Evaluation of the effectiveness and safety of combined oral and topical photoprotection with a standardized extract of Polypodium leucotomos (Fernblock®) in a Moroccan population with xeroderma pigmentosum.

BACKGROUND: Xeroderma pigmentosum (XP) is a rare autosomal-recessive genodermatosis resulting from a DNA-repair defect syndrome. The purpose was to evaluate the prevention on new malignant lesions in patients taking a supplement with Fernblock® (Polypodium leucotomos extract [PLE]) and secondarily correlation with the photoprotective behavior. METHODS: A prospective, single-center and open cohort study was conducted over a 12-month period. The study was performed in Morocco. Optimal photoprotection behavior was recommended. Patients were instructed to take one capsule containing 480 mg of Fernblock® and 5 mcg vitamin D and to apply sunscreen with a SPF50+ and Fernblock® every 2 h during sun exposure. The demographic, clinical, and dermatoscopic patient data were collected at baseline (T0) and following visits at 3 months (T3), 6 months (T6), and 12 months (T12) when it was assessed: Investigator Global Assessment (IGA), Patient/Guardian Global Assessment (PGA), Patient/Guardian Satisfaction Questionnaire, and Photographic and Adverse Events Registration. Pertinent statistical study was performed. RESULTS: Eighteen patients completed the study. Eleven patients (61%) finished the study without new lesions. Seven patients developed new lesions by the end of the study. Among them, only 30% showed an ideal photoprotective behavior. The lack of an optimal photoprotective behavior increased the probability of developing lesions by 2.5 times with 95% confidence interval. CONCLUSIONS: In our study, more than 60% of patients taking a supplement with Fernblock® did not develop new lesions, and furthermore, we detected that patients following almost ideal photoprotection were 2.5 times less likely to develop NMSC lesions.

Linear IgA bullous dermatosis following Oxford AstraZeneca COVID-19 vaccine.

A case of linear IgA bullous dermatosis developing 3 days after the second dose of Oxford AstraZeneca COVID-19 vaccine in an adult patient, suggesting a possible causal association. It is worth keeping in mind that COVID-19 vaccination could induce immune-mediated bullous disease in susceptible people.

Erysipelas of the leg: A cross-sectional study of risk factors for recurrence.

BACKGROUND: Erysipelas is a common infection of the superficial layer of skin. The main causative agent is group A ß-hemolytic streptococci. One of the most challenging aspects of this disease is its high rate of recurrence. OBJECTIVES: To identify risk factors for recurrence of erysipelas of the leg. PATIENTS AND METHODS: We included in a cross-sectional study all patients hospitalized for erysipelas of the leg seen at the dermatology department of the Ibn Rochd University Hospital in Casablanca, Morocco, from January 2015 to April 2020. Patients were divided into two groups: those with a single episode (SE) and those with recurrent erysipelas (RE). These two groups were compared for clinical and laboratory characteristics, with particular focus on systemic and local risk factors. RESULTS: The study included 270 patients, of whom 132 had SE and 138 had RE. Age, gender, hospital stay as well as laboratory findings did not differ significantly between the two groups. In multivariable analysis, cutaneous disruption [adjusted odds ratio (aOR)=1.9; 95% confidence interval (95% CI): 1.0-3.71], lymphedema [aOR=19.6; 95% CI: 8.0-57.2], and obesity [aOR=2.3; 95% CI: 1.1-5.2] were significantly associated with RE. Venous insufficiency and diabetes were not found to be associated with risk of recurrence. CONCLUSION: Our results suggest that erysipelas is a potentially chronic disease and patients with identified local risk factors or obesity should be considered as exposed to recurrence and considered as targets for antibiotic prophylaxis and other preventive methods.

[Oral tranexamic acid in the treatment of lichen planus pigmentosus: A prospective study of 20 cases]. / Acide tranexamique oral dans le traitement du lichen pigmentogène.

INTRODUCTION: Lichen planus pigmentosus is a variant of lichen planus that is particularly difficult to treat. The aim of this study was to evaluate the efficacy and safety of tranexamic acid in lichen planus pigmentosus. PATIENTS AND METHODS: This prospective study, conducted at the University Hospital of Casablanca from August 2017 to June 2019, included 20 patients with histologically confirmed lichen planus pigmentosus. The exclusion criteria were pregnancy, breastfeeding, known hypersensitivity to tranexamic acid, and thromboembolic disease. Evaluation was carried out by means of clinical examination and measurement of the affected area using the Visioface® RD hardware package at 6 and 12 months (M6 and M12). RESULTS: Eighteen women and 2 men with an average age of 49 years (range: 26-65 years) were included. All patients were phototype 3 or 4. The average disease duration was 2.9 years (range: 2 months-15 years). Pigmented, slate-grey, well-delineated macules were observed in all patients on the face (n=19), neck (n=14) or arms (n=2). Pruritus was present in 9 patients. All patients received oral tranexamic acid 250mg/d for 4 to 6 months with external photoprotection that was prolonged beyond 6 months. At M6, partial improvement was noted in 10 patients, 3 patients showed no improvement, and 7 patients were lost to follow-up. Pruritus disappeared in all patients and no relapse was seen at M12. DISCUSSION: Our study suggests that tranexamic acid could be an effective treatment for lichen planus pigmentosus with a good safety profile. However, the limitations of the study are the limited population and the high number of patients lost to follow-up at M6. Larger-scale studies are needed to provide more detailed results.

[Successful combination of topical corticosteroids and trichloroacetic acid for the treatment of verrucous lichen planus]. / Succès de l'association dermocorticoïdes et acide trichloroacétique dans le traitement du lichen plan verruqueux.

BACKGROUND: Verrucous or hypertrophic lichen planus is a chronic inflammatory skin disease characterized by extremely pruritic thick hyperkeratotic plaques and is resistant to topical treatment. PATIENTS AND METHODS: Herein, we report three clinical cases of hypertrophic lichen planus successfully treated with a combination of topical steroids daily in occlusion and trichloroacetic acid (TCA) 50% with peeling every week. DISCUSSION: TCA is involved in regulating inflammation and scarring. Through its keratolytic properties it enhances the efficacy of topical steroids, whose action is hindered by hyperkeratosis. CONCLUSION: The combination of TCA and topical steroids offers a good alternative for the treatment of hypertrophic lichen planus.

[Acute pancreatitis under acitretin]. / Pancréatite aiguë sous acitrétine.

INTRODUCTION: Retinoids are vitamin A derivatives with numerous indications in dermatology. Acute pancreatitis is a rare adverse effect of systemic retinoids. We report a case occurring during acitretin treatment for psoriasis. PATIENTS AND METHODS: A 27-year-old male patient with no history of diabetes, obesity, alcohol consumption or medication consulted for extensive pustular psoriasis. The lipid balance and liver tests were normal. The patient was treated with acitretin at a dose of 25mg/d. Four days after the start of treatment, the patient was admitted to the surgical emergency room for piercing epigastric pain with vomiting of bile, without transit problems. Serum lipase was 20 times the normal value (1278 IU/L). CRP was raised at 155mg/L and triglycerides were normal at 0.66g/L. Ranson's score was 1 and the abdominal scan revealed Balthazar Grade B pancreatitis with a small amount of peritoneal effusion. The ultrasound examination showed absence of gall stones, without dilation of either the intra- or extra-hepatic bile ducts. Acitretin was discontinued due to its possible causative role. The patient was treated by means of parenteral feeding, strict fasting and a proton-pump inhibitor, and a good clinical outcome with gradual normalization of serum lipase and CRP was achieved in 10 days. The patient was subsequently treated with infliximab for psoriasis, with good results. DISCUSSION: In the event of acute abdominal pain in a patient treated with retinoids, a diagnosis of acute pancreatitis should be considered. This complication can occur in the absence of hypertriglyceridemia.

[Pediatric cutaneous leishmaniasis in Morocco: Clinical and epidemiological features]. / La leishmaniose cutanée de l'enfant au Maroc : particularités cliniques et épidémiologiques.

INTRODUCTION: Cutaneous leishmaniasis (CL) is a parasitic disease caused by a flagellate protozoa of the genus Leishmania and is a public health problem throughout the world. In Morocco, CL may be sporadic or endemic depending on the species of Leishmania and the region involved. Casablanca is a non-endemic city but many children with CL come from elsewhere in the country. The aim of our study is to describe the clinical and epidemiological particularities of CL diagnosed in children at the Casablanca University Hospital. MATERIALS AND METHODS: A cross-sectional study was conducted between 2010 and 2016. All patients with one or more skin lesions suggestive of CL underwent parasitological and molecular investigation. Epidemiological and clinical data from patients with positive genotyping were collected and analyzed. A comparative study was made of epidemiological and clinical variables between children and adults and between different species found in children. RESULTS: One hundred and six cases of CL were diagnosed, of which 40 in children (37.7%): 29 due to L. tropica (72.5%), 7 due to L. major (17.5%), and 4 due to L. infantum (10%). CL caused by L. tropica was significantly associated with children (P=0.009) and was distinguished by the predominance of the nodular form (P=0.04) and a facial location (P=0.007). Compared to adults, childhood CL lesions were characterized by their small size (P=0.001) and facial location (P=0.003). Children were treated mainly with pentavalent antimonials, with good safety and clinical response. CONCLUSION: In our series, CL due to L. tropica was predominant in children, reflecting the epidemic nature and wide geographical distribution of this species in Morocco. CL due to L. tropica was significantly associated with facial location and nodular form in children.

[Cutaneous leishmaniasis of the vermilion border of the upper lip extending to the oral mucosa]. / Leishmaniose cutanée du vermillon avec atteinte muqueuse de la lèvre supérieure.

BACKGROUND: Cutaneous leishmaniasis is endemic in Morocco. Mucosal involvement is rare. We report a case in Morocco of cutaneous leishmaniasis of the vermilion border of the upper lip extending to the oral mucosa due to Leishmania tropica. PATIENTS AND METHODS: A 15-year-old girl was seen with 2 ulcerated lesions, present for 4 months, situated on the left cheek and vermilion border and extending to the oral mucosa. The diagnosis of leishmaniasis was confirmed by direct examination revealing high numbers of Leishmania amastigotes. Culture of the offending organism in NNN medium and isoenzymatic characterization resulted in identification of L. tropica. Treatment with meglumine antimoniate (Glucantime) was ineffective. The outcome was good after treatment with fluconazole. CONCLUSION: In Morocco, cutaneous leishmaniasis with mucosal involvement is rare, and usually develops as a complication of cutaneous leishmaniasis via direct extension.

[Chronic mucocutaneous candidiasis with STAT1 gain-of-function mutation associated with herpes virus and mycobacterial infections]. / Candidose cutanéo-muqueuse chronique avec mutation gain-de-fonction du gène STAT1 associée à des infections herpétiques et à mycobactérie.

INTRODUCTION: Chronic mucocutaneous candidiasis (CMC) is characterized by susceptibility to chronic or recurrent infections with yeasts of the genus Candida affecting the skin, nails and mucous membranes. We describe a Moroccan patient presenting CMC with heterozygous STAT1 gain-of-function (GOF) mutation. PATIENTS AND METHODS: A 5-year-old boy with no consanguinity presented recurrent episodes of oral thrush, chronic nail candidiasis and herpetic gingivostomatitis from the age of 8 months. He also had mycobacterial adenitis secondary to BCG vaccination and atypical rosacea. Genetic analysis revealed GOF mutation of the STAT1 gene. DISCUSSION: CMC was diagnosed in our patient despite poor clinical features. Sequencing of the genome revealed STAT1GOF mutation. This mutation affects production of IL-17, an important cytokine in mucocutaneous defense against Candida. The association with mycobacterial adenitis is rare and continues to be poorly understood. The presence of atypical rosacea in this setting is suggestive of this entity. Antifungal therapy and prevention of complications are necessary to reduce the morbidity and mortality associated with this condition. CONCLUSION: CMC due to STAT1GOF mutation is characterized by a broad clinical spectrum and should be considered in all cases of chronic or recurrent fungal infection, whether or not associated with other infections.

[Pseudotumoral sublingual leishmaniasis caused by Leishmaniainfantum]. / Leishmaniose sublinguale pseudotumorale à Leishmaniainfantum.

INTRODUCTION: Mucosal leishmaniasis is endemic in certain parts of Latin America and are usually absent in Morocco. Herein we report a case of Leishmaniainfantum in a Moroccan patient. PATIENTS AND METHODS: A 61-year-old male patient working as a tourist bus driver presented with a sublingual endobuccal tumor. He reported a history of treated cutaneous leishmaniasis of the lower lip in 2009 and had presented the sublingual oral tumor since December 2011. The histopathological findings as well as the species-specific PCR analysis confirmed the diagnosis of sublingual mucosal leishmaniasis due to L. infantum. HIV serology was negative. Our patient was then treated with intra-muscular meglumine antimoniate for 25 days, resulting in complete disappearance of the oral lesion. CONCLUSION: Our case thus has several peculiarities: the strictly mucosal character of the lesion, occurring in an immunocompetent subject, the unusual pseudotumoral form, and the causative agent, L. infantum, not known for its mucosal tropism in our country.

Comparison of PCR-based methods for the diagnosis of cutaneous leishmaniasis in two different epidemiological scenarios: Spain and Morocco.

BACKGROUND: Cutaneous leishmaniasis (CL) is a disfiguring and stigmatising disease occurring in more than 70 countries across the world including Spain and Morocco. The use of sensitive tests that can differentiate Leishmania species is advised. OBJECTIVE: To evaluate the influence of the epidemiological scenario on the reliability of the PCR techniques and contribute to the selection of the most efficient one for CL diagnosis. METHODS: The sensitivities of parasitological methods and four PCRs were compared in cutaneous samples from 77 patients from Spanish (PSH) and Moroccan hospitals (PMH). Exudates and fresh or paraffin-embedded tissue biopsies were used. RESULTS: None of the PCRs used in this study allowed the diagnosis of all CL cases, showing also some drawbacks. Lmj4/Uni21-PCR displayed the best sensitivity with PMH, but it did not provide positive results in PSH with CL confirmed by other PCRs. Conversely, JW13/JW14-PCR and L. infantum-PCR-ELISA displayed good sensitivities with PSH that were not achieved with PMH. Nested-ITS-1-PCR did not show enough sensitivity with paraffin-embedded tissue biopsies. False-negative results were obtained in 19% of PSH due to unspecific hybridizations of ITS-1 primers with human chromosome1. CONCLUSIONS: PCR should be routinely used in patients with cutaneous lesions compatible with CL and furthermore, the combination of two PCR techniques is advisable. The selection of these PCRs will be influenced by the epidemiological scenario: In areas where L. infantum is endemic, the use of the PCR-ELISA joint with JW13/JW14-PCR seems an appropriate choice, whereas in areas such as Morocco, Lmj4/Uni21 and ITS-1 provide satisfactory results.

[Giardia duodenalis and its involvement in skin diseases]. / Giardia duodenalis et son implication dans diverses dermatoses.

BACKGOUND: Over the last thirty years, the scientific community has become increasingly interested in the intestinal flora, whether commensal or pathogenic, and its impact on other organs. In dermatology, the correlation between intestinal microbial agents and cutaneous lesions is well established. Giardia duodenalis, an intestinal parasite, has been particularly widely studied. The aim of this work is to provide a review of studies demonstrating the involvement of G. duodenalis in various forms of dermatosis. PATIENTS AND METHODS: The data were obtained by an English-language literature search of Medline, PubMed and Google Scholar for the period 1975-2015. Among the thirty case reports since 1976, we selected the twenty most objective and clinically relevant. RESULTS AND DISCUSSION: This review demonstrates that intestinal giardiasis may be an etiological factor, either alone or in combination with other agents, of various dermatoses through inflammatory and allergic mechanisms or intestinal hyperpermeability. The mucocutaneous lesions are varied: urticaria, angioedema, atopic dermatitis, erythema nodosum, Wells syndrome, among others. The role and origin of the infection are often unknown, and it is thus difficult to determine the interval between parasite infestation and the onset of skin lesions. Consequently, a fecal examination to identify G. duodenalis should be considered in chronic urticaria or angioedema, and where atopic dermatitis occurs in adulthood without any specific etiology. Therapeutic test should be done in every suspicion.

[Hidradenocarcinoma of the heel associated with inguinal metastases]. / Hidradénocarcinome du talon associé à des métastases inguinales.

BACKGROUND: Hidradenocarcinoma is a rare malignant tumour involving the sweat glands. It classically arises de novo, only rarely resulting from pre-existing hidradenoma. The literature contains few reports of lymph node metastasis in this tumour. We report a case of a patient with hidradenocarcinoma of the heel associated with inguinal node metastases. PATIENTS AND METHODS: We report the case of a 64-year-old patient with a history of chronic smoking, who in the last two years developed a painless nodule in his right heel, with no prior injury, and which gradually increased in size to become an ulcerated tumour. Physical examination revealed a rounded tumour mass, ulcerated in the centre, and associated with multiple inguinal adenopathies. Histological and immunohistochemical examination was suggestive of hidradenocarcinoma. The patient had undergone extensive local excision with inguinal lymphadenectomy. Histological examination showed infiltration of lymph nodes by the tumour with capsular rupture. Radiotherapy was subsequently given. The outcome was good without recurrence after 34 months of follow-up. DISCUSSION: Hidradenocarcinoma is a rare malignant tumour. Diagnosis is based on histological and immunohistochemical examination. However, hidradenocarcinoma may on occasion be difficult to differentiate from hidradenoma, a benign tumour, hence the interest of complete surgical resection with safety margins even in the absence of cytological malignancy. Local recurrences are common. The occurrence of lymph node metastasis during hidradenocarcinoma has been described only rarely in the literature. Such metastases usually occur after tumour resection. The specific features of our case are the rarity of lymph node metastases in hidradenocarcinoma coupled with the fact that these metastases were discovered upon diagnosis of the primary tumour.

[Nicolau livedoid dermatitis with severe neurological involvement in a child]. / Dermite livédoïde de Nicolau avec atteinte neurologique sévère chez un enfant.

BACKGROUND: Nicolau syndrome is a rare condition consisting in tissue ischemia and necrosis following intramuscular, intra-articular or subcutaneous injection. PATIENTS AND METHODS: Immediately after gluteal intramuscular injection of benzathine-penicillin, a 10-year-old male child presented an extensive painful violaceous lesion on the left buttock associated with urinary incontinence and left lower-limb paresis. Additional underlying muscular damage was supported by high serum levels of creatine kinase and lactate dehydrogenase. Treatment was based on fluid expansion, intravenous steroids and anticoagulants, resulting in improvement of cutaneous and muscular lesions. Improvement in terms of neurological dysfunction was obtained after regular neuromuscular rehabilitation. DISCUSSION: This case underlines the need to prevent Nicolau syndrome by means of compliance with the technical recommendations for intramuscular injections.