Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: a case report

Abstract Background Paraneoplastic pemphigus is a rare multiorgan disease of autoimmune causes, usually triggered by neoplasias, mainly of lymphoproliferative origin, such as leukemia and lymphoma. This disorder is categorized by the presence of autoantibodies that react against proteins, such as desmoplakins, desmogleins, desmocollins, and others that exist in cellular junctions. Paraneoplastic pemphigus can manifest clinically in a variety of ways, ranging from mucositis to lesions involving the skin and pulmonary changes. The diagnosis depends on the correlation between the clinical and histopathologic evaluations. Currently, the treatment of this disease is still very difficult and ineffective. The prognosis is poor, and the mortality rate is very high. Case presentation We report a case of a Caucasian patient who had chronic lymphocytic leukemia and developed paraneoplastic pemphigus with severe impairment of skin and mucosa. The initial diagnostic hypothesis was Stevens-Johnson syndrome. The histopathological examination of the skin biopsy was compatible with paraneoplastic pemphigus, and the definitive diagnosis was made on the basis of clinical-pathological correlation. Conclusions With the presence of multiorgan lesions in patients with lymphoproliferative neoplasia, paraneoplastic pemphigus should always be considered among the possible diagnostic hypotheses, because diagnosis and early treatment may allow a better prognosis for the patient

The Brazilian version of Skindex-16 is a valid and reliable instrument to assess the health-related quality of life of patients with skin diseases

<div><p>Introduction</p><p>The aim of this study was to assess the psychometric properties of the Brazilian version of Skindex-16 in patients with various skin diseases.</p><p>Methods</p><p>Dermatologic assessments were performed for the diagnosis and classification of the severity of skin conditions. The clinical feasibility of Skindex-16 was assessed based on the time required to complete the questionnaire and the number of unanswered items. The participants (n = 110) answered the Hospital Anxiety and Depression Scale (HADS), the Dermatology Life Quality Index (DLQI) and the Skindex-16 (Portuguese/Brazil version) questionnaires. Convergent validity was assessed based on the correlation of the Skindex-16 with the DLQI and HADS subscales. Known-groups validity was assessed based on the comparison of the mild, moderate and severe disease groups using the Kruskal-Wallis test. Internal consistency was assessed using Cronbach’s alpha and test-retest reproducibility using the intraclass correlation coefficient (ICC) obtained with 29 participants who answered the Skindex-16 a second time 3 to 10 days after the first assessment.</p><p>Results</p><p>The mean time to answer the questionnaire was 2 min 41 sec. Cronbach’s alpha scores were 0.867, 0.930 and 0.888 for the Skindex-16 domains symptoms, emotions and functioning, respectively. The ICCs were 0.947, 0.860 and 0.843 for the Skindex-16 domains symptoms, emotions and functioning, respectively. All three Skindex-16 scales exhibited strong correlations with DLQI. Moderate correlations were found between HADS subscales and the Skindex-16 emotions domain. Known-groups validity showed differences in all three Skindex-16 domains between the mild and moderate skin disease groups (emotions: p < 0.001; symptoms: p = 0.049; functioning: p < 0.001) and between the mild and severe skin disease groups (emotions: p = 0.002; symptoms: p = 0.001; functioning: p = 0.002).</p><p>Conclusion</p><p>The Portuguese/Brazil version of Skindex-16 is a valid and reliable instrument to assess the quality of life of patients with skin diseases.</p></div

Known-groups validation analyses of Skindex-16.

<p>Known-groups validation analyses of Skindex-16.</p

Convergent analyses between Skindex-16, HADS and DLQI.

<p>Convergent analyses between Skindex-16, HADS and DLQI.</p