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Resume Introduction La mucinose papuleuse generalisee est une affection rare, de pathogenie encore obscure et de traitement difficile. Nous en rapportons un cas resistant au traitement par corticoides et controle par des immunoglobulines intraveineuses (IVIG). Observation Une femme de 60 ans consultait pour l’apparition progressive de lesions papuleuses de 2 a 5 mm de diametre, d’allure cireuse, de couleur chair, infiltrees et prurigineuses. Le reste de l’examen clinique etait normal. Il existait une dysglobulinemie de signification indeterminee IgG lambda. L’examen histologique d’une biopsie cutanee confirmait la suspicion clinique de mucinose papuleuse. La maladie progressait malgre une corticotherapie a 0,5 mg/kg par jour de prednisone et un traitement par thalidomide mal tolere cliniquement. Une serie de six cures mensuelles d’IVIG a la posologie de 2 g/kg repartis sur cinq jours permettait une regression de plus de 50 % des lesions papuleuses. A l’issue de ces cures, une corticotherapie a faible dose etait maintenue, mais une recidive des lesions survenait deux mois apres la derniere cure. Un traitement d’entretien par IVIG a la meme posologie etait alors administre toutes les six semaines, permettant de stabiliser la maladie et d’eviter les recidives. Discussion L’evolution de la mucinose papuleuse generalisee reste imprevisible et son traitement particulierement difficile. Les IVIG ont recemment ete rapportees pour etre efficaces dans cette pathologie, mais il semble que le traitement ne soit que suspensif et que des cures d’entretien soient necessaires. L’efficacite observee chez notre malade, mais egalement la recidive rapide des lesions semblent confirmer ces donnees.

Resume Introduction Le mycosis fongoide est un lymphome cutane T epidermotrope. Les stades precoces ont un bon pronostic mais posent des problemes therapeutiques par leur chronicite et les recidives frequentes. Nous avons realise une etude retrospective anatomo-clinique sur 10 malades atteints de mycosis fongoide afin d’evaluer l’efficacite du laser excimer a 308 nm dans cette indication. Methodes Dix malades dont le diagnostic de mycosis fongoide avait ete confirme histologiquement, ont ete inclus dans l’etude. Un traitement par laser excimer a 308 nm a ete realise sur un total de 29 lesions dont 25 lesions peu ou pas infiltrees, trois plaques infiltrees et un nodule. Apres determination de la dose erythemateuse minimale, les seances ont ete debutees au rythme de deux par semaine avec une augmentation progressive des doses en fonction de la tolerance. Un suivi clinique a ete ensuite realise. Des biopsies cutanees ont ete realisees, lorsque cela etait possible, avant, a la fin et a distance du traitement afin de realiser une etude histologique et immuno-histochimique. Resultats Quatre-vingt-six pour cent des lesions peu ou pas infiltrees ou en plaques etaient en remission complete a la fin du traitement et 14 p. 100 en remission partielle avec un taux global de reponse de 100 p. 100. Le nombre moyen de seances a ete de 15 (6 a 46 seances), soit une duree moyenne de traitement de 2 mois, et la dose cumulee de 5 J/cm2 (1,3 a 16,1 J/cm2). Dix-neuf lesions ont pu etre suivies a distance du traitement avec une duree mediane de suivi de 15 mois (8 a 26 mois). Une reponse complete s’est maintenue pour 13 lesions (12 lesions peu ou pas infiltrees et une plaque) sur 19, soit 68 p. 100. Une reponse partielle s’est maintenue pour trois lesions (une lesion peu ou pas infiltree et deux plaques) sur 19, soit 16 p. 100. Une rechute est survenue pour deux lesions (deux lesions peu ou pas infiltrees) sur 19, soit 11 p. 100. Ces deux rechutes ont toutes deux ete observees a 7 mois. Le seul nodule traite n’a pas repondu au traitement. Les resultats histologiques et immuno-histochimiques concordaient avec l’aspect clinique montrant une guerison histologique en cas de guerison clinique hormis un cas qui a montre la persistance de quelques cellules de type mycosis en exocytose alors que la lesion paraissait guerie cliniquement. Discussion Cette etude montre l’efficacite du laser excimer dans les mycosis fongoides au stade Ia. Compte tenu de la faible disponibilite de ce traitement et de son caractere onereux, le laser excimer a 308 nm reste indique en seconde intention apres echec ou recidive d’un traitement par dermocorticoides bien conduit.

Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG).A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease.The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurrence of lesions following withdrawal.

Mycosis fungoides is a cutaneous T-cell lymphoma. The early stages have a good prognosis but pose therapeutic problems due to chronic disease status and the frequent recurrence of lesions. We performed a retrospective clinical and histological study in 10 patients presenting mycosis fungoides in order to evaluate the efficacy of 308 nm excimer laser in this indication.Ten patients with mycosis fungoides confirmed by histological examination were included. Treatment by 308 nm excimer laser was performed on a total of 29 lesions (25 patch-stage, 3 plaques and 1 nodule). After determination of the minimal erythemal dose, sessions were started twice weekly gradually increasing in accordance with tolerability. Clinical follow-up was performed and histological and immunohistological analysis was done, if possible, before and after treatment and at the end of the follow-up.Eighty-six percent of patch-stage or plaque lesions had completely cleared up by the end of treatment and 14% had cleared up partially, with a global response rate of 100%. The mean number of sessions was 15 (6 to 46 sessions) corresponding to a mean treatment duration of 2 months. The mean cumulative dose was 5 J/cm2 (1.3 to 16.1 cm2). Follow-up was performed in 19 lesions for a median period of 15 months (8 to 26 months). Persistent complete clearance was observed in 13 of 19 (68%) lesions (12 patch-stage and 1 plaque). Continued partial clearance was noted in 3 lesions (1 patch-stage and 2 plaques) (16%). Two lesions (both patch-stage) (11%) showed relapse 7 months after the end of treatment. The sole nodule in the study showed no response to treatment. Histological and immunohistological were consistent with clinical results and showed histological healing where lesions had clinically disappeared, except for one case with persistence of a few mycosis cells in the epidermis despite a clinical appearance of healing.This study demonstrates the efficacy of 308 nm excimer laser in stage Ia of mycosis fungoides. Given its limited availability and high cost, 308 nm excimer laser may be used as second-line therapy after failure of treatment with topical steroids.

We report the case of a patient in whom the first manifestation of Waldenström' s macroglobulinemia was specific skin lesions, treated with chlorambucil chemotherapy.A 76-years old woman was referred to us because of chronic red nodular lesions on her face. A biopsy specimen showed a dense lymphocytic dermal infiltrate and immunohistochemistry identified a monoclonal B lymphoid population with an IgM-kappa phenotype. The patient's disease was diagnosed as Waldenström's macroglobulinemia with cutaneous localization, on the basis of a high level of circulating macroglobulinemia and a lymphoplasmocytic infiltrate in the bone marrow expressing the same monoclonal IgM-kappa as in blood and skin. Treatment with radiotherapy (12 Grays) was unsuccessful. Chlorambucil (16 mg per day, 7 days per month) was then introduced with rapid disappearance of the skin lesions. Neutropenia led to withdrawal of this treatment after 4 courses. The skin lesions relapsed 18 months later and were cured with chlorambucil at a lower dose.Specific skin infiltrates have been rarely described during Waldenström's macroglobulinemia. Review of the literature showed eight cases of such lesions treated by chemotherapy with only two successes with oral cyclophosphamide and polychemotherapy (cyclophosphamide, vincristine and CCNU). Chlorambucil was used unsuccessfully three times. We hypothesize that primary resistance to alkylating-agent and the small number of cases of cutaneous Waldenström's macroglobulinemia may explain the poor response to systemic chemotherapy previously reported.

Human dirofilariosis is endemic in southern France. The causal agent is Dirofilaria repens transmitted to man by mosquitos. The human organism is an accidental host while the dog is the reservoir. Approximately 60 cases have been reported in France, mainly in southern continental areas and Corsica. Most cases involve subcutaneous and occular manifestations.A 47-year old woman living in the Var department in southern france consulted for a subcutaneous nodule with the aspect of an epidermoid cyst. At incision, the nodule was found to contain a helminth found at parasite examination to be Dirofilaria repens.The prevalence of endemic subcutaneous dirofilariosis is probably underestimated as the clinical expression is non-specific and spontaneous cure is common. The diagnosis of epidermoid cyst is frequently suggested. History taking does not reveal travel to intertropical areas. The helminth is discovered when the nodule is opened, also providing successful cure.

Monoclonal gammopathy and edema are features encountered during several diseases, especially systemic capillary leak syndrome. The diagnoses of POEMS syndrome, edematous systemic scleroderma and a fortuitous association may be also discussed. We report the cases of two patients which did not fulfill the criteria for such diagnoses.Although the 2 cases share some discrepancies, they have also similar and particular features: association of chronic edema, monoclonal gammopathy and profuse and acquired telangiectasias.The meaning of these cases remains to be clarified. It might be an entity close to the systemic capillary leak syndrome but characterized by the chronicity of edema and by a distinct cutaneous sign, the occurrence of numerous telangiectasias. These cases emphasize that the acquired and profuse telangiectasias belong to the wide range of cutaneous abnormalities which may be associated with monoclonal paraproteinemia.

The case of a patient originated from Angola who presented subcutaneous and neurologic cysticercosis is reported.The patient was referred for the etiological diagnosis of recurrent seizures. The diagnosis was made by histologic examination of a subcutaneous lesion.We review the clinical characteristics of subcutaneous cysticercosis. The association of subcutaneous nodules and signs of central nervous involvement occurring in a patient originating from an area where cysticercosis is endemic, must be suspected of cysticercosis.

Acquired elastolysis of the papillary dermis simulating pseudoxanthoma elasticum and white papulosis of the neck are two skin diseases with clearly limited borders. The two entities can be distinguished clinically when papulae converge into yellowish-white patches and histologically by complete absence of the elastic network in the papillary dermis without thickening of the collagen network in the superficial and mid dermis.We observed a patient with the clinical and histological criteria for acquired elastolysis of the papillary dermis. Histological examination showed a thick collagen network as described in white papulosis of the neck.This case clearly demonstrates the histological continuum between these two entities. A review of the literature also provided evidence of borderline forms, leading us to propose grouping them together. Elastolysis of the papillary dermis would appear to be a relatively specific histological sign differentiating white papulosis of the neck, in a general sense, and acquired elastosis of the mid dermis. Two factors could explain the rare observations of acquired elastosis of the mid dermis in patients with acquired elastosis of the papillary derma: use of the Verhoeff Van Gieson stain without an internal control and imprecise localization of the biopsy.

Multiple skin localization of phaeohyphomycoses are rare and are encountered in immunodepressed subjects. We report a case due to Alternaria tenuissima in a patient given corticosteroids and IgA deficiency.A 73-year-old man presented with ulceronecrotic lesions on the two lower limbs which had developed over 15 days and poor general health. He had been taking corticosteroids for retroperitoneal fibrosis for 15 days. Skin biopsies demonstrated monoclonal dysglobulinaemia with IgG kappa and IgA deficiency. Itraconazol treatment was successful.Identification of Alternaria is in a skin sample, it not sufficient to determine its pathogenic nature. Strict criteria, especially histological criteria, are required. Most cases of cutaneous alternariosis are localized infections due to direct inoculation. They are seen in healthy patients or more often in immunodepressed subjects. Disseminated skin disease is rare and has been reported in 2 highly immunodepressed patients. Our patient was taking corticosteroids, but his deficiency state in IgA may have played a role in the development of the mycosis. Itraconazol appears to be effective treatment.

Targetoid hemosiderotic hemangioma (THH) is a new vascular disorder, rarely reported. The lesion presents as violaceous papule surrounded by an ecchymotic ring usually measuring less than 1 cm in diameter. Epithelioid endothelial cells with a hobnail appearance, vascular papillary projections and hemosiderin deposits are the histological hallmarks of this entity.We report a case fulfilling these criteria. Particular morphologic features were the presence of a very prominent lymphocytic infiltrate, with numerous intraluminal lymphocytes closely associated with the neoplastic endothelial cells, scarcity of siderophages, rought of retiform pattern, predilection of newly formed vessels for muscles of hair erection and eccrine ducts.Having regard to these worrisome histologic features the differential diagnosis between THH, Kaposi's sarcoma and newly recognized entities such as lymphangioendothelioma and retiform hemangioendothelioma is discussed.

The recent development of studies applied to ancient materials may be explained by the application of molecular biology techniques on the extracted ancient DNA mainly the polymerase chain reaction (PCR), the ultrasensitive DNA-amplification technique that hit the headlines in the late 1980s. PCR was used to amplify human immunodeficiency virus (HIV), genetic material in stored tissue specimens and to document people who died in 1976. In addition, recent discoveries of mummified bodies in ice of the Tyrolean Alps or Greenland allowed a new approach in archeological studies. Mummies are a good material for investigations of ancient tissues. The studies concern the techniques of embalming, tissue preservation and palepathological aspects. In cutaneous paleopathology, mummies from Egypt, South or North America and Europe were considered. Various skin lesions were characterized: histiocytoma, Chagas' disease, smallpox, syphilis. Recently drugs (cocaine, hashish and nicotine) were extracted from skin and head hair of Egyptian mummies. Only a few studies were concerned with the ultrastructure of the skin of such mummies.We had the opportunity to obtain skin samples of two Egyptian mummies. One of them was embalmed between 150 BC-90 AC. The skin was studied by transmission electron microscopy. As the mummified materials had dried out and shrunk, it was necessary to rehydrate them for ultrastructural observation. Skin samples were fragmented into small pieces before fixation with 2 p. 100 glutaraldehyde in sodium cacodylate buffer for 10 days (allowing for removal of the materials used for embalming). The samples were then washed in the same buffer for 10 days. After washing the pieces were post-fixed with 1 p. 100 osmium tetroxyde, dehydrated and embedded in Epoxy medium.With this process, it was possible to observe the excellent preservation of the cutaneous structures. The epidermis was well preserved. It was possible to observe the different cell layers and mainly the upper layers. The nuclei and the desmosomes of keratinocytes were recognized. Intercellular spaces were narrow. Desmosomes showed dense thickenings of the cell membrane on both sides and an intercellular band with narrow lucent spaces adjacent to the dense cell membrane. The nuclei showed dense spots of chromatin and in the cytoplasm recognizable tonofilament bundles were identified. Langerhans cells and melanocytes were not observed. In the dermis, the collagen fibrils formed thick bundles and showed the characteristic axial periodicity. Elastic fibers were also recognized showing two main components: the amorphous substance and the fibrils. Throughout the dermis, a number of round or oval structures were found. They had the typical appearance of spores of bacteria. In the centre, they had an electron dense and granular core surrounded by an inner membrane and a spore coat.Only a few investigations were performed on the ultrastructure of the skin of the Egyptian mummies. In the majority of cases, the epidermis was not preserved. The present work demonstrates the good preservation of epidermal structures and specially desmosomes and intercellular connections. The presence of spores of bacteria was previously reported. These spores enter a highly resistant resting phase in order to survive in a dormant state for a long period of starvation or other adverse environmental conditions. Similar investigations were performed on skin obtained from Eskimo mummies preserved by the extremely cold and dry polar weather. In these conditions, the authors reported the observation of melanocytes, vessels and nerves. Additionally, biochemical investigations demonstrated the very good preservation of collagen and glycosaminoglycans of the dermal extracellular matrix.

Pustular vasculitis is a classical manifestation of Behçet's disease. We report a case where the papulopustular eruption formed an arch on the skin and the histology examination showed predominant lymphocytic infiltration.A 29 year-old with a past history of recurrent buccal aphthosis had developed episodic papulopustular eruptions over the past 3 years. The diagnostic of Behçet's disease was made on the basis of an association with lymphocytic meningis, uveitis and erythema nodosum. General corticosteroid therapy was effective, but did not prevent skin relapse, controlled with colchicine.Pustular vasculitis usually presents with pustular eruptions on purpuric lesions and polynuclear infiltration of the dermis. Predominant lymphocytic infiltration is usually due to a pathergy phenomenon and would be specific for Behçet's disease. In our patient, colchicine was effective, although the mechanism remains to be explained.

Extra-cutaneous manifestations of neutrophilic dermatosis are rare. Symptomatic cases are usually multiform and confusing. We report a case with an aseptic lymph node abscess associated with pyoderma gangrenosum.A 28-year-old woman with past history of pyoderma gangrenosum was seen for abdominal symptoms and fever related to an epigastric mass. Due to signs indicating abscess formation, surgery was performed and led to total regression of the symptomatology. Recurrence was evidenced 3 months later with skin lesions which were negative on bacteriological examinations. The diagnosis of lymph node manifestations of pyoderma gangrenosum was retained. General corticotherapy was very effective. Relapse occurred again 6 years later and was treated with thalidomide.Lymph node involvement has been described in Sneddon-Wilkinson's disease associated with pyoderma gangrenosum. In neutrophilic dermatosis, pulmonary manifestations appear to be the least exceptional of the extra-cutaneous lesions. The pathophysiology has not been elucidated although recent hypotheses suggest that G-CSF could be involved. Corticotherapy is remarkably effective in most cases but thalidomide could be an interesting alternative treatment.

We report a case of dermatitis forming a halo around a dermatofibroma. This phenomenon has initially been described around a naevocellular naevus and called Meyerson's naevus, then around other non naevus skin lesions. So far, it had not yet been reported around a dermatofibroma. The condition seems to be non-specific reaction of unknown mechanisms that does not modify the course or the prognosis of this circumscribed lesion.

We report a case of focal epithelial hyperplasia in a child born in France of Algerian parents. The clinical appearance was unusual in that certain lesions were verrucous and pediculate. A virological study revealed the presence of papillomavirus 32, one of the two types of HPV specifically associated with this entity.

We report a case of erythroderma revealing lymphoblastic leukaemia occurring immediately after myelodysplasia. The patient was an 87-year old man admitted for poor general condition, lymph node enlargement and pruriginous oedematous erythroderma. Laboratory examinations showed tricytopenia, bone marrow invasion by lymphoblasts in a myelodysplastic environment of the refractory anaemia type with excess of blasts, and a cutaneous lymphocytic infiltrate with non-blastic lymph node reaction. Owing to its morphological and immunohistological features, the lymphocytic infiltrate was regarded as non-specific. We could not help comparing this case to the other non-specific cutaneous lesions of refractory anaemia which usually reflect transformation into leukaemia.

Embryonal rhabdomyosarcoma is the most frequent of tissue sarcomas in children. Its location in the subcutaneous tissue makes it a dermatological diagnosis. In childhood, the exact histological type of the tumour, sometimes difficult to determine, is absolutely necessary since prognosis and treatment differ according to the histogenetic form. Rhabdomyosarcoma is rare in adults. One must rule out malignant pleomorphic histiocytoma which has a more favourable prognosis. Among the antisera recently made available, those directed against desmin, foetal skeletal myosin and/or specific skeletal muscle myofilament seem to be most useful when associated with the anti-myoglobin antibody.