[Streptococcal impetigo at topical tacrolimus application sites in a woman with atopic dermatitis]. / Impétigo streptococcique apparu sur les sites d'application du tacrolimus chez une jeune atopique.

BACKGROUND: We report a case of staphylococcal impetigo in a girl treated with tacrolimus ointment (Protopic) for atopic dermatitis. OBSERVATION: A 15 year-old girl was receiving treatment with tacrolimus 0.03% (Protopic) for an episode of atopic dermatitis. On reduction of applications of tacrolimus, a vesicular-pustular rash appeared and was treated with pristinamycin and valaciclovir. At the end of antibiotic and antiviral therapy, the vesicular-pustular rash recurred while the goal was receiving treatment once more with tacrolimus ointment 0.1%. The bacteriological and virological skin samples revealed B-haemolytic streptococcus group A. The negative results for cutaneous virological samples ruled out Kaposi-Juliusberg syndrome and a diagnosis of staphylococcal impetigo was made. The intrinsic imputability of tacrolimus was I3 (C3 S2). DISCUSSION: The most obvious specific feature of this impetigo was its limitation to areas of eczema treated by application of tacrolimus. In prospective studies in large patient cohorts, tacrolimus ointment has been associated with two types of adverse effect: local irritations and skin infections chiefly caused by Staphylococcus aureus. To date, there have been no reports in the literature of impetigo due to haemolytic B streptococcus following application of tacrolimus. Because of its immunodepressant effect, tacrolimus ointment may result in increased incidence of skin infections even though a number of studies have shown a reduction in such infections.

[Mouth ulcers induced by enteric-coated mycophenolate sodium (Myfortic]. / Ulcérations buccales après prise de mycophénolate de sodium (Myfortic).

BACKGROUND: Mycophenolate sodium (Myfortic) is an enteric-coated formulation of the immunosuppressant therapy mycophenolic acid. We report a case of diffuse mouth ulceration in a patient treated with Myfortic presenting recurrence after another dose of drug. PATIENTS AND METHODS: We report the case of a 26-year-old female patient with systemic lupus erythematosus, initially treated with corticosteroids and mycophenolate mofetil, but which was stopped because of varicella-zoster dissemination and leucopoenia. She consulted for mouth ulcers occurring two weeks after the introduction of Myfortic. There were no signs of opportunist infection or lupus activity. Mucosal ulcerations disappeared when Myfortic was stopped. Several weeks later, the patient presented recurrence of mouth ulcerations after another treatment of Myfortic. DISCUSSION: Myfortic is a new enteric-coated formulation of mycophenolic acid developed to reduce gastrointestinal upset associated with Cellcept. In certain cases, Cellcept toxicity can present as a number of oral ulcerations. Direct toxicity is involved in these cases. This side effect has never been described with Myfortic. In our case, the distinctive characteristic is that the patient was never treated with Cellcept without mucosal toxicity despite equivalent systemic mycophenolic acid exposure.

[Myxofibrosarcoma associated with anetoderma]. / Myxofibrosarcome dans une forme anétodermique.

BACKGROUND: Association of malignant cutaneous tumor and secondary anetoderma is rare. Secondary anetoderma in myxofibrosarcoma has not been described to date. We report a case below. CASE REPORT: A 80-year-old woman presented with a 40 x 40 mm, round, flesh-colored lesion on her left buttock. Physical examination showed a soft, protuberant lesion, without firm underlying subcutaneous mass. Pathologic examination of the surgical specimen revealed a myxofibrosarcoma, with focal loss of elastic fibers in the overlying dermis. There was no evidence of systemic involvement. One year later, she developed a recurrent tumor at the same site, with similar clinical presentation, which was treated by broad excision. DISCUSSION: Secondary anetoderma is usually seen in association with cutaneous infections and benign skin tumors. An anetodermic presentation of myxofibrosarcoma has not been reported to our knowledge. Myxofibrosarcoma (formerly referred to as myxoid malignant fibrous histiocytoma) is characterized by an abundant myxoid background in at least one half of the tumor. The tumor recurs in almost two-thirds of cases and metastasizes in one-fourth. Our case confirms that a unique, acquired anetodermic lesion can reveal a malignant tumor. A large deep biopsy should be performed systematically when this variety of anetoderma is observed.

[Drug reaction with eosinophilia and systemic symptoms (DRESS) following imatinib therapy]. / DRESS (Drug reaction with eosinophilia and systemic symptoms) après traitement par imatinib (Glivec).

BACKGROUND: Imatinib (Gleevec) is a tyrosine kinase inhibitor used to treat chronic myeloid leukemia. We describe a case of drug reaction with eosinophilia and systemic symptoms (DRESS) after institution of treatment with imatinib. PATIENT AND METHODS: A 78-year-old woman was treated with low-dose imatinib for chronic myeloid leukemia since November 2003. A macular and pruritic eruption appeared on the patient's trunk after 7 weeks of treatment and gradually worsened. After 1 month, she was admitted for generalized skin eruption with fever and diffuse lymphadenopathy. Laboratory data showed hypereosinophilia and blood cultures positive for Staphylococcus aureus. Imatinib was stopped and replaced with hydroxyurea (Hydrea). Improved clinical and laboratory results were seen with antibiotics and topical steroids. DISCUSSION: To our knowledge, this is the first case of DRESS following treatment with imatinib. Cutaneous reactions to imatinib are frequent and are usually mild, comprising maculopapular eruption, pruritus and facial edema. Few cases of serious skin reactions have been reported until now. Several authors suggest that the prevalence and severity of cutaneous manifestations are related to a pharmacologic effect of imatinib. Our observation cannot rule out an underlying immunologic mechanism. Septicemia may also play a part in the development of DRESS.

[Skin diseases observed in the dermatology departments of three French university teaching hospitals]. / Activité de consultation de trois services de dermatologie hospitalo-universitaires français.

BACKGROUND: We recently carried out a study concerning consultations by French dermatologists in private practice. We evaluated consultations at the dermatology departments of 3 university teaching hospitals in France. MATERIALS AND METHODS: This was a 2-month prospective study conducted in 2003 at the dermatology departments of the university teaching hospitals of Amiens, Lille and Rouen. Each consultant completed a questionnaire covering the duration of the study. The following data were recorded: consultation date, function of the consultant, study centre, type of consultation, type of disease, and whether or not the patient was hospitalised after the consultation. RESULTS: 7296 files were examined during the study. 38% of the consultations were performed by part-time hospital consultants, 29% by dermatology interns, 18% by hospital practitioners, 9% by university professors and 6% by clinical heads or assistant heads. The most commonly encountered diseases were allergies (17%), cancer (16%), arteriovenous disease (15%) and infectious disease (11%). Three types of consultation were identified: emergency consultations without an appointment, consultations by appointment for a specific problem and consultations by appointment without a specific problem. The number of resulting hospital admissions ranged from 2 to 10% of consultations, depending on the type of consultation and the role of the consultant in question. DISCUSSION: This study shows that in France, consultations at hospital dermatology departments differ greatly from those of dermatologists in private practice. The main diseases seen (cancer, arteriovenous disease, allergy, infectious dermatosis) accounted for the majority of hospitalisations in these departments. The organisation of hospital consultations is increasingly tending both towards treatment of highly specialised diseases through specifically oriented consultations and also towards the emergency treatment of certain forms of acute dermatosis.

Post-transplant cutaneous T-cell lymphomas.

Post-transplant cutaneous lymphomas are rare. Cutaneous T-cell lymphomas account for 30% of these lymphomas. The clinical appearance of the skin lesions is identical to cutaneous lymphomas observed in non-immunosuppressed patients, with infiltrated plaques, nodular and ulcerated tumors, but with an increased frequency of erythroderma. Standard histology and immunohistochemistry are also consistent with the features of mycosis fungoides and CD30+ cutaneous lymphomas observed in the general population. However, the pronostic differs from the usually favourable outcome of cutaneous T-cell lymphomas, as 8 out of the 13 patients of our series died, in less than 1 year for 6 of them. This unfavourable course appears to be the same as that observed for systemic T-cell lymphoma in transplant recipients. In contrast to post-transplant B-cell lymphomas (systemic and primary cutaneous), the link to a virus has not been demonstrated. The prognosis is also less favourable for post-transplant cutaneous T-cell lymphomas than for post-transplant cutaneous B-cell lymphomas.

Epidermolysis bullosa acquisita and Crohn's disease. A case report with immunological and electron microscopic studies.

A review of the literature shows that the association of epidermolysis bullosa acquisita and Crohn's disease is rare. A 27-year-old man developed cutaneous blisters on the trauma-prone areas that were consistent with the diagnosis of epidermolysis bullosa acquisita. Immunological and electron-microscopic studies of the skin showed intense IgG deposits located beneath the basal lamina. Three years later, Crohn's colitis was diagnosed. Prednisolone and sulfasalazine treatment resulted in an improvement of the bowel disease but without appreciable effect on the skin lesions.

[Topical silver sulfadiazine-induced acute renal failure]. / Insuffisance rénale aiguë après application topique de sulfadiazine argentique.

INTRODUCTION: Prolonged topical application of silver sulfadiazine cream can induce argyria and adverse effects of sulphonamides. We report a case of a woman with acute renal failure following repeated applications of topical silver sulfadiazine on pyoderma gangrenosum wounds. CASE REPORT: A 61 year-old woman suffering from rheumatoid arthritis, Sjogren's syndrome and scleroderma was treated with corticosteroids (1 mg/kg/day) and topical application of silver sulfadiazine cream (200 g/day) for extensive pyoderma gangrenosum wounds on the legs. Three weeks later, the patient was transferred to intensive care because of pulmonary edema, oligoanuria and disrupted consciousness. Laboratory data revealed leukopenia (1100/mm(3)) with neutropenia and renal failure (serum creatinine 316 micromol/l). Proteinuria was moderate and ultrasonography of the kidneys was normal. Silver concentration in blood was 1818 nmol/l (N < 92 nmol/l) and 1381 nmol/l (N < 9 nmol/l) in urine. Sulfadiazine concentration in blood was undetectable. All the signs regressed after withdrawal of silver sulfadiazine and after several sessions of hemodialysis. DISCUSSION: Various causes of renal failure are discussed in our patient. However, direct silver-induced renal toxicity is the most likely and is confirmed by the high concentration of silver in blood and urine and the improvement on withdrawal of the topical cream, without modification in the oral treatment. The absence of red corpuscles and crystals in the urine and undetectable concentrations of sulfadiazine in blood are not in favor of sulphonamide renal toxicity. Furthermore, the autoimmune diseases of our patient were well-controlled. Leukopenia could be secondary to silver sulfadiazine medullar toxicity. This observation confirms that this topical cream should not be used for long periods on extensive wounds.

[Perforating granuloma annulare with transfollicular perforation]. / Granulome annulaire perforant unique avec phénomène d'élimination transfolliculaire.

INTRODUCTION: The perforating granuloma annulare is a rare form of granuloma annulare. The clinical diagnosis is difficult and the confirmation is histological. The localisation is unique in less than 10% of all cases. We report a documented case with a histological suspicion of transfollicular perforation. OBSERVATION: A 36 year-old woman, without any particular antecedent, presented on her upper arm a single ulcerated nodular lesion that had evolved for one year. The clinical examination and biological investigations were normal. The histological examination led to the diagnosis of perforating granuloma annulare with a large epidermic ulceration. Treatment with a topical corticosteroid was disappointing and the removal was decided. DISCUSSION: In our observation, the single localization of this lesion raises the problem of differential diagnosis such as cutaneous tuberculosis, atypical mycobacteriosis, skin sarcoidosis, foreign body granuloma, epidermoid carcinoma or perforating dermatitis. The histological examination permitted diagnosis of a perforating granuloma annulare with large epidermic ulceration. The infiltration and destruction of a hair follicle evoked the possible transfollicular elimination of the necrotic material. Other pathologies with the histological aspect of a palisading granuloma were excluded from this context. No associated pathology such as diabetes nor any other favouring factors such as ultraviolet light or insect bites or traumas were identified. The physiopathology of perforating granuloma annulare and the process of perforation remain unknown. Numerous therapies have been proposed with variable results.

Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group.

BACKGROUND AND DESIGN: The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 months in three university dermatologic centers in Amiens, Limoges, and Tours, France, that correspond to a cumulative reference population of 3.55 x 10(6). RESULTS: Using data from these regions, we found a mean annual incidence of autoimmune subepidermal bullous diseases to be 10.4 per million people and, therefore, estimated the overall number of new cases of these disorders in France to be about 590 cases per year. According to clinical and immunoelectron microscopic criteria, a precise diagnosis was established in 94 cases, distributed as follows: bullous pemphigoid, 69 cases; cicatricial pemphigoid, 12 cases; linear IgA dermatosis, five cases; herpes gestationis, four cases; epidermolysis bullosa acquisita, two cases; and vesiculobullous systemic lupus erythematosus, two cases. CONCLUSION: Our prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data. Our estimated incidence of bullous pemphigoid (seven new cases per million people per year) is large enough to establish bullous pemphigoid as the major autoimmune subepidermal bullous disease for the purpose of therapeutic trials. On the contrary, all other disorders, particularly epidermolysis bullosa acquisita (estimated annual incidence, 0.17 to 0.26 per million people), were very rare and reflect the paucity of patients available for short-term clinical studies in France.

Leg ulcers and hydroxyurea: forty-one cases.

BACKGROUND: Hydroxyurea is an antitumor agent used to treat chronic myeloproliferative disorders. Leg ulcerations have been reported in patients undergoing long-term hydroxyurea therapy for myeloproliferative diseases. To better define this dermatological adverse effect of hydroxyurea therapy and to try to understand the pathophysiological process of this disease, we collected medical information for such patients in a multicenter retrospective study. OBSERVATIONS: Forty-one patients (mean age, 67 years) developed leg ulcerations while undergoing hydroxyurea therapy (mean therapy duration, 5 years). The sex ratio was 1, and there was no underlying vascular disease. Hematologic abnormalities were identified. Complete recovery from the ulcerations occurred quickly after withdrawal of treatment in 33 (80%) of the cases. CONCLUSIONS: This longest-reported series of patients confirms the role of hydroxyurea therapy in the onset of leg ulcerations. Healing or improvement requires cessation of treatment. Cutaneous atrophy and impaired wound healing may explain the relationship between hydroxyurea and leg ulcers. In addition, the megaloblastic erythrocytes resulting from the presence of hydroxyurea may circulate poorly through the capillary network. A prospective study in hematologic centers would be valuable.

[Leg ulcers in Werner's syndrome. Report of one case]. / Les ulcères de jambe du syndrome de Werner. A propos d'un cas.

Werner's syndrome (adult progeria) is a rare autosomal recessive condition characterized mainly by a characteristic habitus (short stature, light body weight) scleroderma like changes of the limbs and premature aging. Chronic leg ulcers appears in about fifty per cent of the patients. These ulcers can be related to the combination of mechanical factors on atrophic subcutaneous tissue and skin of the feet and leg associated with early arteriosclerosis (20%) and diabetes mellitus (60%).

[Melanocytes in epiloia; ultrastructural aspects (about 7 cases) (author's transl)]. / les mélanocytes dans l'épiloïa; aspects ultrastructuraux (a propos de 7 cas).

Hypopigmented macules of epiloia have been studied by photonic and electron microscopy in 7 cases. The number of melanocytes was not decreased but these cells were hypotrophic and had a low tyrosinae activity. The melanosomes were smaller in size and their maturation rate was greatly reduced; many abnormal round and granular melanosomes, phaeomelanosome-like, were observed. In one case, such abnormal melanocytes were also occasionally found in normally pigmented control skin.

[Acute febrile neutrophilic dermatitis (Sweet's syndrome) during therapeutic agranulocytosis in acute myeloblastic leukemia]. / Dermatose aiguë fébrile neutrophilique (syndrome de Sweet) en période d'agranulocytose thérapeutique au cours d'une leucémie aiguë myéloblastique (LAM).

INTRODUCTION: the association of acute febrile neutrophilic dermatosis (Sweet's syndrome) with malignant haemopathies is well known and characterized by an usual lack of hyperleukocytosis: indeed, moderate neutropenia is often reported. However, cases of Sweet's syndrome in the agranulocytosis stage are exceptional (7 in the literature). CASE-REPORT: We report the case of a woman with acute myeloblastic leukaemia who had presented with Sweet's syndrome in the phase of therapeutic aplasia during induction of treatment, in the absence of white blood cells transfusion or treatment with haematopoietic growth factor (GM CSF, GCSF). COMMENTS: the physiopathology of Sweet's syndrome is unknown. Various mechanisms have been suggested, including immune reaction type III, increased interleukin-1 synthesis, increased chemotaxis of neutrophils, action of haematopoietic growth factors, iatrogenic effect of some drugs (e.g. cotrimoxazole, furosemide or minocycline). Yet none of these mechanisms involving circulating polymorphonuclears or their bone marrow precursors can explain the occurrence of Sweet's syndrome in the phase of agranulocytosis. CONCLUSION: the diagnosis of Sweet's syndrome must be considered in patients with agranulocytosis in order to avoid ineffective antibiotics and to initiate a corticosteroid therapy that will accelerate the cure of this benign dermatosis.

[Accidents caused by iontophoresis]. / Incidents de l'ionophorèse.

Ionophoresis is a well-established treatment for idiopathic hyperhidrosis. Modern apparatuses are reliable as long as the electrical equipment is used correctly. The authors present five cases of cutaneous incidents resulting in burns and secondary necrosis. Two of the cases could be explained by defective protection and the other three were apparently of the same type although non confirmation could be established. In comparison with uneventful incidences (pruritus, erythematous reactions, dysaesthesia) these spectacular burns should not exist. Machines must be regularly check and only electrodes with optimal protection should be used.

[Genital leiomyomas of the male nipple: two cases]. / Léiomyome dartoïque du mamelon: deux cas chez l'homme.

BACKGROUND: Genital leiomyoma is a rare benign solitary skin tumor, not painful, developed from smooth muscle. Genital leiomyoma arising from the nipple is extremely rare, especially in males. CASES REPORT: A 47 year-old male had a 2 cm cutaneous plaque with nodules located on the right nipple. This plaque was circumscribed, erythematous, pruriginous and was not painful. The lesion had been noticed by the patient two years ago. A 37 year-old male showed a 1.5 cm cutaneous plaque located on the left nipple. The plaque was slightly erythematous, pruriginous, not painful and had been noticed by the patient 5 years earlier. Histology provided the diagnosis of genital leiomyoma in both cases. No surgical therapy was performed. DISCUSSION: Cutaneous leiomyomas are classified in 3 types regarding their origin: multiple or solitary piloleiomyoma, arising from arectores pilorum muscles, solitary genital leiomyoma, arising from the dartoic, vulvar, or mammillary muscles, and solitary angioleiomyoma, arising from the vein muscles. Clinically, genital leiomyoma is a 1 cm diameter solitary erythematous, firm nodule. According to many authors, genital leiomyoma is asymptomatic, but in the 2 patients, the lesions were pruriginous. Surgical excision is usually performed.

[Plaque variant of trichoblastic fibroma]. / Fibrome trichoblastique en plaque.

BACKGROUND: Tricoblastic fibroma is a rare benign skin tumor originating in the hair follicle. There are two clinical presentations: nodular and plaque variants. The plaque variant is almost exclusively located on the face with deep tissue infiltration. CASE REPORT: A 38-year-old man presented with a 2 cm diameter cutaneous plaque located on the right cheek. It had first been noted by the patient 3 years earlier and had enlarged slowly. On examination, the plaque was well defined, yellowish and slightly indurated. The first clinical diagnosis was basal cell carcinoma and the plaque was removed. Histology provided the diagnosis of trichoblastic fibroma. The patient remains well, with no evidence of recurrence, 10 months after excision. DISCUSSION: Trichoblastoma is an inclusive term for all benign cutaneous neoplasms that are mostly composed of follicular germinative cells. According to Altman, the plaque variant of trichoblastic fibroma is a poorly circumscribed neoplasm, particularly at its lateral and deep margins. This author also states that mitotic figures are more numerous in the plaque variant of trichoblastic fibroma and considers this clinical variant as a low-grade follicular malignancy.

[Cutaneous T-cell lymphoma following renal transplantation]. / Lymphome T cutané après transplantation rénale.

INTRODUCTION: Post-transplant cutaneous lymphomas are the second skin cancer after cutaneous carcinoma and are usually of the B-cell type. Post-transplant cutaneous T-cell lymphomas are extremely rare. We described a case of a cutaneous T-cell lymphoma in a renal transplant recipient. CASE REPORT: A 52-year-old woman was hospitalized for an erythematous infiltrated eruption. Seven years earlier, she had undergone kidney transplantation. No palpable lymphadenopathy or hepatosplenomegaly was present. The patient's skin biopsy specimen was histologically suggestive of CD30- fungoid mycosis. The same clonal TCR-rearrangement was identified in the blood and in the skin. No EBV was detected within the cutaneous lesion on immunohistochemical analysis or by PCR in the blood. Chlorambucil (Chloraminophène) was associated with a topical treatment with chlormethine (Caryolysine) and corticosteroids while tacrolimus (Prograf) was reduced and stopped. There was no evidence of recurrence of the lymphoma after 12 months of follow-up. DISCUSSION: The particularity of our observation is the apparition, 7 years after transplantation, of a CD30-, EBV-fungoid mycosis with a blood and cutaneous clonal TCR-rearrangement. Despite this poor prognosis factor, the cutaneous lymphoma regressed after reduction of the immunosuppressive treatment reduction and institution of topical corticosteroids, chlormethine and chlorambucil.

[Pigmented pemphigoid]. / Pemphigoïde pigmentée.

BACKGROUND: Pigmented bullous pemphigoid has many clinical manifestations. We report a pigmented erythematous form with disseminated bullae. CASE REPORT: A 70-year-old woman with a history of breast adenocarcinoma developed an eruption of pigmented macules on the trunk and members of 72 hour duration. At five days, there was an eruption of tight bullae. The diagnosis of bullous pemphigoid was retained because of the association of infraepidermic bullae, linear deposits of C3 along the basal membrane and the presence of the 180 kDa minor bullous pemphigoid antigen on immunoblotting. DISCUSSION: The initial pigmented aspect of this bullous pemphigoid suggested disseminated pigmented bullous erythema, but histology data with immunotransfer corrected the diagnosis. This very atypical presentation led us to look for a particular etiology. There was no argument in favor of a malignancy in this patient in complete remission after treatment of her breast adenocarcinoma. The fact that the patient was phototype IV would probably explain the pigmented nature of the initial lesions.

[MELAS syndrome (mitochondrial encephalopathy with lactic acidosis and stroke-like episodes]. / Syndrome MELAS (Encéphalopathie Mitochondriale avec Acidose Lactique et pseudo-accidents vasculaires cérébraux).

BACKGROUND: The MELAS syndrome (Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes) belongs to the category of mitochondrial disorders. The most common molecular etiology of the syndrome is a mutation A to G transition at base pair 3243 in the mitochondrial genome. The phenotype is varied and depends on the proportion of DNA muted and which organ on aerobic metabolism suffers most. CASE-REPORT: An 17 year-old woman had successively neurosensory hearing loss, renal disease, cardiomyopathy, diabetes mellitus, lactic acidosis and stroke-like episodes that evoked a MELAS syndrome. DISCUSSION: The skin manifestations of patients with MELAS syndrome are scaly, pruritic, diffuse erythema, reticular pigmentation, moderate hypertrichosis, seborrheic eczema, atopy and vitiligo. Our patient presented severe hirsutism and reticular pigmentation of the limbs. No abnormal histologic and electron microscopic findings were noted in the skin or the follicles involved.