Bullous systemic lupus erythematosus in a patient with human immunodeficiency virus infection: a paradox of autoimmunity and immunodeficiency

Bullous lupus erythematosus is a rare variant of systemic lupus erythematosus (SLE) and is characterized by autoantibodies to type VII collagen.  Co-existence of SLE and human immunodeficiency virus (HIV) infection is extremely rare; the development of bullous lupus in the setting of HIV has been, to our knowledge, reported in the literature only once.We describe a 26-year-old man with an 8-year history of HIV infection who developed bullous SLE.  The patient presented with widespread, tense bullae as well as oral ulcerations.  Clinical, laboratory, histological, and cutaneous immunofluorescence findings confirmed the diagnosis of bullous SLE. Given the immunological consequence of HIV infection, the co-occurrence of these two diseases would, theoretically, be unusual. Theories pertaining to the interplay of immunologic mechanisms of the seemingly paradoxical occurrence of autoimmunity in the setting of HIV infection are discussed

Bullous systemic lupus erythematosus in a patient with human immunodeficiency virus infection: a paradox of autoimmunity and immunodeficiency

Bullous lupus erythematosus is a rare variant of systemic lupus erythematosus (SLE) and is characterized by autoantibodies to type VII collagen.  Co-existence of SLE and human immunodeficiency virus (HIV) infection is extremely rare; the development of bullous lupus in the setting of HIV has been, to our knowledge, reported in the literature only once.We describe a 26-year-old man with an 8-year history of HIV infection who developed bullous SLE.  The patient presented with widespread, tense bullae as well as oral ulcerations.  Clinical, laboratory, histological, and cutaneous immunofluorescence findings confirmed the diagnosis of bullous SLE. Given the immunological consequence of HIV infection, the co-occurrence of these two diseases would, theoretically, be unusual. Theories pertaining to the interplay of immunologic mechanisms of the seemingly paradoxical occurrence of autoimmunity in the setting of HIV infection are discussed

Hodgkin lymphoma presenting as generalized pruritus in an adolescent.

Pruritus is a common manifestation of Hodgkin lymphoma (HL), and given its high frequency, inclusion of itching as a B symptom of HL has been proposed. We present a 16-year-old adolescent boy with treatment-refractory eczema of 2 years\u27 duration. Physical examination revealed a thin adolescent boy with widespread excoriations, but no eczematous or primary cutaneous lesions were identifiable. Lymph node examination revealed palpably enlarged nodes in the cervical and supraclavicular regions. Laboratory studies revealed leukocytosis and an elevated lactate dehydrogenase level. Diffuse lymphadenopathy was detected on a chest radiograph, and excisional lymph node biopsy revealed HL (nodular sclerosing subtype). The patient was classified as HL stage IIIB (Ann Arbor staging classification) after further evaluation. Chemotherapy was initiated followed by radiation therapy. The patient\u27s pruritus markedly improved within 2 cycles of chemotherapy; however, his HL relapsed and additional salvage combination chemotherapy followed by high-dose chemotherapy and autologous stem cell transplant were required. This case underscores the need for a complete history as well as a careful skin and systemic evaluation in patients presenting with long-term pruritus, including children and adolescents

A Case of Syringolymphoid Hyperplasia with Follicular Mucinosis

Syringolymphoid hyperplasia (SLH) is an extremely rare histopathological entity with fewer than 40 cases reported in the literature. SLH have been seen as both benign lesions and in association with T-cell lymphoproliferative lesions. A 20-year-old male presented with a solitary, infiltrated plaque on the left cheek initially diagnosed as a sebaceous carcinoma at an external institution. A repeat biopsy demonstrated prominent follicular mucinosis (FM), squamous metaplasia of the eccrine coils, and a moderately dense perieccrine lymphocytic infiltrate mimicking eccrine carcinoma. The lesion was subsequently diagnosed as SLH with associated FM, an entity that has been previously reported in 12 cases, including this current case. This case highlights the characteristic features of a rare entity, emphasizes the potential for misdiagnosis of SLH, and adds to the current series of SLH described in the literature

Clinical end points and response criteria in mycosis fungoides and sezary syndrome: a consensus statement of the international society for cutaneous lymphomas, the United States cutaneous lymphoma consortium, and the cutaneous lymphoma task force of the European organisation for research and treatment of cancer

Mycosis fungoides (MF) and Sézary syndrome (SS), the major forms of cutaneous T-cell lymphoma, have unique characteristics that distinguish them from other types of non-Hodgkin's lymphomas. Clinical trials in MF/SS have suffered from a lack of standardization in evaluation, staging, assessment, end points, and response criteria. Recently defined criteria for the diagnosis of early MF, guidelines for initial evaluation, and revised staging and classification criteria for MF and SS now offer the potential for uniform staging of patients enrolled in clinical trials for MF/SS. This article presents consensus recommendations for the general conduct of clinical trials of patients with MF/SS as well as methods for standardized assessment of potential disease manifestations in skin, lymph nodes, blood, and visceral organs, and definition of end points and response criteria. These guidelines should facilitate collaboration among investigators and collation of data from sponsor-generated or investigator-initiated clinical trials involving patients with MF or SS