Cutaneous Involvement by Refractory Celiac Disease Type 2 Histologically Mimicking Mycosis Fungoides.

ABSTRACT: Refractory celiac disease (RCD) is a rare condition characterized by persistent malabsorptive symptoms and villous atrophy despite a gluten-free diet. While RCD type 1 has a normal intraepithelial lymphocyte phenotype, RCD type 2 is defined by the presence of immunophenotypically aberrant and monoclonal intraepithelial T lymphocytes, with a high propensity to transform to enteropathy-associated T-cell lymphoma (EATL). Although dermatological manifestations of celiac disease are common, presentation with cutaneous involvement by abnormal lymphocytes of RCD type 2 or EATL is rare, with few histologic descriptions in the literature. We describe the case of a 66-year-old man with a history of celiac disease presenting with a generalized, erythematous papular rash over his torso, upper arms, and legs. Biopsy of his skin lesions showed prominent hyperkeratosis with underlying spongiosis and interface change. Increased intraepithelial (epidermotropic) lymphocytes were observed, out of proportion to the level of spongiosis, but not overly atypical in appearance. Immunohistochemistry revealed an aberrant T-cell immunophenotype (CD3/2/7 positive; CD5/4/8 negative), raising suspicion for a cutaneous T-cell lymphoproliferative disorder. A duodenal biopsy demonstrated total villous atrophy with a morphologically bland population of epitheliotropic T lymphocytes showing the same aberrant immunophenotype. Similar cells were also identified by flow cytometry in the peripheral blood. In conjunction with the history of celiac disease, a diagnosis of RCD type 2 or 'EATL in situ' with cutaneous involvement was made. Cutaneous RCD type 2 or EATL should be considered as differential diagnoses in patients with a history of celiac disease and histopathology reminiscent of epidermotropic forms of cutaneous T-cell lymphoma.

Life and treatment goals of individuals hospitalized for first-episode nonaffective psychosis.

First-episode psychosis typically emerges during late adolescence or young adulthood, interrupting achievement of crucial educational, occupational, and social milestones. Recovery-oriented approaches to treatment may be particularly applicable to this critical phase of the illness, but more research is needed on the life and treatment goals of individuals at this stage. Open-ended questions were used to elicit life and treatment goals from a sample of 100 people hospitalized for first-episode psychosis in an urban, public-sector setting in the southeastern United States. Employment, education, relationships, housing, health, and transportation were the most frequently stated life goals. When asked about treatment goals, participants' responses included wanting medication management, reducing troubling symptoms, a desire to simply be well, engaging in counseling, and attending to their physical health. In response to queries about specific services, most indicated a desire for both vocational and educational services, as well as assistance with symptoms and drug abuse. These findings are interpreted and discussed in light of emerging or recently advanced treatment paradigms-recovery and empowerment, shared decision-making, community and social reintegration, and phase-specific psychosocial treatment. Integration of these paradigms would likely promote recovery-oriented tailoring of early psychosocial interventions, such as supported employment and supported education, for first-episode psychosis.