H Syndrome: Three New Cases from Morocco.

A 19-year-old girl presented with symmetric and bilateral hyperpigmentation, an indurated lesion that initially appeared on the axillary fold at the age of 14, which then extended to the lower back, anterior aspect of both thighs, and popliteal fold. No hypertrichosis was observed (Figure 1).The patient was the youngest of the four children, born from the first-degree consanguineous marriage. She was born at full term and weighed 2,420 g at birth. No similar patient was present in the family. The patient experienced delayed motor acquisition and stature growth (3rd percentile) until the age of 4. Right hypoacusis was diagnosed at the age of 6. She developed hallux valgus, flexion contracture of the fin-gers and toes, barrel deformity of the anterior thorax, and recurrent fever. The laboratory tests, including fasting blood glucose, -triglycerides, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were normal. Her abdominal, pelvic, and transthoracic ultrasound scans were normal, with no hepatosplenomegaly, lymphadenopathy, or cardiac abnormalities. Histologic analysis demonstrated patchy acanthosis of the epidermis, with orthokeratotic hyperkeratosis. Keratinocyte hyperpigmentation and spongiosis at certain areas were observed with moder-ate inflammation because of the infiltration of lymphocytes, histiocytes, and plasma cells. Immunohistochemical analysis showed macrosialin (CD68+) and common gamma chain (γc) CD132. Germline mutations in the SLC29A3 gene were not analyzed. The patient was prescribed dermocorticoids with depigmentation therapy, which demonstrated moderate clinical evolution.

Evaluation of serum levels of sestrin 2 and betatrophin in type 2 diabetic patients with diabetic nephropathy.

BACKGROUND: Diabetic kidney disease (DKD) is one of the most serious microvascular complications of diabetes mellitus (DM) and the leading cause of chronic kidney disease (CKD) worldwide. Since obesity and type 2 DM (T2DM) are considered as inflammatory conditions, thus reducing their accompanied systemic inflammation may lessen their complications. Sestrin 2 belongs to a group of stress induced proteins which are produced in response to oxidative stress, inflammation and DNA damage. Betatrophin; a hormone that stimulates the growth, proliferation and mass expansion of pancreatic beta-cells and improves glucose tolerance. The objective of the study was to evaluate levels of serum Sestrin 2 and betatrophin in patients with different stages of diabetic nephropathy (DN)) and compare results with healthy control. METHODS: This cross sectional study was carried out on 60 patients above 18 years old, recruited from Tanta University hospitals out patients clinics and 20 apparently healthy individuals of matched sex and age as a control group. Participants were divided into two groups: group I: 20 normal subjects as control group and group II: 60 patients with type 2 DM,. further subdivided in to three equal groups: group 1IIA(20 patients) with normo-albuminuria (ACR < 30 mg/g), group IIB (20 patients) with micro albuminuria (ACR = 30 to 300 mg/g) and group IIC (20 patients) with macro albuminuria (ACR > 300 mg/g). They were subjected to detailed history taking, careful clinical examination and laboratory investigations including blood urea, serum creatinine, estimated glomerular filtration rate (eGFR), urinary albumin creatinine ratio, and specific laboratory tests for Sestrin 2 and Betatrophin by using ELISA technique. RESULTS: Serum Sestrin 2 significantly decreased, while serum betatrophin level significantly increased in macroalbuminuric group compared to control and other 2 diabetic groups (P value < 0.05). The cut off value of serum sestrin 2 was 0.98 ng/ml with sensitivity 99%, specificity 66% while the cut off value of serum betatrophin was > 98.25 ng/ml with sensitivity 98%, specificity 82%. Serum betatrophin positively correlated with age, fasting, 2 h postprandial, BMI, triglyceride, total cholesterol, serum creatinine, blood urea, UACR, and negatively correlated with eGFR and serum albumin. Serum Sestrin 2 positively correlated with serum albumin. BMI, serum urea, UACR and serum albumin. Serum betatrophin are found to be risk factors or predictors for diabetic nephropathy. CONCLUSIONS: Patients with DN, particularly the macroalbuminuria group, had a significant increase in betatrophin levels and a significant decrease in serum Sestrin 2 level. The function of Sestrin 2 is compromised in DN, and restoring it can reverse a series of molecular alterations with subsequent improvement of the renal functions, albuminuria and structural damage.

An atypical form of pediatric pityriasis rosea of the diaper area: Pityriasis marginata et circinata of Vidal.

Pityriasis rosea is a common, self-limited disease which in its typical form should not raise diagnostic doubts. Nevertheless, its atypical presentations can be a challenge for the clinician, and hence it is important to be aware of them. Recognition of these forms may enable a correct diagnosis to be made and avoid unnecessary procedures. We present two patients for whom we believe atypical PR was the most likely the diagnosis.

Effectiveness of Narrowband Ultraviolet Light in Chronic Kidney Disease-Associated Pruritus.

INTRODUCTION:  Chronic kidney disease-associated pruritus (CKDaP) is a prevalent and challenging symptom in individuals suffering from advanced chronic kidney disease (CKD). Its underlying mechanism remains inadequately understood, leading to a limited array of unsatisfactory therapeutic interventions. Despite various attempts, identifying the most effective treatment remains inconclusive. Nevertheless, there is a growing interest in employing ultraviolet phototherapy, particularly for non-responsive patients, although its efficacy is not definitively established. To investigate the potential benefits of narrowband ultraviolet B (NB-UVB) phototherapy on individuals experiencing CKDaP, we report our experience with NB-UVB light in management of CKDaP in dialysis patients. MATERIALS AND METHODS: The study group consisted of patients with end-stage chronic kidney disease who underwent hemodialysis. These patients received dermatological consultations and follow-ups for itching. They were all unresponsive to the conventional treatment (emollients and antihistamines). Screening laboratory examinations, including complete blood count, liver function test, thyroid function, electrolytes, and others, were also arranged to exclude systemic etiologies. The main potential pruritogens were dosed: calcium, phosphate, and parathyroid hormone. Itch intensity was evaluated with a numerical rating scale (0-10), based on the worst level of itching in the past two weeks. They had sessions of NB-UVB light (311 nm, TL01) twice per week. After UVB exposure, patients were advised to use topical emollients. A questionnaire was employed to document the extent, intensity, frequency, and sleep disruption experienced to evaluate the efficiency of the treatment, using a scale from 0 to 10.  Results: In a group of 38 patients, the average age of the patients was 56 years (16-80); 63.2% were female and 36.8% were male. Median duration of pruritus was 4.7 years, and that of dialysis was 8.4 years. Pruritus was intermittent and diffuse in most cases, localized to the arteriovenous fistula site in two cases, and exacerbated by heat in all cases. Itch intensity was evaluated with a numerical rating scale (0-10) based on the worst level of itching in the past two weeks and showed a moderate average score (5/10). Xerosis was found in 63%, and scratch lesions such as excoriation in 34%. NB-UVB phototherapy was used twice per week on nonconsecutive days, with protection of the genital area and also the eyes using UVB-blocking goggles. The initial dose was 0.4 J/cm2 and further doses were introduced according to the erythema response until a maximum of 2 J/cm2. No sunburn, hyperpigmentation, or blistering was noted. Emollients were maintained in patients with xerosis. Average number of sessions was 13 (6-24) and reduction of itch intensity was observed starting from the sixth session. Total improvement was obtained at the end of treatment duration except for three patients who required additional sessions. One patient had recurrence one year later.  Conclusion: In conclusion, phototherapy represents a significant advancement in the treatment options for CKD-associated pruritus. Its positive impact on reducing itching and improving the quality of life for many patients is undeniable. However, to fully unlock its potential, ongoing research is needed to optimize dosing, understand relapse mechanisms, and identify the patients who will benefit most from this therapy.

Vitamin B12 induced acneiform eruption.

A 47-year-old, north african, male patient, has recently been diagnosed with pernicious anemia, treated with weekly intramuscular hydroxocobalamin. 6 weeks after its initiation, the patient presented a sudden, extensive and monomorphic eruption of inflammatory papulo-pustules and nodules, affecting the face, and the trunk. The eruption was pruritic, and comedones were also present, on the chest. The patient was diagnosed with vitamin B12-induced acneiform eruption. Levels of vitamin B12 were normalized. Hydroxocobalamin was therefore stopped and lymecycline was started, allowing a complete resolution of the lesions within 3 months. Drug intake, sudden and uncommon age of onset, pruritus, a monomorphic pattern and an involvement of extra-seborrheic areas are features that distinguish acneiform eruptions from acne vulgaris.

Focal Dermal Hypoplasia: Case Series.

Background: Focal dermal hypoplasia (Goltz syndrome) is a genetic multisystem disorder characterized primarily by involvement of the skin associated to face, skeletal, and eyes anomalies. The objective of the present series is to shed light on this rare syndrome and these atypical manifestations. Methodology: Our study reports the case of five Moroccan patients who present typical clinical picture of the Goltz syndrome with some rare manifestations. Results: A total of 5 patients with Goltz syndrome were evaluated. All of them are female with one familial case. The age ranged from 8 months to 35 years. A characteristic Blaschkoid hypo- and hyper-pigmented skin lesions, congenital nodular fat herniation, and skin atrophy were present in all patients. Ocular manifestations were present in 80% of patients. Cranio-facial deformity was seen in 80% of patients. Short stature and intellectual delay were documented in 80% and 40% of patients, respectively. Limb abnormality was found in all patients. Two patients had a cleft lip, one of which unusual lateral facial cleft. Limitations: Genetic testing could not be performed in the present series. Conclusions: Through this work we will discuss the different clinical signs and genetic aspects of Goltz syndrome and the interest of a good clinical expertise.

COVID-19 in Three Patients with Pemphigus.

COVID-19 is an infectious disease caused by the Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2). It has various clinical presentations, ranging from mild and moderate to severe manifestations. This infection has caused disruptions worldwide, particularly for healthcare professionals, regarding the prescribing of immunosuppressive drugs for chronic autoinflammatory and autoimmune pathologies, such as pemphigus. We report three pemphigus patients who being treated with immunosuppressive therapy developed the SARS-CoV-2 infection. (SKINmed. 2022;20:428-429).

Development and validation of knowledge of caring for COVID-19 tool.

AIM: We aimed to examine the validity and reliability of a newly developed tool that measures nursing and allied health students' knowledge of caring for patients with COVID-19. METHOD: We developed in five phases (literature review, item development, expert evaluation using content validity index, revisions based on a pilot test, and exploratory factor and reliability analyses on the data from the main study). We administered online surveys (the pilot test & the main study using a cross-sectional design) to students in a governmental health sciences college in Oman. RESULTS: The pilot test had 43 participants, and the main study had 507. The item content validity index scores ranged from 0.81-1.00, and the scale content validity index for 20 items was 0.95. The factor analysis revealed a three-factor solution. The overall Cronbach's alpha for the final (19-item) version of the tool after item deletion was 0.76. The reliabilities for Subscales 1, 2, and 3 were 0.83, 0.67, and 0.51, respectively.

Ameliorative effect of vitamins (E and C) on biochemical alterations induced by sublethal concentrations of zinc oxide bulk and nanoparticles in Oreochromis niloticus.

The comparison between bulk and nano ZnO particles were antecedently studied but describing the dose-dependent toxicity and the ameliorative effect of vitamins (E and C) in Oreochromis niloticus, have not been previously documented. Therefore, the present study was designed to investigate the ameliorative effect of vitamins (E and C) against oxidative stress and biochemical alterations induced by sublethal concentrations of zinc oxide bulk particles (ZnOBPs) and zinc oxide nanoparticles (ZnONPs). Toxicity tests were carried out on O. niloticus and showed that 96 h LC50 values of ZnOBPs and ZnONPs were 84 mg/l and 5.6 mg/l respectively. Exposure of the studied fish to these sublethel concentrations for 7, 14, 21 & 28 days showed a significant increase (p < 0.05) in serum glucose, AST, ALT, creatinine, urea and uric acid compared to control groups while, fish groups exposed to ZnOBPs or ZnONPs and supplemented with vitamins E and C, their serum enzyme concentrations were decreased compared to the groups without supplementation after 7, 14, 21 and 28 day. On the other hand, antioxidant defense enzymes (SOD, CAT and GST) activity in O.niloticus fish were increased significantly (p < 0.05) when exposed to sublethal concentrations of ZnOBPs or ZnONPs compared to the control value. However, fish groups supplemented with vitamins (E and C) have a decrease in SOD, CAT and GST enzymes activity when compared to unsupplemented groups and the values returned to similar levels established in the control at low concentrations but still higher than control at the high concentrations.

Effect of treatment with direct antiviral agents (DAAs) on glycemic control in patients with type 2 diabetes mellitus & hepatitis C virus genotype 4.

BACKGROUND AND AIMS: It is widely recognized that chronic hepatitis C is a metabolic disease that is strongly associated with type 2 diabetes mellitus (T2DM) and insulin resistance (IR). The evidence behind the effect of Direct Anti-Viral Agents (DAAs) therapy on T2DM is conflicting. The aim of the present study was to evaluate the effect of treatment with DAAs on glycemic control in patients with type-2 diabetes mellitus and chronic hepatitis C virus genotype 4. METHODS: This study was a prospective study that conducted on 100 patients with chronic hepatitis C and Type-2 diabetes mellitus, selected from Kafr El-Sheikh Liver Research Center treated with Direct Anti-Viral Agents (DAAs) during the period from September 1, 2017 to last of August 2018. All patients in the study were subjected to the following: Full history taking stressing on the age, gender, previous treatment; clinical examination and laboratory investigations. HBA1C was assessed before and after DAAs treatment. RESULTS: In the present study, there was a significant decrease of baseline fasting blood glucose levels after treatment when compared with before treatment. Also, there was a significant decrease of 2 h post prandial blood glucose after treatment when compared with before treatment. There was significant decrease of HBA1c levels after treatment when compared with before treatment. CONCLUSIONS: DAAs treatment significantly improved the fasting blood glucose and help better glycemic control. This study augments the importance and the benefits of new Direct Anti-Viral Agents interferon free regimens in diabetic HCV infected patients.

Hopes and Limits of Adipose-Derived Stem Cells (ADSCs) and Mesenchymal Stem Cells (MSCs) in Wound Healing.

Adipose tissue derived stem cells (ADSCs) are mesenchymal stem cells identified within subcutaneous tissue at the base of the hair follicle (dermal papilla cells), in the dermal sheets (dermal sheet cells), in interfollicular dermis, and in the hypodermis tissue. These cells are expected to play a major role in regulating skin regeneration and aging-associated morphologic disgraces and structural deficits. ADSCs are known to proliferate and differentiate into skin cells to repair damaged or dead cells, but also act by an autocrine and paracrine pathway to activate cell regeneration and the healing process. During wound healing, ADSCs have a great ability in migration to be recruited rapidly into wounded sites added to their differentiation towards dermal fibroblasts (DF), endothelial cells, and keratinocytes. Additionally, ADSCs and DFs are the major sources of the extracellular matrix (ECM) proteins involved in maintaining skin structure and function. Their interactions with skin cells are involved in regulating skin homeostasis and during healing. The evidence suggests that their secretomes ensure: (i) The change in macrophages inflammatory phenotype implicated in the inflammatory phase, (ii) the formation of new blood vessels, thus promoting angiogenesis by increasing endothelial cell differentiation and cell migration, and (iii) the formation of granulation tissues, skin cells, and ECM production, whereby proliferation and remodeling phases occur. These characteristics would be beneficial to therapeutic strategies in wound healing and skin aging and have driven more insights in many clinical investigations. Additionally, it was recently presented as the tool key in the new free-cell therapy in regenerative medicine. Nevertheless, ADSCs fulfill the general accepted criteria for cell-based therapies, but still need further investigations into their efficiency, taking into consideration the host-environment and patient-associated factors.

Addictive behaviors, cardiovascular and metabolic comorbidities in North African psoriatic patients: case-control study.

We propose to study the epidemiological aspects of North African psoriasis and determine the cardiovascular comorbidities and addictive behaviors associated with psoriasis. This is a North African case-control study which was conducted over a five year period (October 2008 through August 2013), involving 671 psoriatic patients and 1,242 controls identified in various Algerian, Tunisian and Moroccan university hospitals. For each patient, epidemiological characteristic, addictive behaviors, and cardiovascular pathologies associated with psoriasis were noted. Six hundred and seventy one psoriasis patients and 1,242 controls were included in this study. The average age was 47.24 years and the M/F sex-ratio was 1.11 (354 men and 317 women). Statistical analysis showed that psoriasis patients were more likely to develop addictive behaviors than controls (smoking p<10-5 and alcohol consumption: p < 10-5), together with dyslipidemia (30.1% of patients p < 10-5), obesity (23.8% of patients p < 10-4), hypertension (22.3% of patients p < 10-5), diabetes (21.7% of occurrences p < 10-5) and metabolic syndrome (37.4% of patients p<10-5). The relative risk for developing psoriasis was 1.9 in hypertensive patients, 1.7 in diabetic patients, 3.9 in dyslipidemic patients, 1.8 in obese patients, 2.6 in those with metabolic syndrome, 2.1 in smokers and 2.8 in alcoholics. Our work confirms the high incidence of addictive behaviors and of cardiovascular and metabolic comorbidities during the North-African psoriasis, hence the need for a multidisciplinary comprehensive care based on a guideline suited to the characteristics of North-African psoriatic patients.

Multisystem Langerhans Cell Histiocytosis in Adults Revealed by Skin Lesions.

A 37-year-old woman with no remarkable medical or family history presented with papules and vesicles on an erythematous background involving the neck, sacrum, and folds (postauricular, axillary, inguinal, and under the breasts) (Figure 1). During the previous year, she was treated with local and systemic antifungals without improvement. Her history included a secondary amenorrhea, polydipsia, and polyuria (6 L/d) that started 2 years prior. Physical examination revealed chronic bilateral purulent otorrhea with thick eardrums. Histologic examination of skin biopsy revealed a highly suggestive appearance of multisystem Langerhans cell histiocytosis (LCH) with immunohistochemistry (anti-PS100 and anti-CD1a), which were positive (Figure 2A and 2B). Pituitary magnetic resonance imaging showed a thickening of the pituitary stalk in relation to a location histiocytic (Figure 3). Bone gaps were objectified on two radiographic tibial diaphyseal. Results from computed tomography (CT) scan showed a magma coelio mesenteric, axillary, and inguinal lymph nodes.

EPIMAG: International Cross-Sectional Epidemiological Psoriasis Study in the Maghreb.

BACKGROUND: Psoriasis is a common skin disorder that is characterized by red plaques covered with silvery scales and is associated with considerable psychosocial impact. It has been described in several studies worldwide, but specific data from the Maghreb (Algeria, Morocco and Tunisia) are unavailable. OBJECTIVES: To characterize the frequency of new psoriasis cases and to describe the epidemiological and clinical profile of psoriasis in the Maghreb. METHODS: A psoriasis working group for the Maghreb initiated the EPIMAG international multicentre cross-sectional observational epidemiological study coupled with a 2-week psoriasis screening study via medical consultation. Data were collected via questionnaires. RESULTS: The total analysis population included 373 pre-existing and 326 new psoriasis cases, described by 261 participating investigators. The frequency of new psoriasis cases was 10.26/1,000 in Algeria, 15.04/1,000 in Morocco and 13.26/1,000 in Tunisia, and thus 12.08/1,000 in the Maghreb. In all 699 psoriasis subjects, the mean age was 46 years, the mean BMI was 26.6, and 55.7% of subjects were men. Two thirds of the subjects had never smoked, and 85.0% had never consumed alcohol. Half had brown skin, and 28.6% had a family history of psoriasis. Three quarters had localized psoriasis, 85.8% had plaque psoriasis, coupled with pruritus in over 70.0% of cases. Flares or outbreaks were most often triggered by stress (79.4%) and change of season (43.1%). The majority of subjects used topical therapy, and the investigators considered overall treatment efficacy to be partial in over half of the cases. Among patients with pre-existing psoriasis, secondary analyses showed that 73.2% had severe psoriasis, and that quality of life was severely affected in 40.1% of cases. The mean number of missing school or work days over 6 months was 3.2 (±12.1) days. CONCLUSIONS: Our study provides novel information relative to psoriasis epidemiology and characterization in the Maghreb and highlights the need to improve psoriasis screening and management in the region. The data will help optimize psoriasis management, to ensure appropriate national health care policies.

Validation of analytical breast cancer microarray analysis in medical laboratory.

A previously reported microarray data analysis by RISS algorithm on breast cancer showed over-expression of the growth factor receptor (Grb7) and it also highlighted Tweety (TTYH1) gene to be under expressed in breast cancer for the first time. Our aim was to validate the results obtained from the microarray analysis with respect to these genes. Also, the relationship between their expression and the different prognostic indicators was addressed. RNA was extracted from the breast tissue of 30 patients with primary malignant breast cancer. Control samples from the same patients were harvested at a distance of ≥5 cm from the tumour. Semi-quantitative RT-PCR analysis was done on all samples. There was a significant difference between the malignant and control tissues as regards Grb7 expression. It was significantly related to the presence of lymph node metastasis, stage and histological grade of the malignant tumours. There was a significant inverse relation between expression of Grb7 and expression of both oestrogen and progesterone receptors. Grb7 was found to be significantly related to the biological classification of breast cancer. TTYH1 was not expressed in either the malignant or the control samples. The RISS by our group algorithm developed was laboratory validated for Grb7, but not for TTYH1. The newly developed software tool needs to be improved.