[Lichenoid drug eruption with antituberculosis drugs associated with an anonychia]. / Toxidermie lichénoïde aux antituberculeux associée à une anonychie.

INTRODUCTION: Lichenoid cutaneous reactions to antituberculosis drugs are rare. Herein we report a new case. PATIENTS AND METHODS: A 41-year-old patient was seen for a profuse and pruriginous rash occurring 2 weeks after administration of rifampicin and isoniazid for pulmonary tuberculosis. Dermatological examination revealed polymorphic erythemato-squamous plaques with lichenoid, psoriatic and eczematous features, associated with cheilitis, erosions on the cheeks and diffuse onychodystrophy. The skin biopsy confirmed a lichenoid reaction. The pharmacovigilance investigation incriminated isoniazid and rifampicin. The patient was treated with topical corticosteroids and UVB phototherapy. The outcome involved complete regression of the eruption but with secondary anonychia. DISCUSSION: Antituberculosis drugs including isoniazid and rifampicin are known to induce lichenoid reactions. It is difficult to distinguish the results from lichen planus. The clinical polymorphism of the rash as well as the patient's drug intake militate in favour of a diagnosis of lichenoid reaction. Widespread ungual involvement, which is extremely rare, warranted early management in order to avert irreversible anonychia.

[Frontal fibrosing alopecia: A prospective study of 20 cases]. / Alopécie frontale fibrosante : étude prospective de 20 cas.

BACKGROUND: Frontal fibrosing alopecia (FFA) is a variant of lichen planopilaris predominantly affecting postmenopausal women. We report a series of 20 cases of FFA and describe the epidemiological, clinical, dermoscopic features and progress under treatment. PATIENTS AND METHODS: This was a prospective study conducted over a period of 16 months in patients seen at the dermatology department of the Hassan II University in Fez, Morocco. RESULTS: Mean patient age was 46 years. Patients were premenopausal in 65% of cases. Dermoscopic examination revealed specific signs of the disease. Skin biopsy guided by dermoscopy confirmed the diagnosis of lichen planus pilaris in its FFA variant in all cases. Immune dysfunctions and other disorders were noted in half of the cases. Various treatments had been initiated, including topical corticosteroids, tacrolimus ointment, minoxidil 2%, hydroxychloroquine, and oral finasteride. The results were satisfactory with a decline within one year. CONCLUSION: FFA is increasingly widely described in premenopausal women. Dermoscopy may be used to facilitate diagnosis, guide biopsy, evaluate treatment efficacy and establish a prognosis.

[Extensive ulcerations in children: The difficulty diagnosing pyoderma gangrenosum]. / Ulcérations extensives chez l'enfant : penser au pyoderma gangrenosum.

Pyoderma gangrenosum is an amicrobial neutrophilic dermatosis of unknown cause with a chronic course. We report a case in a 30-month-old child who presented with progressive and painful skin ulcers. Lesions were quickly extensive, refractory to local and systemic antibiotic therapy. Histopathology of the skin biopsies confirmed the diagnosis of ulcerative pyoderma gangrenosum. Only 4% of the cases reported in the literature are in children below the age of 4 years. This neutrophilic dermatosis is associated in half of the cases with a systemic disease. The treatment is based on corticosteroids. Immunosuppressant drugs such as tacrolimus, cyclosporine, and mycophenolate mofetil may be effective for pyoderma gangrenosum refractory to corticosteroids. Currently, anti-TNF is a promising treatment for refractory PG.

[Nasoethmoidal psammomatoid cemento-ossifiying fibroma with intraorbital extension]. / Fibrome cémento-ossifiant psammomateux nasoéthmoïdal à extension orbitaire.

INTRODUCTION: Psammomatoid cemento-ossifying fibroma (PCOF) is a rare benign fibro-osseous lesion. It affects the maxilla, the nasal cavity, paranasal sinuses, and the ethmoid. It is slow-growing, progressive, and benign but it can be locally extended and mimic a malignant tumor. OBSERVATION: We report the unusual case of a 36-year-old woman with a nasal and ethmoid PCOF with orbital and endocranial extension. The tumor was revealed by exophthalmia and nasal obstruction. The diagnosis was proven histologicaly on biopsies. The treatment was transfacial surgery. There was no recurrence 1 year later. DISCUSSION: Intraorbital and endocranial extension of PCOF are rare aggressive behaviours. Histologically, the differential diagnosis is difficult between fibrous dysplasia or psammomatoid meningioma. Complete surgical excision is necessary to prevent recurrence.

[Pseudo-Kaposi's sarcoma secondary to superficial arteriovenous malformation: Stewart-Bluefarb syndrome]. / Pseudomaladie de Kaposi secondaire à une malformation artérioveineuse superficielle: syndrome de Stewart-Bluefarb.

BACKGROUND: Pseudo-Kaposi's sarcoma or Stewart-Bluefarb subtype acroangiodermatitis is uncommon and is caused by arteriovenous fistula and malformation. We report a new case. CASE REPORT: A 33-year-old man presented with painful red-violet plaque on the dorsum of the toes with angiomatous nodules on the sole. Histological and immunohistochemical studies for CD34 were consistent with Kaposi's sarcoma. Doppler ultrasonography and femoral angiography showed multiple distal arteriovenous shunts. Free-flow embolisation with fragments of Ethibloc gelatin sponge was performed and arteriography, performed immediately afterwards, showed delayed venous drainage. The outcome was good with complete drainage of the angiomatous lesions. DISCUSSION: Pseudo-Kaposi's sarcoma Stewart-Bluefarb subtype begins early in life in male subjects, with unilateral skin lesions. It bears clinical and histological resemblance to Kaposi's sarcoma. Doppler ultrasonography and angiography show arteriovenous fistulas that classically develop at shunts, explaining the role of traumatism and high vascular pressure in the genesis of this disease.

[Desmoplastic ameloblastoma in a 7 year old child]. / Améoblastome desmoplastique chez un enfant de 7 ans.

Desmoplastic ameloblastoma is a benign, locally aggressive neoplasm of proliferating odontogenic epithelial origin. It is seen among old patients from 17 to 72 years with an average age 42 years and without predilection of sex. We report the case of a 7 year old child, having presented since the 5 years age, a gingival tumefaction on the left higher incisivo-canin group which increased volume gradually. The stomatologic examination showed a gingival tumefaction covered with a healthy mucous membrane, ovoid form and measuring 3 cm on its horizontal axis. The tomodensitometry of the jawbone showed in front of the 21st and the 22nd tooth, the presence of an osseous lesion associating of the hearths of osteolysis and osteocondensation with rupture of cortical and invasion of the soft tissue. A curetting of the lesion was carried out and the anatomopathologic examination retained the diagnosis of desmoplastic ameloblastoma. The characteristic of our observation is the youth of the patient. In addition, the desmoplastic ameloblastoma is relatively rare, is characterized by an anatomical distribution, a radiological appearance and a morphological aspect differents from the traditional ameloblastoma. A radical surgical treatment is suggested for this tumour to avoid recurrency.

[Cutaneous blastomycosis revealing intravascular B-cell lymphoma: a case in Morocco]. / Blastomycose cutanée révélant un lymphome B intravasculaire: une observation marocaine.

Blastomycosis is a systemic fungal infection caused by a thermally dimorphic fungus, Blastomyces dermatitidis. The incidence in immunocompromised patients has increased in the last two decades. A 55-year-old man consulted for inflammatory nodules on the forearm. Biopsy of one nodules showed a pseudoepitheliomatous hyperplastic epidermis overlaying a dense agranulomatous inflammatory infiltrate containing free-formed ovoid bodies enclosing giant macrophageous cells. These findings were consistent with blastomycosis. After a month of treatment cutaneous lesions regressed partially but the patient's general status continued to deteriorate with the appearance of an edematous-ascitic syndrome and icterus. Laboratory blood testing demonstrated cholestasia and abdominal ultrasound showed hepatosplenomegaly. Needle liver biopsy revealed giant B-cell lymphomatous infiltration of the hepatic ducts. The patient's condition worsened rapidly and he died five months after diagnosis despite four rounds of chemotherapy. Blastomycosis is rare in Morocco. Primary infection is usually a pneumonic process. Isolated cutaneous infection is possible but uncommon. To our knowledge the association of blastomycosis and intravascular lymphoma has not been previously reported. In immunocompromised patients, clinical findings can be alarming and the outcome can be rapidly fatal.

[Laryngeal tuberculosis: a case report]. / La tuberculose laryngée: à propos d'un cas.

The laryngeal tuberculous is the most frequent granulomatous disease of the larynx. It is generally associated with a pulmonary cave, and represents a prognosis element extremely serious and can be complicated extreme dysphagia and pains. Among all the extrapulmonary localisations. The isolated laryngée localisation is rare. It is secondary the dissemination coming hematogen, air or lymphatic way. All the structures of the larynx can be reached. The beginning is progressive and insidious and appears by the dysphony, cough, the odynophagy and dyspnea. We report the case of laryngeal tuberculosis in a 21 year old young man. The authors point out the characteristics of this localization and insist on the biopsy in front of any chronic laryngitis no aetiology. The treatment is medical.

[Primary nasal tuberculosis: a case report]. / La tuberculose nasale primaire: a propos d'un cas.

We report a case of a 36 year old woman who presented a chronic rhinitis and a hypertrophy of the inferior turbinates. Primary nasal tuberculosis was discovered by chance after the inferior turbinectomy. Primary nasal tuberculosis is very rare and is more frequent in women. Symptomatology is often unilateral with nasal obstruction, anterior rhinorrhea or epistaxis. The clinical examination may discover ulceration or a polyp located generally in the nasal septum or the inferior turbinate. Diagnosis relies on the anatomopathologic and bacteriological examinations. The treatment is mainly medical based on antituberculosis drugs. In the light of this case report, a review of the literature was made.

[Ganglioneuroma of the zygoma]. / Le ganglioneurome de l'os zygomatique.

INTRODUCTION: Ganglioneuroma is an uncommon benign tumor that arises from the sympathetic nervous system accounting for less than 1% of all soft-tissue neoplasms. CASE REPORT: We report the case of a 22 year-old man who presented a ganglioneuroma localized in the zygoma. DISCUSSION: Ganglioneuroma generally develop in the abdomen or thorax soft tissue. The occurrence of ganglioneuroma in the bone is exceptional. To date less than ten cases have been reported, six of which involved the mandible. We discuss the possible causes of tumor development at this site.

[Thalidomide in adult multisystem Langerhans cell histiocytosis: a case report]. / Efficacité du thalidomide dans le traitement de l'histiocytose langerhansienne multisystémique de l'adulte: à propos d'une observation.

BACKGROUND: Adult multisystem Langerhans cell histiocytosis is an excepted disorder, which have several treatments. The purpose of this study was to test the disease-controlling effect of thalidomide in a case of adult multisystem Langerhans cell histiocytosis with cutaneomucous and hypothalamic localizations at dermatology department, of Mohammed-V military hospital, Rabat. CASE REPORT: A 43-year-old women, presented multifocal chronic Langerhans cell histiocytosis confined to cutaneous, oral cavity, perianal, mastoid and hypothalamic areas, with severe disabling ulcers in intertriginous areas, diabetes insipid and amenorrhoea. We treated with thalidomide 200 mg/day after neurological examination. A rapid initial response with total diminution of the involved skin area, and diminution of diabetes insipid. She remained in remission for 1 year. No adverse effects from treatment were observed at clinical and electrophysiological examinations. CONCLUSION: Thalidomide treatment is an adequate therapeutic measure in adult Langerhans cell histiocytosis, which is rare and difficult to treat. Our case showed the efficacy of thalidomide at cutaneomucosal and hypothalamic manifestations.

Laryngeal leiomyosarcoma.

Laryngeal leiomyosarcoma is rare. The authors report the case of a 48-year-old patient who presented with progressive hoarseness. This symptomatology was associated with a laryngeal tumour. Immunohistopathological study indicated a leiomyosarcoma. Acute dyspnoea resulted in an urgent tracheostomy, followed by total laryngectomy without neck dissection later. In the light of this observation and a literature review, histopathological characteristics, clinical aspects and therapeutic indications will be discussed for this localisation.

[Chronic alveolar syndrome]. / Syndrome de condensation alvéolaire chronique.

We report a case of primary pulmonary Hodgkin's disease in a 20 year-old woman. The chest x-ray showed a chronic alveolar syndrome. The diagnosis was established from a pulmonary biopsy. The radiological features and the options for diagnosis of primary pulmonary Hodgkin's disease are discussed.

[Benign granular cell anal tumor]. / Tumeur anale bénigne à cellules granuleuses.

INTRODUCTION: Granular cell tumors (GCT) are rarely located in the perianal area. OBSERVATION: Over the past 3 years, a 56 year-old man presented a papule of the right margin of the anus that had progressively increased in size (1.5 cm). Cell proliferation was located in the dermis and strongly expressed the S100 protein. It was covered by a pseudo-epitheliomatous hyperplasia of the overlying epidermis. Forty months after local surgical excision, there was no sign of recurrence. COMMENTS: Granular cell tumors are rare and usually benign. When cutaneous or mucosal, the pseudo-epitheliomatous hyperplasia of the overlying epithelium may, on superficial samples, be mistakenly diagnosed as squamous cell carcinomas. Malignant GCT may, histologically, appear identical to a benign GCT and only the appearance of metastases (generally after local recurrence) permits the subsequent diagnosis of malignancy.

[Botryoide sarcoma of the uterine cervix. Apropos of 1 case]. / Sarcome botryoïde du col utérin. A propos d'un cas.

Sarcoma botryoide (SB), a variant of rhabdomyosarcoma, is a rare tumor who are rarely localized in the uterine cervix. Only 99 cases have been described to date. The authors reported this case with review of the literature in order to insist on diagnosis progress and a new combined modality approach using: radiotherapy, surgery and chemotherapy which have improved prognosis in the localized disease especially.

[Nodular fasciitis or pseudosarcoma]. / Fasciite nodulaire ou pseudosarcomateuse.

Nodular fasciitis is a benign neoplastic and reactive proliferation of fibroblasts of soft tissues, which is often mistaken for a sarcoma because of its rapid growth, rich cellularity and mitotic activity. A case is reported that provides the opportunity to discuss the specific clinical and pathological features of nodular fasciitis.

[Systemic manifestations of Sweet's syndrome: a case report]. / Manifestations systémiques du syndrome de Sweet. A propos d'un cas.

Sweet's syndrome belongs to the group of neutrophilic dermatoses. We report the case of a 36-year-old man admitted for stiff neck and fever. He had a history of recurrent oral aphtous ulcers, orchitis, phlebitis, two episodes of febrile acute polyarthritis with interstitial pneumonia. He presented a stiff neck and a temperature of 40 degrees C for two days associated with an erythematonodular eruption of the right periocular region. Laboratory exams showed an inflammatory syndrome with hyperleukocytosis. Skin biopsy showed dermic neutrophilic infiltrates, confirming the diagnosis of Sweet's syndrome. The patient improved dramatically with corticosteroids: the temperature fell and neck stiffness and skin lesions disappeared. In light of this case with a rich cohort of extracutaneous manifestations, we reviewed the literature on the characteristics of Sweet's syndrome. This syndrome is commonly associated with inflammatory and neoplastic diseases.

[Testicular metastasis of prostatic adenocarcinoma: report of 2 cases]. / Métastases testiculaires d'un adénocarcinome de la prostate: à propos de deux cas.

Prostatic carcinoma metastasizing to the testis is an extremely rare occurrence. In this study, two new cases have been reported of unilateral testicular metastases from an adenocarcinoma of the prostate. Unlike primary tumors of the testis, this type of tumor develops later in life (between 50 and 60 years of age), and is in most instances unilateral. It is generally discovered accidentally during orchiectomy or autopsy. It is not always easy to establish a morphological diagnosis, as the macroscopic appearance of the tumor may be normal. However, microscopic investigation reveals the presence of neoplasms of the testis.