Mycosis Fungoides and Granulomatous Slack Skin: A Single Entity With Distinct Clinical Phenotypes.

Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in the WHO classification as a subtype of mycosis fungoides (MFs). 1 These overlapping entities have shared clinical and histopathologic features which can present a diagnostic challenge. The dominance of the granulomatous infiltrate and the often sparse lymphocytic infiltrate frequently with minimal cytological atypia are features that distract from the correct diagnosis, even when raised by the clinician.

Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoproliferative Disorder or Primary Cutaneous Marginal Zone B-Cell Lymphoma? Two Distinct Entities With Overlapping Histopathological Features.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder and primary cutaneous marginal zone B-cell lymphoma are 2 distinct entities with several overlapping features which can result in diagnostic uncertainty. Clinically, they both follow an indolent course and present with solitary or multiple papules or nodules. Histologically, they are characterized by polymorphous dermal infiltrates rich in mixed populations of B cells and T cells, often in similar proportions.

Prominent Blasts in Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoproliferative Disorder. A Reconsideration of Diagnostic Criteria.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-LPD), recently downgraded from a T-cell lymphoma, is a poorly characterized histopathological entity. Presenting as a solitary lesion that often grows rapidly, it may raise suspicion for a cutaneous B-cell lymphoma. However, classically, the dermal lymphoid proliferation is predominantly CD4+ with a follicular T-helper profile and a smaller B-cell fraction.

Management of skin cancer in recipients of solid organ transplants.

Recent improvements in post-transplant care have led to an increased life expectancy for recipients of organ transplants. These patients require lifelong immunosuppression, which is associated with an increased incidence of malignant disease. Skin cancers are the most common malignancies seen in recipients of organ transplants and are associated with significant morbidity and mortality.

The Results of Low-Dose Total Skin Electron Beam Radiation Therapy (TSEB) in Patients With Mycosis Fungoides From the UK Cutaneous Lymphoma Group.

Purpose: Total skin electron beam radiation therapy (TSEB) is a very effective treatment of mycosis fungoides. Following reports of similar durations of response to lower doses of TSEB, a low-dose schedule of TSEB was introduced in the United Kingdom.

Methods And Materials: A protocol of 12 Gy in 8 fractions over a period of 2 weeks was agreed on by use of the Stanford University technique.

Chromosomal anomalies in primary cutaneous follicle center cell lymphoma do not portend a poor prognosis.

Background: The t(14;18)(q32;q21) chromosomal translocation is found in the majority of nodal follicular lymphomas but only rarely in primary cutaneous follicle center cell lymphomas (PCFCL). Recent studies have postulated that the translocation is more prevalent in PCFCL than previously described and that it might be a molecular prognostic marker.

Objectives: The purpose of our study was to analyze cases of PCFCL for the presence of a t(14;18) translocation using fluorescence in situ hybridization to detect balanced translocations involving either the BCL2 or MALT1 loci and to correlate the results with growth pattern, immunophenotype, and clinical outcome.

Results of a 5-week schedule of modern total skin electron beam radiation therapy.

Purpose: To report the outcomes of a 5-week schedule of total skin electron beam radiation therapy (TSEB) for mycosis fungoides (MF).

Methods: Over 5 years, 41 patients with confirmed MF were treated with a modern TSEB technique delivering 30 Gy in 20 fractions over 5 weeks to the whole skin surface. Data were collected prospectively and entered into the skin tumor unit research database.

Solitary mycosis fungoides: a distinct clinicopathologic entity with a good prognosis: a series of 15 cases and literature review.

Background: Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma (CTCL), accounting for almost 50% of all primary cutaneous lymphomas. The occurrence of solitary lesions, which are clinically and histopathologically indistinguishable from classic MF has been described.

Objective: We describe 15 cases of solitary MF and discuss the relationship to classic MF, "reactive" processes and to other, rarer forms of CTCL that may present with solitary lesions.

Expanding the spectrum of frontal fibrosing alopecia: a unifying concept.

Background: In frontal fibrosing alopecia (FFA), scalp alopecia dominates the clinical picture. However, eyebrow loss and hair loss in other body sites may also occur; this has been documented clinically, but rarely histopathologically. We describe the clinicopathological findings of 13 cases of FFA, with histopathologic data from the scalp, eyebrow, and body hair.

Cutaneous collagenous vasculopathy with generalized telangiectasia in two female patients.

Cutaneous collagenous vasculopathy is characterized by generalized cutaneous telangiectasia and unique microscopic and ultrastructural vascular changes, consisting of marked collagen deposition within the vascular walls of the post-capillary venules in the superficial dermis. There are only 4 previous cases described in the medical literature, all in males, mostly middle-aged. We have recently seen two female patients with clinical and histopathologic features diagnostic of cutaneous collagenous vasculopathy, indicating that it is not restricted to males.

Downregulation of Fas gene expression in Sézary syndrome is associated with promoter hypermethylation.

Sézary Syndrome (SS) is an aggressive leukemic variant of primary cutaneous T-cell lymphoma characterized by the presence of tumor or Sézary cells that generally display a mature memory T-cell immunophenotype. Sézary cells proliferate poorly and therefore their accumulation may be due to defective T-cell homeostasis involving resistance to apoptosis. In this study, we analyzed Fas expression in CD4+ lymphocytes at the mRNA and protein levels in a large cohort of SS patients as compared with healthy controls.

Congenital anetoderma in a preterm infant.

Isolated, small lesions of anetoderma presenting at birth have been reported in twins born at 25 weeks of gestational age. We report the first case of widespread congenital anetoderma occurring in an infant born at 24 weeks of gestation weighing 640 g and discuss the potential factors in its etiology.

Genital herpes masquerading as a cutaneous T-cell lymphoma: a report of two cases.

Genital herpes simplex virus (HSV) infection is usually a straightforward clinical diagnosis, rarely requiring histological confirmation. We report two cases of immunosuppressed patients in which the clinical and pathological features were initially suspicious for cutaneous lymphoma with a T-cell clone detected in one case. A diagnosis of HSV infection was eventually made on the basis of histological features and confirmed with immunohistochemistry.

Three cases of lymphomatoid papulosis with a CD56+ immunophenotype.

We report 3 cases of lymphomatoid papulosis (LyP) with a CD56+, cytotoxic immunophenotype. All 3 patients presented with clinical histories typical of LyP, with one patient having associated mycosis fungoides. Histologically, two cases were type A LyP and one was type B.

Mycosis fungoides with a CD56+ immunophenotype.

We report 3 cases of mycosis fungoides (MF) with a CD56+ cytotoxic immunophenotype. Each patient presented with a different clinical phenotype: one exhibited limited poikilodermatous patches (skin stage T1); one, widespread hypopigmented lesions (skin stage T2); and one, poikiloderma with a single cutaneous tumor (skin stage T3). MF was confirmed both histologically and by the presence of a T-cell receptor clone in lesional skin in all cases.

Fine mapping of chromosome 10q deletions in mycosis fungoides and sezary syndrome: identification of two discrete regions of deletion at 10q23.33-24.1 and 10q24.33-25.1.

Previous cytogenetic studies in mycosis fungoides (MF) and Sezary syndrome (SS) have identified a large and poorly defined area of chromosomal deletion on chromosome 10q. We report an extensive fine-mapping allelotyping study using 19 microsatellite markers in the region 10q22.3-10q26.

Outcome in 34 patients with juvenile-onset mycosis fungoides: a clinical, immunophenotypic, and molecular study.

Background: Mycosis fungoides (MF) is predominantly a disease of older patients, but occasionally occurs in children. The aims of the current study were to describe the clinical presentation, pathologic features, and disease progression (DP) in patients who developed MF before age 16 years.

Methods: A retrospective study was performed.