, histopathologic, and immunohistochemical features; to consider its possible relationship to the Epstein-Barr virus (EBV); and to clarify its classification within the spectrum of angiocentric lymphomas., Methods: The clinical, histopathologic, and immunohistochemical features of four cases of ACTCLC were identified and analyzed. In addition in situ hybridization for EBV was performed in all cases., Results: The clinical features were similar to previous cases reported under different names, such as hydroa-like lymphomas, edematous, scarring vasculitic panniculitis. Histologically, all showed angiocentric infiltrates composed mainly of T cells. In all cases there were variable numbers of CD30+ cells. The EBV was present in three of the cases., Conclusion: ACTCLC is a distinct type of T-cell lymphoma. It affects mainly children, and the EBV appears to play a role in the pathogenesis of this disease.

Background: Follicular mycosis fungoides (FMF) is a recently described variant of cutaneous T-cell lymphoma, histopathologically characterized by infiltrates of atypical CD4+ T lymphocytes around and within the epithelium of the hair follicles (folliculotropism). To date, only four cases of FMF have been reported., Objective: Our goal was to identify cases of FMF and compare them with those previously reported., Methods: The clinical and histopathologic features of three cases of FMF were analyzed. In addition, in one case immunohistochemical analysis in paraffin-embedded and frozen tissue as well as genotypic studies were performed., Results: The three reported cases showed typical histologic findings of FMF, namely, perifollicular and intrafollicular infiltrates of atypical lymphocytes, in the absence of either epidermotropism or follicular mucinosis. Clinically, the lesions were similar to classic MF, but also contained follicular papules. Immunohistochemistry and gene rearrangement studies showed findings similar to classic MF., Conclusion: FMF is a rare variant of cutaneous T-cell lymphoma. Including our three cases, only seven patients have been reported. We hypothesize that the follicular epithelium in patients with FMF may express increased levels of skin-selective homing receptors and adhesion molecules. These may be involved in the induction of the folliculotropism of atypical lymphocytes.

Primitive neuroectodermal tumors are rare, highly aggressive neoplasms that affect both sexes and occur in all age groups. They are a large group of neoplasms with neuroepithelial differentiation, including cutaneous neuroblastomas. Histopathologically they are characterized by a rather uniform population of small, dark cells, with or without Homer Wright rosettes. Immunohistochemically they stain for one or more of the reputed neural markers. Ultrastructurally they show interdigitating processes containing neurosecretory granules, intermediate filaments, and microtubules. We describe a patient with unusual multiple peripheral neuroectodermal tumors localized to the skin, with benign biologic behavior. The patient related the appearance of new lesions for several years without evidence of visceral dissemination or systemic complications. The histopathologic, immunohistochemical, and ultrastructural findings support the diagnosis of a peripheral neuroectodermal tumor.