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Background: Median raphe cysts are rare benign lesions of the male genitalia. They may be present along the entire raphe, from the urethral meatus to the anus, in the median position. We report herein a case of median raphe cyst with an atypical clinical and histological presentation., Patients and Methods: An 11-year-old boy consulted in dermatology for an asymptomatic perineal lesion. The clinical examination revealed a linear cystic lesion with clear content extending from the base of the scrotum to the anus. The histological diagnosis was a superficial dermal glandular cyst suggestive of a possibly apocrine origin in this topography, or possibly due to malformation., Discussion: Different clinical and histological features of median raphe cysts have been reported in the literature. Treatment is mainly surgical., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Introduction: Chondroid syringoma (CS) is a rare cutaneous tumor characterized by mixte epithelial and mesenchymal component. The confident histological diagnosis can be obtained by immuno-histochemistry study. Here we present 10 new cases with their clinico-hystological characteristics., Methods: The 10 cases were observed between January 2000 and august 2013, in Fort-de-France and Louis-Mourier universitary hospitals. For all the cases a controlled histological study was performed by a dermatopathologist expert and immuno-histochemistry was added. Clinical and immuno-histological data were analyzed., Results: The lesions were almost localized on the face (3/10) and the extremities (3/10). The size was about 1.2 to 5.2cm. Every case was treated by surgery, no malignant case was diagnosed. Histologically, all the 10 cases presented as a well-limited dermic tumor with a mixte epithelial and mesenchymal component. The stroma was myxo-chondroid, and the epithelial component consisted in epithelial cavities lined by one or two cell layers with eccrine (4/10) or apocrine (5/10) features. Immuno-chemistry study reveals positivity for EMA, ACE and CK7 for the internal cells, and positivity for S100 protein and vimentin of the extern cell layer., Discussion: Chondroid syringoma is characterized by a mixte epithelial with eccrine and apocrine cells and a myxo-chondroid stroma. Our study has some clinical and histological particularities (lesions on the extremities, epidermic connecting…). The main differentials diagnoses are the other annexial tumors. The treatment is surgical., Conclusion: The histological diagnosis of CS is quite easy, but in case of doubt, immuno-chemistry will help, showing a double mesenchymal and epithelial differentiation., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Background: Tuberculosis is the most common mycobacterial disease in the world. The cutaneous form is rare in low endemic countries. The occurrence of several cutaneous tuberculosis cases in our dermatology department during 2011-2012 led us to investigate whether there was a resurgence of cutaneous tuberculosis in France. The aim was to analyse changes in cutaneous tuberculosis and the related clinical, microbiological and therapeutic data., Patients and Methods: We conducted a retrospective study in our hospital between 2005 and 2012 by querying the PMSI database (code: A 18.4). Epidemiological, clinical, paraclinical and therapeutic data were collected. Erythema induratum was regarded as a variety of cutaneous tuberculosis., Results: Thirteen patients presented cutaneous tuberculosis between 2005 and 2012. The most frequent clinical forms were erythema induratum of Bazin (n=6) and scrofuloderma (n=3). Microbiological evidence was provided in only 4 cases., Discussion: Diagnosis is difficult due to the varied clinical forms and to the relatively high frequency of paucibacillary forms. Further, the set of additional examinations is non-specific. In some cases, it is only therapeutic tests that allow diagnosis to be made. The place of new diagnostic tools must be clarified and a universally acceptable definition of erythema induratum devised., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Background: Mycosis fungoides during large cell transformation to lymphoma has a poor prognosis with mean survival of 36 months. Autologous stem cell transplantation is rarely proposed in this indication. We report the case of a young man still in complete remission for transformed mycosis fungoides 14 years after autologous stem cell transplantation., Case Report: A 25-year-old man presenting eczema-like patches since childhood was treated by chemotherapy for multiple lymphadenopathies considered as Hodgkin's lymphoma. He was referred with diffuse skin tumours and infiltrated patches. Histology of tumour samples revealed atypical T-cell infiltrate with epidermotropism and presence of more than 25% of large CD30-positive cells. Non-infiltrated patches showed small T-cell lymphoma with epidermotropism. Histological verification of a previous lymphadenopathy confirmed the diagnosis of transformed mycosis fungoides. Despite multiple courses of chemotherapy, the disease progressed, with neurological involvement in particular. Because of tumour aggressiveness, autologous stem cell transplantation was performed and resulted in rapid regression of the tumours, lymphadenopathy and neurological symptoms. Non-transformed mycosis fungoides patches persisted but were controlled with topical mechlorethamine. This patient is still in complete remission for tumour and extracutaneous lesions 14 years after the autograft., Discussion: This was probably a case of juvenile mycosis fungoides diagnosed and transformed in adult age. Neurological involvement by mycosis fungoides is rare and usually carries a drastic prognosis. To our knowledge, this is the longest remission of transformed mycosis fungoides seen after autograft. It highlights the value of this method in aggressive transformed mycosis fungoides, especially in patients ineligible for allograft.

Introduction: Usually, temporal arteritis progresses as a chronic disease., Case Report: The authors report the observation of a 74-year-old woman who presented with two acute flares of temporal arteritis with headache, fever and inflammatory syndrome, which have spontaneously resolved., Discussion: The observations of auto-inflammatory attacks of arteritis disease are rare, but maybe underestimated. The pathophysiology remains unclear.

We report two cases of intravenous leiomyomatosis, a rarely described benign tumor, which illustrate potential complications resulting from metastatic dissemination. Both cases were fortuitously discoveries. We discuss the diagnostic problems, etiological factors, therapeutic management and prognosis. Hysterectomy with bilateral salpingo-oophorectomy and regular follow-up with cardiac and pulmonary monitoring were performed. Pelvic pain disappeared and the patients are recurrence-free. The main difficulties concerned early diagnosis, complete resection and prognosis. These cases illustrate the importance of careful pathology examination of all uterine myoma resection specimens.

Infantile myofibromatosis is the most frequent fibromatosis in childhood. It is a benign proliferation of fibroblasts and myofibroblasts. This case report concerns a newborn who presented at birth with a purple cutaneous nodule on the scalp. Surgical excision was performed at the age of 16 months. Infantile myofibromatosis was diagnosed on histology. Infantile Myofibromatosis (IMF) was first described by Enzinger in 1981. Three types can exist. Solitary MFI, the most frequent, is a solitary lesion, cutaneous/subcutaneous, osseous or involving soft tissues. Multicentric disease is characterized by multiple locations and generalized form by visceral involvement. Morphological features are identical in all types. The histological diagnosis relies on the identification of two separate components, a fascicular myofibroblastic pattern at the periphery with a hemangiopericytoma like pattern in the centre. Both components are positive for alpha-smooth muscle actin. Atypia, or mitotic activity, are not observed usually but features of intravascular growth can be seen in the centre of the lesion. Infantile MF carries a good prognosis when solitary but death frequently occurs in generalized MF with visceral involvement.

Introduction: Xanthoma disseminatum is a non-Langerhans histiocyte proliferation, described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis, associated with diabetes insipidus and normal lipid metabolism. In this case report, vinblastine produced the regression of the lesions., Observation: A 51 year-old man presented in 1999 with a four-year history of progressive xanthomatous papulonodular lesions of his trunk, axillary and inguinal folds, neck and face (leonin facies). Treatment with thalidomide for 6 months was ineffective. A rapid extension of the lesions to the pharynx, larynx and trachea with dyspnea occurred and required a tracheotomy. Histopathological study showed a dense histiocytic infiltrate within the upper and mid dermis with Touton giant cells and inflammatory cells. The histiocyte cells were positive for CD68. Neither lungs nor hypophysis were involved. A treatment with 6 cures of cyclophosphamide was insufficient. Vinblastine therapy (32 cycles: 0.1 mg/kg/cycle) produced a spectacular regression of the mucous-cutaneous papulonodular lesions leaving cheloid scars. The patient required a transitory tracheotomy and a bilateral commissuroplasty., Discussion: Prognosis of xanthoma disseminatum is in related to the mucous membrane manifestations (50 p. 100 of cases) and involvement of the upper respiratory tract. The response to any form of therapy in xanthoma disseminatum is unsatisfactory. Surgical excision or laser therapy can improve physical and functional aspects but the evolution is characterized by very frequent relapses. Treatment with antimitotic drugs seems to be ineffective in many cases. In our patient, vinblastine induced a spectacular regression of mucocutaneous lesions without neurologic toxicity. To our knowledge, this is the first report of vinblastine efficacy in this rare and severe disorder.

Nora's lesion, also known as bizarre parosteal osteochondromatous proliferation (B.P.O.P.), involves mostly the small tubular bones of the hands and feet. Histologically, it is characterized by a proliferation of chondroid, bony and fibrous tissues, sometimes with high cellular density, bizarre chondrocytes but is devoid of cellular atypia and necrosis. Distinct blue color is noted at the interface of bone and cartilage. The most important lesions that present differential diagnostic problems are chondrosarcoma, parosteal osteosarcoma and florid reactive periostitis. The lesion is benign but may recur locally in as many as 55%. The clinical and pathological findings of four cases of Nora's lesion are presented.

Objectives: Mycobacterium marinum is responsible for "fish tank granuloma", a chronic and sometimes disabling skin infection, which in France would be the most frequent "atypical" cutaneous mycobacteriosis. Tropical fish salesmen are concerned about this disease because they might sell contaminated fish and be infected themselves. A review of the literature indicated that one quarter of the patients were aquarium workers. This original study tested the salesmen's knowledge and behaviour regarding "fish tank granuloma"., Method: This national study consisted in an anonymous questionnaire with 24 questions., Results: Among forty salesmen, six of them (15 p. 100) knew this human disease well. Thirty (75 p. 100) ignored it. Among the few salesmen (22.5 p. 100) who had studied to become aquarium workers, only one third were taught about Mycobacterium marinum. Eight salesmen (20 p. 100) were concerned about the human disease: three of them were personally infected and five others had known infected colleagues or purchasers. Most of the aquarium workers immersed their hands without gloves in the fish tanks every day. Only a few destroyed all the fishes from a contaminated tank., Conclusions: Most of the tropical fish salesmen ignored "fish tank granuloma" although 20 p. 100 of them were concerned. This chronic disease, sometimes disabling, should be better known by aquarium salesmen. The national tropical fish salesmen were informed of the results of this study.

Background: Prostatic adenocarcinoma is exceptionally associated with cutaneous lesions. We describe a patient with cutaneous lymphangitis and paraneoplastic ichtyosis related to prostatic cancer., Case Report: A 92 year-old man had been treated for five years for a prostatic carcinoma. An angiomatous lesion developed with in 3 months near the right breast. Physical examination revealed axillary node enlargement, a large skin angiomatous lesion, and ichtyosis. The skin biopsy of the angiomatous skin lesion demonstrated its prostatic origin with carcinomatous metastases in the lymphatic vessels. The ichtyosis was considered as paraneoplastic., Discussion: Cutaneous metastases from prostatic carcinoma are rare. Less than 1 p. 100 of cutaneous metastases are of prostate origin despite the high frequency of this cancer in the general population. The clinical aspects - angiomatous lesion and paraneoplastic ichtyosis - are exceptional.

Malignant pilomatricoma is a rare malignant hair follicle tumor, that was initially described in 1980. Histologically, it shares common features with the more frequent, which benign pilomatricoma, makes its diagnosis difficult. It is a deep dermis-hypodermis epithelial tumor, well circumscribed, with no relation with the epidermis. It is composed of nodular structures with rows of basaloid cells in their periphery, as well as focal necrosis and mummified "ghost" cells in their central parts. Immunohistochemistry is of little value and can not confirm malignancy. The diagnosis remains essentially morphological. Histological examination must stress on the evaluation of the degree and extent of infiltration of the surrounding tissues, the degree of necrosis, the presence of atypical mitotic figures, and the presence or not of peri-neural or vascular invasion. Surgical wide resection is the recommended treatment. It reduces the risk of local recurrence by 50%. Malignant pilomatricoma carries a high risk of metastases to the bones, lungs, and lymph nodes. No feature is specific to confirm wether a malignant pilomatricoma arises de novo, or whether it is a malignant transformation of a pre-existing benign pilomatricoma.

Postmenopausal frontal fibrosing alopecia is a rare aspect of scarring alopecia concerning elderly women. It appears as a receding anterior hair line localised in the frontal and temporal regions. It is a particular pathologic and clinical form of lichen planopilaris. The histologic aspect is that of a lichenoïd inflammatory infiltrate affecting the dermal follicular junction, accompanied by a fibrous scarring aspect, the latter contributing to the diagnosis and individualization of this entity. Discoïd lupus erythematous is the main histologic differential diagnosis. Postmenopausal period is the only associated condition found in affected women. Evolution is unpredictable and does not seem to be modified by treatment.

Introduction: Diagnosis of retroperitoneal fibrosis is generally delayed and revealed by various non-specific signs. We report the case of an isolated lymphedema of the lower limb revealing retroperitoneal fibrosis complicating a metastatic squamous cell carcinoma., Case Report: In an 83-year-old women, a lymphedema appeared that remained isolated for several months before being associated with alteration in general health. Morphological examinations showed bilateral compression of the urinary excretory tracts and led to the diagnosis of retroperitoneal fibrosis. Histological examination of a sub-clavicular adenopathy that had evolved over 9 months, confirmed the diagnosis of a metastatic squamous cell carcinoma of pulmonary cancer., Discussion: Retroperitoneal fibrosis is an exceptional etiology that must be recognized in isolated lymphadomas of the lower limbs. In view of the possible tumoral origin of retroperitoneal fibrosis, any evocative sign accompanying the lymphedema must be searched for.

Introduction: Immune restoration syndrome was first described in 1998 and involved mycobacterium avium complex. We report the case of a patient with acquired immunodeficiency syndrome who had disseminated cutaneous lesions due to Mycobacterium tuberculosis, following initiation of highly active antiretroviral therapy., Case Report: A 42 year-old HIV-infected man, was admitted for fever, cough, nocturnal sweat and impaired of general condition. He had a viral load of 127,200 copies/ml and 199/ml CD4 T-cells. He was treated with triple tuberculosis combination therapy according to tuberculous contagium, positivity of the tuberculin intradermoreaction (15 mm) and right upper lung nodule on thoracic scan. M. tuberculosis was not found. Fever improved at day 3. Highly active antiretroviral therapy with zidovudine, lamivudine, indinavir, was started at day 11 and 33 days after, fever and dermohypodermal nodules with necrotising evolution appeared. Skin biopsy specimen showed tuberculoid granuloma. The levels of viral load and CD4 T-cells were less than 200 copies/ml and 497/ml respectively. Fever and cutaneous lesions spontaneously resolved without changing therapy., Discussion: Immune restoration syndrome appears after initiation of antiretroviral therapy, in patients with advanced HIV infection and without prophylactic treatment versus MAC. This case report probably involves mycobacterium tuberculosis. Bacterial lysis and immune restoration take part in cutaneous pathogenesis. Subclinical mycobacterial infection should be monitored during initiation of antiretroviral therapy in patients with advanced HIV infection.

Background: Systemic corticosteroid is the main treatment of severe forms of pemphigoid gestationis. We report a case of generalised pemphigoid gestationis successfully treated with very potent topical corticosteroid., Case: A 37-year-old woman developed during her third pregnancy with a new partner an urticated generalised eruption associated with bullous lesions. The diagnosis of pemphigoid gestationis was confirmed by direct immunofluorescence which detected a linear C3 deposition along the basement membrane zone and the positivity of Herpes Gestationis Factor (10 units). Local treatment with potent corticosteroid (betamethasone dipropionate 0.05 p. 100) failed and the patient was successfully treated by clobetasol propionate 0.05 p. 100 cream. The infant, in good health, was not delivered prematurely., Discussion: Severe form of pemphigoid gestationis are currently treated with 0.5 to 1 mg/kg/day of systemic corticosteroids, with maternal and pediatric possible side effects. As in bullous pemphigoid, this observation underlines the efficiency and good tolerance of very potent corticosteroid in severe forms of pemphigoid gestationis.