Report of Eleven Patients of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Clinicopathologic Features, 18F-FDG PET/CT Findings and Outcome

ObjectivesSubcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a fairly rare subtype of primary cutaneous lymphoma. This study aims to investigate the clinicopathologic features, 18F-FDG PET/CT findings, and outcome of patients with SPTCL.MethodsA retrospective single-center study enrolled 11 patients with SPTCL between August 2010 and March 2020. A total of 26 18F-FDG PET/CT scans were performed, and the initial and follow-up PET/CT imaging features, clinicopathologic and immunohistochemical characteristics, and outcome were analyzed.ResultsThe male-to-female ratio was 1.2. The mean age at diagnosis was 24.2 years (age range: 13–48 years). Histopathological examinations revealed atypical T-lymphocyte rimming of individual subcutaneous adipocytes, mostly with CD2+, CD3+, CD4−, CD5+, CD8+, CD56−, T-cell intracellular antigen-1+, Granzyme B+, and high Ki-67 index. Multiple large skin ulcerations with a maximum diameter of 10 cm were observed in one of the 11 patients (9.1%, 1/11), and hemophagocytic syndrome was found in another one. At initial PET/CT scans, the lesions in all 11 patients showed increased uptake of 18F-FDG with a wide range of maximum standard uptake value (SUVmax) from 2.0 to 14.9. The morphology of the lesions presented as multiple nodules and/or disseminated plaques mainly involving the trunk and/or limbs. Five patients had extracutaneous non-lymph node lesions with SUVmax of 5.6 ± 2.8 on 18F-FDG PET/CT. No significant correlation between SUVmax and Ki-67 index was observed (r = 0.19, P > 0.05). Follow-up 18F-FDG PET/CT scans in six patients showed complete remission of the disease in two, partial remission in three, and progressive disease in one. During the follow-up period, there was no death except for the patient with multiple ulcerations who died 4 months after diagnosis of SPTCL.ConclusionsSPTCL may be a group of heterogeneous diseases with varying degrees of 18F-FDG uptake. 18F-FDG PET/CT demonstrates its usefulness in detecting disease extent, providing diagnostic work-up, staging, and evaluating treatment response of SPTCL. Multiple large skin ulcerations may be a factor of poor prognosis for patients with SPTCL.

Incidence of major complication following embolo-sclerotherapy for upper and lower extremity vascular malformations

Objective The current literature on the major complications of embolo-sclerotherapy of upper and lower extremity vascular malformations is scarce. Evaluating and understanding the rates and types of potential major complications of embolo-sclerotherapy of vascular malformations help treatment planning and informed consent. Therefore, this study reviewed major complications following embolo-sclerotherapy of all upper and lower extremity vascular malformations in a single specialized multidisciplinary vascular malformation center over a 5-year period. Methods All patients with vascular malformations underwent multidisciplinary directed intervention. Demographic, procedural, follow-up, and complication data were collected prospectively in a dedicated database, and reviewed retrospectively. Major complications for upper and lower extremity vascular malformations from 1 January 2013 to 31 December 2017 were analyzed. All embolo-sclerotherapies of high-flow vascular malformations (HFVMs) were performed under selective catheter angiography and direct injection, but low-flow vascular malformations (LFVM) with direct injection only. Major complications were defined as any tissue or functional damage caused by direct injection, distal embolization, or tissue reaction. Results Seventy patients (median age of 25 years; 44 males and 26 females) had 150 embolo-sclerotherapy procedures for upper extremity vascular malformation. Of these, 28 patients had embolo-sclerotherapy for HFVM and 42 patients for LFVM; total 78 and 72 procedures, respectively. A total of 107 patients (median age of 26 years; 42 males and 65 females) had 160 embolo-sclerotherapy interventions for lower extremity vascular malformations. Of these, 18 patients had embolo-sclerotherapy for HFVM and 89 patients for LFVM; total of 30 and 130 procedures, respectively. The overall major complication rates following embolo-sclerotherapy of upper and lower extremity vascular malformations were 14.3% and 4.7%, respectively ( P = 0.030). In the upper extremity HFVM group, major complications from embolo-sclerotherapy occurred in five patients; three ischemic fingers requiring amputation and two skin ulcerations. Meanwhile, in the upper extremity LFVM group, major complications occurred in five patients; one median nerve injury requiring nerve grafting and hand therapy, one hand contracture requiring tendon release, and three skin ulcerations. There was only one major complication, which was cellulitis in the lower extremity HFVM group. In the lower extremity LFVM group, major complications occurred in four patients; two skin ulcerations, one cellulitis, and one deep vein thrombosis. Conclusions Embolo-sclerotherapy is relatively safe for upper and lower extremity vascular malformations in a high-volume experienced center where our major complication rates were 14.3% and 4.7%, respectively, which compare favorably or similar to those reported in most recent literature. These outcomes will direct treatment strategies to avoid local and systemic toxic complications in the upper and lower extremity, for both HFVM and LFVM, and to improve informed consent.

Post-copulatory behavior of olive baboons (Papio anubis) infected by Treponema pallidum

In nonhuman primates pathogens are known to exert a profound and pervasive cost on various aspects of their sociality and reproduction. In olive baboons (Papio anubis) at Lake Manyara National Park, genital skin ulcers caused by Treponema pallidum subsp. pertenue lead to mating avoidance in females and altered mating patterns at a pre-copulatory and copulatory level. Beyond this level, sexual behavior comprises also post-copulatory interactions among the sexual partners. To investigate whether the presence of genital skin ulcers has an impact at the post-copulatory level, we analyzed 517 copulation events of 32 cycling females and 29 males. The occurrence of post-copulatory behaviors (i.e., copulation calls, darting [female rapid withdraw from the male] and post-copulatory grooming) was not altered by the presence of genital skin ulcerations. Similarly to other baboon populations, females of our group were more likely to utter copulation calls after ejaculatory copulation. The likelihood of darting was higher after ejaculatory copulations and with the presence of copulation calls. Post-copulatory grooming was rarely observed but when it occurred, males groomed females for longer periods when females uttered copulation calls during, or preceding mating. Our results indicate that despite the presence of conspicuous genital skin ulcers, the post-copulatory behavior was not affected by the genital health status of the dyad. This suggests that infection cues play a major role before and during mating but do not affect post-copulatory behavior.

Anti-MDA5 juvenile idiopathic inflammatory myopathy: a specific subgroup defined by differentially enhanced interferon-α signalling

Objectives JDM and juvenile overlap myositis represent heterogeneous subtypes of juvenile idiopathic inflammatory myopathy (JIIM). Chronic evolution can occur in up to 60% of cases, and morbidity/mortality is substantial. We aimed to describe the clinical, biological, histological and type I IFN status in JIIM associated with anti-melanoma differentiation-associated protein 5 (anti-MDA5) autoantibodies at presentation (group 1) in comparison with other JIIM (group 2). Methods This was a retrospective and prospective study of patients with JIIM ascertained from three French paediatric rheumatology reference centres between 2013 and 2019. Muscle biopsies were reviewed. Type I interferon pathway activity was assessed by dosage of IFNα serum protein and the expression of IFN-stimulated genes. Results Sixty-four patients were included, 13 in group 1 (54% JDM and 46% juvenile overlap myositis) and 51 in group 2 (76% JDM and 24% juvenile overlap myositis). Group 1 patients demonstrated more arthritis, skin ulcerations, lupus features and interstitial lung disease, and a milder muscular involvement. Serum IFNα levels were higher in group 1 than 2, and decreased after treatment or improvement in both groups. Outcome was similar in both groups. Unconventional treatment (more than two lines) was required in order to achieve remission, especially when skin ulceration was reported. Conclusion This study indicates a higher frequency of arthritis, skin ulcerations and interstitial lung disease, but milder muscular involvement, in JIIM with positive anti-MDA5 autoantibodies compared with other JIIM. Our data support an important role of systemic IFNα in disease pathology, particularly in the anti-MDA5 auto-antibody-positive subgroup. In severe and refractory forms of JIIM, IFNα may represent a therapeutic target.

Shabbir Syndrome: Case Report of a Rare Disease

Purpose: To report a case of Laryngeal Onycho Cutaneous Syndrome in a 10 year old child presenting with an ocular surface mass. Methods: A 10 year old boy presented in the out-patient department with a painless mass in left eye. There was history of airway obstruction requiring tracheotomy in early childhood and recurrent skin ulcerations. Aslit lamp examination revealed fleshy mass arising from the supero-nasalconjunctiva of left eye. Systemic examination revealed ulcerative lesions on cheeks and ears with dystrophic nails in hands and feet. Results: The ocular surface lesion was treated successfully with topical steroid drops. Conclusion: Steroids may be useful in treating early lesion of ocular surface in LOCS. The importance of regular follow must be emphasized for timely management of frequent granulation tissue outgrowth in bid to avoid development of severe symblepharon and blindness.

Moderne Behandlungsverfahren der Varikose der unteren Extremität

Varicosis of the lower limb is a common disease that can lead to edema, trophic disturbances and skin ulcerations. In addition to the classic surgical procedure of crossectomy with stripping of the saphenous vein, a growing number of minimally invasive endoluminal treatment methods has been established over the past 15 years, offering a colorful array of procedures to the modern phlebologist. It is important to choose the optimal treatment option for each individual patient.