[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.

[Clinical characteristics of nail lichen planus and follow-up: a descriptive study of 20 patients]. / Caractéristiques cliniques et évolutives du lichen plan unguéal : étude descriptive de 20 patients.

BACKGROUND: Nail lichen planus (NLP) occurs in 10% of patients with disseminated disease but it can also occur in isolation. The outcome of treatment is usually disappointing and data about its long-term prognosis are lacking. STUDY AIM: To study clinical features, response to treatment and follow-up of a series of 20 patients with NLP. PATIENTS AND METHODS: A descriptive study was conducted of 20 patients with histologically confirmed NLP seen at consultations for nail disorders between September 2009 and April 2013. RESULTS: The mean age was 35 years (9-56 years) with no gender preponderance. Forty percent of patients were children. Stress was an evident triggering factor in 20% of patients. The mean duration was around 48 months (2 months to 10 years). Only 25% of patients had extra-ungual lesions. All 20 nails were affected in 55% of patients. Nail matrix involvement was observed in 85% of cases and 55% had nail-bed involvement. Twenty-five percent of patients were presenting severe involvement such as pterygium or anonychia. Intramuscular corticosteroids were given to 10 patients. In 80% of patients, the NLP was limited or regressed rapidly from the third injection. The average follow-up was 24 months. DISCUSSION: Our study highlights the frequency of paediatric forms and of often aesthetically unacceptable nail scarring. Early diagnosis of NLP, notably in children, would allow initiation of adequate treatment that could perhaps limit the risk of such sequelae.

[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.

[Xanthoma disseminatum with asymptomatic multisystem involvement]. / Xanthoma disseminatum avec atteinte viscérale multifocale asymptomatique.

BACKGROUND: Xanthogranulomas belong to non-Langerhans histiocytosis of the second group in the Histiocyte Society classification. They comprise a heterogeneous group of rare entities frequently involving cutaneous tropism. Xanthoma disseminatum belongs to this group of non-Langerhans histiocytosis. We report a case of xanthoma disseminatum (XD) in which localized skin and mucous impairment revealed multisystem involvement. PATIENTS AND METHODS: A 28-year-old man presented with a two-year history of progressive yellow-orange and infiltrated xanthomatous papulonodular lesions of the face. Lesions of the oral mucosa and genital region were seen, with no functional repercussions. No ophthalmic or other complications were found. Histopathology showed a dense histiocytic infiltrate within the dermis with Touton giant cells, foamy multinucleated giant cells and inflammatory cells, without necrobiosis. Histiocytes were positive for CD68 but negative for CD1a. Gastric and lung involvement was seen and was confirmed at histology. Bone scintigraphy showed suspicious left ulnar hyperfixation suggesting bone involvement. No monoclonal gammopathy or diabetes insipidus was seen. Our patient was treated with corticosteroids 1mg/kg/day and thalidomide 100 mg/day. The outcome was marked by regression and exfiltration of the cutaneous lesions from the second week of treatment, with subsidence continuing at 3 months. DISCUSSION: This case involves a very rare form of xanthoma disseminatum. The localized facial skin lesions revealed multifocal non-Langerhans histiocytosis that was in fact asymptomatic. The diagnosis of XD was based on clinical, histological and immunohistochemical criteria. Xanthoma disseminatum is a non-Langerhans histiocytic proliferation first described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis in which the facial involvement is common, together with diabetes insipidus and normal lipid metabolism. The prognosis is determined by the presence of mucosal xanthomas and visceral involvement. Thus, all xanthogranulomas involving multiple lesions warrant screening for visceral involvement. Diagnosis of this entity can be difficult and is usually based on clinical and histopathological findings. In addition, treatment is complex and non-consensual.

[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.

[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.

Risk factors in facial hyperpigmentation in Maghrebian population - a case-control study.

Triggering factors seem to be multiple in the pathogenesis of facial hyperpigmentation (FH), as dark skin types, pregnancy, sun exposure… The aim of this study was to investigate the risk factors for FH in Maghrebian population. Patients with FH were included in a case-controlled study. Following data were recorded: age, sex, parity, phototype, FH and hormonal-related history, endocrinological features, sunlight exposure, specific cultural behaviours, use of topic corticosteroids and sunscreens. One hundred women (mean age 29.5 ± 13.7 years) were included with 200 age-matched controls. Duration of FH was 32.9 ± 42.2 months. There was a statistically significant relation between FH and hirsutism (P = 0.009), troubles of menstruations (P = 0.008), but not with acne (P = 0.23) and contraceptive oral (P = 0.06) drugs or with history of thyroid disorders (P = 0.13). For cultural factors, there were a statistical significant relation with rubbing by flannel glove (P < 0.05), use of dark soap (P = 0.009) and traditional masks (P < 0.05) but both groups were used to go to hammam. A strong relation was observed between FH and use of topical corticosteroids: 40% in the FH group vs. 5% in the control group (P < 0.05). Both groups used to apply sunscreens: 70% in the FH group and 67% in the control group where as there were no differences in the two groups for sun exposure. No statistical differences were observed regarding to age, phototypes and grade of parity. Our study demonstrated a relation between FH and hyperestrogenemia, rubbing with a friction glove, use of moroccan traditional masks and application of topical steroids. The eviction of all these triggering factors could be an adjuvant recommendation in the assessment of FH.

[Successive cutaneous adverse reactions to nilotinib and imatinib in a single patient]. / Toxidermie à l'imatinib suivie d'une toxidermie au nilotinib.

BACKGROUND: Since the advent of targeted molecules, the treatment and prognosis of many cancers, especially chronic myeloid leukemia (CML), have been substantially modified through the introduction of first- and second-generation tyrosine kinase inhibitors. Skin effects constitute the most common adverse effects of these new substances. Although such skin changes are not life-threatening, they can have extensive clinical impact, in some cases leading to discontinuation of treatment. PATIENTS AND METHODS: A 47-year-old woman with no past medical history was followed for chronic phase CML since 26/11/2010 with the presence of the t(9; 22) karyotype. Imatinib (IM) was started at a dose of 400mg/day and haematological response was good. After 4 months of treatment with IM the patient presented with erythematous plaques on both upper limbs and on the oral and vaginal mucosa. These lesions disappeared after discontinuation of IM. The patient was then put on nilotinib 400mg/d and skin lesions reappeared after 3 weeks in the more serious form of erythema multiform with acral distribution, but with no involvement of the mucosa, resulting in immediate cessation of nilotinib. Skin biopsy was consistent with a drug-induced eruption. The lesions disappeared after discontinuation of nilotinib. DISCUSSION: In case of intolerance to IM, a second-generation ITK (dasatinib or nilotinib) may be substituted, and while cross-sensitivities seem infrequent, therapy is problematic in these patients presenting potentially curable blood dyscrasias.

[Unusual clinical presentation of cutaneous leishmaniasis in three diabetic patients]. / Leishmanioses cutanées d'aspect clinique inhabituel chez trois patients diabétiques.

INTRODUCTION: Cutaneous leishmaniasis (CL) is a parasitic infection characterized by significant clinical variability. Unusual and atypical clinical aspects of infection have been reported in immunodeficient patients or associated with particular parasite species. We report three cases of CL from foci of Leishmania major with a particular clinical presentation in diabetic patients. OBSERVATIONS: Patient 1: a 37-year-old man was admitted to the dermatology department for cutaneous vegetative ulcers spreading to the dorsal surface of the foot. History-taking revealed a stay in Er-Rachidia (East of Morocco, a known focus of CL L. major) six weeks earlier. Diabetes mellitus type I was discovered during hospitalization. The patient's 43-year-old sister (patient 2), diabetic for 6 years, consulted for a single leg ulcer appearing 3 months after the same trip to Er-Rachidia. Patient 3: a 61-year-old patient with a 7-year history of diabetes and under oral antidiabetics presented an extended vegetative lesion of the posterior surface of the leg 2 months after staying in Er-Rachidia. A diagnosis of CL was retained on the basis of epidemiology and history (living in an endemic areas of leishmaniasis), coupled in patients 1 and 3 with microbiological evidence involving identification of Leishmania bodies in skin smears or skin biopsies. All patients were treated with two intra-lesional injections per week of meglumine antimony (Glucantime(®)) for 6 weeks. The outcome was marked in all cases by healing of lesions and persistent pigmented scarring. DISCUSSION: We described three particular clinical aspect of CL emerging from a known focus of L. major, where infection is classically associated with vegetative or ulcero-vegetative lesions. This unusual profile suggests the role of factors related to parasite species and/or diabetes found in our three patients.

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.

[Stevens-Johnson and Lyell syndromes: mucocutaneous and ocular sequels in 43 cases]. / Séquelles cutanéomuqueuses et oculaires des SJS et de Lyell.

INTRODUCTION: The ophthalmic sequelae of Stevens-Johnson and Lyell syndromes are deemed serious, and their mucocutaneous syndromes can adversely affect social and functional outcomes. The aim of this study is to describe these sequelae in Morocco. METHODS: This retrospective study involved all patients hospitalized in the dermatology department of the Ibn Rochd University Hospital in Casablanca for toxic epidermal necrolysis or Stevens-Johnson syndrome (SJS). The study period lasted from January 1986 to December 2006. Mucosal, cutaneous and ocular sequelae of these diseases were identified during follow-up. RESULTS: During this 21-year period, 43 cases of Lyell syndrome and SJS were analyzed. Twenty-five women and 18 men were included and the average age was 28 years. Clinical forms were as follow: Lyell's syndrome: 32 cases; SJS: six cases; intermediary Lyell-SJS form: five cases. Average follow-up was 3 years. Concerning mucocutaneous sequelae, 72% of patients had diffuse hyperchromic macules with scarring, photosensitivity was noted in 70% of cases, telogen effluvium was seen in 10 cases, abnormal sweating was noted in eight cases, nail loss was seen in eight cases, and vaginal bands were noted in two cases. In the chronic phase, 70% of patients had photophobia with chronic lacrimation. Eyelid malposition was noted in 21% of cases: ectropion (n=6), entropion with trichiasis (n=3) and symblepharon in 13 cases (30%). Corneal complications involving superficial punctate keratitis were noted in 42% of cases and were associated with corneal neovascularisation in five cases. Finally, bilateral dry eye syndrome was seen in 24 cases (56%). CONCLUSION: Our study confirms the reality of ocular, and mucocutaneous sequelae of Stevens-Johnson and Lyell syndromes. Ocular sequelae are dramatic, while cutaneous sequelae are unsightly. These sequelae have marked repercussions on the social and professional integration of our patients, most of whom were young.

[Familial histoid leprosy]. / Lèpre histoïde familiale.

BACKGROUND: Histoid leprosy is a very particular and rare anatomoclinical form of lepromatous leprosy. Familial cases are extremely rare. We report two new cases of histoid leprosy in a sister and brother born of a non-consanguineous marriage. PATIENTS AND METHODS: We report the case of a sister and brother, aged 21 and 25 years respectively, who were admitted to the National Leprology Centre for multibacillary leprosy. Questioning revealed the existence of family contact with an uncle and two maternal cousins, all of whom had histoid leprosy. On clinical examination, multiple erythematous papules and nodules were noted, some of which were umbilicated while others were ulcerated. Palpation also revealed subcutaneous nodules on the thighs and forearms. No neurological disorders were found in the two patients. Histopathological analysis of cutaneous biopsy samples of nodular lesions for the two patients showed dermal-hypodermic nodular infiltrates comprising spindle cells with a few macrophages. Ziehl-Neelsen staining showed numerous alcohol-acid resistant bacilli (BAAR) without globi. Both patients were placed on the multi-drug regimen recommended by the WHO for multibacillary leprosy. An extremely favourable therapeutic response was obtained after 3 months of treatment. The patients had presented no relapse of erythema nodosum leprosum after two years of follow-up. CONCLUSION: Histoid leprosy is a clinical entity that must be clearly differentiated through its clinical and histopathological features, which distinguish it from polar lepromatous leprosy. There may be a genetic predisposition to this clinical leprosy form, but this requires confirmation by genetic studies.

[Fatal erythroderma in a young Moroccan]. / Erythrodermie fatale chez un jeune marocain.

The purpose of this report is to describe a case involving a young Moroccan who abruptly developed pruritic papulo-vesicular lesions with erythroderma. Secondary development of jaundice and tumoral syndrome lead to diagnosis of an acute form of adult T-cell leukemia/lymphoma associated with HTLV-1 infection. The patient died within three months. To o ur knowledge, this is the first such case reported in Morocco.

[Histoid leprosy in Morocco: retrospective study of 18 cases]. / Lèpre histoïde au Maroc: etude rétrospective de 18 cas.

INTRODUCTION: Histoid leprosy is a special and rare anatomoclinical form of multibacillary leprosy. Most of the few large series describing this entity In literature have been reported from India. The purpose of this study was to analyze the epidemiological, clinical and therapeutic characteristics of patients with histoid leprosy in Morocco. MATERIAL AND METHODS: This retrospective study was conducted at the National Centre of Leprology (CNL) in Casablanca from January 1991 to December 2006. Only histologially confirmed cases of histoid leprosy were included. Epidemiological, clinical, histological and therapeutic characteristics were compiled from records and analyzed using EPI-Info version 6. RESULTS: Confirmed histoid leprosy accounted for 18 of the new cases of leprosy recorded at CNL recorded during the 16-year study period. There were 13 men and 5 women with a mean age of 35.2 years. Family exposure was noted in 9 patients (50%) including two who had more than two relatives with leprosy. Nodules/subcutaneous nodules were the most common morphological pattern (88.8%). Neurological involvement was found in 10 patients. Seven patients had grade 1 deformities. Slit skin smears from histoid lesions revealed abundant bacilli with a high bacteriological index ranging from 3 + to 5 + according to the Ridley index. All patients received antibiotic treatment according to the Moroccan protocol. Outcome was favorable in all patients. Six patients (33.3%) developed erythema nodosum leprosum during the course of disease. With a average follow-up of 9 years, no recurrence has been observed. DISCUSSION: Because of its multibacillary character and despite its rarity, histoid leprosy poses a challenge to the leprosy eradication in Morocco. Like other forms of multibacillary leprosy, histoid leprosy requires early detection and prompt multidrug therapy. These requirments should be made a priority in the national program against leprosy.

[Maxillary bone sarcoidosis]. / Sarcoïdose osseuse maxillaire.

INTRODUCTION: Sarcoidosis is an unexplained systemic granulomatosis. Bone localizations of the disease are rare. We report a maxillary localization. CASE: A 35-year-old patient consulted for facial dysmorphia first observed 2 years before. This swelling was associated to nodular panniculitis lesions and xerostomia. The facial asymmetry was due to maxillary gingival and alveolar swelling. It was associated with scarring in the legs and a purple nodular facial lesion. Lip and jaw biopsies revealed epithelioid and giant cell granulomas without caseous necrosis. The panoramic dental X-ray showed diffuse horizontal alveolar ridge lysis and CT scan revealed an osteolytic lesion of the right maxilla associated to a bone-condensing lesion of the left hemi-mandible. DISCUSSION: The diagnosis of sarcoidosis is made in case of epithelioid and giant cell granulomas without caseous necrosis and the ruling out of other possible diagnoses, including tuberculosis. Bone involvement is rare; face and maxillary localization are extremely rare. The recommended treatment is corticosteroids. Facial remodeling surgery is not recommended.

[Ingrown nail: A new cause of chronic perionyxis]. / Une nouvelle cause de périonyxis chronique : la rétronychie.

BACKGROUND: Retronychia is the phenomenon of ingrown nail resulting in inflammation of the proximal subungual fold and is a newly described entity. Herein, we report a new case revealed through chronic proximal perionyxis. PATIENTS AND METHODS: A 52-year-old woman followed up for hypothyroidism, for which she was receiving treatment, consulted for pain in the lateral fold of the left big toe that had been ongoing for four months and for which glomic tumour was the suspected cause. Questioning revealed repeated microtrauma due to wearing of tight shoes and lack of growth of the toenail over the previous four months (in contrast with the contralateral toenail). Examination showed painful proximal perionyxis of the left big toe, with discharge from the subungual fold. Ultrasound examination of the subungual fold revealed effusion of fluid around the matrix, and this was confirmed by MRI. Surgical avulsion of the nail was carried out, and recovery was complete. DISCUSSION: Retronychia is the result of a loss of continuity between the nail plate and the nail matrix, generally as a result of mechanical factors (distal trauma). However, unlike onychomadesis or Beau's lines, there is a disturbance of alignment between the two parts of the nail, which is normally maintained by the proximal subungual fold. This prevents newly formed nail from progressing towards the distal part of the plate, which itself is pushed into the nail bed and forced towards the proximal fold, causing inflammation of the latter structure. In our case, the pain recorded in the lateral fold was misleading, hence the ultimately unnecessary use of MRI to rule out the hypothesis of glomic tumour. Paronychia, discontinuation of nail growth and weeping through the subungual fold all argued in favour of a diagnosis of retronychia, which was confirmed by surgery. Surgical nail removal is the curative treatment for retronychia and results in complete cure without relapse or occurrence. CONCLUSION: Retronychia should be suspected in the event of persistent proximal perionyxis, particularly in a setting of trauma with interrupted nail growth. Nail avulsion constitutes both a diagnostic and a therapeutic procedure.

[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.

[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.