Characteristics of DRESS Syndrome in the Elderly: A Comparative Study of 55 Patients.

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare drug reaction characterized by a skin rash, eosinophilia, and organ involvement. Objective: Our purpose is to focus on the clinical and epidemiological characteristics of DRESS in the elderly and to identify the incriminated drugs. Methods: This is a retrospective study including patients, hospitalized for DRESS with a RegiSCAR ≥4. The population was divided into 2 groups according to age: 65 years or older (G1) and <65 years (G2). The statistical study was performed using the comparative and multivariate analysis. Results: We included 55 patients (30.9% G1 and 69.1% G2). Skin manifestations were comparable in both groups. Lymphadenopathy was less common in G1 with a statistically significant difference (P = 0.012). Renal impairment was more frequent in the elderly with a statistically significant result (P = 0.005). DRESS in the elderly group was significantly associated with the occurrence of sepsis (P = 0.008). Allopurinol was the most common culprit associated with DRESS in G1 (P = 0.001). Relapses and recurrences were comparable in both groups (P = 0.71). Conclusions: DRESS in the elderly is associated with a high risk of complications, mainly kidney involvement and sepsis. Allopurinol is the most incriminated drug.

Follicular Basaloid Hamartoma and Associated Cutaneous Tumors: Clinical, Histopathological and Dermoscopic Aspects.

Background: Basaloid follicular hamartoma is an uncommun benign neoplasm of the hair follicule. The linear form of basaloid follicular hamartoma can be associated with basal cell carcinoma.OBJECTIVE: We report a case of a patient with multiple basal cell carcinomas developing on a congenital lesion allowing the diagnosis of basaloid follicular hamartoma on histopathology. Methods: Each lesion was evaluated by two dermatologists. All biopsy specimens were routinely stained with hematoxylin-eosin. Results: A 76 year-old-man consulted our dermatology departement for erythematous papules spread over the left lower limb. The lesion had been evolving since birth with flesh-colored to pinkish papules following Blaschko's lines from the root of the thigh to the foot. Three months before consultation, the papules increased in size leading to nodules with sometimes an ulcerated center. Dermoscopy under polarized mode displayed for ulcerated lesions (A) yellow-brownish crusts and ulceration surrounded by blue-grey ovoid nests, subtle short white streaks, brown dots and linear/arborising vessels. For nodular lesions (B), dermoscopic features are white pinkish hue, dotted and linear vessels, brown dots, blue-grey structureless areas and white prominent shiny streaks. There were some more erythematous inflamed and eroded areas in the background with a reversed honeycomb white network on dermoscopy (C), polymorphous vessels, whitish scales, ulcerations and milia-like cysts. The background lesion showed varied dermoscopic structures on a flesh colored slightly pinkish bottom (D). Histolopathology concluded for lesions A to an infiltrating and nodular basal cell carcinoma, (B) and (C) to fibroepithelioma of Pinkus and (D) to basaloid follicular hamartoma. Conclusion: Several case reports have documented dermoscopic features of a solitary basaloid follicular hamartoma. However, further studies are required to specify any reproducible features.

Bullous Pemphigoid Associated With Dipeptidyl Peptidase-4 Inhibitors: A Case Report and Review of Current Evidence.

Dipeptidyl peptidase-4 inhibitors (DPP-4i), or gliptins, are a widely used glucose-lowering agents. A growing amount of evidence pointed to a possible role of DPP-4i in the induction of bullous pemphigoid (BP), which is an auto-immune skin blistering disease that mainly affects the elderly. In this article we discuss a case of DPP-4i associated BP and we provide an updated review of the current knowledge regarding this emerging entity. Use of DPP-4i, particularly vildagliptin, was found to significantly increase the risk of BP. BP180 would be in the center of the aberrant immune response. DPP-4i induced BP is thought to be associated with male gender, mucosal involvement, and milder inflammatory phenotype especially in Asian population. Generally, patients may not remit fully after DPP-4i withdrawal only and require either topical or systemic glucocorticoid courses.

Predicting Waist Circumference From a Single Computed Tomography Image Using a Mobile App (Measure It): Development and Evaluation Study.

Background: Despite the existing evidence that waist circumference (WC) provides independent and additive information to BMI when predicting morbidity and mortality, this measurement is not routinely obtained in clinical practice. Using computed tomography (CT) scan images, mobile health (mHealth) has the potential to make this abdominal obesity parameter easily available even in retrospective studies. Objective: This study aimed to develop a mobile app as a tool for facilitating the measurement of WC based on a cross-sectional CT image. Methods: The development process included three stages: determination of the principles of WC measurement from CT images, app prototype design, and validation. We performed a preliminary validity study in which we compared WC measurements obtained both by the conventional method using a tape measurement in a standing position and by the mobile app using the last abdominal CT slice not showing the iliac bone. Pearson correlation, student t tests, and Q-Q and Bland-Altman plots were used for statistical analysis. Moreover, to perform a diagnostic test evaluation, we also analyzed the accuracy of the app in detecting abdominal obesity. Results: We developed a prototype of the app Measure It, which is capable of estimating WC from a single cross-sectional CT image. We used an estimation based on an ellipse formula adjusted to the gender of the patient. The validity study included 20 patients (10 men and 10 women). There was a good correlation between both measurements (Pearson R=0.906). The student t test showed no significant differences between the two measurements (P=.98). Both the Q-Q dispersion plot and Bland-Altman analysis graphs showed good overlap with some dispersion of extreme values. The diagnostic test evaluation showed an accuracy of 83% when using the mobile app to detect abdominal obesity. Conclusions: This app is a simple and accessible mHealth tool to routinely measure WC as a valuable obesity indicator in clinical and research practice. A usability and validity evaluation among medical teams will be the next step before its use in clinical trials and multicentric studies.

[Predictive factors for anastomotic leakage after colon cancer surgery]. / Facteurs prédictifs de fistule anastomotique après colectomie pour cancer.

Introduction: in colon cancer surgery, anastomotic fistula (AF) is considered the most feared complication. The purpose of this study was to identify predictive factors associated with anastomotic fistula after colon cancer surgical resection and to describe the impact of this complication on mortality and postoperative length of stay. Methods: we conducted a retrospective, descriptive and analytical study in the Department of General Surgery at the Habib Bourguiba Hospital in Sfax, Tunisia from 1st January 2013 to 31 December 2020. Results: we collected data from the medical records of 163 patients who had undergone surgery for colon cancer. The average age of patients was 62.7 years with a sex ratio of 1.36. The postoperative course was uneventful in 64.4% of cases and complicated in 35.6% of cases. Surgical morbidity was mainly due to anastomotic fistulas (22 patients). This study demonstrated that predictors of the development of this complication were: diabetes p = 0.04, smoking p = 0.01, hypoalbuminaemia p = 0.01, preoperative haemoglobin less than 10g/dl, p < 0.01, anastomotic fistula located in the left colonic angle p = 0.02, perioperative transfusion p <0.01, and duration of surgery longer than 180 min p = 0.04. Moreover, the occurrence of anastomotic fistula was associated with specific mortality rate (9%) and significantly prolonged postoperative length of stay. Conclusion: the prevention of anastomotic fistulas should be part of a multimodal approach based on the correction of nutritional deficiencies and possible pre-operative anemia.

[Cutaneous leishmaniasis after renal transplantation: Report of 7 cases and review of the literature]. / Leishmaniose cutanée après transplantation rénale : à propos de 7 cas et revue de la littérature.

Leishmaniasis is a zoonosis acquired from the bite of a sandfly that introduces the amastigote forms of leishmania into the bloodstream. It is a frequent infection in the countries of the Mediterranean basin and in Tunisia, where it is rife in an endemo-epidemic mode. However, it is rare after kidney transplantation. It constitutes a challenge due to the diagnostic difficulty, the variability and the polymorphism of the clinical picture in immunocompromised patients. We report seven observations of cutaneous leishmaniasis after kidney transplantation through which we try to identify diagnostic and therapeutic difficulties.

Primary retroperitoneal hydatid cyst with intraperitoneal rupture: a case report.

BACKGROUND: Hydatid disease is endemic in Mediterranean countries and most commonly occurs in the liver followed by the lung. A primary localization in the retroperitoneum is extremely rare. CASE PRESENTATION: We report the case of a 29-year-old Tunisian patient presenting with progressive left flank pain and skin urticaria. On abdominal ultrasonography and computed tomography scan, a ruptured retroperitoneal hydatid cyst was diagnosed, which was confirmed by positive hydatid serology. The treatment consisted of resection of protruding dome. The evolution was favorable. No local recurrence was detected during postoperative follow-up. CONCLUSIONS: Primary retroperitoneal hydatid cyst is extremely rare and has uncommon presentation, but we should learn the keys to its diagnosis. In endemic regions, high suspicion for this disease is justified regardless of localization.

Mesenteric lipoma revealed by chronic abdominal pain: A rare case report.

Mesenteric lipoma is a rare entity. It can be asymptomatic or revealed by unspecific clinical symptoms. Complete resection of the lipoma is often proposed to prevent complications. We report a case of mesenteric lipoma revealed by chronic abdominal pain, and we performed a literature review regarding this rare condition.

Duplication cyst and diverticulum of the stomach: A case report of an unusual association.

Gastric duplication cysts are uncommon findings in adult patients. Accurate diagnosis of these cysts is difficult. Presenting symptoms are often non-specific, and complications are rare. We report an uncommon case of a non-communicating duplication cyst associated to a diverticula of the stomach in 38-year-old women.

Perforated jejunal diverticulitis with extensive diverticulosis: A case report.

Jejunal diverticulitis is a rare condition that almost occurs in the elderly. An extensive diverticulosis associated is exceptional and can make the treatment more challenging.

Strangulated obturator hernia: Report of two cases.

Obturator hernia is a rare condition, which can be life-threatening if misdiagnosed. We must consider the diagnosis in case of a bowel obstruction especially in elderly women.

Autoantibodies against Desmoglein 1 and 3 in South Tunisian pemphigus.

BACKGROUND: Desmoglein (Dsg) 1 and 3 are the 2 major autoantigens in pemphigus foliaceus (PF) and pemphigus vulgaris (PV). AIM: We aimed to determine anti-Dsg1 and 3 Abs'usefulness in the diagnosis of pemphigus and to assess the correlation of these antibodies (Abs) with clinical phenotype and disease activity in south Tunisian patients. METHODS: We retrospectively analyzed 131 samples from 82 patients (52 with PF and 30 with PV) during follow-up. Anti-Dsg1 and 3 Abs were measured by ELISA. Consecutive anti-Dsg1 and 3 Abs were correlated with disease activity. Receiver operating characteristics (ROC) curve were calculated to determine anti-Dsg1 and 3 Abs'cut-offs with optimal sensitivity and specificity for disease activity. RESULTS: Anti-Dsg1 and 3 levels were associated to in PF and PV patients respectively (p<0,001). Anti-Dsg1 and 3 Ab were associated with skin (95%) and mucosal (60%) lesions, respectively. A significant decrease of anti-Dsg1 Abs was observed in patients with PF in clinical remission (36 ± 62 U/mL; (p=0,04). No correlation was found between anti-Dsg3 Abs and the course of mucosal lesions in PV (p=0,3). During follow-up, anti-Dsg1 Abs correlated with relapses (177 ±60 U/mL ; p=0,04). The 161,5 U/mL cut-off for anti-Dsg1 Abs provided 100% specificity and 86,4% sensitivity in PF disease activity. The 30,7U/mL cut-off for anti-Dsg3 provided 89,5% sensitivity and 100% specificity in PV. CONCLUSIONS: High anti-Dsg3 Abs values are not always associated with PV disease activity. Anti-Dsg1 Abs showed a closer relationship with skin activity in PS and should be therefore taken into account in management of pemphigus patients.

Update on immunogenetics of Tunisian endemic pemphigus foliaceus.

Pemphigus foliaceus (PF) is an autoimmune blistering skin disease characterized by the presence of bullous skin lesions, the absence of mucous tissue involvement, and the production of auto-antibodies directed against a keratinocyte transmembrane protein localized in the desmosome and member of the cadherines, desmoglein 1. These pathogenic auto-antibodies are responsible for the intra-epidermal formation of blisters through the loss of keratinocyte adhesion, the so-called acantholysis process. The endemic form of PF observed in the south of Tunisia is characterized by a significantly higher incidence rate compared to the sporadic form in northern countries, occurrence mainly in young women and the absence of cases during childhood. Tunisian endemic PF is an ideal research model for the decryption of the puzzle of genetic and environmental factors and their interactions in the development of autoimmune diseases. In this review, we will summarize recent findings regarding the epidemiologic and immunologic features of Tunisian PF and its genetic and environmental factors.

Pemphigus herpetiformis in South Tunisia: a clinical expression of pemphigus foliaceus?

BACKGROUND: Pemphigus herpetiformis (PH) is a rare subtype of pemphigus combining clinical features of dermatitis herpetiformis and the immunopathologic characteristics of pemphigus. We aimed to analyze the epidemiological, clinical, and immunological presentation and management of the disease in a cohort of south Tunisian patients with a long-term follow-up. METHODS: We included all patients with confirmed PH followed from January 1987 to December 2015 in the dermatology department. RESULTS: We included 24 south Tunisian female patients with a mean age of 36 years (20-63), mainly from rural origin (84%). All patients had exclusive skin lesions. Clinical features combined erythematous, vesicular, or bullous lesions, with circinate borders mainly on the trunk and limbs associated with severe pruritus (91%). Peripheral hypereosinophilia was noted in 31% of cases. Histological examination demonstrated no intraepithelial (50%), suprabasal (35%), or subcorneal cleavage (15%). Eosinophilic spongiosis was seen in 42% of cases. On direct immunofluorescence, there was an intercellular staining in 96% of cases composed mainly of IgG and C3 (79%). Indirect immunofluorescence was positive in 71% of cases. Reactivity was mainly directed against Dsg1. Dapsone alone was inefficient. All patients received systemic corticosteroids. Most patients (64%) relapsed. Iatrogenic complications and cutaneous infections were noted in 40% and 16% of cases, respectively. CONCLUSION: PH is characterized by the occurrence of exclusive skin lesions in young women with a good response to corticosteroids but not to Dapsone. Reactivity mainly against Dsg1 suggests that PH in south Tunisia is a particular variant of nonendemic pemphigus foliaceus.

Malignancy in a retrospective cohort of 17 patients with Dermatomyositis or Polymyositis in southern Tunisia.

INTRODUCTION: The prognosis of dermatomyositis (DM)/ polymyositis (PM) in adults is partly related to their association with neoplasia. The aim of our study was to report the epidemiologic, clinical, paraclinical, therapeutic and evolutionary aspects of DM associated with malignancy in patients from Sfax, south eastern of Tunisia. METHODS: A retrospective cohort study of patients with DM or PM admitted in Dermatology and Internal Medicine Departments of Hedi Chaker University Hospital of Sfax between 1996 and 2015. Cases of DM or PM associated with malignancy were retained. RESULTS: Seventeen cases (13.5%) of DM or PM associated with malignancy were noted. Fourteen patients had DM and 3 patients had PM. The Sex ratio M / F was 0.3 and the mean age at diagnosis was 56.5 years. In DM patients, malignancy preceded the myositis in 64.2% of cases. In PM patients, only one patient was known to have breast cancer and the myositis revealed the cancer for 2 others. Treatment consisted of corticosteroids associated with methotrexate in 4 cases. Outcome was fatal in 5 cases (29.4%), due to the underlying cancer in 3 cases. Swallowing disorders related to DM/PM were responsible for death in 2 cases. CONCLUSION: There are no specific clinical or biological features in paraneoplastic DM. In our series, breast neoplasm represented the first cancer associated with DM. Cancers of nasopharynx, colon and urinary tract had the second position.

Oxalosis in a Patient with Livedo Reticularis.

A 27-year-old man with terminal renal failure requiring peritoneal dialysis for the past 2 years was referred to the dermatologist for evaluation of red violaceous macular skin lesions consistent with livedo reticularis. These lesions had appeared suddenly on his legs (Figure 1). He had first experienced recurrent nephrolithiasis at the age of 14. Results from urine analysis and abdominal ultrasound revealed chronic kidney failure. Because the patient had a sister with similar findings, primary hyperoxaluria (PH) was suspected and genetic testing was performed in all members of his family. The results confirmed PH type 1 (PH1) in both our patient and his sister, who had died 8 years after the establishment of the diagnosis. A biopsy of the livedo reticularis lesions revealed deposits of a yellowish brown crystalline material within the lumen and the media of medium vessels in the hypodermis associated with a histiocytic giant cell reaction (Figure 2a). There was no evidence of extravascular calcium deposition in the sections examined. The deposits were intensely birefringent under polarized light, and classic speculated crystals of oxalate salts were observed (Figure 2b). No focal epidermal or superficial dermal necrosis was seen. On the basis of the histopathologic findings, the diagnosis of oxalate crystal-induced vascular disease was established. The patient subsequently developed complications including pancreatitis and peritonitis. He underwent hemodialysis until a combined liver-kidney transplant could be performed.