C utaneous T-cell lymphoma (CTCL) is the general term used to describe extracutaneous non-Hodgkin's lymphomas characterized by monoclonal expansion of skin-homing malignant T cells that can further be defined by the use of immunophenotypic markers and the presence of specific rearrangements of the T-cell receptor.' The most frequently diagnosed form of CTCL is mycosis fungoides (MF) and its leukemic variant, Sezary syndrome (SS).2 MF most often presents as chronic eczematous or psoriasiform patches or plaques and can remain stable for many years. In some patients, MF progresses to involve lymph nodes, peripheral blood, bone marrow, and visceral organs.' SS may present de novo with persistent exfoliative erythroderma accompanied by staphylococcal colonization, perivascular atypical lymphocytes in the dermis, and ;:::1000 atypical T lymphocytes/mm' in the blood, a criterion recently defined as a B2 blood rating by the International Society for Cutaneous Lymphomas.Y Extracorporeal photopheresis (ECP) combines blood apheresis with ultraviolet-A irradiation of psoralen-photosensitized peripheral blood mononuclear cells. ECP was first introduced in 1987 for the treatment of erythrodermic MF and SS. ECP was the first therapy to receive FDA approval for the treatment of CTCL based on a multicenter trial. 6 Twenty-seven of 37 patients (76%) achieved a 25%-100% reduction in their erythroderma skin score. The Yale-New Haven Hospital experience has been reviewed by Heald et at.7 who treated 32 patients with ECP. Of 19 erythrodermic patients who were treated with ECP as their first therapy at Yale, five had greater than 75% improvement of their skin score, ten had 25%-50% improvement, and four had less than 25% improvement. The majority of patients had improved quality of life. Heald et al. 8 also evaluated long-term followup in 29 original CTCL patients in the original multicenter study. Their mean survival from the time of diagnosis was 60.3 months and from beginning of therapy it was 47.9 months, which exceeds their expected mean overall survival of 24-30 months reported in the litera
Acral erythema has been associated with a number of chemotherapeutic agents, but there are no reports of clofarabine-induced acral erythema in the literature. We describe two patients who developed acral erythema after receiving clofarabine. One patient had myelodysplastic syndrome while the other had acute lymphoblastic leukemia.
We report the case of a 21-year-old white male with an ectopic sebaceous gland and duct located within the bulb of an anagen hair follicle. This is an incidental finding in one of two biopsies from his chin, performed as part of the workup for a recalcitrant perioral acneiform eruption. The embryogenesis and development of sebaceous glands in human scalp hair follicles are reviewed. To our knowledge, this is the first report of such a developmental anomaly.
A 38‐year‐old woman presented with nodules on the face, scalp, ears, hands and elbows. She also developed fevers, chills, fatigue, weakness, myalgia, joint pain, nausea and weight loss of 40 pounds in eight months. Histology revealed large histiocytes with smooth eosinophilic ground glass cytoplasm, consistent with multicentric reticulohistiocytosis. She was treated with methotrexate 25 mg IM per week and prednisone 10 mg PO QD, with relief of her symptoms and improvement in her skin. Multicentric reticulohistiocytosis is a rare histiocytic systemic disease, with facial and hand skin nodules and symmetric arthritis. Pathognomonic signs include “coral beads” and vermicular erythematous papules bordering nostrils. The histologic hallmark for this disease is a nodular infiltrate of histiocytic cells, including multinucleated giant cells containing an eosinophilic, ground glass cytoplasm. Immunohistiochemistry is positive for CD68 and CD3, and negative for B cell markers as well as Langerhans cell markers. Approximately twenty percent of patients with multicentric reticulohistiocytosis have concurrent malignancy, but there is no one predominant type. The disease usually waxes and wanes for many years, with 30% of patients developing arthritis mutilans. It often regresses approximately seven years after its first appearance.
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