[Reactive perforating collagenosis]. / Collagénose perforante réactionnelle.

BACKGROUND: 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. PATIENTS AND METHODS: 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. DISCUSSION: 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.

[Annular elastolytic giant cell granuloma]. / Granulome élastolytique annulaire à cellules géantes.

BACKGROUND: 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. PATIENTS AND METHODS: 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. DISCUSSION: 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.

[Cutaneous leishmaniasis due to Leishmania major involving the bone marrow in an AIDS patient in Burkina Faso]. / Leishmaniose cutanée à Leishmania major avec atteinte de la moelle osseuse chez un malade infecté par le VIH au Burkina Faso.

BACKGROUND: 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. CASE REPORT: 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. DISCUSSION: 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.

[Epidermodysplasia verruciformis: clinicaland epidemiological features of 45 cases in the Department of Dermatology at the University Hospital Center of Yalgado Ouedraogo, Ouagadougou]. / Epidermodysplasie verruciforme: aspects epidémiologiques et cliniques de 45 cas dans le service de Dermatologie du Centre Hospitalier Universitaire Yalgado Ouédraogo, Ouagadougou.

INTRODUCTION: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: This study confirmed that the epidermodysplasia verruciformis was rare. Genetic factors or immunodeficiency would support the appearance of the disease.

[Nevocytic nevi associated elastic fibers hyperplasia: a type of "twin nevus"]. / Naevus naevocellulaires avec hyperplasie des fibres élastiques: une forme de naevus jumelé.

BACKGROUND: 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. DISCUSSION: 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".

[Actinomycosis of the buttock]. / Actinomycose fessière.

INTRODUCTION: 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. CASE REPORT: 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. DISCUSSION: 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.

[Disseminated histoplasmosis: an atypical ulcerous form in an HIV-infected patient]. / Histoplasmose disséminée: forme ulcéreuse atypique chez un malade infecté par le VIH.

INTRODUCTION: 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. OBSERVATION: 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. DISCUSSION: 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.

Induction of tolerance to urushiol by epicutaneous application of this hapten on dinitrofluorobenzene-treated skin.

The application of a sensitizing dose of urushiol on a dinitrofluorobenzene (DNFB)-treated skin area significantly diminished the intensity of the urushiol challenge test in guinea pigs. Furthermore, the animals which had been first exposed to urushiol through DNFB-treated skin failed to become sensitized in a second sensitization attempt even when painted on a previously untreated area. This tolerance is hapten-specific and may be reversed by treatment with cyclophosphamide (200 mg/kg) shortly before another contact sensitization attempt to urushiol. In a previous work, we have shown that most of the Langerhans cells present in the DNFB-treated skin area are ATPase-negative and that there exists a link between the membranous ATPase system and the formation of Langerhans cell granules. The latter seem to develop in the course of a mechanism of adsorptive pinocytosis during which ATPase activity "disappears." Thus we suggest that the "unavailability" of ATPase-negative Langerhans cells for adequate processing a second hapten may result from the incapacity of cells lacking their ATPase system to activate the intracellular events that depend on this system and that normally lead to sensitization.

ATPase Langerhans cell staining: a technique allowing progression from light to electron microscope observation.

A technique which enables good visualization of the membranous ATPase activity of epidermal Langerhans cells is described. The method has the advantage of keeping intact most of the ultrastructural details. It may allow the observation, under pathologic conditions, of ultrastructural modifications in ATPase-negative Langerhans cells still recognizable by their Langerhans cell granules.

ATPase and morphologic changes induced by UVB on Langerhans cells in guinea pigs.

We have devised, in guinea pigs, an improved ATPase technique which enables one to proceed from light to electron microscope study while preserving, on the ultrastructural level, the various membranous structures, in particular the Langerhans cell (LC) granules. Using this method, we have been able to confirm the action of acute, low-dose UVB on the surface enzymatic marker, ATPase. Moreover, this study has shown that the ATPase-negative LC contain abnormal LC granules or, more often, are deficient in LC granules. In a previous work, we have shown that, after epicutaneous application of a hapten, one successively observes an extensive adsorptive pinocytosis process, the disappearance of the membranous ATPase system, and the appearance of LC granules in the cytoplasm. Therefore we may suppose that, after UVB irradiation, the disappearance of the ATPase system and/or the possible alteration of the adsorptive pinocytosis process interrupts or alters the formation of LC granules. These successive events might play a vital role in the formation of the hapten--carrier protein-Ia antigen complex. In their absence in a large number of LC, following UV irradiation, epicutaneous application of a hapten would lead to the development of a state of immune tolerance.

ATPase and morphologic changes in Langerhans cells induced by epicutaneous application of a sensitizing dose of DNFB.

We have previously described an ATPase Langerhans cell (LC) staining technique allowing progression from light to electron microscope observation. Using this technique we have studied, following epicutaneous application of a sensitizing dose of a hapten, 2,4-dinitro-1-fluorobenzene (DNFB), the fate of the epidermal LC located in the sensitization zone. We wanted to know, under the light microscope, if the density and/or morphology of the LC are modified by such a treatment and, under the electron microscope, what are the ultrastructural changes accompanying the possible light microscope modifications. Under the light microscope, the observation of LC during the 5 d necessary for the development of contact sensitivity to DNFB shows that their number drops in the course of the first 24 h to normalize again 3 d later. Under the electron microscope, observations over the first 24 h revealed that LC remained in the epidermis, but were ATPase-negative. The disappearance of the membrane ATPase activity took place while the LC presented an increased number of coated pits, coated vesicles, endosomes, and lysosome organelles which characterize, at the ultrastructural level, the process of receptor-mediated endocytosis (RME). Following RME, many Birbeck granules (BG) appeared in the cytoplasm. Thus, epicutaneous application of DNFB leads to an endocytic activation of LC. However, the ligand(s) and/or the cell-surface components, which probably internalize during the RME process, remain unknown.

Human epidermal Langerhans cells internalize by receptor-mediated endocytosis T6 (CD1 "NA1/34") surface antigen. Birbeck granules are involved in the intracellular traffic of the T6 antigen.

Using immunogold staining of a suspension of living human epidermal cells to identify the Langerhans cell membrane-associated antigen T6 (revealed by the monoclonal antibody BL6), we have observed internalization of T6 antigen in Langerhans cells. This phenomenon is at least partly due to receptor-mediated endocytosis involving coated pits, coated vesicles, endosomes, the smooth endoplasmic reticulum, and lysosomes. These ultrastructural results suggest that T6 antigen may be part of a receptor site. Following receptor-mediated endocytosis, the appearance in the cell center of the first labeled Birbeck granules suggests that Birbeck granules could represent T6 intracellular transport organelles carrying T6 from the central part of the cell to an unknown destination.

The Schnitzler syndrome. Four new cases and review of the literature.

The Schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver and/or spleen, elevated erythrocyte sedimentation rate, and leukocytosis. The mean delay to diagnosis is more than 5 years, and this syndrome is of concern to internists and many medical specialists. Patients with this syndrome are often initially considered to have lymphoma or adult-onset Still disease, which are the main differential diagnoses. However, hypocomplementic urticarial vasculitis, systemic lupus erythematosus, cryoglobulinemia, acquired C1 inhibitor deficiency, hyper IgD syndrome, chronic infantile neurologic cutaneous and articular (CINCA) syndrome, and Muckle-Wells syndrome should also be excluded, because diagnosis relies on a combination of clinical and biologic signs and there is no specific marker of the disease. The disease pursues a chronic course, and no remissions have yet been reported. Disabling skin rash, fever, and musculoskeletal involvement are the most frequent complications. Severe anemia of chronic disease is another serious complication. The most harmful complication, however, is evolution to an authentic lymphoplasmacytic malignancy, which occurs in at least 15% of patients. This hematologic transformation can occur more than 20 years after the first signs of the disease, thus patients deserve long-term follow-up. Treatment is symptomatic and unsatisfactory. The skin rash is unresponsive to treatment, and nonsteroidal antiinflammatory drugs, antihistamines, dapsone, colchicine, and psoralens and ultraviolet A (PUVA) therapy give inconstant results. Fever, arthralgia, and bone pain often respond to nonsteroidal antiinflammatory drugs. In some patients, these symptoms and/or the presence of severe inflammatory anemia require steroids and/or immunosuppressive treatment, which ameliorate inflammatory symptoms but do not change the course of the skin rash.

Epithelial sheath neuroma: a new entity.

The authors describe four examples of a peculiar cutaneous lesion characterized histopathologically by a proliferation of enlarged nerve fibers ensheathed by squamous epithelium involving the superficial dermis. The perineural epithelial sheaths were composed of uniform squamous epithelium with evidence of cornification in the form of dyskeratotic cells or resulting in orthokeratotic basket-weave corneocytes. Immunohistochemical studies confirmed the epithelial and neural nature of the two components of the lesions, with the nerve fibers expressing immunoreactivity for S-100 protein, neurofilaments, CD57, and nerve growth factor receptor, whereas the perineural epithelial sheaths showed immunoreactivity for cytokeratins. The authors propose the term "epithelial sheath neuroma" for this lesion and believe that it is a distinct and a previously undescribed benign neoplasm of both cutaneous nerves and epithelial elements.

Acquired blaschkolinear dermatoses.

Congenital and/or nevoid skin disorders following the lines of Blaschko may have a delayed onset after birth. They have to be differentiated from acquired dermatoses exhibiting the same linear pattern. In common dermatoses, such as psoriasis or lichen planus, lesions in a blaschkolinear distribution most often occur together with scattered lesions, but occasionally they may be isolated. Less common self-limited dermatoses such as lichen striatus and adult blaschkitis always present in a blaschkolinear fashion. In these diseases, or some other conditions occasionally distributed along these lines (chronic graft versus host reaction, fixed drug eruption, lupus erythematosus, atopic dermatitis, etc.), the cause of the disease may lead to the unmasking of tolerance to an abnormal keratinocyte clone that remained hidden in these lines. In addition to epithelial cells, other cells may be involved in the occurrence of acquired blaschkolinear dermatoses. In linear atrophoderma and linear fibromatosis, the histogenesis seems to involve hypothetic dermal clones. The extension of an acquired dermatosis on a preexisting linear nevoid disorder is an argument in favor of an early embryonic somatic mutation of a skin cell line.

Combination chemotherapy of dacarbazine and fotemustine in disseminated malignant melanoma. Experience of the French Study Group.

A total of 70 patients presenting with a disseminated malignant melanoma were entered into a multicentric study of combination chemotherapy using dacarbazine and fotemustine. In all, 63 patients were evaluable, 31.8% of whom had previously received cytotoxic chemotherapy. The protocol consisted of induction treatment with a weekly infusion of 100 mg/m2 fotemustine on days 1 and 8 and a daily infusion of 250 mg/m2 dacarbazine on days 15/18 followed by a 4- to 5-week rest period. Responding and stabilized patients were given maintenance treatment comprising fotemustine (100 mg/m2, day 1) and dacarbazine (250 mg/m2, days 2/5) every 3 weeks. The response rate was 33.3% (9 complete responses (CRs) and 12 partial responses (PRs)) and was outstanding among pretreated patients (34.9%). Responses were also documented in cerebral (28.6%), visceral (23.1%) and nonvisceral (43.3%) metastatic sites. Toxicity was mainly hematologic (22.2%, grade III/IV leukopenia; 20.3%, grade III/IV thrombocytopenia) and was acceptable. These results are encouraging in terms of the antitumor activity against nonvisceral metastases (43.3%) and the percentage of CRs obtained (23.3%), and they confirm the activity of fotemustine in cerebral metastatic sites.

Allergic contact dermatitis caused by skin painting (pseudotattooing) with black henna, a mixture of henna and p-phenylenediamine and its derivatives.

BACKGROUND: Skin painting (pseudotattooing) with henna is traditionally performed mainly in Muslim or Hindu persons. Recently, transient artists have begun using black henna mixtures to temporarily paint the skin. Emergence of allergic contact dermatitis after application indicates the presence of a skin sensitizer in such preparations and poses future risks. OBSERVATIONS: Four patients developed allergic contact dermatitis after skin painting with black henna performed in France, Egypt, and the United States. The delay of symptoms suggested previous sensitization in 1 patient and active sensitization in 3 patients. Of 3 patients who underwent patch testing, the results were positive for p-phenylenediamine in 3 patients and for p-toluylenediamine in 1 patient. These sensitizers are found in hair dye preparations. CONCLUSIONS: The mixtures used by the artists possibly contained natural henna, a rare and weak skin sensitizer, and likely contained chemical coloring agents, diaminobenzenes, such as p-phenylenediamine and/or diaminotoluenes. The long duration of skin contact, the high concentrations of sensitizing materials, and the lack of a neutralizing agent dramatically increase the risk of skin sensitization, which is why such substances are prohibited for direct skin application. Because of the worldwide vogue of skin painting, future cases of sensitization to p-phenylenediamine and diaminobenzenes or diaminotoluenes are expected.

Nail changes in patients infected with human immunodeficiency virus. A prospective controlled study.

OBJECTIVES: To study the frequency of nail changes in a population of human immunodeficiency virus (HIV)-infected patients and to evaluate the specificity of these findings by comparison with HIV-negative control subjects. DESIGN: Prospective controlled study. Nail changes were recorded by a standardized clinical examination (curvature, nail plate, color, onychomycosis). In case of clinical diagnosis of onychomycosis, mycological culture was performed. SETTING: Primary care university hospital. PATIENTS: A total of 155 HIV-1-positive patients and 103 healthy HIV-negative control subjects of comparable age and sex ratio. INTERVENTION: None. MAIN OUTCOME MEASURE: Clinical examination findings. RESULTS: Nail symptoms were present in 67.7% of HIV-positive patients vs 34.0% of controls (P << .001). The following symptoms were significantly more frequent in the HIV group: clubbing (5.8%) (P < .05), transverse lines (7.1%) (P < .01), onychoschizia (7.1%) (P < .05), leukonychia (14.3%) (P < .001), and longitudinal melanonychia (14.8%) (P < .01). The main finding was onychomycosis in 30.3% of patients vs 12.6% of controls (P < .001). Trichophyton rubrum was present in 48% of onychomycoses and unusual Candida species were also recorded. Multiple fungi were frequently cultured in a single patient. The mean CD4+ cell count was lower in patients with onychomycosis and the frequency of onychomycosis increased in advanced stages of HIV disease. Acquired total leukonychia of the 20 nails was present in 4% of patients. CONCLUSION: Nail symptoms are much more frequent in patients with HIV than in healthy controls, and some of them could be linked to the level of immunosuppression.