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.

Compound heterozygous SLC29A3 mutation causes H syndrome in a Moroccan patient: A case report.

H syndrome is an autosomal recessive syndrome, which affects the skin and some vital organs, it is caused by mutations in the SLC29A3 gene, encoding the human equilibrative nucleoside transporter hENT3. This report describes a patient with typical features of H syndrome. Based on the patient's clinical features, SLC29A3 was selected for molecular investigation. Through direct sequencing, a compound heterozygous alteration in the SLC29A3 gene was found. The c.243delA frameshift mutation leading to a premature termination, resulting in a truncated protein, and a splice site mutation c.300+1G>C predicted to cause a splicing error. This contribution extends the clinical variability of compound heterozygous SLC29A3 mutations resulting in an additional multisystemic manifestation of the clinical spectrum of SLC29A3 disorders.

[Actinomycosic mycetoma of the foot in Morocco due to Actinomycetes viscosus]. / Mycétome actinomycosique du pied à Actinomycetes viscosus au Maroc.

We present the case of an actinomycotic mycetoma of the foot due to Actinomycetes viscosus. It evolved for nine years on the foot of a 26-year-old patient from a rural environment: Douar Inezgane (city in southern Morocco). Bacteriological study of the skin and grains confirmed the diagnosis. It showed positive bacilli on direct examination and on Gram staining and in positive culture. Histological study showed a polymorphous granulomatous inflammation without signs of malignancy with actinomycotic grains. Then we retained the diagnosis of primary cutaneous actinomycosis without visceral locations. The treatment was based on antibiotics: penicillin G by intravenous infusion for five weeks, relayed orally by amoxicillin associated with trimethoprim-sulfamethoxazole for long periods. After six months of treatment, we observed a favorable outcome with reduction of the swelling, nodules, lymphadenopathy, fistula's number and extension of time of issue of grains. The current follow up is 15 months. The primary cutaneous actinomycosis is still relevant in Morocco.

[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.

[Palmoplantar keratoderma: a rare manifestation of myxoedema]. / Kératodermie palmo-plantaire : une manifestation rare du myxœdème.

BACKGROUND: Herein we report a rare case of acquired palmoplantar keratoderma in association with myxoedema and hypothyroidism. PATIENTS AND METHODS: A 53-year-old woman presented with palmoplantar keratoderma, dry skin, muscular weakness and cramps for 9 months. The laboratory work-up revealed autoimmune thyroiditis with hypothyroidism. Skin biopsy showed chronic eczema. Other causes of acquired palmoplantar keratoderma were ruled out. Rapid improvement was achieved within 3 weeks of institution of hormone replacement therapy. DISCUSSION: Although this association is very rare, hypothyroidism must be suspected in patients with acquired palmoplantar keratoderma, particularly when it occurs in association with systemic symptoms.

[Acrodermatitis enteropathica-like disease in a full-term exclusively breast-fed infant]. / Pseudo-acrodermatite entéropathique chez un nourrisson né à terme, exclusivement nourri au sein.

UNLABELLED: Symptomatic zinc deficiency can occur in exclusively breast-fed infants. We report a case in a 5-month-old infant. CASE REPORT: A 5-month-old exclusively breast-fed boy presented with persistent erythema of breech since the age of 2 months as well as perioral and acral erythematous scaly lesions. Laboratory investigations revealed low zinc levels in the infant's serum, the mother's plasma zinc level was normal, and breast milk zinc was low. Rapid healing occurred after oral zinc supplementation. DISCUSSION: Zinc deficiency in breast-fed infants is a rare disease caused by a low level of zinc in the mother's milk. The clinical features resemble those of acrodermatitis enteropathica. Oral zinc supplementation is required until weaning.

[Renal ultrasound in fat necrosis]. / Intérêt de l'échographie rénale dans la cytostéatonécrose sous-cutanée.

Subcutaneous fat necrosis is an uncommon disease that may be complicated with potentially fatal hypercalcemia or with nephrocalcinosis. We report on the case of a patient with a history of significant perinatal asphyxia, hospitalized for a urinary tract infection. Lesions of subcutaneous fat necrosis were noted, with asymptomatic hypercalcemia at 3.9mmol/L. A renal ultrasound was performed and showed echogenic medullary pyramids bilaterally, consistent with nephrocalcinosis and left nephrolithiasis. The treatment of hypercalcemia included hyperhydration, a diuretic and corticosteroids. Progression was characterized by the total regression of skin lesions and normalization of serum calcium. Hypercalcemia is a rare complication of subcutaneous fat necrosis. It develops within days to weeks after the appearance of skin lesions. Nephrocalcinosis appears after several weeks or months. Hypercalcemia must be treated in due time to avoid the impact on the kidney.

[Purpura fulminans, venous thrombosis and constitutional thrombophilia in an infant]. / Purpura fulminans et thrombose veineuse compatible avec une thrombophilie constitutionnelle chez un nourrisson.

The association of idiopathic purpura fulminans (PF) and venous thrombosis (VT) seldom reveals constitutional thrombophilia in an infant. We report a case of PF in an 18-month-old infant. Laboratory tests showed disseminated intravascular coagulation (DIVC) with normal rates of C and S proteins and antithrombin. The echo-Doppler examination conveyed venous thrombosis of the lower limbs, while the genetic study showed heterozygous mutation of Factor II (G 20210A). Precocious and multidisciplinary management included frozen fresh plasma supplementation and necrosectomy with skin grafts. The diagnosis and therapeutic problems posed by PF combined with deep venous thrombosis are discussed.

[Generalized chromomycosis caused by Phialophora verrucosa]. / Chromomycose cutanée diffuse àPhialophora verrucosa.

BACKGROUND: Chromomycosis is a chronic fungal skin infection that generally presents in the form of verrucous or vegetative lesions on uncovered areas of skin. We report an unusual case of generalised chromomycosis due to Phialophora verrucosa. PATIENTS AND METHODS: A 42-year-old town-dwelling housewife was hospitalised for erythematous keratotic nodules on the arm showing sporotrichoid distribution, associated with a crusted ulcerative lesion on the homolateral index finger, as well as subcutaneous papulonodular lesions. In places, the lesions on the patient's back presented an umbilical and molluscoid appearance. The patient had suffered no previous injuries and had not visited any areas in which leishmaniasis is endemic. Her history included insulin-dependent diabetes. Screening for Leishman bodies was negative. Histopathological analysis of the skin biopsy revealed an epithelioid giant-cell granuloma with no caseous necrosis. The mycological study demonstrated the presence of fumagoid bodies and P. verrucosa was isolated. Treatment with terbinafine was initially given, followed by clarithromycin, but in the absence of any improvement, the patient was readmitted to hospital and is currently on itraconazole and amphotericin B. DISCUSSION: The novel features of our case comprise the clinical aspect of chromomycosis, the extent of the lesions, their unusual site on the back and upper limbs, and the isolation of a rare species, P. verrucosa (only the second observation in Morocco). It also highlights the therapeutic difficulties posed by this type of chromomycosis.

[Pyoderma gangrenosum and Behçet's disease: a study of two pediatric cases]. / Pyoderma gangrenosum et maladie de Behçet : étude de 2 observations pédiatriques.

Pyoderma gangrenosum is a rare neutrophilic dermatosis that affects 4% of children. It is characterized by its association in half of the cases with an underlying disease. The association with Behçet's disease is exceptional and may have a very poor prognosis. We report 2 pediatric observations with fatal outcome.

Syringocystadenoma papilliferum: Unusual location.

Syringocystadenocarcinoma papilliferum (SCAP) is rare. We describe a 55-year-old man with a partially eroded lesion on the trunk that developed over one year. Histopathological examination revealed syringocystadenoma papilliferum. A wide excision was performed to remove the tumor. The patient has been well without relapse or metastasis for 8 months.

[Suppurations of the perineal and gluteal region: An aetiological study of 60 cases]. / Suppurations périnéofessières : étude étiologique de 60 cas.

BACKGROUND: The gluteal region is a complex area of the body that may be the seat of several diseases with dermatological manifestations. The purpose of this series was to study the aetiological profile of suppurative lesions of the gluteal region in patients in Morocco. PATIENTS AND METHODS: A retrospective study was carried out on patients presenting suppuration of the gluteal region hospitalised in our department between 1987 and 2008. The epidemiological, clinical and aetiological data for 60 cases were reviewed. Skin biopsies with bacterial, fungal and histological studies, as well as bacterial and fungal cultures of pus and anorectal examination, were performed for all patients. RESULTS: We examined the files for 44 males and 16 females; mean age was 44 years (range: 13 to 70 years) and the average duration of illness was 6.5 years. The various aetiologies were as follows: hidradenitis suppurativa (27 cases), cutaneous tuberculosis (10 cases), Crohn's disease (nine cases), phlegmon-carbuncle (eight cases), tumours (four cases) and deep mycosis (two cases). Medical and surgical treatments were aetiological. DISCUSSION: There are various aetiologies of suppurations of the gluteal region. In Morocco, hidradenitis suppurativa, cutaneous tuberculosis and Crohn's disease are the most frequent causes. Multidisciplinary management is essential.

[Malignant pyoderma with cranial osteolysis]. / Pyoderma malin compliqué d'ostéolyse crânienne.

BACKGROUND: Malignant pyoderma or pyoderma gangrenosum with cephalic involvement is a rare disease characterized by chronic aggressive local progression. We report a case of malignant pyoderma with cranial osteolysis. PATIENTS AND METHODS: A 42-year-old man had been presenting chronic ulcerative dermatosis involving the entire scalp since the age of sixteen. Clinical examination, histopathological analysis of the skin sample and negative aetiological tests suggested a diagnosis of cephalic pyoderma gangrenosum. Different treatments were ineffective. The patient was subsequently admitted with convulsions revealing cranial osteolysis. A cranial scan confirmed the osteolysis and histopathological analysis of the bone sample revealed non-specific suppurative osteitis. Treatment with corticosteroids and thalidomide was effective. Skin and bone grafting was proposed but was not carried out due to the chronic progression and absence of stable wound healing. DISCUSSION: Malignant pyoderma is a rare destructive, ulcerating dermatosis. It is characterized by a chronic course and resistance to treatment. Our case report is original in terms of the involvement of underlying cranial bone revealed by convulsions. This bone involvement makes treatment of the dermatosis more difficult.

[Fatal progression of Bannayan-Zonana syndrome]. / Syndrome de Bannayan-Zonana d'évolution fatale.

Bannayan-Zonana syndrome is a rare entity characterized by macrocephaly and multiple soft tissue and visceral hamartomas and lipomas. Knowledge of this syndrome is important given the risk of sometimes fatal bleeding and visceral neoplasia in adulthood. We report on a new case, original in its sporadic occurrence and rapidly fatal progression.