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Biomedical subjects

E Grosshans

Publications and source records attributed to E Grosshans.

At least 127 records · Page 7Linked to original sources

[Macrophage activation syndrome disclosing leukemic transformation of mycosis fungoides].

BACKGROUND: Mycosis fungoides can mimic pigmented purpuric dermatitis. We report such a case which progressed to peripheral T-cell lymphoma; progression was revealed by reactive hemophagocytic syndrome (RHS). CASE REPORT: A 65-year old male patient was hospitalized for a pigmented and purpuric eruption. The skin lesions appeared 2 years earlier and at that time biopsy had shown pigmented and purpuric dermatitis. One month before hospitalization, general signs appeared. On admission, he had papular and purpuric rash, mainly on the trunk, hepatosplenomegaly, enlarged axillar and inguinal lymph nodes, and fever at 38.2 degrees. A skin biopsy showed histologic changes typical of mycosis fungoides. He also had bicytopenia, hepatitis, and increased triglyceride and ferritin levels suggesting RHS which was proved by means of bone marrow biopsy. These tests also evidenced peripheral T-cell lymphoma. The patient was treated with two courses of chemotherapy (CHOP) but the disease progressed and he deceased. DISCUSSION: Mycosis fungoides can occasionally begin with an eruption very closely resembling pigmented purpuric dermatitis. Therefore, repeated biopsies should be done in case of widespread permanent pigmented purpuric dermatitis of no apparent origin. RHS is a life-threatening disease. The diagnosis should be suspected in any cytopenic patient with fever, increased triglyceride levels and abnormal liver tests. A search for an etiology must then be undertaken a prompt treatment is needed.

Aged↗

[Chloroquine-induced achromotrichia. An ultrastructural study].

BACKGROUND: Electron microscopy examination of scalp biopsies from a patient with chloroquine achromotrichia gave elements concerning the pathogenesis of chloroquine-induced achromotrichia. CASE REPORT: A 21-year old light brown-haired patient developed achromotrichia after four months of treatment with chloroquine for subacute lupus erythematosus. Hair bleaching completely regressed 5 months after discontinuing chloroquine despite replacement with hydroxychloroquine. During the achromatrichia phase, many ultrastructural anomalies were observed in the hair root melanocytes: the nuclei were small and densified, and there was an accumulation of immature melanosomes in the cytoplasm; these melanosomes, mainly in stage II, were rarely transferred to keratinocytes. After recovery from the achromotrichia, melanocytes displayed a normal aspect. DISCUSSION: Pathophysiological disturbances leading to chloroquine induced achromotrichia are still unclear. The ultrastructural study of hair follicles in our patient show that under chloroquine action melanocytes become unable to perform complete melanin synthesis and to produce normally melanized melanosomes which may be transferred to keratinocytes. Non-melanized or poorly melanized melanosomes accumulate in the melanocytes which finally become inactive cells. These findings suggest that achromotrichia is induced by a toxic effect of chloroquine on the melanocyte.

Adult↗

[Rilmenidine in rosacea: a double-blind study versus placebo].

INTRODUCTION: The usual treatments of rosacea (cyclines and metronidazole) are mainly effective on reducing the number of papules and pustules. Clonidine only was employed in order to treat flushes observed in rosacea. Rilmenidine is a central hypotensive drug which acts more specifically than clonidine on imidazoline receptors and which has no sedative side effects. The purpose of this study was to evaluate the efficacy of rilmenidine 1 mg/d in the treatment of rosacea. PATIENTS AND METHODS: A total of 41 patients suffering from typical rosacea were selected in this randomised double blind study rilmenidine versus placebo. The study comprised an 1-month observation period without treatment followed by 3 months of treatment. The major assessment criteria was the proportion of responders at the end of the treatment period. Responders were defined as patients showing a decrease of more than 50 p. 100 in the count of papules and pustules. Minor criterias were the variation of the number of flushes, self-evaluated by the patient and the variation of the redness of the face, noted by the investigator on a scale from 0 to 5. RESULTS: Fifteen patients treated by rilmenidine (R) and 19 patients receiving the placebo (P) were evaluated. The proportion of responders was 69.2 p. 100 in group R and 57.1 p. 100 in group P (p = 0.69). The variations of the number of papules and pustules and of the redness of the face were not significantly different in the two groups. The decrease in the number of flushes was higher in group R (around -13) than in group P (around -5), but the difference was not really significant (p = 0.076). Arterial pressure decreased in 3 patients in group R and in 2 patients in group P. Minor side effects were noted in a similar proportion of patients in the two groups. DISCUSSION: Rilmenidine is not efficient in reducing the number of papules and pustules but could decrease the number of flushes. Nevertheless, many patients were lost for follow-up and because of a major placebo effect, the conclusions of this study are not strong enough. Another study including more patients and using evaluation criteria based on the vascular components of rosacea could perhaps confirm this hypothesis.

Adrenergic alpha-Agonists↗

[Chronic edema, monoclonal dysglobulinemia and profuse telangiectasia: a distinct entity?].

INTRODUCTION: Monoclonal gammopathy and edema are features encountered during several diseases, especially systemic capillary leak syndrome. The diagnoses of POEMS syndrome, edematous systemic scleroderma and a fortuitous association may be also discussed. We report the cases of two patients which did not fulfill the criteria for such diagnoses. CASE REPORT: Although the 2 cases share some discrepancies, they have also similar and particular features: association of chronic edema, monoclonal gammopathy and profuse and acquired telangiectasias. DISCUSSION: The meaning of these cases remains to be clarified. It might be an entity close to the systemic capillary leak syndrome but characterized by the chronicity of edema and by a distinct cutaneous sign, the occurrence of numerous telangiectasias. These cases emphasize that the acquired and profuse telangiectasias belong to the wide range of cutaneous abnormalities which may be associated with monoclonal paraproteinemia.

Capillary Leak Syndrome↗

[The allergic skin].

The main cutaneous manifestation of allergy is eczema. In atopic dermatitis, the epidermal Langerhans cells express receptors for IgE and the eczematous lesions may be associated with other atopic disorders such as asthma or pollinosis. In contact dermatitis, the epidermal Langerhans' cells play the role of antigen-presenting cells; the antigens eliciting the eczematous lesions may be of occupational, vestimentary, cosmetical, therapeutical or other environmental origin. Epicutaneous test procedures enable their identification. Paraptic eczema is a concept including all the cutaneous and systemic complications of contact dermatitis.

Adult↗

Recurrent annular erythema with purpura: a new variant of leucocytoclastic vasculitis responsive to dapsone.

Annular lesions are rarely reported in the clinical spectrum of leucocytoclastic vasculitis, except in the acute haemorrhagic oedema of the skin. We report three patients who suffered from an extraordinary recurrent annular dermatitis, for 4 years in one case and for decades in the other two. The eruption was characterized by purpuric lesions that had a centrifugal evolution, creating target- or polycyclic patches disseminated on the limbs and trunk. The patients' general condition remained excellent during the attacks. All lesions spontaneously disappeared within 2 weeks, but recurred monthly. In all three cases, the histological changes were consistent with leucocytoclastic vasculitis. One patient had ulcerative colitis and another had a benign immunoglobulin A (IgA) monoclonal gammopathy. These two patients showed a good response to dapsone therapy. This dermatosis probably represents a new and rare variant of leucocytoclastic vasculitis.

Dapsone↗

Changes in sexual behaviour of patients attending an HIV testing centre: a prospective study 1988-1994.

OBJECTIVE: To evaluate the sexual behaviour changes in patients attending HIV testing during the period July 1988 to June 1994. DESIGN: In a prospective study, 6824 face-to-face interviews were carried out before the HIV test was performed. The frequency of condom use and the number of sexual partners during the 6 previous months were recorded annually from July to June. The data were analysed according to gender, age class and sexual orientation. SETTING: Strasbourg, Bas-Rhin, France. SUBJECTS: Patients attending the HIV testing centre of Strasbourg. RESULTS: There was a striking increase in the number of attenders of this centre from 358 patients in 1988/89 to 2421 in 1993/94. We observed a significant decrease of homosexuals having more than five partners (p < 0.05) whereas multipartner sex remained unchanged in heterosexuals. There was no change in the proportion of patients having only one partner, except a slight raise in patients under 20 years. All groups showed a very significant increase in condom use, which was especially marked in young heterosexuals aged under 30 years. Nevertheless, condom use remained higher in homosexuals than in heterosexuals in 1993/94. In addition, there was a striking fall of past sexually transmitted disease in heterosexual patients under 20 years during the study period, and a fall in the HIV positivity rate from 1.96% to 0.42%. CONCLUSIONS: A major increase was noted in condom use in all groups and a reduction of multipartner sex in some patients. These data are encouraging in the younger patients, but prevention efforts should also be concentrated on middle aged patients who did not show major sexual changes, although having important risk factors for HIV infection.

Adult↗

[Blaschkolinear eruption with biological signs of lupus].

INTRODUCTION: Acquired inflammatory dermatoses of the adult with a blaschkolinear arrangement are rare. Their cause is unknown. CASE REPORT: We report the case of a 35-year-old man who presented an unilateral skin eruption covering the forearm and with secondary blaschkolinear extension to the arm, the axillary region and the chest. In this particular case, the patient was positive for RNP type anti-nuclear antibodies and direct immunofluorescence was positive in both biopsies of the lesions and of healthy skin. The eruption lasted 6 months and regressed spontaneously. DISCUSSION: Such cases are exceptional and the nosology is difficult to establish. A particular form of lupus erythematosus? Fortuitous coexpression of lupus antibodies and blaschkitis in an adult?

Adult↗

[Plantar hidradenitis].

INTRODUCTION: Plantar hidradenitis, one of several possible causes of painful papulo-nodular lesions of the foot in children and young adults, was recently described as the presence of characteristic lesions of the eccrine sweat glands. CASE REPORT: A 15-year-old boy consulted for a sudden-onset painful eruption on the sole of the right foot. Physical examination revealed papulo-nodular erythromato-violet infiltrated lesions located on the anterior part of the right planta. Histology examination showed dense neutrophil inflammatory infiltration predominating at the dermo-hypodermic junction around the eccrine sweat glands. The excretory ducts of the sweat glands were infiltrated but the secretory glomerulae were intact. A dense infiltration of venular thrombi without vasculitis was also seen. The lesion regressed with aspirin (8 days) and rest. No recurrence has been recorded after 18 months. DISCUSSION: This clinical and histological presentation led to the diagnosis of plantar hidradenitis, confirming the disease entity. Former cases may have been described as trauma-induced plantar urticaria or plantar erythema nodosum. This diagnosis should be entertained in children or young adults with painful papulo-nodular eruptions of the soles. The histology examination should include the eccrine sweat glands.

Adolescent↗

[Porphyria cutanea tarda and hepatitis C virus infection. Clinical and virological study].

INTRODUCTION: The role of hepatitis C virus (HCV) infection in porphyria cutanea tarda (PCT) is probable since the global antibody prevalence among PCT patients is about 70 p. 100. The purpose of this study was to evaluate the virological characteristics in 12 patients with sporadic PCT and one with familial PCT. METHODS: Anti-HCV antibodies were detected by enzyme-linked immunosorbent assay and confirmed by recombinant immunoblot assay. Hepatitis B virus (HBV) and anti-human immunodeficiency virus (HIV) markers were also determined. The polymerase chain reaction (PCR) was performed in order to detect: 1) both positive and minus HCV strands, 2) HCV RNA titer and 3) HCV RNA genotype. RESULTS: Seven of the 12 patients with sporadic PCT were HCV positive and the patient with familial PCT was HCV negative. The age of onset of PCT was significantly lower in HCV positive patients than in HCV negative patients (p < 0.02). The HCV RNA was detected in all patients who had HCV antibodies, and the replicative intermediate of HCV was detected in 3 of them. The positive RNA titer ranged from 1/10 to 1/10(6). Four patients were infected by HCV genotype I, 2 by genotype II and 1 patient was coinfected by type I and type II. Three of the 7 HCV positive patients also had HBV antibodies, but HBV DNA was never detected. All patients were HIV negative. DISCUSSION: The HCV infection rate was high in this series (58 p. 100), and all HCV infected patients had HCV RNA, reflecting an active replication of the virus. The young age of onset of PCT suggests that HCV is a major triggering factor of PCT. Nevertheless, the clinical changes of PCT were not related to the virological findings, suggesting an indirect role of HCV.

Adult↗

Examination of cutaneous macroglobulinosis by immunoelectron microscopy.

Cutaneous macroglobulinosis is a rare cutaneous manifestation of Waldenström's disease. Lesions are though to result from accumulation of macroglobulin in the dermis and are therefore called IgM storage papules. Ultrastructural findings in the previously reported cases were contradictory and the nature of the deposits was not established by electron microscopy. The purpose of this study was to analyse such deposits by the use of immunoelectron microscopy. A 60-year-old woman had multiple erythematous papules for 1 year. The histopathological changes consisted of plasmocytic infiltration of the dermis and eosinophilic deposits. The skin changes and other investigations led to a diagnosis of Waldenström's disease. Samples from normal and diseased skin were analysed by electron microscopy and by immunogold labelling with anti-IgM antibodies, after Lowicryl K4M embedding. An extracellular electron dense granular and filamentous material was observed in the mid- and upper dermis, between and within the collagen bundles. No periodicity was noted and no deposits were seen at the dermoepidermal junction. Immunoelectron microscopy showed a positive labelling located only on these deposits, in both normal-appearing skin and in lesions. In this patient, immunoelectron microscopy clearly demonstrated the presence of large amounts of IgM in the dermis, which were found in the lesions of cutaneous macroglobulinosis and in normal skin. These results suggest that the IgM storage papules result from a greater density of deposits rather than a site-specific accumulation.

Female↗

Abnormal urinary coproporphyrin levels in patients infected by hepatitis C virus with or without human immunodeficiency virus. A study of 177 patients.

OBJECTIVE: Many cases of porphyria cutanea tarda have been described in association with human immunodeficiency virus (HIV) infection in young individuals. The link between hepatitis C virus (HCV) and porphyria cutanea tarda is even stronger as more than 50% of patients who have this diagnosis in Italy, France, or Spain are also infected by HCV. To study the role of viral infections on the metabolism of porphyrins, we measured the urinary porphyrin levels in patients with HIV and HCV infections. DESIGN: Survey; prospective study. SETTING: University Hospital of Strasbourg, France. PATIENTS: Sixty-one HIV-positive patients, 56 HCV-positive patients, 60 HIV- and HCV-positive patients, and 51 HIV- and HCV-negative control subjects were randomly selected. None had clinical signs of porphyria or a familial history of porphyria. MAIN OUTCOME MEASURES: The porphyrin-excretion profile was determined by high-performance liquid chromatography on fresh urine samples. The HIV and HCV viremias were quantified in the serum by the branched DNA assay. Measures were planned before data collection began. RESULTS: The porphyrin-excretion profile typical of porphyria cutanea tarda was found in only 1 of 177 patients. In the remaining 176 patients, the mean coproporphyrin level was significantly raised in HCV-positive patients and even higher in patients who were HIV- and HCV-positive. The coproporphyrin level was not correlated to the alanine aminotransferase level, the CD4+ cell count, or the HCV and HIV viremias. CONCLUSIONS: In cases of infection with HIV, HCV, or both, the development of a porphyria cutanea tarda urinary profile is a rare event (0.56% in this study), but coproporphyrin excretion is increased. This could be related to hepatic changes induced by the viruses. Our results do not support the hypothesis of a direct viral effect on the porphyrin metabolism. Infection with HIV, HCV, or both may be a major triggering factor, but is not sufficient to induce porphyria.

Adolescent↗