Biomedical subjects
E Heid
Publications and source records attributed to E Heid.
Severe hypernatremic dehydration in an infant with Netherton syndrome.
Netherthon syndrome is a rare autosomal recessive disease characterized by ichthyosis, the characteristic hair abnormality trichorrhexis invaginata and atopic manifestations. We report a female child with the severe hypernatremic dehydration form of the Netherton syndrome born as the first child of consanguineous parents. Ichthyosis was present at birth. She was admitted to the intensive care unit at the age of 4 days with important loss of weight and dehydration. Severe hypernatremia and convulsions occurred. Despite intensive care the baby died at the age of 11 days. The diagnosis of Netherton syndrome was confirmed by the finding of the pathognomonic hair shaft anomaly trichorrhexis invaginata (bamboo hair) and premature lamellar body secretion and foci of electron-dense material in the intercellular spaces of stratum corneum as relatively specific markers for Netherton syndrome. Netherton syndrome is characterized by a large variability in phenotypic expression. The major neonatal complication is the hypernatremic dehydration, which can be fatal as in this patient or complicated by neurologic signs (intracranial hemorrhage) and secondary sequellae. Molecular studies revealed a mutation in SPINK 5, encoding a serine protease inhibitor. Prenatal diagnosis was performed in the second pregnancy and showed that the fetus was equally affected.
[Survival after cutaneous metastasis: a study of 200 cases].
BACKGROUND: Cutaneous metastatic disease is uncommon and the outcome after cutaneous metastasis has rarely been thoroughly studied. The objective of this work was to study the survival after diagnosis of cutaneous metastasis in a large series of patients and to evaluate survival according to the type of cancer. PATIENTS AND METHODS: This retrospective study was conducted out in the Laboratoire d'Histo-pathologie Cutanée of Strasbourg. Between 1950 to 1996, 228 patients with cutaneous metastasis were diagnosed on the basis of typical histopathology, confirmed by two dermatopathologists. We excluded lymphoma or leukaemia with secondary skin involvement. Medical and demographic data were collected from hospital data, and the "Registre du Cancer du Bas-Rhin". The type of neoplasm, the time of diagnosis of primary cancer and the time of death (or survival at 12/31/1996) was established in 200 patients, 99 men and 101 women with a mean age 62.4 +/- 13 years. We found 64 cases of breast carcinoma, 36 cases of lung carcinoma, 31 cases of melanoma and 69 cases of other cancers. Long term actuarial survival after cutaneous metastasis was calculated using by the Kaplan-Meier method. RESULTS: The median survival after cutaneous metastasis was 6.5 months (mean 22.8 +/- 43.8 months). The mortality rate was 13 p. 100 at 1 month, 48 p. 100 at 6 months and 64.5 p. 100 at 12 months. Median survival was calculated according to the primary neoplasm: breast carcinoma: 13.8 months, melanoma: 13.5 months, lung carcinoma: 2.9 months (36 cases). The outcome of patients with cutaneous metastasis of lung carcinoma was worse than those with melanoma (p < 10(-4)) and breast cancer (p < 10(-4)). Survival after cutaneous metastasis of other cancers could not be compared because of the small size of the subgroups: median survival after cutaneous metastasis of non cutaneous squamous cell carcinoma of the head and neck: 8.8 months (5 cases), cutaneous squamous cell carcinoma: 6.5 months (12 cases), carcinoma of oesophagus: 4.7 months (2 cases), colo-rectal cancer: 4.4 months (9 cases), pancreatic cancer: 3.3 months (2 cases), stomach cancer: 1.2 months (7 cases) and liver and gall bladder carcinoma: < 1 month (3 cases). Survival beyond 10 years was observed in 9 patients: 3 melanoma, 2 breast cancers, 2 prostatic carcinomas, 1 larynx carcinoma and 1 cutaneous squamous cell carcinoma. CONCLUSION: This is the first study in which the survival after the occurrence of skin metastasis was systematically analysed in a large series of patients. It shows that half of patients with cutaneous metastasis die within the first 6 months after the diagnosis. Those cases due to lung carcinoma have the poorest prognosis.
[Basal cell carcinoma of a light exposed area in a dark skinned African woman].
Explore the source record for details and available documents.
Helicity dependence of gammap --> Npi below 450 MeV and contribution to the Gerasimov-Drell-Hearn sum rule.
The helicity dependence of the single pion photoproduction on the proton has been measured in the energy range from 200 to 450 MeV for the first time. The experiment, performed at the Mainz microtron MAMI, used a 4pi-detector system, a circularly polarized, tagged photon beam, and a frozen-spin target. The data obtained provide new information for multipole analyses of pion photoproduction and determine the main contributions to the Gerasimov-Drell-Hearn sum rule and the forward spin polarizability gamma(0).
[Ulceration or erosion of the oral and genital mucosa. Diagnostic approach].
Explore the source record for details and available documents.
Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions.
The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy. It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. Immunoblotting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were performed in three patients with the Schnitzler syndrome. Western blotting on epidermal extracts showed the presence of IgM-kappa antiskin autoantibodies in two patients. These antibodies displayed the same isotype as the monoclonal components and recognized a 280-290-kDa antigen in one patient and a 200-kDa antigen in the other patient. IEM studies showed sparse IgM deposits in the epidermis, around the keratinocytes, near the desmosomes in one patient and dense deposits below the lamina densa, in the region of the anchoring fibrils, in another patient. Antiskin IgM autoantibodies of the same isotype as their monoclonal gammopathies can be present in the serum of some patients with the Schnitzler syndrome. These IgM antibodies seem to deposit in vivo in the epidermis and at the dermal-epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammopathy plays a part in the pathophysiology of the skin rash. They also suggest patient heterogeneity both in the skin antigens that are recognized as well as in their localization.
Striking increase of thin melanomas contrasts with stable incidence of thick melanomas.
OBJECTIVE: The aim of this study was to analyze the temporal evolution of melanoma incidence in the department of the Bas-Rhin, France, and to study the evolution of tumor thickness. DESIGN: Retrospective study including all histologically proven melanomas recorded at the cancer registry of the department of the Bas-Rhin between January 1980 and December 1992 and at the Cutaneous Histopathology Department of the University Hospitals, Strasbourg, between January 1980 and December 1997. SETTING: Population-based cancer registry and academic cutaneous histopathology department. PATIENTS: A total of 1254 patients with histologically proven melanomas. INTERVENTION: None. MAIN OUTCOME MEASURES: Temporal evolution of melanoma incidence and tumor thickness. RESULTS: The mean (SD) and median tumor thicknesses were 1.48 (1.59) mm and 0.87 mm, respectively, and they decreased during the study period. The increase in the number of melanomas was mainly related to an increase of superficial spreading melanomas in both sexes. The number of intermediate melanomas (1-2 mm) in both sexes and the number of melanomas with a Breslow index between 2 and 4 mm in women increased only slightly. The number of melanomas with a Breslow index greater than 2 mm in men and greater than 4 mm in women remained stable during the reference period. CONCLUSION: A striking increase in incidence of thin melanomas contrasts with a stable incidence of thick melanomas.
Erythrodermic bullous pemphigoid.
Explore the source record for details and available documents.
Cutaneous lupus erythematosus following the lines of Blaschko.
Explore the source record for details and available documents.
[Cutaneous lymphoma manifesting as pigmented, purpuric capillaries].
INTRODUCTION: A few cases of patients with both purpuric pigmented dermatitis and cutaneous lymphoma have been reported. The aim of this study was to evaluate the prognosis of purpuric pigmented dermatitis. MATERIAL AND METHODS: This is a monocentric retrospective study at the dermatology department of the university hospital of Strasbourg. The records of all patients hospitalized for purpuric pigmented dermatitis between 1967 and 1997 have been reviewed. RESULTS: Eight women and 9 men aged between 17 and 84 years were hospitalized for purpuric pigmented dermatitis during the reference period. Except for one patient, all had had a cutaneous biopsy showing the typical features of purpuric pigmented dermatitis. On the basis of clinical signs and course, one patient was thought to have contact dermatitis and three patients were thought to have a purpuric pigmented dermatitis-like drug eruption (meprobannate, pefloxacine and lorazepam or aspirin). The mean follow-up was one year. During follow-up, two patients developed cutaneous T-cell lymphoma after two and four years respectively and one patient developed Hodgkin's disease with skin and lymph node involvement. Another patient who suffered from purpuric pigmented dermatitis for four years had a monoclonal T cell population within the dermal infiltrate. Two patients died of their lymphoma. DISCUSSION: This study shows that purpuric pigmented dermatitis can be associated to or evolve into lymphoproliferative disease. This course should be suspected when purpuric pigmented dermatitis is extensive, long-lasting (> 1 year), has a reticular arrangement and negative patch-testing. In this situation, a long-term follow-up and treatments indicated in the early patch stage of mycosis fungoides (PUVA therapy, chlormethine) seem adequate.
The significance of the 'dust-like particles' pattern of immunofluorescence. A study of 66 cases.
Subacute cutaneous lupus erythematosus (SCLE) is a marker of a unique subset of lupus erythematosus patients. A 'dust-like particles' direct immunofluorescence (DIF) staining pattern, which consists of fine granular particles of immunoglobulin(s) scattered through the epidermis and the cellular infiltrates of the dermis, was reported to be specific for SCLE. In this study, we assessed the real specificity of this staining pattern, which had not yet been evaluated. We systematically searched for the dust-like particles staining pattern among the 4374 skin biopsy specimens submitted for direct cutaneous immunofluorescence during a 7-year period (1989-96). The corresponding patient records were reviewed. Dust-like particles were observed in 66 samples originating from 60 patients. Only 53% of the patients had SCLE. The remaining patients had systemic lupus erythematosus with visceral involvement (17%), discoid lupus erythematosus (3%), mixed connective tissue disease (2%). Sjögren syndrome (2%) and other diseases. Eighty-five per cent of the patients had connective tissue disease. Seventy-seven per cent of the patients were positive for antinuclear antibodies, but only 36% were positive for anti-Ro (SSA) antibodies. This study shows that the dust-like particles staining pattern is not specific for SCLE, but is highly suggestive of connective tissue disease. The nature of the antigen responsible for the immunoglobulin deposition and the prognostic significance of this DIF pattern remain to be established.
Erythema nodosum and associated diseases. A study of 129 cases.
BACKGROUND: Erythema nodosum (EN) is associated with many infectious diseases. The purpose of this study was to evaluate the relative prevalence of associated diseases in a large series of EN, and to review the previously described causes of EN. MATERIALS AND METHODS: A total of 157 inpatients with a diagnosis of EN made in Strasbourg, France between 1960 and 1995 were studied retrospectively, but only 129 patients with confirmed EN were evaluated. A biopsy was taken in 30 patients with atypical clinical symptoms. Chest radiography, blood cell count, throat swab, and anti-streptolysin dosage were performed systematically. Viral investigations and serodiagnoses for various bacterial infections were carried out in approximately half of the patients. All investigations were analyzed retrospectively and compared with the world literature. RESULTS: The female: male ratio was 5 : 1 and the mean age was 31 years. We found 28% confirmed streptococcal infections, 11% sarcoidosis, 1.5% enteropathies, 1.5% Chlamydia infections, 0.8% Mycoplasma infections, 0.8% Yersinia infections, 0.8% hepatitis B, and 0.8% tuberculosis (one case). The causative factor could not be determined in 55% of patients. CONCLUSIONS: Our data confirm the predominance of streptococcal infections and sarcoidosis among patients with EN. Tuberculosis has virtually disappeared, since the last case was observed in 1962. Various viral or bacterial diseases are rarely associated with EN, but all patients were not thoroughly investigated. A large and prospective study should be performed in order to determine the true prevalence of associated diseases in EN. In the absence of specific symptoms, exhaustive investigations are not cost-effective.
Systematic cutaneous examination in hepatitis C virus infected patients.
The purpose of this study was to evaluate the frequency of skin changes among 100 patients from the Hepatogastroenterology Department of the University Hospital, Strasbourg, France who were hepatitis C virus-positive (HCV) and HIV-negative. Their clinical data were compared to those of 50 HCV-, and HIV-negative patients from the same Department, who suffered from various liver diseases. Psoriasis, rosacea, seborrheic dermatitis, cherry angiomas, spider nevus and skin cancers were noted in similar proportions in the two groups. In 15% of HCV-positive patients vs. 4% of controls, chronic pruritus was noted (p < 0.05). In 9 HCV-positive patients, pruritus was not related to itching dermatosis, and only 2 of these patients had mild cholestasis. Four cases of lichen planus vs. 0 in the control group were recorded. The virological data of patients with pruritus or lichen planus were not different than those of the rest of the group. Our findings indicate that systematic skin check-up in HCV-positive patients is valuable.
Chronic urticaria and IgA myeloma.
Explore the source record for details and available documents.
Photocontact dermatitis from ketoprofen and tiaprofenic acid: cross-reactivity study in 12 consecutive patients.
The arylpropionic acid derivatives (APADs) ketoprofen and tiaprofenic acid can provoke photoallergic dermatitis. Possible cross-reactivity between APADs is of importance in patients using nonsteroidal anti-inflammatory drugs. Because of the similarities in chemical structures, we investigated patients with photoallergy to ketoprofen or tiaprofenic acid, in order to study cross-reactivity between APADs and a possible pattern of cross-reactivity between benzophenone-containing molecules, so as to determine the molecular basis of photoallergy to ketoprofen or tiaprofenic acid. 10 patients with photoallergy to topical ketoprofen, 2 with photoallergy to oral tiaprofenic acid, and 15 control subjects with no history of contact dermatitis from APADs, nor from benzophenone-containing molecules, were photopatch tested in triplicate with ketoprofen, tiaprofenic acid, other APADs (alminoprofen, fenoprofen, flurbiprofen, ibuprofen and naproxen), benzophenone-containing molecules (fenofibrate, oxybenzone, sulisobenzone), and unsubstituted benzophenone. 1 set was irradiated with UVA light, 1 with solar-simulated irradiation and 1 dark control. Tests were read at 2, 3 and 4 days. Patients reacted to both ketoprofen and tiaprofenic acid (12/12), fenofibrate (8/12), oxybenzone (3/12) and unsubstituted benzophenone (11/12), but not to other APADs, nor to sulisobenzone. Tests remained negative in control patients. Photoallergy is due to the benzophenone moiety of ketoprofen, or to the very similar thiophene-phenylketone of tiaprofenic acid, but not to their arylpropionic function. This induces cross-reactivity to fenofibrate and oxybenzone but not to APADs without a benzophenone moiety, which may therefore probably be used in such patients. Unsubstituted benzophenone should be added to standard phototesting series.
[Erythroderma with immunoglobulin deposits along the basal membrane. Pemphigoid erythroderma?].
INTRODUCTION: There are many clinical forms of bullous pemphigoid. Nodular, localised, vegetating, vesicular and dysidrosiform variants have been described. The appartenance to the spectrum of pemphigoid was confirmed by the demonstration of antibodies directed against the 230 and 180 kDa BP antigens. Three cases of erythrodermic pemphigoid were recently described. OBSERVATIONS: We describe two cases of patients suffering from erythroderma for several months associated with a linear deposit of immunoglobulins and complement along the basement membrane. In the first observation, the patient suffered from bullae of the legs before developing erythroderma. The positive direct immunofluorescence of the skin was associated with circulating anti basement membrane antibodies, making therefore the diagnosis of erythrodermic pemphigoid possible. In the second case, the patient never developed blisters. We also found a linear deposit along the basement membrane, localized on the dermic side of a skin treated by molar NaCl. No circulating antibodies were found by indirect immunofluorescence or by immunoblott. Direct immuno electron microscopy showed a deposit of immunoglobulins into the deep layers of the lamina lucida and into a part of the lamina densa. CONCLUSION: These two cases suggest that there are true erythrodermic pemphigoid without blisters. The value of the systematic direct immunofluorescence examination of the skin of erythroderma should be evaluated.
[Bullous erythema nodosum leprosum. A case report in French Guiana].
BACKGROUND: Polar and borderline lepromatosis leprosy can be complicated by type 2 reactional states, including erythema nodosum leprosum. CASE REPORT: A 36-year-old man was treated for lepromatous leprosum. He consulted for recurrent erythema nodosum leprosum. Certain nodular lesions were bullous. Histopathology demonstrated major dermal edema causing bullous blisters. Treatment with thalidomide led to rapid regression. DISCUSSION: The bullous form of erythema nodosum leprosum is rarely described in the literature. It raises the diffential diagnosis with other bullous dermatoses, particularly Sweet's syndrome.