[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.

[Frontal fibrosing alopecia: Report on three pediatric cases]. / Alopécie frontale fibrosante : à propos de trois cas pédiatriques.

BACKGROUND: Frontal fibrosing alopecia is a topographic form of lichen planopilaris, which most commonly affects postmenopausal women. We report on three original pediatric cases of this scarring alopecia, including one case of female twins. OBSERVATIONS: The first observation concerns twin sisters, 14 years of age, with frontotemporal symmetric and progressive alopecia, beginning at the age of 5 years, with follicular facial noninflammatory micropapules. Histological examination showed a depletion of hair follicles with dermal fibrosis and perivascular infiltrate. The treatment decided was monthly intralesional infiltrations of betamethasone. The second case is a 7-year-old girl, with a frontotemporal alopecia band and partial alopecia of the eyebrows. Histological examination was consistent with lichen planopilaris. The outcome was favorable after six monthly oral boluses of steroids. COMMENT AND CONCLUSION: The initial description of frontal fibrosing alopecia was made in postmenopausal women. Some cases have been reported subsequently in premenopausal women as well as in men, with recent identification of familial cases. To our knowledge, these are the first pediatric cases of this form of alopecia, which is not only postmenopausal.

[Tinea capitis in the military hospital Avicenna (Morocco): Review of 8 years (2006-2013)]. / Teignes du cuir chevelu à l'hôpital militaire Avicenne de Marrakech (Maroc): bilan de 8ans (2006-2013).

INTRODUCTION AND OBJECTIVE: Tinea capitis are due to fungal infection by dermatophytes. They are common in developing countries including Morocco. The objective of this study intended to describe the epidemiology, clinical and mycological profile of tinea capitis in Avicenna military hospital of Marrakech. PATIENTS AND METHODS: This is a retrospective study over an 8-year period (from 1st January 2006 to 31st December 2013). All patients targeted through this study presented to the laboratory with the suspicion of tinea capitis, they were under a detailed investigation with a careful mycological analysis; diagnosis of tinea capitis was established as the direct examination and/or the sampling proved positive. RESULTS: Of the 334 patients investigated, 216 had a TC with an overall prevalence of 64.67%. The average age was 6 years. The M/F sex ratio was 0.55. The isolated dermatophytes were Microsporum canis with 105 cases (63.26%), Trichophyton violaceum in 44 cases (26.51%), T. mentographytes in 8 cases (4.81%), M. langeronii in 5 cases (3.01%), T. verrucosum in 3 cases (1.8%) and T. schoenleinii in 1 case (0.61%). The contact with animals was found in 40% of cases and immunosupression in 3.47% of cases. We verify through our investigation that tineas predominate among school age children with a female predominance. The epidemiological profile of TC in our study is similar to that of other studies in Moroccan and Maghrebian countries investigations. CONCLUSION: The TC is relatively a mild infection but can be confused with other dermatoses not easy to diagnose. For this reason, their treatment necessitates a mycological analysis.

[Profuse sarcoidosis associated with multiple cutaneous squamous cell carcinomas]. / Sarcoïdose profuse associée à des carcinomes épidermoïdes cutanés multiples.

BACKGROUND: The cutaneous signs of sarcoidosis are polymorphous and occasionally misleading. Herein, we report a rare case of profuse sarcoidosis involving various rare cutaneous signs specific to the disease associated with multiple cutaneous squamous cell carcinomas. PATIENTS AND METHODS: A seventy-three-year-old man had been presenting dry ichthyosiform erythroderma for 2 years. The examination also revealed diffuse cutaneous atrophy with ulceration, superficial adenopathy and pseudotumoral hypodermic masses. Ophthalmological examination revealed conjunctival nodules. Biopsy samples for the various cutaneous lesions, salivary glands, conjunctival nodules, a hypodermic nodule and an adenopathy yielded similar images evocative of epithelioid granulomas and giant cells without caseous necrosis. Infectious causes were ruled out. Respiratory function tests showed a restrictive respiratory disorder. ACE levels were extremely high. Proteinuria was noted for 24-hour urine tests and renal punch biopsy showed an appearance of membrane-proliferative glomerulonephritis. Oral corticosteroids produced clinical and laboratory improvement. Furthermore, the patient was also presenting several invasive cutaneous squamous cell carcinomas in a setting of marked helioderma. DISCUSSION: Erythrodermic, ichthyosiform, atrophied, ulcerated and pseudotumoral forms of cutaneous sarcoidosis are very uncommon and their simultaneous appearance in the same patient is extremely rare. Further, the existence of numerous cutaneous carcinomas in this patient raises the question of an association between sarcoidosis and cancer, with fortuitous association appearing unlikely.

[Necrotic leg ulcer revealing vasculitis induced by vitamin K antagonists]. / Ulcère de jambe nécrotique révélant une vasculite induite par les antivitamines K.

UNLABELLED: Vitamin K antagonists are widely used in thromboembolic diseases. Hemorrhagic complications related to drug overdose represent their main side effect. We report a rare side effect, a severe and unexpected type of skin vasculitis - necrotic leg ulcer - induced by vitamin K antagonist. CASE REPORT: A 63-year-old female with a history of diabetes developed hyperalgesic necrotic ulcerations on the lower limbs one month after starting an acenocoumarol-based treatment for ischemic heart disease. Histological examination revealed lymphocytic vasculitis with fibrinoid necrosis. Etiological explorations searching for vasculitis were negative. In the absence of a precise etiology, drug-induced ulcer was suspected. Low molecular weight heparin was prescribed to replace acenocoumarol. The lesions slowly resolved with topical treatment. DISCUSSION: The chronological criteria and the negativity of etiological explorations allowed the diagnosis of vitamin K antagonist-induced necrotic skin ulcer. Clinicians should be aware of this rare complication induced by oral anticoagulants because of its practical therapeutic implications. This is the first case of necrotic leg ulcer induced by acenocoumarol corresponding histologically to necrotising lymphocytic vasculitis.

[Cutaneous cryptococcosis mimicking basal cell carcinoma and revealing systemic involvement in acquired immunodeficiency]. / Cryptococcose cutanée mimant un carcinome basocellulaire et révélant une atteinte systémique sur immunodéficience acquise.

BACKGROUND: Cryptococcosis is a rare and a serious opportunistic infection that occurs primarily on the field of immunodeficiency. We report a case of disseminated cryptococcosis in acquired immunodeficiency syndrome revealed by unusual skin lesions. OBSERVATION: A 52-year-old patient consulted for two crusty ulcerative lesions situated on the left supraorbital and on the nasal tip that appeared 6 months ago. He also reported respiratory symptoms present since one year, with dry cough and dyspnea, chronic headache and vomiting with no alteration in visual acuity. The mycological study of the skin biopsy on both lesions isolated Cryptococcus neoformans as well as in the sputum and cerebrospinal fluid. Serology of human immunodeficiency virus infection was positive. Treatment with fluconazole, local care and antiretroviral triple therapy was implemented. DISCUSSION: Skin lesions during cryptococcosis are rare and observed in 2-10% of cases. Cutaneous symptoms were the reason for consultation in our patient. This is a rare form of cutaneous cryptococcosis leading to the discovery of both pulmonary and central nervous system locations, and to diagnosis of HIV infection.

[Ophthalmic manifestations after Lyell and Stevens-Johnson syndromes]. / Manifestations ophtalmiques au décours des syndromes de Lyell et de Stevens-Johnson.

PURPOSE: To describe the ocular complications at the end of serious drug eruptions such as Lyell syndrome or toxic epidemic necrolysis (TEN) syndrome, Stevens-Johnson syndrome (SJS), and SJS/TEN overlap syndrome; to analyze their relationship using disease severity scores. PATIENTS AND METHODS: A retrospective study carried out in the dermatology department in collaboration with the ophthalmopathy department in a series of 81 dossiers collated over a 10-year period. The severity of ophthalmological involvement was evaluated using the Foster score, and the drug eruption score was assessed using the Bastuji-Garin classification and the SCORTEN prognostic score. RESULTS: The average patient age was 36 years; the clinical forms seen were Lyell's syndrome in 57.8 % of cases, Stevens-Johnson syndrome in 32.8 % and overlap syndrome in 9.4 % of cases. According to the Foster classification, 34 % of patients were in stage I, 43 % in stage II, and 22 % in stage III. Stage I was seen in 50 % of cases presenting with overlap syndrome and in 42 % of patients with Stevens-Johnson, whereas stage III accompanied Lyell's syndrome in 27 % of cases, followed by SJS/TEN overlap syndrome in 16.6 % of cases. Photophobia persisted in 36 patients, and there were 17 cases of decreased visual acuity and 13 cases of eyelid malposition. Dry eye syndrome was noted in 39 cases and corneal perforation in 3 cases. DISCUSSION: The ocular manifestations of drug eruptions are daunting. It was suggested that ocular involvement is more serious when the peeled skin surface is extensive.

[An uncommon cause of delayed walking: idiopathic palmoplantar hidradenitis]. / Une cause inhabituelle de retard d'acquisition de la marche : l'hidradénite palmo-plantaire idiopathique.

INTRODUCTION: Idiopathic palmoplantar hidradenitis is an uncommon neutrophilic dermatosis, occurring in children and young adults. Its pathogenesis is incompletely understood. It is characterized by spontaneous resolution, but it can relapse in 50% of cases. We describe a case of a child with atypical idiopathic hidradenitis remarkable for its impact on his motor development. OBSERVATION: A 3-year-old boy was admitted to the pediatric unit for etiological assessment of delayed walking. Medullary MRI and TSH were normal. He was referred to a dermatologic consultation for recurrent and painful palmoplantar lesions, giving an equinus antalgic posture. Examination found erythematous tender plantar nodules. The palms were not affected. The exam was otherwise normal. Diagnosis of idiopathic plantar hidradenitis, pressure urticaria, and plantar erythema nodosum were discussed, leading to a skin biopsy. The histopathologic findings of nodular, neutrophilic infiltrates around the eccrine glands confirmed the diagnosis of idiopathic plantar hidradenitis. Anti-inflammatory treatment was given, followed by complete resolution of the lesions, but persistent equinus posture. COMMENTS: Idiopathic palmoplantar hidradenitis is an uncommon neutrophilic dermatosis, distinct from neutrophilic eccrine hidradenitis. It corresponds to neutrophilic infiltrates of the eccrine sweat glands. It is more frequently reported in children and young adults with no medical history. Its pathogenesis is not completely explained. The lesions are usually painful, hindering walking for a few days or even delaying it, as for our patient. These lesions typically involute, but they may recur in more than half of the cases. CONCLUSION: Through this observation, we highlight the atypical impact of idiopathic hidradenitis and the value of a dermatological examination in case of delayed walking.

[Myxoid neurofibroma of the thumb]. / Neurofibrome myxoïde du pouce.

UNLABELLED: Myxoid neurofibroma (MN) is a benign tumor of poorly identified perineural cell origin. We report a case of NM of left thumb and discuss the main differential diagnosis. CASE REPORT: A 34-year-old woman presented with a painless, slowly progressive tumor of the left thumb of 6 months duration. This tumor had recurred after resection done 26 years before. Clinical examination revealed a tumor on the anterior surface of the left thumb, painless, firm, 4×3cm, ulcerated and covered with thin telangiectasia. The X-ray of the phalanx showed no alteration to the underlying bone. The histopathological study of a skin biopsy reported a myxoid neurofibroma. Excision of the lesion was performed with preservation of the nail. Neither the patient nor her family members had neurofibromatosis. The outcome was favorable and no recurrence was noted after 18 months of follow-up. COMMENTS: The usual sites of the MN are the face, shoulders, arms, and periungual regions. It is usually a solitary lesion; however, lesions may be multiple or recur after initial incomplete excision as in our patient. It must be considered in the differential diagnosis of tumors of the extremities. We report this case because of the rarity of both the tumor and its site.

Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 52 cases.

Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined due to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52 patients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied in 31 patients and an activating mutation of the BRAF or NRAS gene was researched in 22 patients. 30 men and 22 women, with a mean age of 33 were studied. Forty-three tumors (82.69%) were located in the extremities, specially the foot (19 tumors). Median initial tumor size was 4.8 cm (1 to 15 cm). Necrosis involving more than 50% of the tumor cells was found in 14 cases (26.92%). High mitotic rate (>10) was found in 25 cases (48.07%). The EWS/ATF-1 translocation was found in 28 (53.84%) of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%). Among the tumor-associated parameters, only tumor size (>4 cm) emerged as a significant prognostic factor. Forty-nine patients had a localized disease at diagnosis (94.23%) and underwent surgical resection immediately (90%) or after neoadjuvant chemotherapy (CT) (10%). Various CT regimens were used in 37 patients (71.15%) with no significant efficacy. The 5- and 10-year OS rates were 59% and 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the optimal treatment for this aggressive chemoresistant tumor.

[Bilateral elastofibroma dorsi]. / Élastofibrome dorsal bilatéral.

BACKGROUND: Elastofibroma dorsi (EFD) is a rare form of benign soft-tissue tumour that is almost always located at the lower pole of the scapula, deep to the serratus anterior muscle. There are few reports in the dermatological literature since it rarely develops in skin or subcutaneous tissue. CASE REPORT: We present the case of a 59-year-old man with bilateral elastofibroma dorsi. Physical examination showed two bilateral scapular masses, better visualized in abduction or antepulsion. They were firm, painless, mobile, covered by normal skin, and measured 40/35 mm on the right and 50/55 mm on the left. Ultrasound of soft tissue and a chest CT scan showed two heterogeneous masses (tissue and fat) within the serratus anterior muscle measuring 30/15 mm on the right and 60/19 mm on the left. Histopathological study of a deep biopsy done on the right helped to confirm the diagnosis of EFD. Tumour resection was done on the right. DISCUSSION: Elastofibroma dorsi is an uncommon, benign, non-encapsulated pseudo-tumour occurring in connective tissue of the infrascapular region of elderly patients. Another mass must be sought in all cases in the corresponding contralateral site since this form of tumour is often bilateral. CONCLUSION: EFD must be considered in the differential diagnosis of shoulder masses in elderly patients.

[Severe necrotizing myopathy subsequent to Merkel cell carcinoma]. / Myopathie nécrosante sévère consécutive à un carcinome à cellules de Merkel.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare tumour with a poor prognosis. Rare cases of paraneoplastic neurological syndrome have been associated with this type of tumour, namely myasthenic syndrome of Lambert-Eaton and encephalomyelitis. We report the first case of severe necrotizing myopathy with anti-Hu antibodies complicating MCC. CASE REPORT: We describe the case of a 58-year-old woman with Merkel cell carcinoma (MCC) of the forearm complicated by severe necrotizing myopathy associated with the presence of anti-Hu antibodies. This myopathy occurred 3 months after complete remission of MCC. The patient was treated with high-dose corticosteroids combined with two intravenous infusions of immunoglobulins. Her neurological status deteriorated despite this treatment. Pararaneoplastic syndrome (anti-Hu antibodies, necrotizing myopathy) complicating MCC was suspected. There was no visible tumour relapse. After multidisciplinary discussion, it was decided to supplement treatment with chemotherapy (carboplatin and VP-16). The patient died 20 days after the first course of chemotherapy. DISCUSSION: Severe necrotizing myopathy with anti-Hu antibodies may be added to the list of possible paraneoplastic syndromes associated with Merkel cell carcinoma.

Unusual presentation of a rare tumor of the dorsal surface of the foot.

Calcifying aponeurotic fibroma (CAF) was originally described by Keasbey in 1953 as juvenile aponeurotic fibroma, most commonly occurring in the palms of the hands and soles of the feet in children and adolescents. It usually presents as a firm, painless mass without preceding trauma. We report a case of this rare condition with an unusual presentation in a 60-year-old woman affecting the dorsal surface of the foot. It is a relatively benign condition with a good prognosis following complete surgical excision. It may have a slightly increased incidence in males. The accurate diagnosis is based only on histology but it is essential to differentiate it from other sinister lesions such as fibrosarcoma that may lead to amputation.

A case of pemphigus herpetiformis in a 12-year-old male.

Pemphigus herpetiformis (PH) is one of the less common forms of pemphigus. PH in children is unreported. We describe a case of a child who developed PH. Observation. A 12-year-old boy was seen at our department with erosive plaques, vesicles, and crusted cutaneous lesions associated with severe itching persisting for six months. Histologic examination showed an intraepidermal bulla containing rare acantholytic epidermal cells with eosinophilic spongiosis. Direct immunofluorescence demonstrated intercellular Ig G and C3 deposit. The serum titer of antibodies against intercellular epidermal was 1/200 UI/l. Diagnosis of PH was made, and treatment with Dapsone 2 mg/kg per day resulted in total clinical remission. However, two months later, new vesicles reappeared and treatment was begun with prednisone at a dose of 2 mg/kg daily. There was a very good response. Discussion. Childhood pemphigus herpetiformis is a rare disease, often initially misdiagnosed. It must not be forgotten that the disease is a possible cause of erosive mucocutaneous disease in children.