[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.

Trichoblastic carcinoma of the pinna. A rare case.

Adnexal tumors of the hair follicle can be regarded as lesions that show similar differentiation to one or more portions of the hair follicle. Trichoblastic carcinoma is a rare malignant adnexal tumor, which usually occurs on the scalp. There have been reported cases with regional lymph node metastasis. We report a case of a 65 years old man with a painless irregular mass of the posterior surface of the right pinna, which was slowly growing over a 5-year period. He had a history of a similar lesion on the same site, which was removed 8 years before he presented to us. We excised the lesion and the defect was covered with an advancing flap. Because of the histology result the patient was re-operated and reviewed for over 3 years and there wasn't any sign of recurrence.

[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.

[Superficial granulomatous pyoderma]. / Pyoderma granulomateux superficiel.

INTRODUCTION: 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. CASE REPORT: 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. DISCUSSION: 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.

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.

[Primary cutaneous non T non B CD4+ CD56+ leukemia (2 cases): an original anatomoclinical syndrome]. / Leucémie aleucémique non T non B CD4+ CD56+ (2 cas). Une entité anatomoclinique originale.

INTRODUCTION: 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. CASE REPORTS: 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. DISCUSSION: 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.