Faculty Bibliography

We present a 46-year-old man with a greater than 15-year history of erythroderma. A definitive diagnosis has not been established. The differential diagnosis is discussed.

Lichen planus (LP) is a relatively common papulosquamous disorder that is characterized by pruritic, polygonal papules in a characteristic distribution. We present a case of a 71-year-old man with erythroderma, who was ultimately diagnosed with severe, generalized LP. Treatment of severe LP is challenging, and there are few, robust, clinical trials in the literature to guide the selection of appropriate treatment. We discuss the treatment options for generalized LP and the evidence in support of these agents.

Poikilodermatous mycosis fungoides is a rare form of cutaneous T-cell lymphoma that is characterized clinically by localized or diffuse patches, which consist of telangiectases, mottled hyper- and hypopigmentation, and atrophy. The immunophenotype of neoplastic cells is similar to that observed in classic mycosis fungoides. Therapeutic options used in poikilodermatous and classic mycosis fungoides include both skin-directed and systemic treatments. We present a case of poikilodermatous mycosis fungoides in a 53-year-old woman, who initially presented with erythroderma and who has failed multiple treatment modalities

Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma that is characterized by solitary or localized nodules or plaques. Histopathologic features include a diffuse, non-epidermotropic infiltrate with cohesive sheets of large anaplastic CD30+ tumor cells. This entity must be distinguished from systemic ALCL with cutaneous involvement and lymphomatoid papulosis. Treatment modalities include clinical monitoring, radiation therapy, and surgical excision, with systemic chemotherapy reserved for disseminated or extracutaneous disease

In this article, the management of cutaneous T-cell lymphoma will be presented in terms of the strategies that guide treatment. With the strategies and goals in mind, treatment options to achieve a measurable goal will be presented. The treatments presented in this article are those utilized to reliably achieve a remission. If remission is not achieved, a patient's management plan must be changed. The landmarks that help guide the therapy plan will be discussed

Strong evidence supports that CNS-specific CD4(+) T cells are central to the pathogenesis of multiple sclerosis and experimental autoimmune encephalomyelitis (EAE). Using a model of spontaneous EAE, we demonstrated that myelin basic protein (MBP)-specific CD4(+) T cells up-regulate activation markers in the CNS-draining cervical lymph nodes at a time when there is no T cell activation anywhere else, including the CNS, and before the appearance of clinical signs. In spontaneous EAE, the number of MBP-specific T cell numbers does not build up gradually in the CNS; instead, a swift migration of IFN-gamma-producing T cells into the CNS takes place approximately 24 h before the onset of neurological signs of EAE. Surgical excision of the cervical lymph nodes in healthy pre-EAE transgenic mice delayed the onset of EAE and resulted in a less severe disease. In EAE induced by immunization with MBP/CFA, a similar activation of T cells in the draining lymph nodes of the injection site precedes the disease. Taken together, our results suggest that peripheral activation of T cells in draining lymph nodes is an early event in the development of EAE, which paves the way for the initial burst of IFN-gamma-producing CD4(+) T cell into the CNS

A 46-year-old man presented with a 1-year history of asymptomatic papules on the right arm, without an antecedent event. Initial clinical and histopathologic features were consistent with a pseudolymphoma without gene rearrangements, and the patient was treated with intralesional glucocorticoids. Four months later, the patient developed additional papules and plaques on the right arm, and, at this time, clinical and histopathologic features were most consistent with a T-cell-rich, large B-cell lymphoma, with monoclonal immunoglobulin light chain gene rearrangement. Systemic evaluation showed no evidence of extracutaneous involvement. The transformation of a pseudolymphoma into a large B-cell lymphoma is a rare event. This patient's subtype, diffuse large B-cell lymphoma-other, carries an intermediate prognosis when compared to the more aggressive leg subtype and more indolent folliculocentric subtype. Potential therapeutic options include local radiotherapy, chemotherapy, and rituximab

A 56-year-old woman presented with small, skin-colored papules on the trunk and thighs. Histopathologic findings were consistent with papular mucinosis. Laboratory evaluation did not show an associated paraproteinemia. Treatment with topical glucocorticoids was unsuccessful. Papular mucinosis, also known as localized lichen myxedematosus, has been categorized into 4 subtypes. The discrete papular form, as seen in our patient, is characterized by a few to multiple, 2-5-mm, skin-colored, firm, waxy, dome-shaped papules on the trunk and proximal aspects of the extremities. By definition there is no associated paraproteinemia, but this form may be associated with human immunodeficiency virus infection. Focal or diffuse mucinous deposits are seen on histopathologic examination. The usual clinical course is slow cutaneous progression without spontaneous resolution. Treatment is empiric and is usually unsuccessful

A 64-year-old woman presented with 2 years of pruritic and ulcerated nodules and tumors on the trunk and arms. Histopathologic examination showed a diffuse infiltrate that consisted of predominantly small lymphocytes and scattered large atypical multinucleated cells positive for CD30. These findings were consistent with a diagnosis of anaplastic large-cell T-cell lymphoma, which is a CD30+ cutaneous lymphoma. This case highlights the importance of considering both histopathologic and clinical criteria in diagnosing a patient with a CD30+ cutaneous lymphoma