We report two cases of cutaneous granuloma induced by anti-TNF-alpha therapy: a 47-year-old man suffering from psoriatic arthritis treated with infliximab and a 56-year-old woman treated with ...adalimumab for polyarticular juvenile rheumatoid arthritis. The biospies confirmed the diagnosis of a 'sarcoidosis-like' reaction. No systemic involvement was observed. Such cases of noninfectious granulomatous diseases occurring during anti-TNF-alpha therapy are becoming increasingly frequent.
Summary
Variations in the clinical and histological presentation of cutaneous T‐cell lymphoma (CTCL) can hamper diagnosis. We report two cases of a novel presentation of CTCL characterized by an ...aberrant immunophenotype with complete loss of pan T‐cell antigens including T‐cell receptor β chain and showing the clinical and histopathological appearance of erythrodermic and plaque‐stage mycosis fungoides.
What's already known about this topic?
Phenotypic aberrations with loss of some surface antigens are common findings in malignant cutaneous T‐cell lymphomas.
The almost complete absence of T‐cell marker expression in mycosis fungoides has never been reported previously.
What does this study add?
We describe two original observations of cutaneous T‐cell lymphoma characterized by an aberrant immunophenotype with loss of pan T‐cell antigens including the T‐cell receptor β chain.
Lymphoma behaviour and evolution does not appear to be modified in these cases.
Summary
Background
Genetics discoveries have allowed for a better understanding of capillary malformations (CMs) associated with overgrowth syndrome. However, molecular analyses are still not easy to ...perform or interpret. Other analytical methods are needed.
Objectives
To identify clinical and haemodynamic factors associated with leg length discrepancy (LLD) in children with CMs of the lower limbs.
Methods
Data were obtained from the multicentre French national cohort CONAPE (COhorte Nationale d'enfants atteints d'Angiome Plan de membrE inférieur), from children aged 2–12 years old with CMs of the lower limbs. Clinical characteristics were prospectively collected. Haemodynamic factors were measured by an sonographer who calculated the arterial blood flow (ABF) in both lower limbs. An ABF difference ≥ 50% between the two lower limbs was considered relevant. LLD ≥ 2% was determined by the same radiologist on centralized radiographs.
Results
We analysed data at baseline for 96 children. The mean ± SD age was 5·6 ± 3·1 years; 49 (51%) were male; and 14 (15%) showed LLD. In total, 32 patients (33%) had venous anomalies, 13 (14%) lymphatic anomalies and in one child a diagnosis of Parkes Weber syndrome was made. Only an increased circumference above the knee was more frequent with than without LLD (43% vs. 13%, P = 0·02). In all, 10/79 patients (13%) showed a difference in ABF ≥ 50%: four had LLD. The frequency of differences in ABF ≥ 50% was greater with than without LLD 33% (n = 4/12) vs. 9% (n = 6/67), P = 0·04.
Conclusions
ABF measured by Duplex ultrasonography is a simple, low‐cost and noninvasive complementary examination for help in detecting LLD, with a difference of ≥ 50% possibly associated.
What's already known about this topic?
Capillary malformations (CMs) might be associated with venous, lymphatic or arteriovenous anomalies or with overgrowth syndrome such as leg length discrepancy (LLD).
Molecular analyses have allowed for better understanding.
What does this study add?
In children with CMs of the lower limbs the frequency of differences in arterial blood flow ≥ 50% between the two limbs was greater with than without LLD.
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Vedolizumab is a humanized monoclonal antibody that binds to the human a4β7 integrin and is approved for use in inflammatory bowel diseases. We describe a patient with severe, refractory erosive ...gingivostomatitis, which appeared a few days after the first dose of vedolizumab and resolved after discontinuation of the drug. We believe the gingivostomatitis to be a direct side effect of vedolizumab, rather than an extraintestinal manifestation of the underlying inflammatory bowel diseases. The clinicians need to be aware of this adverse event, which could be mistakenly considered as an extraintestinal manifestation of inflammatory bowel diseases.
Summary
Calciphylaxis is a rare cause of skin ulcerations and necrosis in patients with both normal renal and parathyroid function. Although calciphylaxis appears to be on the increase, treatments ...are mainly empirical, especially for wound care. The lesions in calciphylaxis are typically very painful and carry a high risk of infection, with sepsis being the leading cause of death in this serious disease. We report two cases of nonuraemic calciphylaxis, which responded to treatment with pamidronate and wound management by negative pressure system.
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