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Resume Introduction Le granulome elastolytique annulaire a cellules geantes est une dermatose granulomateuse rare. Elle est caracterisee histologiquement par la phagocytose des fibres elastiques par des cellules multinucleees. Nous rapportons l’evolution favorable d’un cas apres PUVAtherapie et traitement par antipaludeens de synthese. Observation Un homme de 67 ans, ancien viticulteur, avait des lesions annulaires a bordures surelevees des epaules et du tronc, tres prurigineuses, evoluant depuis plusieurs mois. L’examen histologique d’une biopsie de la bordure erythemateuse etait caracteristique d’un granulome elastolytique annulaire a cellules geantes. Differents traitements topiques s’etaient averes inefficaces et une corticotherapie generale avait attenue le prurit mais sans effet sur les lesions cutanees. Une PUVAtherapie entrainait une regression des lesions du tronc, mais l’eruption s’etendait sur les bras et se couvrait de microkystes epidermiques. La PUVAtherapie etait arretee et un traitement par antipaludeens de synthese (hydroxychloroquine 400 mg/j) etait entrepris, suivi d’une regression complete des lesions. Discussion Le granulome annulaire elastolytique a cellules geantes a ete isole en 1979 par Hanke et al. a propos de cinq cas feminins. Il s’agit d’une dermatose rare dont une trentaine de cas ont ete rapportes dans la litterature anglo-saxonne. L’aspect clinique est assez evocateur, avec des lesions erythemato-papuleuses isolees ou groupees en anneaux a bordures surelevees et a centre plus clair d’evolution atrophique. Les lesions predominent souvent sur les zones photoexposees. L’aspect histologique est caracteristique, avec une image de granulome elastophagique a cellules geantes qui donne son nom a cette affection. Cet aspect permet de le differencier d’autres maladies granulomateuses. L’etiologie est inconnue et le traitement empirique. L’evolution spontanee vers la guerison est possible et aucun traitement n’a obtenu des resultats constants. Dans notre cas, la PUVAtherapie a obtenu un resultat partiel et les antipaludeens de synthese une regression complete des lesions residuelles.

Resume Introduction Les leishmanioses comprennent trois formes bien individualisees dues a des especes differentes dont la distribution geographique est connue. Nous rapportons le premier cas de leishmaniose cutanee a Leishmania major avec une atteinte de la moelle osseuse chez un malade infecte par le VIH au Burkina Faso. Observation Un homme de 38 ans seropositif pour le VIH avait des lesions papulonodulaires generalisees, cuivrees, indolores, infiltrees, prurigineuses, evoluant depuis dix mois. L’examen histologique d’une biopsie cutanee etait en faveur du diagnostic de leishmaniose cutanee diffuse. Une ponction sternale de la moelle osseuse decelait de nombreuses leishmanies. La culture etait positive et l’identification montrait L. major . Le malade recevait une tritherapie antiretrovirale et de l’antimoniate de meglumine. L’evolution etait faite d’une alternance de poussees et de remissions espacees de trois semaines en moyenne. Discussion L. major est l’espece de leishmanie qui a ete identifiee au Burkina Faso. Elle est responsable de formes cutanees pures. Cependant, avec l’immunodepression induite par l’infection a VIH, des formes cliniques particulieres ont ete decrites, notamment a type de leishmaniose cutanee diffuse. L’atteinte de la moelle osseuse par L. major temoigne de la possibilite d’atteinte viscerale de la leishmaniose due a une espece consideree comme purement dermotrope. Un risque de multiplication des cas de leishmaniose viscerale a L. major dans le cadre de la pandemie VIH ne peut donc pas etre ecarte et justifie une vigilance particuliere.

Resume Introduction Dans les naevus naevocellulaires les cellules naeviques peuvent etre associees a d’autres tissus, cellules ou lesions. Dans de rares cas, une hyperplasie intranaevique des fibres elastiques peut leur etre associee. Observations et resultats Dans une serie de 11 naevus, composes ou intradermiques, dont un naevus bleu, nous avons observe une hyperplasie des fibres elastiques, circonscrite au naevus, sans anomalie des fibres elastiques perinaeviques. Ces fibres etaient plus epaisses que les fibres elastiques normales du derme reticulaire et contractaient des rapports etroits avec les cellules naeviques. L’hyperplasie observee n’avait aucune traduction clinique. Elle etait sans rapport avec l’âge, le sexe et la localisation du naevus. Elle etait presente dans 0,1 p. 100 des naevus excises. Discussion Cette hyperplasie elastique intranaevique est une curiosite a classer dans les lesions occasionnellement jumelees aux naevus, au meme titre que certaines formations vasculaires, nerveuses, epitheliales ou conjonctives. Elle est a ajouter a la liste des « naevus jumeles ».

Resume Introduction Le pyoderma granulomateux superficiel est une forme particuliere de pyoderma gangrenosum, qui est souvent diagnostiquee tardivement en raison de son tableau clinico-histologique particulier. Nous rapportons un nouveau cas de cette maladie rare. Observation Un homme de 67 ans avait des lesions inflammatoires puis ulcerees des jambes evoluant depuis 4 mois, malgre une antibiotherapie prolongee. L’aspect histologique etait initialement celui d’un granulome palissadique evoquant un granulome annulaire, puis d’un granulome necrosant compatible avec une mycobacteriose. Les differents examens complementaires permettaient d’eliminer une etiologie infectieuse ou une maladie systemique. Devant l’aggravation clinique avec ulceration extensive et des douleurs intolerables, une nouvelle biopsie montrait une dermatose neutrophilique, accompagnee de necrose dermique et d’un granulome. L’ensemble des donnees permettait le diagnostic de pyoderma granulomateux superficiel. Les lesions guerissaient apres 3 mois de corticotherapie generale a la dose de 1 mg/kg/j. Discussion Le pyoderma granulomateux superficiel a un aspect clinique proche de celui du pyoderma gangrenosum classique, mais sur le plan histologique il est plus superficiel et s’associe a des granulomes dermiques. Ces granulomes sont inhabituels dans les dermatoses neutrophiliques, evoquant plutot une maladie infectieuse. Ceci explique les erreurs frequentes de diagnostic, avec des traitements anti-infectieux inappropries et surtout des exereses chirurgicales inutiles.

Resume Introduction La survenue spontanee et recurrente de bulles hemorragiques a la face inferieure des seins est une presentation clinique inhabituelle de lymphangiectasies acquises. Observations Chez 2 femmes, âgees de 64 et de 85 ans, sont apparues de facon recurrente pendant plusieurs mois des bulles hemorragiques des regions sous-mammaires. L’examen histologique, immunohistochimique et ultrastructural montrait que les dehiscences bulleuses intradermiques etaient en rapport avec des lymphangiectasies du derme superficiel, paralleles a l’epiderme. Leur dilatation extreme et leur rupture paraissaient etre la cause de l’aspect inflammatoire et hemorragique des lesions, qui n’ont pas recidive apres, respectivement, 1 et 3 ans de suivi. Discussion La presence de lymphangiectasies sous-papillaires parait etre la cause de decollements bulleux intradermiques recidivants. La cause de ces lymphangiectasies hemorragiques, leur localisation strictement sous-mammaire, l’evolution spontanement resolutive des symptomes, apres plusieurs poussees, restent inexpliquees. La presentation inhabituelle des lesions observees chez ces 2 femmes est a verser au diagnostic differentiel des bulles dermolytiques acquises.

Hard keratins are expressed in normal hair and nails, and are characterized by a higher cysteine content than cytokeratins. Previous studies have suggested a coexpression of hard keratins and cytokeratins in pilomatrixoma, a benign follicular tumour which could originate from the hair matrix. Human hair keratin basic 1 (hHb1) is a newly characterized hair keratin which is expressed specifically by cortical cells of the normal hair shaft. A preliminary study has suggested that hHb1 could be expressed in pilomatrixoma. In order to confirm this hypothesis, we have studied a series of 128 pilomatrixomas by in situ hybridization, using a 35S-labelled hHb1-specific probe. The anti-sense probe was used as a negative control. Among these pilomatrixomas, six were early cases, 60 were classified into the intermediate stage (either fully developed or early regressive cases) and 62 were late regressive tumours made of shadow cells only. Forty-seven tumours showed hHb1 expression (37%), all being intermediate stage pilomatrixomas. The areas positively stained by the probe were band-like structures made of transitional cells only, which were very close to cells showing tricholemmal keratinization features. Neither the basophilic matrix cells nor the shadow cells expressed hHb1. Our results suggest that pilomatrixomas can differentiate towards cortical cells during their maturation process, as this keratin is specifically expressed in the cortex of the normal hair shaft. These data are consistent with previous studies which showed the expression of a hard keratin group in transitional cells by immunohistochemistry. The histogenesis of basophilic cells of pilomatrixoma is controversial, but it is likely that transitional cells represent an equivalent of the hair cortex.

Many nail abnormalities have traditionally been described in association with rheumatoid arthritis (RA), but their specificity has never been assessed in a controlled study. Our purpose was to evaluate the frequency and the specificity of nail changes associated with RA in a case-controlled study including 50 patients suffering from RA and 50 controls. For each patient, a general skin examination was performed and the 20 nails were examined. The nail features were noted and classified. A chi 2 test or a Fisher test was used to compare the two groups. The only nail abnormalities significantly associated with RA were longitudinal ridging on nine or 10 finger nails (29 patients in the RA group vs. three in the controls, chi 2: P < 0.001) and clubbing on at least one nail (24 patients vs. 10, chi 2: P < 0.01). Other nail changes were noticed but were not frequent enough to be significant. The presence of longitudinal ridging on the finger nails was significantly associated with RA.

Sarcoidosis or granulomatous reactions have rarely been reported in association with malignant melanoma (MM). We describe seven patients who presented with both granulomatous disease and MM, and discuss the physiopathological and prognostic significance of this association. In three patients, the granulomatosis was diagnosed as true sarcoidosis and in one patient, as tumour-associated granuloma. In three cases, designated here as atypical tumour-associated granulomatoses, the presence of clear-cut pulmonary granulomatous nodules was typical neither for sarcoidosis nor for tumour-associated granuloma and was highly suggestive of melanoma metastases. Mediastinal lymphadenopathy was present in every patient. In all seven patients, the question of mediastinal or pulmonary involvement or relapse of the MM was raised, but could be confirmed in only one patient. MM can be associated with granulomatous disease. Knowledge of this association has implications in the management of patients with MM.

In 1995, the French Societies of Dermatology, Plastic Surgery, and Cancer organised a consensus conference on the follow-up of patients who had received surgery for stage I melanoma. The following guidelines were approved: paraclinical tests are of limited value for detecting metastases during the patient's initial staging, but chest radiography and liver sonography can be performed. During follow-up, physical examinations should be scheduled at fixed intervals—determined according to tumour thickness—to detect any locoregional recurrence and/or a second melanoma early. Medical imaging technologies should not be pursued without clinical suspicion of metastases. In all cases, patients should be taught self-examination and exhorted to practice it routinely. No adjuvant therapy (lymph-node dissection, chemotherapy, immunotherapy, radiotherapy) has proven effective. To detect familial melanomas, it is recommended that the parents, children, brothers and sisters of patients suffering from sporadic melanoma undergo a complete skin examination that should be repeated systematically in high-risk families.

Reactive perforating collagenosis (RPC) belongs to the group of perforating dermatoses, which comprises elastosis perforans serpiginosa, RPC, perforating folliculitis and Kyrle's disease. RPC was initially described as a distinctive form of transepithelial elimination of altered collagen related to superficial trauma. Two types are distinguished: a hereditary type (MIM 216700), which is rare and begins during early childhood, and a second type, called acquired RPC, which is more frequent, appears in adults and is associated with other diseases, diabetes mellitus, renal insufficiency, solid tumors, lymphomas and AIDS. We report the case of a young man whose illness began during infancy, militating in favor of a diagnosis of a hereditary form of RPC. The description of similar lesions in the patient's brother confirmed our diagnosis.A 26-year-old man, the child of consanguinous parents, presented crusted papular lesions on his hands. The cutaneous lesions, located on the external side of the limbs, had been present since childhood, with flares during winter. Histologic analysis showed a cup-shaped depression in the epidermis containing keratinous material with extruded degenerated collagen towards the cutaneous surface. Treatment with topic retinoids did not result in any real resolution of the disease. The patient reported the presence of similar lesions in his brother, which was consistent with our diagnosis.The pathogenesis of hereditary RPC is still unknown, even if superficial trauma is suspected as the cause of RPC. In contrast, in diabetes, acquired RPC pathogenesis has recently been related to advanced glycation end-products of collagen.

An illustrative case report and a series of 18 well-documented cases of lichen striatus are presented. The mean age at diagnosis was 3 years (6 months to 14 years; median 2 years). The lesions were predominantly distributed on the trunk in 33% of cases and on the limbs in the remaining two thirds (upper limb: 48%; lower limb: 19%). Pruritus was noted in only 1 of 18 cases. Six cases were associated with clinical features of atopy and/or minor signs of atopic dermatitis (e.g., pityriasis alba). Two cases were considered to be clinically associated with lesions consistent with psoriasis. The mean duration was 9.5 months (4 weeks to 3 years; median 6 months). In one patient, two relapses occurred in 4 years. Hypochromic sequelae were noted in 50% of cases. Lichen striatus is the most common acquired self-limited linear eruption in childhood that follows Blaschko's lines. A new acronym is proposed to emphasize the developmental background of the disease: BLAISE for Blaschko linear acquired inflammatory skin eruption.

The epidermodysplasia verruciformis is a rare, autosomic, recessive, genodermatose characterized by a chronic, disseminated, cutaneous infection with human papillomavirus. The majority of these patients have a genetic or acquired immunodeficiency.This retrospective study was conducted on the records of all patients who presented in our dermatology department with an epidermodysplasia verruciformis in a 13 years and 6 months period, from January 1st, 1992 to June 30th, 2005.We have collected 45 cases of epidermodysplasia verruciformis. They were aged from 3 to 57 years, with a mean of 24.6 years. The most concerned age bracket was that from zero to 9 years. They were 29 women (64.4%) and 16 men (35.6%). The eruption presented as papules of 2 to 3 mm size, associated with hypochromic, finely squamous macules with the same size. We noted three cases of itching. We found 37.7% of family cases. We observed 14 cases of HIV positive patients and one case of cancer.This study confirmed that the epidermodysplasia verruciformis was rare. Genetic factors or immunodeficiency would support the appearance of the disease.

Annular elastolytic giant cell granuloma (AEGCG) is a rare form of granulomatous dermatosis. It is characterised histologically by phagocytosis of elastic fibres by multinucleated cells. We report a favourable outcome in a case of AEGCG following PUVA therapy and treatment with synthetic antimalarials.A 67-year-old retired wine grower presented with highly pruritic annular lesions with raised borders on the shoulders and trunk that had been present for several months. Histological examination of a biopsy sample from the erythematous border was characteristic of AEGCG. Various topical treatments proved ineffective and systemic corticosteroids attenuated the patient's pruritus but had no effect on the skin lesions. PUVA therapy resulted in regression of lesions on the trunk, but the rash spread to the patient's arms and was covered with epidermal microcysts. PUVA therapy was discontinued and treatment with a synthetic antimalarial (hydroxychloroquine 400mg/d) was initiated, resulting in complete regression of the lesions.AEGC was isolated in 1979 by Hanke et al. on the basis of five cases seen in females. This is a rare form of dermatosis with some 30 cases being reported in the English literature. The clinical aspect is fairly evocative, with erythematous papular lesions, either alone or in groups, with a raised border and a lighter centre tending towards atrophy. In most cases, the lesions are found predominantly in areas exposed to sunlight. The histological appearance is characteristic, with an image of giant cell elastophagic granuloma from which the name of the disease is taken. This appearance allows the disease to be differentiated from a number of other granulomatous diseases. The aetiology is unknown and treatment is empirical. Spontaneous cure can occur and consistent results have not been obtained with any treatments. In our case, PUVA was partly successful, and the synthetic antimalarials resulted in complete regression of residual lesions.

Leishmaniasis covers three well-individualized clinical variants, each due to individual species found in different geographic areas. Herein we report the first case of cutaneous leishmaniasis due to Leishmania major involving bone marrow in an AIDS patient in Burkina Faso.A 38-year-old HIV-positive man presented with generalized, copper-coloured, painless, infiltrated, itching, papulonodular lesions present over the previous 10 months. Skin biopsy confirmed the diagnosis of diffuse cutaneous leishmaniasis. The bone-marrow smear showed numerous leishmania. The culture was positive and L. major was identified. The patient was being treated with antiretroviral medication and a pentavalent antimonial compound. The disease progression consisted of attacks and remissions separated by an average of three weeks.L. major is the Leishmania species identified in Burkina Faso. It is responsible for typical cutaneous leishmaniasis but particular clinical forms have been described in immunodeficient patients, especially with diffuse cutaneous involvement. The spread of L. major infection to bone marrow could represent a public health problem in our country, where the HIV epidemic is still not under control, and particular vigilance is thus called for.

Striated muscle hamartoma is a rare entity which was described only recently. It is a congenital malformation characterized by the presence of striated, mature muscle fibers in the reticular dermis and hypodermis. We describe a striated muscle hamartoma of the perianal region in a female infant associated with an hemangioma in the same area that regressed completely. Including the present patient, only 14 cases of striated muscle hamartomas have been reported, but this association with an hemangioma is unique. The lesion was polypoid and histopathologic examination showed numerous striated muscle fibers located within the dermis.

In common intradermal or compound nevi, nevocytes may be associated with other kinds of cells, tissues or lesions. In rare cases, they are associated with notable hyperplasia of the elastic fibers. CASE-REPORTS AND RESULTS: In 11 nevi (10 intradermal or compound nevi and 1 blue nevus), we observed striking hyperplasia of the elastic fibers strictly limited to the nevi, with normal aspect of the elastic fibers in the surrounding reticular dermis. These fibers were thicker than normal elastic fibers and showed tight connexions with nevus cells. This hyperplasia was not clinically relevant. It was correlated neither with the age and sex of patients nor with the site of the excised nevi. It was seen in 0.1% of excised nevi.Hyperplasia of elastic fibers in some common nevi is a curiosity which may be classified together with other vascular, nervous, epithelial or connective twin lesions occasionally associated with the nevocytic nevi. It has to be added to the list of so-called "twin nevi".

Cutaneous actinomycosis of the buttocks is a rare granulomatous bacterial infection that usually starts in the perianal area. We present an exceptional case in the form of a pseudo-tumor.A 69 year-old woman, in general good health, developed an indurate mass on the supra-external quadrant of the right buttock. The tumor was centered by an ulcerated nodule with a diameter of around 10 centimeters. Imaging showed invasion of the soft tissue of the skin in the internal psoas muscle, the adipose tonality of which was compatible with a liposarcoma. The skin biopsy revealed characteristic bacterial grain in the center of a cholesterol granuloma. Subsequent culture in aerobic milieu identified Actinomyces gerencseriae. Cure was obtained following complete exeresis of the fibrous tissue and 8 months of antibiotic amoxicillin-clavulanic acid therapy.Other than the most unusual clinical aspect, the originality of this case of actinomycosis of the buttocks is based on its potential appendix origin, 4 years after acute appendicitis, with slow posterior fistulation. Other cases of actinomycosis of appendix origin have been reported and its delayed onset following the intervention has been documented. The pseudo-sarcomatous aspect was responsible for diagnostic wandering. The histological image and, subsequently, the results of the bacteriological culture confirmed the diagnosis.

Histoplasma capsulatum var capsulatum is a dimorphic fungi predominating on the American continent. It is responsible for disseminated histoplasmosis associated with AIDS. The presentation in the form of cutaneous ulceration is uncommon and misleading.A 25 year-old man presented with 3 ulcerations, of 2 to 4 cm in diameter, localized on the lower lip and knees. The patient exhibited fever, alteration in his general status of health and a pulmonary interstitial syndrome. He was seropositive for the human immunodeficiency syndrome (HIV). His lymphocyte CD4+ level was of 1/mm3. Diagnosis of histoplasmosis was established by direct examination and culture of the cutaneous ulcerations and bronchoalveolar washing fluid.The clinical aspect of cutaneous localizations of disseminated histoplasmosis is usually multiple, disseminated, papular or nodular-type lesions. Ulcerations represent less than 20% of the cases described. In our patient, the aspect of the lesions at first evoked cutaneous leishmaniosis. Direct mycological examination followed by culture confirmed the final diagnosis.

Granuloma have already been described in the context of immunosuppression. We report six cases of widespread granuloma annulare occurring in patients with drug-induced immunosuppression.There were four women and two men, with mean age of 61 years (35-76). Three patients were treated with chemotherapy for breast (2 cases) and liver cancer. One woman was given chemotherapy for Hodgkin's disease. One had undergone liver transplantation and was given cyclosporine, another was treated with systemic steroids for polyarthritis. All had generalized non-photoexposed granuloma annulare, composed of multiple erythematous papules, sometimes with annular pattern. The lesions were localized on the trunk, legs and arms. Histopathology revealed granuloma annulare in each patient.These six cases suggest a relationship between drug-induced immunodeficiency and generalized granuloma annulare. The immune dysregulation induced by the drugs may have been responsible for the formation of granuloma annulare.

Superficial granulomatous pyoderma is a rare variant of pyoderma gangrenosum, which is often diagnosed at a late stage because of misleading clinical-histopathological features. We report a new case of this rare disease.A 67 year-old man presented with inflammatory lesions that had become ulcerated over the past 4 months despite prolonged antibiotic therapy. The histopathological aspect was initially suggestive of palisading granuloma annulare and subsequently a necrotizing granuloma compatible with a mycobacteriosis. The various supplementary examinations eliminated the possibility of an infectious or systemic disease. The lesions became painful and ulcerated. A second biopsy showed a neutrophilic dermatosis with dermal necrosis and a granuloma leading to the diagnosis of superficial granulomatous pyoderma. The lesions healed after 3 months of systemic corticosteroids at the dose of 1 mg/kg/day.The clinical features of superficial granulomatous pyoderma are similar to those of classical pyoderma gangrenosum. However, its histological profile is more superficial and is associated with dermal granulomas. The latter are unusual in neutrophilic dermatosis and would suggest an infectious disease. This explains the frequent diagnostic errors, with inappropriate anti-infectious treatments and notably unnecessary surgical exeresis.

Familial cylindromatosis: we report a daughter with turban tumor and her mother with cylindromatosis. The dermal eccrine cylindroma arose as small, solitary lesions on the head of the mother when she was 28 years old. The following years other tumors became apparent. She was operated on several times. The first lesions appeared on the frontal part of the scalp of the daughter when she was 23 years old. Other tumors grew on the scalp. Histopathological examination of the excised tumors showed the same lesions in both the mother and the daughter: dermal eccrine cylindromata. Family history showed that the daughter's maternal aunt had a few tumors. Dermal eccrine cylindroma should be differentiated from malignant syndromes such as basal naevoid carcinoma or metastases and from neurofibromata. The gene of familial cylindromatosis was localised to chromosome 16q12-q13 and it was proposed that this gene is a tumor supressor gene.

Non T non B CD4+ CD56+ leukemia is often revealed by cutaneous lesions. We report 2 patients with this disorder who had characteristic anatomoclinical findings.An 81 year-old female and a 75 year-old man presented with erythematous macules which increased in number and progressed to infiltrated plaques and nodules. The lesions became ecchymotic, but the patients remained in good general condition and blood count as well as bone marrow examination were unremarkable. A cutaneous biopsy revealed a lymphomatous mononuclear cell infiltrate. The cells expressed CD4 and CD56, but not CD3. The cutaneous lesions preceded for 10 and 9 months respectively the appearance of overt leukemia and medullar involvement. At this stage, the patients deceased rapidly from their leukemia.This is an original anatomoclinical syndrome. The histopathologist must pay attention to the unusual CD4+ and CD3- immunophenotype and search for CD56 expression. The malignant cell responsible for this type of leukemia is now individualized and corresponds to a type II dendritic cell precursor.

Spontaneous occurrence of hemorrhagic blisters on the lower side of the breasts is an unusual clinical presentation of acquired lymphangiectasia.A 64 Year-old and a 85 Year-old woman had developed recurrent bleeding blisters in the sub-mammary region for several Months. Histological, immunohistochemical and electron microscopic examination revealed a subpapillary dermal bullous dehiscence, parallel to the epidermis, which was connected to lymphangiectasias of the superficial dermis. Their extreme dilatation and rupture were probably responsible for the clinical inflammatory and bleeding aspect of the lesions, which have not recurred after 1 and 3 Years, respectively.The presence of subpapillary inflammatory lymphangiectasias might be responsible for recurrent dermal blister formation. The reason for the presence of these hemorrhagic lymphangiectasias, restricted to the sub-mammary location and their spontaneous regression after several flare-ups, remain unclear. The unusual clinical presentation of the lesions observed in these two women constitutes a differential diagnosis of acquired dermolytic blisters.

Increased level of homocysteine has been shown to be associated with atherosclerotic disease and venous thrombosis. There are only exceptional reports of cutaneous disease due hyperhomocysteinemia.A 71-year-old man presented with an acral purpura mainly located under the nail plates and resulting in onycholysis. Histologic examination of a skin biopsy specimen showed thromboses of dermal vessels without vasculitis. Laboratory tests revealed highly elevated homocysteinemia due to a mutation in the methylene-tetra-hydrofolate-reductase gene. No other cause of purpura or thrombophilia was found. When oral folic acid was given, both homocysteinemia and cutaneous lesions were controlled. However, a biological and clinical recurrence occurred when therapy was discontinued.Hyperhomocysteinemia has probably a causal role in this original case of acral purpura. Since treatment is effective, the detection of hyperhomocysteinemia should be proposed in patients with cutaneous lesions secondary to distal vascular thromboses.

Malignant blue nevus is a very rare tumor. Argyrophilic nucleolar organizer regions (AgNORs) have been reported to be both a diagnostic and prognostic clue in various tumors, especially if standardized using an image analysis systems.Seven cases of malignant blue nevus were retrospectively recorded between 1974 and 1999, and their clinical and pathological features described. Using an image analysis system, AgNOR measurements were studied in all cases. These results were compared with those obtained in 10 cases of commun blue nevus, 10 cases of cellular blue nevus and 10 cases of malignant melanoma.The most frequent location of malignant blue nevus was the scalp. Clinically, the tumor generally consisted of a blue nodule, 2.5 cm in diameter. There was no single histopathological criterion for the diagnosis of malignant blue nevus. AgNOR measurement was significantly higher in malignant blue nevus in comparison with commun blue nevus (p0.0004) or cellular blue nevus (p0.012), whereas there was no difference between malignant blue naevus and malignant melanoma (p0.50).Our results confirm the severe prognosis of malignant blue nevus and highlight the necessity of removing all blue tumours located on the scalp. AgNOR measurement using an image analysis system appears to be a useful tool for the diagnosis of malignant blue nevus, but further studies remain necessary.

Pachydermodactyly is an acquired, asymptomatic swelling of the lateral sides of the fingers and is seen mainly in young men. The formal pathogenesis is a benign fibromatosis. The casual pathogenesis was until now unclear, although we believe, that exogenic factors are the main reason. Consequently Pachydermodactyly has to be considered as a result of compulsion neurosis.