[Juvenile hyaline fibromatosis]. / Fibromatose hyaline juvénile.

BACKGROUND: Juvenile hyaline fibromatosis and infantile systemic hyalinosis are two rare autosomal recessive diseases arising from mutation in the capillary morphogenesis factor-2 gene. They are characterized by accumulation of hyaline material, in the skin in the first instance and in other organs in the second. We describe a case of juvenile hyaline fibromatosis. CASE REPORT: A 2-year-old girl presented gingival hyperplasia, skin papules, subcutaneous nodules and joints and bones lesion. A diagnosis of juvenile hyaline fibromatosis was suggested and this was confirmed by histopathology and genetic analyses. The patient presented frequent episodes of diarrhoea, which is evocative of infantile systemic hyalinosis. DISCUSSION: This case clearly illustrates the wide phenotypic range of juvenile hyaline fibromatosis. Diagnosis must be made as soon as possible to avoid cosmetic and functional handicap.

[Cerium-induced granulomatous dermatitis]. / Granulomatose cutanée au cérium.

BACKGROUND: The presence of inorganic foreign bodies in granulomatous cutaneous lesions is not infrequent. In this paper, we describe the first case of cerium-induced cutaneous granulomatous dermatitis. CASE REPORT: A 57-year-old woman seriously burned four years ago presented with papulonodular lesions affecting only the burned areas treated with topical cerium nitrate-silver sulfadiazine cream (Flammacérium). Biopsies revealed sarcoidal granuloma associated with exogenous particles. Electron probe X-ray microanalysis demonstrated a high cerium content. Screening for systemic sarcoidosis was negative. The patient was treated with hydroxychloroquine. After four months of follow-up, clinical and histological evidence of decreased infiltrate was noted. DISCUSSION: Cerium nitrate-silver sulfadiazine cream (Flammacérium) is widely used for the topical treatment of burns. The main effect of cerium is to create superficial calcification, which decreases wound colonization and prevents the formation of granulation tissue (no hypertrophic scar formation) in burns. Prior to our case, no cutaneous side-effects of cerium had been encountered. Inoculation of foreign matter may or may not induce granuloma formation or sarcoidosis in different subjects, according to their immunologic status. The favorable outcome in this case could in fact be due to a change in the pattern of cytokinin production (TH1=> TH2) rather than the effects of hydroxychloroquine therapy.

[Splenic lymphoma with villous lymphocytes revealed by purpura pigmentosa progressiva associated with cryoglobulinemia and chronic hepatitis C infection]. / Lymphome splénique à lymphocytes villeux révélé par un purpura vasculaire lié à une cryoglobulinémie associée à une hépatite C.

BACKGROUND: Splenic lymphoma with villous lymphocytes is occasionally associated with chronic hepatitis C infection. Antiviral hepatitis C therapy has been recently reported to be efficacious against splenic lymphoma with villous lymphocytes. We report a new case revealed by cutaneous symptoms. CASE REPORT: A 53-year-old patient with arthritis and neuropathy of the lower limbs consulted for vascular purpura on both legs. The blood picture showed an increase in villous lymphocytes leading to a diagnosis of splenic lymphoma with villous lymphocytes. Histologic examination of a cutaneous biopsy specimen showed thrombosis of the superficial dermal vessels, associated with cryoglobulinemia with renal and neurologic failure, a satellite of hepatitis C virus infection. The patient was treated with interferon, ribavirin and plasmapheresis. DISCUSSION: Vascular purpura, often associated with cryoglobulinemia, may reveal chronic hepatitis C infection. The efficacy of interferon and ribavirin treatment for splenic lymphoma with villous lymphocytes associated with hepatitis C infection has already been documented, and results in remission of cryoglobulinemia and lymphoma as well a eradication of viral load in 78% patients.

[Dermatological aspects in the risk of biological warfare]. / Aspects dermatologiques du risque biologique provoqué

The possible cutaneous manifestations of infectious biological warfare are multiple and vary depending on the agent used. An ulcerous and/or necrotic syndrome and/or regional lymphadenitis syndrome are possible with anthrax, tularaemia, bubonic plague and emission of trichotecene mycotoxins. A vesiculo-pustular syndrome with fever is provoked by smallpox, melioidosis and glanders. A purpural and/or haemorrhagic syndrome is seen during haemorrhagic fever viruses and septicaemic plague. These cutaneous manifestations are excellent markers that orient and alert when they occur in a context of a situation at risk, when several cases are observed in a usually non-exposed population and with extra-dermatological syndromes. They permit the early initiation of treatment.

[Neutrophilic dermatosis of the hands. A localized subset of Sweet's syndrome: 3 cases]. / Dermatose neutrophilique des mains: une forme régionale du syndrome de Sweet: 3 observations.

INTRODUCTION: The term "pustular vasculitis of the hands" described by Strutton for an acute eruption of the hands was recently revisited. "Neutrophilic dermatosis of the hands" has been preferred. We observed 3 new cases. OBSERVATIONS: Two women and a man, without notable past history, consulted for an acute and painful eruption of both hands with fever. Clinical examination showed erythematous papular or nodular or bullous lesions developed on thenar and hypothenar eminences, and on the finger. Histological findings were predominantly neutrophilic infiltration in the dermis, papillary dermal edema, with or without the presence of leukocytoclastic vasculitis. Diagnosis was Sweet's syndrome. No disease was associated. With indometacine (50-100 mg per day), all the lesions disappeared rapidly without relapse. DISCUSSION: These 3 cases add to 19 other cases (review of literature) which confirm the existence of a homogenous entity that we think it a localized subset of Sweet's Syndrome, with a fast healing. Our observations are specific by the first localization on thenar and hypothenar eminences and by the excellent response to treatment with a non steroidal antiinflammatory agents such indometacine (50-100 mg per day). This treatment could be proposed as a first line therapy.

Erythema dyschromicum perstans: report of a new case and critical review of the literature.

Erythema dyschromicum perstans (EDP), described by Convit et al. in 1961, is a rare dermatosis. Its relationship with ashy dermatosis (AD), described by Ramirez in 1957, is still a matter of debate. We report a typical case of EDP. The patient, of North African origin, had a dyschromic (hypo- and hyperpigmented) eruption on the chest and limbs for 2 years. The lesions were occasionally surrounded by a papular border which spread slowly and centrifugally. Histological examination showed a lichenoid infiltrate. A carcinoma of the lung was simultaneously discovered. No treatment was given, EDP is infrequent and often considered identical to ashy dermatosis in the literature. However, the clinical aspects of the two diseases differ. The main features of these two diseases are reviewed and compared on the basis of a literature review. We conclude that EDP and AD are distinct clinical entities.

[Pityriasis rotunda]. / Pityriasis rotunda.

Pityriasis rotunda (PR) is a well-known skin disease in Japan, but it is seldom observed in Europe. Because it is often associated with an underlying, sometimes malignant disease, it is regarded by some authors as a clinical form of acquired ichthyosis. We report the case of a 20-year old male Congolese presenting with PR and ichthyosis vulgaris. This rarely described association raises the problem, discussed here, of whether PR belongs to ichthyosis vulgaris.

[Infiltrating lipoma]. / Lipome infiltrant.

BACKGROUND: Deep or infiltrating lipoma is an often misdiagnosed clinical entity. We report two typical cases. CASE REPORTS: Two women, aged 92 and 62 years, were seen for a tumefaction on an upper limb with progressively increasing volume. The clinical presentation suggested deep lipoma, confirmed by magnetic resonance imaging and histology which eliminated liposarcoma, the main differential diagnosis. Surgical excision was successful. There has been no recurrence. DISCUSSION: These two observations recall the clinical, diagnostic and therapeutic features of infiltrating lipoma.

[African tick-bite rickettsiosis: the 1st case of autochthonous infection?]. / Rickettsiose à tique africaine: première contamination autochtone?

BACKGROUND: African tick-bite fever is caused by R. africae. All cases reported to date in France have occurred among patients who came from southern Africa, the endemic zone. We report the first case, to our knowledge, of a patient infected in France. CASE REPORT: A 69-year-old white man who had never left France developed fever and 3 "black spots" on the legs with lymphangitis and enlarged nodes. The clinically suspected diagnosis was confirmed by positive serology reactions to R. africae. As cross immunity with R. conorii can occur, the diagnosis was further confirmed by western blot for R. africae and by the persistence of the reaction after adsorption of the R. conorii serum. DISCUSSION: African tick-bite fever was identified as a clinical entity different from Mediterranean spotted fever by Kelly in 1992 who demonstrated the causal role of R. africae. The typical clinical presentation associates fever, several black spots, lymphangitis and multiple node enlargement. The serological diagnosis is difficult owing to cross immunity with R. conorii. Western blot and polymerase chain reaction are required for definitive diagnosis. In our case, the infection was probably due to a R. africae imported into France in the luggage of the patient's daughter who had spent 3 months in Zimbabwe.

[Xanthogranuloma and cutaneous mastocytosis in adults]. / Xanthogranulome de l'adulte et mastocytose cutanée.

Xanthogranuloma (XG) is a rare disease in adults. The authors report a case of XG in an 20 years-old woman. This observation is particularly interesting: First by the pathology of the cutaneous lesion which showed a dermal infiltrate composed of histiocytes and a few foamy cells but without Touton giant cells. Second by is association with a mastocytosis which disappeared when XG appeared. These data led the authors to discuss the misleading histology pattern of XG and also the pathogenesis of XG. In our case, it would appeared as a benign reactive process against mastocytosis.