A purpuric eruption may be an unusual early manifestation of mycosis fungoides (MF). On the other hand, persistent pigmented purpuric dermatoses (PPPD) may, occasionally, evolve to cutaneous T-cell lymphoma. Coexistence of these two conditions has been reported, but it is extremely rare. We present the case of an elderly woman with a long-standing pruritic, pigmented purpuric eruption. On 1-year follow-up, histological features suggesting early MF were observed and molecular analysis of the rearrangement of T-cell receptor genes revealed clonality. Our patient may represent a case of PPPD evolving to MF, a case of MF clinically featuring PPPD, or an intermediate condition in a nosological continuity extending from PPPD to MF. A persistent pigmented purpuric eruption may rarely be a harbinger of cutaneous T-cell lymphoma. Therefore, vigilant long-term follow-up of PPPD is highly recommended.
Background: Among rheumatic disease-related lung disease, interstitial lung disease (ILD) is the most prevalent and contributing to the mortality and morbidity. Increasing number of recent reports dealing with ILD of rheumatoid arthritis (RA), Sjögren's syndrome (pSS) and systemic sclerosis (SSc), multidisciplinary discussions (MDDs) have been growing interest for diagnostic accuracy with dedicated service. There was little evidence indicating that multidisciplinary outpatient clinic resulted in improvements in clinical outcomes including satisfaction and belief. Objectives: We have designed validated a scale of evaluating coping strategies about satisfaction and belief in patient of ILD and rheumatic disease with multidisciplinary approach. Methods: From December 2015 to September 2016, we evaluated 20 patients of rheumatoid disease with ILD and 20 patients of idiopathic pulmonary fibrosis. Patient perceptions of illness, treatment beliefs, and moods were measured via the multiple choice questionnaires presenting brief Illness perception questionnaire, beliefs about medicines questionnaire, and patient health questionnaire 2 for comparing the effectiveness of MDDs and routine ILD management. Results: In univariate analysis, beliefs in necessity and concerns of medication differed significantly high in multidisciplinary outpatient clinic for people with ILD (intentional or unintentional). When controlling for other factors that may impact medication nonadherence, more belief in necessity of medication and greater positive emotional response to disease were presented in multidisciplinary outpatient clinic for people with ILD (OR 1.51, CI 1.01-1.82). Conclusions: The MDD including rheumatologist and pulmonologist allows a satisfactory management comparing routine ILD management. It showed better coping improvements about the emotional distress, pain, and beliefs about treatment. Further research to investigate long-term clinical outcomes of multidisciplinary outpatient clinic for people with ILD is required, overlying the enhancement of mutual communication. References: [1] Vliet Vlieland TP. Multidisciplinary team care and outcomes in rheumatoid arthritis.
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