Juvenile Dermatomyositis: Diagnosis and Management.

Juvenile dermatomyositis (JDM) is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Seven patients with JDM found in the records from 1998-2019 of the Department of Dermatology Farhat Hached Hospital, Sousse, Tunisia. Our study concerned a total of six girls and one boy with a median age at disease onset of 8,16 years. The average time before diagnosis was 8,8 months. The onset of the disease was acute in 2 patients. All patients displayed skin manifestations at diagnosis, with proximal muscular weakness in 4 cases. Four patients had elevated muscle enzymes and all of them showed myopathic findings on electromyography. Oral corticosteroids were prescribed in 6 patients, in association with other systemic therapies. Three patients achieved a good outcome while two others relapsed. The two other patients showed corticosteroids resistance with a fatal outcome in one case. This study highlights the diagnostic features and management of juvenile dermatomyositis.

Acquired Ichthyosis Revealing Lymphocytic Colitis.

A 69-year-old patient with no personal or family history of ichthyosis consulted our dermatology department for diffused cutaneous xerosis with intense pruritus evolving for 3 weeks. Physical examination revealed diffused ichthyosis of large polygonal fine scales on the skin without erythema (Figure 1). The lesions spared the face. Examination of the mucous membranes, hair, and nails revealed no abnormalities. There was no fever or adenomegaly. A skin biopsy revealed an orthokeratotic hyperkeratosis with thinning of granular layer (Figure 2). The initial diagnosis of acquired ichthyosis was maintained. The patient also reported a change in bowel habits since 2 weeks with watery, non-bloody diarrhea and mild steatorrhea. His laboratory investigations presented low serum vitamin B12 level, mild anemia, hypoalbuminemia, and fecal leukocytes; however, antinuclear antibodies, perinuclear anti-neutrophil cytoplasmic antibodies (pANCA), rheumatoid factor, and complement components C3 and C4 were normal. A colonoscopy performed was also normal without any abnormalities. Colon biopsies revealed histologic aspects of lymphocytic colitis with more than 20% increase in lymphocytes in the surface epithelium of colorectal mucosa. Laboratory investigations excluded neoplasia, hemopathies, or autoimmune-associated diseases. The patient was treated with salazopyrin with a remarkable lessening of diarrhea and cutaneous manifestations within 4 weeks (Figure 3).

Cutis verticis gyrata in a 24-year-old young man revealing a T-cell lymphoblastic lymphoma.

T-cell lymphoblastic lymphoma (T-LBL) is frequently revealed by amediastinal mass or peripheral lymphadenopathy. Skin lesions in T-LBLusually present as multiple nodules associated with multiple peripherallymphadenopathy and bone marrow invasion. Our patient is particular bythe revealing presentation of the lesions as Cutis verticis gyrate.

Inflammatory complications after hair transplantation: Report of 10 cases.

BACKGROUND: Androgenetic alopecia (AGA) is a pathology involving the aesthetic prognosis. Hair transplantation is among best treatments. The principle of hair micro-grafts during AGA consists in taking hair from the non-androgen-dependent occipital area to transplant them with their root in the sparse androgen-dependent areas. Herein, we report 10 cases of the different types of post-transplant inflammatory complications. MATERIALS AND METHODS: We included patients referred to our center by their dermatologists or hair transplant surgeons for inflammatory cicatricial alopecia or hair loss observed after the hair transplant. RESULTS: Ten patients (eight men and two women) were included. These patients represented 0.08% of all consultations in our center. The indication for hair transplantation was AGA in all of our patients. The technique used for the transplant was follicular unit extraction (FUE) in seven cases and follicular unit transplantation (FUT) strip in three cases. None of the patients had pathology of the scalp or an inflammatory dermatosis before the operation. The inflammatory complications found were lichen planopilaris (LPP) in seven cases, erosive pustulosis of the scalp (EPS) in two cases, and superficial folliculitis (SF) in 1 case. CONCLUSION: Our series highlight the rarity of inflammatory complications that occur after a hair transplant. We demonstrate through this work that a hair transplant can trigger inflammatory pathology a few months after the act. We show also, the importance of detecting the rough forms of lichen before an intervention, hence the interest of the systematic dermatoscopic examination during the preoperative consultation.

X-linked recessive ichthyosis in 8 Tunisian patients: awareness of misdiagnosis due to the technical trap of the STS pseudogene.

INTRODUCTION: X-linked recessive ichthyosis (XLI) is a genodermatosis, caused by a deficiency of the steroid sulphatase enzyme encoded by the STS gene (OMIM # 300,747). Adopted XLI molecular diagnosis approaches differ from one laboratory to another depending on available technical facilities. Our work aims to figure out a sound diagnostic strategy for XLI. PATIENTS AND METHODS: We collected 8 patients with XLI, all males, from 3 unrelated Tunisian families from central Tunisia. Genetic diagnosis was conducted through a large panel of genetic techniques including: Sanger Sequencing, haplotype analysis of STR markers, MLPA analysis, FISH and array CGH. RESULTS: Direct Sanger sequencing of the STS gene showed the same deletion of 13 base pairs within the exon 4 in all patients resulting in a premature stop codon. However, all patients' mothers were not carriers of this variant and no common haplotype flanking STS gene was shared between affected patients. Sequence alignment with reference human genome revealed an unprocessed pseudogene of the STS gene located on the Y chromosome, on which the 13 bp deletion was actually located. STS MLPA analysis identified a deletion of the entire STS gene on X chromosome for all affected patients. This deletion was confirmed by FISH and delineated by array CGH. CONCLUSION: All our patients shared a deletion of the entire STS gene revealed by MLPA, confirmed by FISH and improved by array CGH. Geneticists must be aware of the presence of pseudogenes that can lead to XLI genetic misdiagnosis.

Domperidone-induced pityriasis rosea-like drug eruption.

Pityriasis rosea is a common, acute, self limiting inflammatory skin disease. Pityriasis rosea-like eruptions (PR-LE) have been reported after drugs. The clinical presentation of PR-LE can be distinguished from pityriasis rosea. We reporte a 41-year-old woman who developed PR-LE 5 days after administration domperidone.

Photodistributed pustular acute febrile neutrophilic dermatosis revealing an acute myeloid leukemia.

Sweet syndrome is a rare inflammatory dermatosis that can be associated with various diseases, including leukemias. Physicians should be aware that a photodistributed clinical presentation of a pustular SS may reveal underlying malignancies, particularly hemopathies. If the hemopathy is known, recurrence lesions should be suspected of a relapse.

The management of appendicular abscesses in a Tunisian Tertiary Care Hospital.

BACKGROUND: This study aims to describe the epidemiological, clinical, and radiological features of appendicular abscesses, compare the different approaches, and assess the safety and utility of laparoscopy in its management. METHODS: This descriptive retrospective study was carried out over 3 years extending from January 2017 to December 2019, reporting 150 appendicular abscesses cases. Data were collected from the register of the general surgery department B of the Rabta hospital. Cases with appendicular abscess were included. Files concerning patients with early appendicitis, non-appendicular abscesses or generalized appendicular peritonitis were excluded. Data were analysed with Statistical Package for the Social Sciences (SPSS) software. In univariate analysis, we used the chi2 test, the Fischer test, the Student t test. The factors retained by the univariate analysis were introduced into a logistic regression model. The significance level was set to 0.05. RESULTS: The mean age was 40.51 years. The gender ratio in patients with appendicular abscesses was M:F 1.94:1. Pain in the right iliac fossa associated with fever was the most common symptom (78% of cases). Clinical examination showed tenderness in the right iliac fossa in 38% of cases, rebound and guarding were found in 77 patients (51.3%), and a palpable mass was noted in 4 cases (4.2%). Imaging was done to confirm diagnosis; 46 patients underwent ultrasonogram and this confirmed the diagnosis in 26 patients (56%), while among the 71 patients who underwent CT abdomen confirmatory diagnosis was made in 65 patients (91.55%). An appendectomy was performed in 148 patients (98.6%) via laparoscopic approach in 94 patients, open Mac burney procedure in 32 cases (21.3%) and midline incision in 24 cases (16%). Two patients had an ileocecal resection. The appendix was most commonly located retrocecally (55.3%) in our cohort. The laparoscopic approach was performed in 94 patients (62.6%), and we had to convert in 44 patients due to dissection difficulties (46.8%). Among patients who underwent laparascopic approach 7 had developed peritonitis.. There were only 2 deaths. The mortality rate was 0,013%. The median duration of outpatient followup was 6 months (4-24 weeks) and was uneventful. CONCLUSION: Appendicular abscess is a disease of young adults more common in men. Location of the appendix in our case series was mostly retroceacal. Laparoscopy was associated with good outcomes; peritonitis was uncommon and mortality was rare. The laparoscopic approach is a safe surgical technique for treating appendicular abscess and it can be considered as the routine approach for this condition In developing countries with limited technical resources, laparoscopy guarantees the absence of recurrence, reduces healthcare costs and decreases the risk of treating a severe disease conservatively.

Acute intraperitoneal rupture of hydatid cysts of the liver: Case series.

ABSTRACT: Hydatid cyst is a parasitic infection caused mainly by Echinococcus granulosus, which is generally considered benign. However, the hepatic hydatid cyst rupture in the abdominal cavity is a life-threatening incident that requires urgent and multidisciplinary management (emergency physicians, radiologists, anesthetists, and surgeons). This study describes clinical and paraclinical liver hydatid cyst rupture in the peritoneal cavity and details the appropriate treatment.A retrospective review of clinical records of patients hospitalized in Jendouba Hospital for liver hydatid cyst was performed over 8 years, from January 1, 2012 to December 31, 2019. Fifteen cases of liver hydatid cyst complicated with acute rupture into the abdominal cavity were collected out of 625 hydatid liver cysts. All patients underwent emergency laparotomy allowing conservative unroofing procedure associated with peritoneal lavage and external drainage combined with necessary intensive care measures. Clinical features, therapeutic procedures' details as well as postoperative outcomes are reported. Statistical analysis was performed using the Statistical Package for the Social Sciences for Windows version 20.There were 9 men and 6 women. Patients' age ranged from 14 to 59 years, with an average of 38 years. Two patients were admitted with abdominal trauma. Acute abdominal pain was the most common complaint. Only 1 patient had an anaphylactic shock. Abdominal ultrasonography and computed tomography scan showed discontinuous cyst wall associated to intraperitoneal fluid in all cases. Intraoperatively, the intraperitoneal effusion was clear in 13 cases and purulent in 2. All patients underwent unroofing procedures associated with intra-operative peritoneal lavage and external drainage. The mean hospital stay was 6.11 days, and the mean follow-up was 19 months. No case of recurrence was reported among the patients.In endemic areas, rupture of a hepatic hydatid cyst in the abdominal cavity should be considered in every case of acute abdominal pain, especially if associated with anaphylaxis signs. Early management starting in the emergency room is needed to ensure good outcome.

Surgical management of a hepatic hydatid cyst fistulized into the duodenum: A case report.

INTRODUCTION AND IMPORTANCE: Hydatid cyst of liver is a disease usually seen in endemic regions. Fistulization into duodenum is one of the most exceptional complications of the hydatid cyst commonly discovered during surgery. This paper aims to present and discuss a rare case of cyst ruptured into the duodenum. This case report has been reported in line with the SCARE criteria 2020. CASE PRESENTATION: A 44-year-old female patient, previously diagnosed with hydatid cyst of liver with deferred care due to the period of COVID 19 containment, presented with the complaints of abdominal pain and fever. Abdominal CT scan showed up a 2 cm multiseptal hydatid cyst in the segment III of the liver with an exovesiculation of 20 cm, communicating with the first duodenum. The patient underwent antrectomy involving the first duodenum and removing the cystoduodenal fistula with a Roux-en-Y anastomosis. She was discharged with full recovery on the postoperative 5th day. CLINICAL DISCUSSION: Clinical features of hydatid cyst fistulized into the duodenum are non-specific. There are two pathognomonic symptoms, hydatidemesis and hydatidenteria.Typically, cysto-duodenal fistula is intra-operatively discovered. The CT scan is the most used morphological examination. The presence of air in the cyst should alert for the gastrointestinal fistula formation. Surgical strategies to perform should be tailored to every patient and to intra operative findings. The post-operative morbidity and mortality are underestimated in the literature. CONCLUSION: The fistulization of Hydatid cyst into the duodenum should be evoked in front of any acute abdominal pain whether or not associated with hydatidemesis or hydatidenteria.

The deceitful diagnosis of gallbladder volvulus: A case report.

INTRODUCTION AND IMPORTANCE: The gallbladder volvulus is a rare but life-threatening condition characterized by an axial torsion of the gallbladder along the cystic pedicle, causing gallbladder ischemia and necrosis. This paper aims to present and discuss a rare case of gallbladder volvulus. This case report has been reported in line with the SCARE criteria 2020 [1]. CASE PRESENTATION: We report the case of a 90-year-old female patient who presented to the emergency room with sharp right upper abdominal quadrant pain of acute onset associated with vomiting, evolving for the last 12 h. She had no fever nor jaundice. Her body mass index (BMI) was 22. She had kyphosis, and scoliosis. Physical examination found tenderness with a palpable mass in the right upper abdominal quadrant. Laboratory test results showed leukocytosis at 11600 /mL and a high C-reactive protein rate at 40 mg/L revealed acute calculous cholecystitis features. However, emergency laparotomy was performed and discovered a gallbladder volvulus. A detorsion and cholecystectomy were performed with a good outcome. CLINICAL DISCUSSION: The preoperative diagnosis of gallbladder volvulus is difficult due to its misleading clinical presentation mimicking acute cholecystitis. The presence of the three highly suggestive triad clinical signs should encourage the radiologist to search for a gallbladder with a horizontal orientation and located outside its anatomical fossa connected to the liver by a conical structure corresponding to the twisted pedicle in ultrasonography. Unlike ordinary acute cholecystitis, which may sometimes tolerate an initial conservative medical treatment, gallbladder volvulus management is always an emergency cholecystectomy. CONCLUSION: Despite the clinical similarities with the classical acute calculous cholecystitis, gallbladder volvulus is more likely to result in fatal outcome. Therefore, a high level of clinical suspicion is necessary to save lives.

Gallbladder adenomyomatosis: Diagnosis and management.

INTRODUCTION: Gallbladder adenomyomatosis is a benign acquired gallbladder disease. It can mimic cancer on radiological findings, leading to a diagnostic dilemma. The management and prognosis of these two gallbladder anomalies are entirely different. Therefore, it is essential to recognize the pathognomonic features of gallbladder adenomyomatosis is in order to accurately diagnose this pathology. This paper presents two encountered cases of gallbladder adenomyomatosis is, their diagnostic modalities as well as the algorithmic approach of their management. These two-case reports have been reported in line with the SCARE Criteria 2020 [1]. PRESENTATION OF CASE: Patient-1 was symptomatic. He was explored by an abdominal ultrasound picturing gallbladder wall thickening while the biopsy showed pleomorphic proliferation of inflammatory cells. An examination of the liver with MRI was indicated, showing diffuse parietal thickening with multiple cystic pockets. He underwent laparoscopic cholecystectomy with simple operative follow-up. Patient 2 was asymptomatic, a staging CT scan of acute pancreatitis revealed focal mural thinking of the gallbladder wall. A liver MRI showed an intramural cystic formation in the vesicular fundus. Given the inconclusive imaging results, laparoscopic cholecystectomy was performed. Histological examination confirmed the diagnosis of GA. DISCUSSION: Adenomyomatosis is usually asymptomatic. Imaging can confirm the diagnosis of gallbladder adenomyomatosis without the need for invasive procedures such as vesicular biopsy. Histologic examination can also confirm the diagnosis when cholecystectomy is done. High resolution ultra-sound is the most efficient radiological examination. Laparoscopic cholecystectomy is the gold standard for symptomatic GA or radiological suspicion of a gallbladder cancer. CONCLUSION: The practitioner should always consider gallbladder carcinoma before confirming the GA, as they share the same features but with a far worse prognosis. The likelihood of malignancy depends on radiological characteristics. In the case of inconclusive findings, cholecystectomy is justified.

Heavy metals accumulation from sewage sludge in the Nile tilapia Oreochromis niloticus (Trewavas, 1983) during a sludge-earthworm-fish short-term cycling.

Municipal sewage sludge from waste-water treatment is an important nutritional source for sustainable agriculture. Here, we report on the assessment of the accumulation of heavy metals in Nile tilapia Oreochromis niloticus (Trewavas 1983) fed on earthworms Eisenia fetida reared on soil treated with different concentrations of sewage sludge (25% and 100%) during sludge-earthworm-fish short-term cycling. In this short-term cycling the Nile tilapia collected from the White Nile were chosen as final consumers, whereas the earthworms reared on loam soil mixed with different ratios of sludge were used as a feed for the final consumers. Our results indicate that the concentrations of Cd2+, Cr2+, Pb2+ and Zn2+ in the sludge treated soil are proportional to the sludge content in the soil. Importantly, the accumulation of these heavy metals was significantly low in the earthworms and the Nile tilapia in comparison with the treated soil and that these concentrations in the Nile tilapia were below the international limits recommended by the US Environmental Protection Agency (2014). Moreover, the growth and overall flesh quality of the fish were improved as indicated by the growth increase up to 146% when fed on earthworm reared in 100% sludge. Additionally, our physico-chemical properties (i.e. pH, soil moisture, electric conductivity and organic matters) evaluation on the soil indicates an improvement of the soil quality when mixed with sewage sludge. These results suggest a sustainable application of sewage sludge in fish culture owing to its high nutritional values, low cost, and low risk of hazardous heavy metals when using primary consumers with heavy metals bioaccumulation capability such as E. fetida.