[Nodulocystic eruption induced by sorafenib]. / Éruption nodulo-kystique induite par le sorafénib.

INTRODUCTION: Sorafenib is a multikinase inhibitor used in the treatment of hepatocellular carcinoma, advanced renal cell carcinoma, and differentiated thyroid carcinoma. Cutaneous adverse events are numerous and occur frequently. PATIENTS AND METHODS: We present two cases of nodulocystic lesions associated with comedones in patients treated with sorafenib for hepatocellular carcinoma. In the first patient, a 64-year-old man, lesions appeared on the trunk one year after beginning sorafenib. Histopathological examination revealed a non-granulomatous, perivascular and perisudoral polymorphic cellular infiltrate associated with comedones and microcysts. These lesions progressed via inflammatory episodes interrupted by long periods of spontaneous remission without any specific treatment. In the second patient, a 53-year-old woman, a rash appeared on the buttocks three months after starting sorafenib and then spread to the lumbar region and thighs. Histopathological examination was consistent with granulomatous acne lesions. The initial treatment (oral tetracycline and zinc) given for 3 months proved ineffective. Patient follow-up over 3 years showed gradual regression without the appearance of any further lesions. DISCUSSION: In the literature, several reports discuss acneiform rashes in patients treated with targeted therapy. In most cases, these lesions were papulopustular without retentional lesions. There are few reports of nodulocystic eruptions associated with comedones following sorafenib therapy. The mechanisms of emergence of these lesions seem to involve inhibition of the RAF pathway, C-KIT, and the PDGF signaling pathway.

Scabietic vasculitis: Report of 2 cases.

BACKGROUND: The infectious causes of cutaneous vasculitis are well known and include streptococcal infections among others. Cases resulting from parasitic infection are less frequent. Scabies, which is currently on the increase, has only been reported in a few isolated cases. Herein, we report two noteworthy cases of profuse scabies complicated by cutaneous vasculitis. PATIENTS AND METHODS: Case 1: a 90-year-old woman, residing in a nursing home, was admitted to our dermatology department complaining of pruritus, present for one month, predominantly on the inside of the thighs and on the buttocks, associated with purpuric lesions on the lower limbs. A skin biopsy revealed leukocytoclastic vasculitis. A diagnosis of scabies was based on severe pruritus and hypereosinophilia and was confirmed by microscopic examination of the parasitology sample and the skin biopsy sample. Despite thorough investigation, no other cause of vasculitis could be found. Complete regression of the skin lesions was achieved with scabies treatment only, without any specific treatment for the vasculitis. Case 2: a 74-year-old man, living in a nursing home, was hospitalized for purpuric papules on the lower limbs, present for one month. Physical examination revealed linear patterns in the interdigital spaces associated with scabies evident on dermoscopic examination. The skin biopsy revealed signs of vasculitis. As in our first case, no aetiology of vasculitis was found and a favorable outcome was achieved by means of scabies treatment alone with no specific treatment for vasculitis. DISCUSSION: Both of our patients presented scabies and vasculitis. In view of the absence of other causes of vasculitis and of the complete regression of lesions due to vasculitis without recurrence achieved with the scabies treatment alone, a diagnosis was made of scabietic vasculitis, probably as a result of cutaneous hypersensitivity reaction to humeral mediators.

Cutaneous basidiobolomycosis: Seven cases in southern Benin.

BACKGROUND: Cutaneous basidiobolomycosis is the most common form of entomophthoramycosis. Herein we report seven cases of cutaneous basidiobolomycosis. PATIENTS AND METHODS: A retrospective observational study was conducted at the Buruli ulcer treatment centre in Pobè and at the national teaching hospital in Cotonou from 2010 to 2015. RESULTS: Seven cases of cutaneous basidiobolomycosis were diagnosed. The mean patient age was 9.53 years. There were 4 female and 3 male patients, all from southeast Benin. Clinically, the disease presented in all cases as a hard, well-defined, subcutaneous plaque with little inflammation, and which could easily be lifted from the deep structures but remained attached to the surface structures. The overlying skin was hyperpigmented. Plaques were localized to the buttocks or thighs. All patients had inflammatory anaemia with an accelerated erythrocyte sedimentation rate (30 to 70mm over the first hour), and a low haemoglobin count (8.7 to 11.4g/dL). Blood hypereosinophilia (650 to 3784elements/mm3) was present in six of the seven subjects. Histopathology (performed for 5 of the 7 subjects) showed granulomatous lesions with foreign-body giant cells, and inflammatory cells, with occasional eosinophils surrounding fungal hyphae (Splendore-Hoeppli phenomenon). Mycological analysis revealed Basidiobolus ranarum in three cases. The patients were treated with ketoconazole (5/7) and itraconazole (2/7), with good outcomes after 10 to 24 weeks of therapy. DISCUSSION: Cutaneous basidiobolomycosis is uncommon in southern Benin, with only seven cases being diagnosed over 6 years. The diagnosis of cutaneous basidiobolomycosis is a challenge in the field in Benin due to the non-specific clinical presentation, the lack of technical resources, and the existence of numerous differential diagnoses. CONCLUSION: Cutaneous basidiobolomycosis is an uncommon fungal infection in southern Benin chiefly affecting children.

[Plaque-like leiomyomas on a chemical burn: First case report]. / Léiomyomes en plaque sur brûlure chimique : première observation.

BACKGROUND: Cutaneous leiomyomas are uncommon benign smooth muscle neoplasms of skin of unknown pathogenesis. We report a sporadic case of multiple cutaneous leiomyomas at the site of a chemical burn. OBSERVATION: A 47-year-old male presented with multiple grouped red nodules on the right calf painful to cold and to touch. The lesions were located on the site of a chemical wound that had occurred 5 years earlier. Histopathological examination revealed a proliferation of smooth muscle fibres, leading to diagnosis of leiomyoma. DISCUSSION: To our knowledge, the literature contains no previous reports of sporadic multiple cutaneous leiomyomas occurring at a chemical burn site. While a chance association cannot be ruled out, there is also the possibility of a physiopathological mechanism similar to that of vascular tumours following exposure to 4-hydrazinobenzoic acid. This compound, a hydrazine derivative of the fungus Agaricus bisporus, can cause smooth-muscle tumours in the aorta and large vessels with morphological characteristics similar to those seen in vascular leiomyomas.

[Desmoplastic melanoma: a study of 23 cases at 3 centres in the Bouches-du-Rhône region]. / Mélanome desmoplastique : une série de 23 cas dans trois centres des Bouches-du-Rhône.

BACKGROUND: Desmoplastic melanoma (DM) is a rare form of melanoma, often with atypical and potentially misleading aspects that result in difficult and late diagnosis. Because of the high likelihood of local relapse, practitioners must have a good knowledge of such tumours. PATIENTS AND METHODS: A retrospective study of the activities of 3 centres in the Bouches-du-Rhône region between 1998 and 2010 enabled us to collate 23 cases of DM and analyse the clinical and histological features of the disease as well as patient survival. RESULTS: Fifteen of the 23 patients (65.2%) were male with a median age of 64.4 years. Mean Breslow thickness was 7.56 mm. The numbers of AJCC (American Joint Committee for Cancer) stages I, II, III, IV were respectively 4.1, 66.7, 20.8 and 4.1%. Thirteen patients presented relapse, with a mean time to onset of 21 months. The initial relapse involved the skin in 1 case, the lymph nodes in 2 cases, the organs in 5 cases, the organs and subcutaneous region in 1 case, the organs and lymph nodes in 2 cases, and the organs, lymph nodes and skin in 1 case. DISCUSSION: While DM presents a higher rate of local relapse than classical melanoma, this trend appears to subside in the case of thick DM, in which relapse also involves the lymph nodes and/or organs.

[Cutaneous events during anti-TNF alpha therapy: a prospective observational study of 41 cases]. / Evénements cutanéomuqueux au cours des traitements par anti-TNF alpha : étude observationnelle prospective de 41 cas.

BACKGROUND: The cutaneous adverse effects of TNFalpha inhibitors and their potential implication in the onset of associated dermatoses remain poorly understood. PURPOSE: To describe the different clinical dermatological situations seen in patients treated with TNFalpha inhibitors. PATIENTS AND METHODS: We conducted a prospective, observational study of patients followed at the Dermatology Department of the CHU Nord university teaching hospital of Marseilles. All patients, referred by various departments, were treated with TNFalpha inhibitors and presented cutaneous events. RESULTS: Forty-one patients were included in the study. Various cutaneous manifestations were observed, including: 15 psoriatic rashes, six skin infections, three eczema rashes, three cases of lupic syndrome, two anaphylactic reactions to infusion and two cutaneous drug reactions. An original case of parapsoriasis was observed. Cutaneous tumors are rarely described. DISCUSSION: This study confirms the multiple clinical dermatological situations observed in patients treated with TNFalpha inhibitors and illustrates the need for good coordination between dermatologists and other specialists in order to ensure optimal management of this population.

Radiosurgery without whole brain radiotherapy in melanoma brain metastases. Club de Cancérologie Cutanée.

To evaluate the effectiveness of radiosurgery without whole brain radiotherapy in the palliative treatment of melanoma brain metastases, we retrospectively assessed the results in 35 patients: 4 with a solitary brain metastasis, 13 with a single brain metastasis and metastases elsewhere and 18 with multiple brain metastases. The local control rate was 98.2% (55/56 metastases) at 3 months. Median survival was 22 months in patients with a solitary brain metastasis, 7.5 months in patients with a single brain metastasis and metastases elsewhere, and 4 months in patients with multiple brain metastases. Complications were unusual and surgery was required in 2 of 35 patients. These results show for the first time that melanoma patients with a unique brain metastasis with or without metastases elsewhere clearly benefit from tumour control easily obtained by radiosurgery. Although the comparison of radiosurgery with surgery and/or whole brain radiotherapy cannot be adequately addressed, radiosurgery alone seems to provide similar results with lower morbidity and impact on quality of life.

Granulomatous mycosis fungoides.

We report a new observation of granulomatous mycosis fungoides. The diagnosis was able to be made only after performing multiple biopsies during the course of the disease. Initial evolution was rapidly favourable with electrontherapy. A granulomatous reaction is, except in Hodgkin's disease, a rare phenomenon in lymphoproliferative disorders, particularly in cutaneous T cell lymphoma. This variant of mycosis fungoides raises the problem of the histological differentiation from other granulomatous dermatoses, mainly sarcoidosis. Its prognostic significance is disputed and its pathogenesis remains unknown.

[Triple tumor association: breast cancer, meningioma and glioblastoma]. / Triple association tumorale: cancer du sein, méningiome, glioblastome.

A 38 year-old woman treated for breast cancer had 10 months later a cerebral tumor diagnosed as a frontal metastasis. Neurosurgical operation showed a meningioma and a malignant glioma. Although exceptional, such an association, is probably not due to chance.

[Cutaneovisceral leishmaniasis following severe nervous depression]. / Leishmaniose cutanéoviscérale au décours d'une dépression nerveuse sévère.

Leishmania is considered an opportunistic agent during cellular immunodeficiency. The authors report the case of a patient living in the south of France presenting visceral leishmaniasis with cutaneous manifestations in the course of a severe nervous depression. This case report illustrates the possible relationships between stress and immunity.

[Edema caused by retroperitoneal and tricuspid fibrosis with sclerodermatous cutaneous involvement disclosing carcinoid tumor. Apropos of a case and review of the literature]. / Oedèmes par fibroses rétropéritonéale et tricuspidienne avec atteinte cutanée sclérodermiforme révélateurs d'une tumeur carcinoïde. A propos d'une observation et revue de la littérature.

A 65-year-old female was admitted with leg edema by retroperitoneal fibrosis and tricuspid valve incompetence by fibrosis, cutaneous fibrosis, moderate flushing over the upper body without diarrhea. It revealed an ileal carcinoid tumor with hepatic metastases. Octreotide (Sandostatine), tumor excision and interferon alpha 2b treatment led to a regression of flushing and edema, a reduction of fibrosis and a stabilization of the metastatic carcinoid, with normal serotonin levels.

[KID syndrome (keratitis, ichthyosis and deafness)]. / Syndrome KID (kératite, ichtyose et surdité).

Keratitis, ichthyosis and deafness are the dominant signs of KID syndrome. The lesions involving cornea, epidermis and internal ear are probably the result of a congenital ectodermal abnormality. Associated signs such as increased sensitivity to infections, and dermoskeleton dystrophies are also useful for the diagnosis. There are no specific biological signs. Most cases are sporadic but familial cases have been described with unclear mode of inheritance. Treatment is disappointing. Thus management mainly relies upon early detection of complications.

[Urticaria caused by cold. 104 cases]. / Urticaire au froid. 104 cas.

INTRODUCTION: Superficial and deep cold urticaria is a heterogeneous group of manifestations induced by exposure to cold (0 to 4 degrees C). Frequency is generally estimated at 2 to 3 p. 100. We studied retrospectively 104 patients meeting clinical, biological and therapeutic criteria of cold urticaria. PATIENTS AND METHODS: From 1981 to 1995, 1802 patients with urticaria were included in a standardized allergy survey. Among these patients, 104 met the criteria for the diagnosis of cold urticaria: positive test with 3 ice cubes placed on the skin for 5, 10 and 15 minutes or positive immersion test (immersion in 4 degrees C water for 15 minutes). Two groups of patients were excluded, those with systemic cold urticaria and those with minimal thermo-differential urticaria. RESULTS: Cold urticaria was found in 5.7 p. 100 of our urticaria patients, predominantly in young women. The triggering effect of cold was found at history taking in 71 p. 100 of the cases. The urticaria was usually superficial, more rarely associated with deep and/or mucosal urticaria. General signs were uncommon. The three ice cube test demonstrated significative biological and immunological abnormalities. In 5 cases, discrete cryoprecipitate was found (4 cryoglobulins, 1 cryofibrinogen). Although the search for an infectious agent was not conducted in absolutely all cases, there was no apparently remarkable association with infection the exception of VIH infection. Anti-H1 agents were given in 88.3 p. 100 of the cases leading to short-term improvement. Follow-up is insufficient to evaluate long-term outcome. DISCUSSION: This retrospective survey of 104 cases of cold urticaria was compared with data in the literature, particularly with the small number of studies including a large number of patients. We conclude that the diagnosis of cold urticaria can be based on history taking alone and the three ice cube tests (prognosis value, indication of reaction threshold). An exhaustive search for the cause is not indicated. A search for cryopathy should however always be done and followed by a complete work-up in case of positivity. Clinical signs other than cold urticaria suggesting a pathological response to cold require complete investigations.

[Acquired zinc deficiency in a breast-fed premature infant]. / Carence acquise en zinc chez un nourrisson prématuré nourri au sein.

Acquired zinc deficiency in exclusively breast-fed premature babies is a recently described entity which surprisingly, has not been reported more frequently. Its pathogenesis would appear to be the result of various factors, prematurity, exclusive breast-feeding and a suspected maternal defect, acquired or inherited, for the transfer of zinc from the blood to the breast milk. We report the case of a girl who was born at 31 weeks, exclusively breast-fed for 28 weeks and who presented a characteristic clinical feature of zinc deficiency. The mother's zinc level in the breast-milk was abnormally low. After zinc therapy and progressive weaning, the lesions dramatically improved in few days. There was no recurrence of the lesions one year after the treatment was completely stopped. With regard to this characteristic case, we outline the many pathophysiological mechanisms involved in acquired zinc deficiency. Recognition of this clinical feature by dermatologists seems essential because the required treatment is spectacularly effective and definitive. Finally, we also examine possible forms with few symptoms and we wonder if serum zinc levels should be checked in at-risk babies.

[Proteolytic enzymes and their inhibitors]. / Enzymes protéolytiques et leurs inhibiteurs.

Tryptase (T), chymase (C), carboxypeptidase A, cathepsin G-like constituent of preformed mediators contained in mastocyte granules, are a group of neutral proteases with proteolytic activity. These enzymes gives differentiation of two groups of mastocytes, MCTC and MCT as a function of the richness of enzymes. Although the functions of these molecules are becoming better and better understood, their exact roles as well as that of their inhibitors, still remain to be explored in urticaria.

[Vulvar dermatoses]. / Dermatoses vulvaires.

Vulvar dermatoses are the localizations to the vulva of a great variety of dermatoses. In genodermatoses, the expression of diseases on the vulva is a part of a complex syndrome. It is non indispensable for the diagnosis. In the immuno-allergic dermatoses or in dermatoses where the pathogenesis is not clearly understood, the vulvar disease is very important because the vulva is often the exclusive localization. The vulvar disease can be the first manifestation before the other expressions of the mucocutaneous disease. Sometimes, there is a risk of scars or transformation in epithelioma. So the diagnosis must be soon established. At least, hidradenitis suppurativa is disease where the choice of the must appropriate treatment is difficult.