Objective SSc and localized sclerosis (LoS) are considered clinically distinct entities. We describe herein the coexistence of SSc and LoS by both a systematic literature review and an observational cohort study of unselected SSc patients. Methods Original studies documenting the coexistence of SSc and LoS were identified in three electronic databases by means of a systematic literature search according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Additionally, the coexistence of SSc and LoS was studied in a prospective cohort of SSc patients visiting the Ghent University Scleroderma Unit for their yearly follow-up visit between January 2018 and January 2019. Results Five studies were finally included for quality appraisal and data extraction. The coexistence of SSc and LoS ranged between 2.4 and 7.4%. RP, scleroderma pattern on nailfold videocapillaroscopy (NVC) and the presence of SSc-specific antibodies were commonly observed in coexistent cases. Additionally, coexistence of SSc and LoS was found in 8/296 (2.7%) consecutive SSc patients of the Ghent University Scleroderma Unit. RP was present in 6/8 coexistent cases; a scleroderma pattern on NVC was observed in all coexistent cases, and SSc-specific antibodies (i.e. cenp-B) were found in 4/8 coexistent cases. Conclusion This is the first systematic literature review with additional cohort evaluation investigating the coexistence of SSc and LoS. A relatively high overlap of SSc and LoS was revealed, which is peculiar because both are rare diseases.

Introduction Some cutaneous manifestations can be the first presentation of an underlying malignancy. These so-called paraneoplastic syndromes can sometimes be very subtle or strongly resemble other benign cutaneous diseases. Aims In this report, we want to emphasize the need for further investigation of eczema-like cutaneous presentations which develop at a later age and are recalcitrant to therapy. Exclusion of an underlying malignancy needs to be considered. Patients and Methods A 53-year-old man with thickening of the skin, more pronounced on the hands, feet and face, was diagnosed with classic nodular sclerosing Hodgkin disease based on cutaneous presentation. Results After two rounds of chemotherapy (adriamycin, bleomycin, vinblastine and dacarbazine), the cutaneous symptoms had disappeared. After six rounds of chemotherapy and 14 months of follow-up, the patient is still in remission without recurrence of the cutaneous symptoms. Conclusions Cutaneous symptoms recalcitrant to adequate treatment should raise suspicion and prompt further investigation to exclude an underlying malignancy. A multidisciplinary approach with the dermatology department can accelerate diagnosis and improve the patient’s prognosis. LEARNING POINTS Skin diseases can reflect internal manifestations or diseases. Vigilance, timely recognition and a multidisciplinary approach are important in a patient with atypical cutaneous manifestations. A paraneoplastic skin syndrome should be considered in persistent skin disease not responding to treatment and starting in middle-aged patients with B symptoms.

Objectives: Fresh frozen plasma (FFP) has been used in angioedema crises, however there is a risk of aggravating the symptoms as well as transmitting infections. In this report, the authors emphasize the dangers of this therapy. Materials and methods:A 25-year-old woman with hereditary angioedema (HAE) was treated with FFP after which her symptoms escalated. Results: Administration of purified C1-inhibitor (C1-INH) resulted in relief of her symptoms. Conclusions: FFP is to be avoided in a HAE crisis. Newer therapies for angioedema are preferred.LEARNING POINTS • Angioedema should be considered when sudden onset facial edema is not associated with urticaria or pruritus, and does not improve with glucocorticoids and H1-antihistamines.• Fresh frozen plasma (FFP) is to be avoided in HAE crises as it can aggravate symptoms and carries the danger of transmitting infectious diseases; it can also induce allergic transfusion reactions, transfusion-related acute lung injury and circulatory overload.• Recombinant or human plasma-derived C1-inhibitor (C1-INH), icatibant or ecallantide are the first line treatments in hereditary angioedema attacks.