Is Neutrophilic Desquamative Erythroderma a Form of Acute Generalized Exanthematous Pustulosis?

Abstract

Delayed-type hypersensitivity to systemically administered corticosteroids is rare. Corticosteroids are unique allergens, potentially leading to atypical manifestations. They are commonly used for treatment of allergic, inflammatory and autoimmune diseases because of intrinsically immunosuppressive and immune-modulatory properties (1). These properties may counterbalance, mask and delay allergic reactions as has been shown (1). It has been speculated that allergic reactions to corticosteroids may remain unrecognized due to their broad clinical variability or frequently atypical presentation (1).

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Facial Swelling and Foreign Body Granulomatous Reaction to Hyaluronic Acid Filler in the setting of Neratinib

Abstract

Cosmetic injection of dermal fillers is common, with late complications increasingly recognized. Herein, we report a granulomatous reaction to hyaluronic acid filler occurring during the use of neratinib. A woman in her fifties, with metastatic adenocarcinoma of unknown primary involving the skin and bones, was referred for new-onset facial swelling. Notable past medical history included progression of disease on carboplatin/paclitaxel and a hemithyroidectomy for papillary thyroid cancer.

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Ultrastructural Aspects of Hairs of Chediak-Higashi Syndrome

Abstract

Chediak-Higashi syndrome (CHS) belongs to a group of partial oculocutaneous albinism and immunodeficiency. Five autosomal recessive conditions are recognized in this group, characterized by hypopigmentation of hair, skin and eyes, associated with recurrent infections. Additionally CHS can present coagulopathies, neurological dysfunction and large granules in many cell types.

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Is there a pathogenetic link between frontal fibrosing alopecia, androgenetic alopecia, and fibrosing alopecia in a pattern distribution?

Abstract

Frequent coexistence of frontal fibrosing alopecia (FFA) and androgenetic alopecia (AGA) has been reported in the literature, but little attention has been given to the pathogenetic mechanisms underlying this potential association. FFA is characterized by progressive cicatricial fronto-temporal hairline recession, mostly affecting peri-menopausal women. Immune mechanisms seem to play a pivotal pathogenetic role.

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Bizarre appearance of chronic cutaneous lupus erythematosus of face mimicking factitial dermatitis

Abstract

Old dermatological wisdom says that syphilis and lupus are great imitators. Cutaneous manifestations of lupus erythematosus (LE) are usually characteristic, however, some patients may present with a less typical clinical picture. Correct diagnosis of cutaneous lupus erythematosus (CLE) requires a high level of clinical suspicion and understanding of broad differential diagnosis. Early lesions of CLE are sometimes difficult to diagnose, both clinically and histopathologically.

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Phototherapy in France: quantitative data (2007-2016) from the National Health Insurance register

Abstract

Phototherapy is a cost-effective treatment with a safe side-effect profile for the management of a wide range of dermatoses. Although one of the most common indications for phototherapy is psoriasis, the use of phototherapy for psoriasis has seen a decline in the U.S.A. Data from the National Ambulatory Medical Care Survey indicate a 94% decrease of physician visits for phototherapy between 1994 and 1998.

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Dermoscopic observations in disseminated cryptococcosis with cutaneous involvement

Abstract

A 26-year-old female patient with a history of intravenous drug abuse, diagnosed with acquired immunodeficiency syndrome (AIDS) 6 months before. AIDS defining disease was Pneumocystis jiroveci pneumonia. The patient presented with severely decreased CD4 cell count of 7 cells/mm³ and relatively low HIV viral load of 281 copies/mL. Shortly after initiation of antiretroviral therapy (ART), was diagnosed with disseminated cryptococcosis with central nervous system and skin involvement.

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Power Doppler ultrasound assessment of vascularization in hidradenitis suppurativa lesions

Abstract

Background

Ultrasound and Power Doppler (PD) ultrasound are useful tools to study and monitor Hidradenitis Suppurativa (HS) patients.

Objective

Describe the Power Doppler signal of HS nodules, abscesses, fistulas.

Methods

A retrospective analysis of PD in mild, moderate, severe HS patients, collecting all demographic and clinical data. The lesions were classified according to their ultrasound morphology, describing the vascular degree - high, moderate, minimal - and distribution - peripheral, internal, mixed. Statistical analysis was performed using odds ratio and bivariate regression.

Results

A total of 241 lesions, 62 nodules, 64 abscesses, 99 simple fistulas, 16 complex fistulas, from 61 HS patients, were included. Vascular distribution was defined peripheral in 143/241, mixed in 55/241, internal in 0/241 lesions, regardless the clinical type. Qualitative Doppler showed high vascularization in 44/241 lesions, moderate in 79/241, minimal in 75/241, despite the clinical type. All lesions showed resistive index <0.7. Age, disease's duration, size of the lesions, high Sartorius score, high BMI, showed positive statistically correlation with both PD signal and mixed vascular distribution. No statistically significance was evidenced for vascular degree measurements.

Limitations

Ultrasound cannot detect lesions <0.1 mm.

Conclusion

Vascular distribution of HS lesions can be evaluated by PD with additional relevant information for earlier and better disease management.

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Progressive osseous heteroplasia in a Chinese infant and a novel mutation in the GNAS Gene

Abstract

Progressive osseous heteroplasia(POH,OMIM 166350) is a rare inherited disease that begins with skin ossification and proceeds into the deeper connective tissues. Most cases of POH are caused by heterozygous inactivating mutations of GNAS gene. Herein, we report a new sporadic case of atypical POH and its underlying genetic basis.

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Pili annulati in a case of Rothmund-Thomson syndrome with a novel frameshift mutation in RECQL4

Abstract

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis characterised by erythematous patches or plaques and sometimes swelling and blisters, which appear during infancy on photo-exposed sites, usually the cheeks, then progress to poikiloderma. Other features include gastrointestinal disturbance, short stature, sparse scalp hair/eyebrows/eyelashes, skeletal abnormalities, juvenile cataracts, hypogonadism and a susceptibility to malignancy. Two forms have been described: Type I, characterised by poikiloderma, ectodermal dysplasia and juvenile cataracts, negative for the RECQL4 mutation, and Type II, with poikiloderma, congenital bone defects and increased risk of osteosarcoma related to deleterious RECQL4 mutations.

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Serious gastric perforation after second stereotactic body radiotherapy for peripheral lung cancer that recurred after initial stereotactic body radiotherapy: a case report

In recent reports, re-irradiation with stereotactic body radiotherapy for lung tumors in patients previously treated with thoracic radiation therapy resulted in several serious toxicities. Serious non-lung tox...

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Antitumor miR-150-5p and miR-150-3p inhibit cancer cell aggressiveness by targeting SPOCK1 in head and neck squamous cell carcinoma

Publication date: Available online 9 December 2017
Source:Auris Nasus Larynx
Author(s): Keiichi Koshizuka, Toyoyuki Hanazawa, Naoko Kikkawa, Koji Katada, Atsushi Okato, Takayuki Arai, Tetsuya Idichi, Yusaku Osako, Yoshitaka Okamoto, Naohiko Seki
ObjectiveOur recent studies have revealed that both strands of pre-miRNAs, the guide strand and the passenger strand, are involved in cancer pathogenesis. Analyses of miRNA expression signatures by RNA sequencing in head and neck squamous cell carcinoma (HNSCC) showed that both of the strands of pre-miR-150 (miR-150-5p and miR-150-3p) were significantly downregulated, and that these miRNAs acted as antitumor miRNAs in HNSCC cells. The aim of this study was to identify oncogenic genes in HNSCC cells that were regulated by miR-150-5p and miR-150-3p.MethodsGenome-wide gene expression studies, in silico analyses and dual-luciferase reporter assays were carried out to predict miR-150-5p and miR-150-3p regulation in HNSCC cells. Knockdown assay was applied to investigate the functional significance of the target gene. Overall patient survival as a function of target gene expression was estimated by The Cancer Genome Atlas (TCGA) database.ResultsA total of 19 genes were putative targets of both miR-150-5p and miR-150-3p regulation. Among them, SPOCK1 (SPARC/osteonectin, cwcv and kazal-like domains proteoglycan 1) was directly regulated by both miRNAs in HNSCC cells. Knockdown studies using si-SPOCK1 showed that expression of SPOCK1 enhanced HNSCC cell aggressiveness. Overexpression of SPOCK1/SPOCK1 was confirmed in HNSCC clinical specimens. Interestingly, analysis of a large number of patients in the TCGA database (n=248) demonstrated that patients with high SPOCK1 expression had significantly shorter survival than did those with low SPOCK1 expression (P=0.0003). Moreover, 15 pathways were identified as SPOCK1-mediated downstream pathways.ConclusionDownregulation of both strands of pre-miR-150 (miR-150-5p and miR-150-3p) and overexpression of SPOCK1 contribute to the aggressive nature of HNSCC. The involvement of passenger strand miRNA in the regulation of HNSCC pathogenesis is a novel concept in RNA research.



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Early-onset childhood atopic dermatitis is related to NLRP2 repression

Publication date: Available online 9 December 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Loreen Thürmann, Konrad Grützmann, Matthias Klös, Matthias Bieg, Marcus Winter, Tobias Polte, Tobias Bauer, Matthias Schick, Melanie Bewerunge-Hudler, Stefan Roeder, Mario Bauer, Dirk K. Wissenbach, Ulrich Sack, Dieter Weichenhan, Oliver Mücke, Christoph Plass, Michael Borte, Martin von Bergen, Irina Lehmann, Roland Eils, Saskia Trump
Early-onset atopic dermatitis (AD) related repression of the immune regulatory NLRP2 is driven by promoter hypermethylation starting already at time of birth providing an early opportunity to modulate innate immunity to potentially mitigate AD development.



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Identification of human papillomavirus (HPV) subtype in oral cancer patients through microarray technology

Abstract

Purpose

Human papilloma virus (HPV) is the main source of cervical cancer. Many recent studies have revealed the prevalence and prognosis of HPV associated with oropharyngeal squamous cell carcinoma, but fewer reports have evaluated HPV in oral squamous cell carcinoma (OSCC). The purpose of this study was to determine the prevalence and prognosis of HPV associated with OSCC according to HPV and tumor types.

Materials and methods

We used a DNA chip kit (MY-HPV chip kit ^®, Mygene Co., Korea) to detect high-risk HPV subtypes (16, 18, 31, 33, 35, 39, 45, 51, 52, 54, 56, 58) and low-risk subtypes (6, 11, 34, 40, 42, 43, 44) among 187 patients. The prevalence was determined by Chi-square and Fisher's exact tests, and the prognosis was calculated by the Kaplan–Meier method and the log-rank test.

Results

The overall prevalence of HPV in OSCC was 7.0% for all HPV positives and 4.3% for high-risk HPV positives. The prevalence of HPV was significantly higher in individuals under 65 years old and in those with tumors in the tongue and gum regions. The prognosis did not differ between the HPV-positive and -negative groups. Although the prevalence of HPV-positive cases in OSCC was low (7.0, 4.3%) and the prognosis did not depend on HPV positivity, HPV-associated OSCC should be considered in the evaluation and treatment of oral cancer patients. In addition, separating high- and low-risk groups based on the HPV status of other body parts might not be appropriate.

Discussion

The DNA microarray method can accurately detect known HPV subtypes simultaneously, but has limitations in detecting new subtypes. Vaccines can also be used to prevent HPV-associated OSCC in patients, so further studies on the prognosis and efficacy of vaccines should be undertaken.

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Pattern of care and impact of prognostic factors on the outcome of head and neck extramedullary plasmacytoma: a systematic review and individual patient data analysis of 315 cases

Abstract

Introduction

Head and neck extramedullary plasmacytoma is a rare localized plasma cell neoplasm. We intended to perform this review of the published literature to assess the demographic profile, pattern of care and survival outcomes.

Methods

Two authors independently searched PubMed, Google search and Cochrane library for eligible studies from 1950 till July 1, 2016, published in English language.

Results

Median age of the cohort was 57 years (range 11–85). Site-wise distributions were paranasal sinuses 22.3% (70), nasal cavity 17.5% (55), nasopharynx 10.8% (34). Median size of SEMP was 3 cm (range 0.3–12 cm). Treatment distribution was radiotherapy (RT) in 52% (164), surgery (S) 19% (60), chemotherapy (C) 5% (16), S + RT 23.49% (74),CRT 1.9% (6), S + C 0.6% (2), S + RT + C 0.95% (3).Radiation was used as a modality in 78.4%(247), surgery in 44.1%(139), chemotherapy in 4.8%(15). Median radiation dose used was 45 Gy with range 20–61 Gy. Median overall survival (OS) was 40 months (range 0.5–298). Median local progression-free survival was 36 months (range 0–298). Median myeloma relapse-free survival was 36 months (range 0.5–298). Five- and 10-year OS was 78.33 and 68.61%. Five-year cause-specific survival (CSS) and 10-year CSS was 90.15 and 83.31%. Five-year LPFS was 94.78%, and 10-year LPFS was 88.43%. Five-year myeloma progression-free survival was 84.46%, and 10-year myeloma PFS was 80.44%. The factors associated with risk of local relapse were site of disease (sinonasal), secretory EMP, type of treatment received (surgery + RT > RT alone > surgery on univariate analysis). Risk factors for myeloma relapse were coexisting diseases, site of disease (sinonasal), bony erosion, size of lesion > 5 cm and type of treatment received on univariate analysis.

Conclusion

Our study shows that combined modality S + RT is superior compared to uni-modality in preventing local recurrence. Radiation dose of 45 Gy is optimal. Nodal irradiation has no impact on local recurrence.

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Association between continuous positive airway pressure and serum aminotransferases in patients with obstructive sleep apnea

Abstract

Introduction

Obstructive sleep apnea (OSA) has been suggested to be a potential contributing factor for nonalcoholic fatty liver disease (NAFLD). Studies on the association between continuous positive airway pressure (CPAP) and NAFLD in OSA patients are limited and controversial.

Objectives

The aim of this study was to assess the relationship between OSA and NAFLD and the effect of CPAP therapy on serum aminotransferase levels in OSA patients.

Methods

A total of 160 consecutive patients who underwent standard polysomnography were enrolled. Blood samples were obtained in the morning after sleep for biological profile measurements. Non-invasive ultrasound techniques were used to assess liver steatosis and fibrosis. Within the OSA group, serum aminotransferases were detected before and after CPAP treatment.

Results

Alanine aminotransferase (ALT), aspartate aminotransferase (AST), gamma-glutamyltransferase, and liver steatosis score increased significantly with an increase in OSA severity. Stepwise multiple regression with liver steatosis score, ALT, AST as dependent variable, respectively, apnea–hypopnea index (β = 0.447, p = 0.020; β = 0.266, p = 0.001; β = 0.351, p = 0.020, respectively) significantly predicted the liver steatosis score, ALT, AST after adjustment for confounders. After 3 months of CPAP treatment, there was a significant decrease in both ALT (54.20 ± 24.34 vs. 46.52 ± 24.95, p = 0.000) and AST (31.82 ± 8.91 vs. 29.00 ± 8.34, p = 0.039).

Conclusions

OSA severity was independently associated with liver steatosis and elevation of serum aminotransferases. 3 months of CPAP therapy were associated with a statistically significant improvement on liver injury in OSA patients.

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Antitumor miR-150-5p and miR-150-3p inhibit cancer cell aggressiveness by targeting SPOCK1 in head and neck squamous cell carcinoma

Our recent studies have revealed that both strands of pre-miRNAs, the guide strand and the passenger strand, are involved in cancer pathogenesis. Analyses of miRNA expression signatures by RNA sequencing in head and neck squamous cell carcinoma (HNSCC) showed that both of the strands of pre-miR-150 (miR-150-5p and miR-150-3p) were significantly downregulated, and that these miRNAs acted as antitumor miRNAs in HNSCC cells. The aim of this study was to identify oncogenic genes in HNSCC cells that were regulated by miR-150-5p and miR-150-3p.

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The Prevalence and Odds of Depression in Patients with Vitiligo: a Meta-Analysis

Abstract

Objective

The objective of this study is to provide a pooled estimate of the prevalence and odds of depression in Vitiligo patients.

Methods

A systematic literature search of Medline, Embase, and Cochrane library databases was conducted. The proportions and mean questionnaire values for depression were pooled according to different evaluation methods. In controlled studies, odds ratio (OR) and standardized mean differences (SMDs) compared depression in vitiligo patients with healthy controls.

Results

One thousand nine hundred and sixty-five patients were identified from 20 eligible cohorts to be included in the present meta-analysis review. Sample sizes ranged from 30 to 308 for each analysis. The pooled prevalence of depression across 17 unique populations (n = 1,711) was 29% (95% CI 20 - 39). The prevalence of clinical depression was 8% (95%CI 2-14%) using Diagnostic and Statistical Manual of Mental Disorders IV (DSM IV) or International Classification of Diseases codes-10 (ICD 10 and the prevalence of depressive symptoms was 33% (95%CI: 23-44%) using validated screening inventory and rating scales. Vitiligo patient were 4.96 times more likely to display depression compared with controls. Subgroup analysis showed that the prevalence of depression in Asian and female vitiligo patients were significantly higher than those in Caucasian and male ones. The pooled prevalence of depressive symptoms was higher (56%, 95%CI: 0.5-0.62) and the heterogeneity was lowering cases with Hamilton Depression Rating Scale (HDRS) questionnaire when compared with other questionnaires.

Conclusions

This meta-analysis review indicates that the prevalence of depression is high in vitiligo patients. The HDRS questionnaire may be the most sensitive tool for testing the depressive symptoms among vitiligo patients.

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Children with atopic dermatitis may have unacknowledged contact allergies contributing to their skin symptoms

Abstract

Background

Whether children with atopic dermatitis have an altered risk of contact allergy than children without atopic dermatitis is frequently debated and studies have been conflicting. Theoretically, the impaired skin barrier in AD facilitates the penetration of potential allergens and several authors have highlighted the risk of underestimating and overlooking contact allergy in children with atopic dermatitis.

Objective

To determine the prevalence of contact allergy in Danish children with atopic dermatitis and explore the problem of unacknowledged allergies maintaining or aggravating the skin symptoms.

Methods

In a cross sectional study, 100 children and adolescents aged 5-17 years with a diagnosis of atopic dermatitis were patch tested with a pediatric series of 31 allergens.

Results

Thirty percent of the children had at least one positive patch test reaction and 17% had at least one contact allergy that was relevant to the current skin symptoms. The risk of contact allergy was significantly correlated to the severity of atopic dermatitis. Metals and components of topical skin care products were the most frequent sensitizers.

Conclusion

Patch testing is relevant as a screening tool in the management of children with atopic dermatitis as they may have unacknowledged contact allergies contributing to or maintaining their skin symptoms. Children with atopic dermatitis seem to be at greater risk of sensitization to certain allergens including metals and components of skin care products.

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Safety and Efficacy of guselkumab in Japanese patients with moderate-to-severe plaque psoriasis: A Randomised, Placebo-Controlled, Ascending Dose Study

Summary

Background

The Interlukin-23 (IL-23)/Interlukin-17 (IL-17) pathway is central in the pathogenesis of psoriasis. The favourable efficacy and safety of guselkumab, an IL-23 specific monoclonal antibody, has been demonstrated in global Phase 3 studies of plaque psoriasis.

Objectives

To evaluate the safety, efficacy and pharmacokinetics of a single-dose subcutaneous guselkumab in Japanese patients with moderate-to-severe plaque psoriasis.

Methods

Patients with ≥ 10% of total body surface area (BSA) involvement and a Psoriasis Area and Severity Index (PASI) ≥12 were randomised (5:1) to receive guselkumab or placebo in 4 cohorts of this double-blind, placebo-controlled, single ascending dose, single-centre study. Safety, pharmacokinetics, and clinical response were monitored at baseline and specific time points over a 24-wk follow-up period.

Results

Through week 24, 54% (11/20) of guselkumab patients and 50% (2/4) of placebo patients experienced ≥1 adverse event (AE). No deaths, serious AEs, or AEs leading to treatment discontinuation were reported. Maximum clinical response was seen at week 16 with PASI 75 response in 2/5 (10 mg), 4/5 (30 mg and 300 mg), and 3/5 (100 mg) patients and PASI 90 in 0/5 (10 mg), 3/5 (30 mg), 2 /5 (100 mg) and 3/5 (300 mg) patients. Mean C_max and AUC_∞ values increased in a dose-proportional manner with mean terminal t_1/2 of 15.6-17.6 days and median t_max of 4-6 days.

Conclusions

Guselkumab was generally well-tolerated and exhibited sustained high levels of clinical response in Japanese patients with moderate-to-severe psoriasis.

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Which outcomes are reported in cellulitis trials? Results of a review of outcomes included in cellulitis trials and a patient priority setting survey

Summary

Background

There is an emerging need to develop consistent outcomes in clinical trials to allow effective comparison of treatment effects. No systematic review has previously looked at the reporting of outcome measures used in randomised controlled trials (RCTs) on treatment and prevention of cellulitis (erysipelas).

Objectives

The primary aim of this review was to describe the breadth of outcomes reported from RCTs on cellulitis treatment and prevention. The secondary aim was to identify outcome themes from patient and health care professionals' feedback from a cellulitis priority setting partnership (PSP).

Methods

We conducted a review of all outcome measures used in RCTs from two recent Cochrane reviews. Free text responses from a cellulitis priority setting survey were used to understand the perspectives of patients and healthcare professionals.

Results

Outcomes from 42 RCTs on treatment of cellulitis and six RCTs on prevention of cellulitis were reviewed. Only 28 trials stated their primary outcome. For trials assessing treatment of cellulitis, clinical response to treatment was categorised in 25 different ways. Five of these trials used an outcome that was in accordance with FDA guidance and only four trials incorporated either quality of life or patient satisfaction. For trials assessing prevention of cellulitis, recurrence was the key outcome measure. From the cellulitis PSP, prevention of recurrence, clinical features and long-term disease impact were the most important outcome themes for patients.

Conclusions

We have shown that in cellulitis treatment and prevention research, there is significant heterogeneity in clinical outcomes, inadequate focus on patient-reported outcomes, and a disparity between what is currently measured and what patients and healthcare professionals feel is important. We recommend that future cellulitis treatment trials consider the use of longer-term outcomes to capture recurrence and long-term morbidity, as well as short-term resolution of acute infection.

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La « rupia syphilitique » existe toujours

Publication date: Available online 8 December 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): J. Revuz




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Quel avenir pour la dermatologie universitaire ?

Publication date: Available online 8 December 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): B. Dréno, N. Dupin




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Sclérodermie systémique de révélation tardive : étude rétrospective de 27 patients diagnostiqués après l’âge de 70 ans

Publication date: Available online 8 December 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): A. Achille, L. Journeau, O. Espitia, J. Connault, A. Espitia-Thibault, C. Durant, F. Perrin, M.-A. Pistorius, A. Néel, M. Hamidou, C. Agard
IntroductionLe but de ce travail était de décrire les particularités de la sclérodermie systémique (ScS) quand le diagnostic est fait après l'âge de 70 ans.Patients et méthodesIl s'agit d'une étude rétrospective des patients âgés de plus de 70 ans au moment du diagnostic de ScS, suivis dans un service de médecine interne entre 2000 et 2015. Les co-morbidités et les caractéristiques démographiques et cliniques ont été analysées, ainsi que la survie à 1, 2 et 3 ans.RésultatsSur les 246 cas de ScS de notre cohorte, 27 (11 %) ont été inclus (89 % de femmes, âgés de 78,3±4,5 ans). Une néoplasie synchrone était notée chez 3 patients. La ScS était majoritairement cutanée limitée (24/27), avec des télangiectasies (63 %), un reflux gastro-oesophagien (59 %), des ulcères digitaux (22 %), et associée à un anticorps anti-centromères (69 % des cas). L'atteinte interstitielle pulmonaire était peu fréquente (29 %). Une hypertension artérielle pulmonaire (HTAP) était suspectée dès le diagnostic de ScS dans 14 cas (52 %), mais seuls 5 patients ont eu un cathétérisme cardiaque, montrant une HTAP sévère dans 3 cas. La survie était de 85,2 % à 1 an et 66,7 % à 3 ans ; elle était moins bonne en cas de suspicion d'HTAP (respectivement 78,6 % et 57,1 %).ConclusionLes cas de ScS diagnostiquée après 70 ans sont majoritairement des formes cutanées limitées. Une suspicion d'HTAP est fréquente, représentant plus d'un cas sur 2, et l'HTAP est un mode d'entrée dans la maladie à cet âge. L'association à un cancer synchrone est possible. La survie est médiocre.BackgroundThe aim of this study was to describe special features of patients with systemic sclerosis (SSc) diagnosed after the age of 70.Patients and methodsThis is a retrospective study of patients aged above 70 years at the time of diagnosis of SSc and followed at an internal medicine unit between 2000 and 2015. Co-morbidities and clinical characteristics were analyzed, as well as survival at 1, 2 and 3 years.ResultsOf 246 patients, 27 (11%) were included (89% women, 96% Caucasians, age 78.3±4.5 years). Synchronous cancer was noted in 3 patients. SSc was mostly limited cutaneous only (24/27), with telangiectasia (63%), gastroesophageal reflux (59%) and digital ulcers (22%), and was associated with anti-centromere antibody (69%). Interstitial lung disease was not frequent (29%). Pulmonary arterial hypertension (PAH) was suspected at diagnosis of SSc in 14 cases (52%), but only 5 patients had undergone heart catheterization, with severe PAH in 3 cases. Survival at 1 and 3 years was 85.2% and 66.7%, and was worse in the case of suspected PAH, at 78.6% and 57.1% respectively.ConclusionCases of SSc diagnosed after 70 years are mostly limited cutaneous forms. Suspicion of PAH is frequent, and PAH may be the main initial sign of the disease for patients at this age. There may be association with synchronous cancer. Survival is poor.



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