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.

Nasolabial Ulcerated Placard Revealing a Primary Cutaneous Aggressive Epidermotropic CD8+ T-cell Lymphoma.

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare entity representing less than 1% of cutaneous lymphomas. It has an aggressive clinical manifestation with a poor prognosis. It is characterized by cytotoxic and epidermotropic CD8+ proliferation. It also expresses the TIA-1 marker. We report a new case for its display and aggressive character, diagnostic difficulty, and good therapeutic response to chemotherapy. This is a 62-year-old female patient admitted to the hospital for a nasolabial ulcerated placard evolving for two years. Clinical examination revealed submandibular lymph nodes. The specimen analysis associated with anatomoclinical manifestation was concluded for a primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. Tumor extent assessment did not show any secondary localization. The blood tests and serology were unremarkable. The patient had benefited from a CHOEP-type multidrug therapy protocol with complete healing of the lesion after three courses of chemotherapy.

[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.

[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.

Giant perianal condyloma acuminatum in an infant without sexual abuse.

Giant condyloma acuminatum is rare in infants. We report a case of giant perianal condyloma in a 10-month-old infant without sexual abuse. The patient was treated by surgical excision associated with electrocoagulation with no recurrence after 2 years. While both sexual and nonsexual transmissions are possible in patients with condyloma acuminatum, sexual abuse must always be considered in children.

Early screening for gestational diabetes mellitus is not associated with improved pregnancy outcomes: an observational study including 9795 women.

AIMS: In addition to screening for hyperglycaemia during pregnancy after 24 weeks of gestation (WG), the current guidelines also suggest screening in early pregnancy and referring women with early gestational diabetes mellitus (eGDM) or overt diabetes (OD) for immediate care. Our aim was to evaluate this strategy. METHODS: This study evaluated, at our hospital (2012-2016), whether the incidence of a predefined composite outcome (preeclampsia, large-for-gestational-age infant, shoulder dystocia) and secondary outcomes was different when women were screened only after 22WG ('late screening only') or before 22WG and treated for eGDM or OD if present, with repeat screening after 22WG if absent ('early ± late screening'). RESULTS: Early ± late screening (n = 4605, 47.0%) increased between 2012 and 2016 (P < 0.0001) and was associated with more risk factors for GDM than late screening only. Glycaemic status differed in both groups (early ± late screening: eGDM 10.3%, GDM 12.1%, OD 0.9% vs. late screening only: GDM 16.8%, OD 1.2%; P < 0.001), with a higher rate of insulin therapy (8.9% vs. 6.0%; P < 0.001) and less gestational weight gain (11.1 ± 5.4 kg vs. 11.4 ± 5.5 kg; P = 0.013) in the early ± late screening group. Rates of those meeting the composite criterion were similar in both groups [11.6% vs. 12.0%, respectively; odds ratio (OR): 1.040, 95% confidence interval (CI): 0.920-1.176; P = 0.53] and remained comparable after adjusting for Propensity Scores (OR: 1.046, 95% CI: 0.924-1.185; P = 0.4790). Rates for secondary outcomes were also similar in both groups. CONCLUSION: While a strategy including early measurement of fasting plasma glucose during pregnancy increases the incidence and care of hyperglycaemia during pregnancy, it may not significantly improve pregnancy outcomes.

Effects of propranolol therapy in Moroccan children with infantile hemangioma.

Infantile hemangiomas are the most common childhood vascular tumors. Propranolol is a ß-adrenergic blocker that has proven effective in the treatment of this tumor. Numerous studies around the world have been published, describing satisfactory responses in pediatric populations with a higher cure rate and fewer adverse effects than when using corticosteroids. The aim of this study was to evaluate the efficacy and adverse effects of propranolol in Moroccan pediatric patients diagnosed with infantile hemangioma who were treated with oral propranolol. A prospective study was conducted from May 2009 to May 2017 in the department of dermatology of a hospital in Casablanca. All the patients who had infantile hemangioma were included. The study comprised 121 patients with infantile hemangioma: 90 girls and 31 boys. The mean age was 6 months. The majority of hemangiomas were mixed (63%) and located on the face and neck. The treatment was well tolerated by all the patients. The dosage of propranolol was gradually increased from 1mg to 2mg/kg/day. We noted a decrease in coloration after 48hours. The healing period for ulcerated hemangiomas was 20 days. A decrease in size was noted after 1 month, while a decrease in palpebral obstruction occurred after 3 days. Treatment with propranolol in this group of Moroccan pediatric patients proved to be safe and effective at a dose of 2 mg/kg/day, reducing the size and coloration of the hemangioma. Treatment should be stopped at an appropriate time, which is determined primarily by the lesion regression rate after propranolol treatment.

[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.

Paronychia-like cutaneous leishmaniasis.

Chronic paronychia is an inflammatory recalcitrant disorder affecting the nail folds. We report one patient with paronychia revealing ungueal leishmaniasis. A 34-year-old man, resident in the north of Morocco, presented with a 6-month history of an inflamed proximal nail fold of the left thumb, resistant to antibiotics and anti-fungal treatments. En bloc excision of the proximal nail fold was done. The histopathological exam showed epithelioid granulomas with giant cells and the presence of leshmania amastigotes, leading to the diagnosis of ungueal leishmaniasis. Clinical aspects of cutaneous leishmaniasis can be very misleading. The paronychial form is rarely described. In endemic areas it is necessary for the physician to be aware of atypical skin presentations of leishmaniasis.

[Deep-seated granulomatous pyoderma gangrenosum: A novel variant?]. / Pyoderma gangrenosum granulomateux profond : une forme originale de pyoderma gangrenosum ?

BACKGROUND: We report an extremely rare case of pyoderma gangrenosum with the clinical features of pyoderma gangrenosum but with unusual histopathological findings and deep massive granulomatous infiltration through the dermis. PATIENTS AND METHODS: A 15-year-old girl presented with a two-month history of deep ulcers on the lower legs and forehead. She also presented abdominal pain with diarrhea and vomiting that disappeared after symptomatic treatment. Histology showed extensive granulomatous infiltration in the dermis and hypodermis and a micro-abscess in the dermis. An abdominopelvic CT scan revealed peritoneal fluid and tissue formation in a lateral carotid cave (6.56 cm/4.56 cm). Colonoscopy was normal. The patient was treated with oral prednisone, resulting in resolution of the problems of cutaneous lesions, peritoneal fluid and tissue formation. DISCUSSION: Our case involves a rare presentation of pyoderma gangrenosum (PG) inconsistent with any of the four clinical variants of PG and characterized histologically by the presence of deep granulomas in the dermis and hypodermis. Despite the atypical clinical presentation and unusual histopathological findings, PG seemed the most likely diagnosis after infectious diseases had been ruled out; the good outcome achieved with oral corticosteroids supported our diagnosis.

[Efficacy of pulsed-dye laser on residual red lesions of cutaneous leishmaniasis]. / Efficacité du laser à colorant pulsé sur les lésions résiduelles rouges de la leishmaniose cutanée.

BACKGROUND: Cutaneous leishmaniasis caused by Leishmania tropica can leave troublesome and unsightly lesions. Treatment of these scars remains difficult. Pulsed-dye laser (PDL) is one therapeutic approach that may improve the clinical appearance of erythematosus lesions. The purpose of this retrospective study was to evaluate the effectiveness of PDL on the residual red lesions of erythematous facial leishmaniasis in three patients. PATIENTS AND METHODS: Case no. 1: a 14-year-old girl presented an ulcerative and erythematous nodular lesion on her left cheek. One month after treatment, an erythematous lesion measuring 3 cm persisted on the patient's cheek, without atrophy or hyperpigmentation. PDL 595nm was used at the following settings: duration: 3ms; spot size: 7mm; energy: 8 j/cm(2). Case no. 2: a 43-year-old woman presented an erythematous papular lesion on her right cheek. Following treatment, a 4-cm hypertrophic, red telangiectasic lesion remained. PDL 595nm was used with the following settings: pulse duration: 3 ms; spot-size: 10mm; energy: 8 j/cm(2). Case no. 3: a 60-year-old woman presented an erythematous papular lesion on her cheek. After treatment, an infiltrated erythematous macule with surface telangiectasia measuring 3.5cm remained. PDL 595nm was also given using the following settings: pulse duration: 3 ms; spot size: 10mm; energy: 8 j/cm(2). All three patients underwent three sessions of PDL. The erythematous and telangiectasic lesions showed improvement after the initial session and had completely disappeared after the third session. Post-laser purpura subsided within around 10 days. Therapeutic response was assessed clinically by comparing photographs taken before and after treatment and follow-up lasted 12 months. DISCUSSION: Cutaneous leishmaniasis caused by L. tropica is endemo-epidemic in Morocco. A number of treatments are available for red residual lesions but thanks to its effect on erythematous and vascular lesions, PDL has been shown to provide the most reproducibly good results and is the laser method of choice for this type of scar. A recent study of the dermoscopic features of DL identified the presence of vascular patterns in 100% of cases in this infection, which may account for the efficacy of PDL. PDL results in selective thermolysis that destroys small vessels. Our study showed improvement with PDL regarding scar size, pliability, erythema and texture. Further larger-scale studies could better determine the place of PDL in treating the sequelae of cutaneous leishmaniasis.