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Biomedical subjects

E Grosshans

Publications and source records attributed to E Grosshans.

At least 109 records · Page 6Linked to original sources

[Photosensitivity as presenting sign of HIV infection. Control with triple antiretroviral therapy].

BACKGROUND: We report a case of photosensitivity which occurred as the presenting sign of HIV infection. Photosensitivity regressed completely after introducing antiretroviral tritherapy. CASE REPORT: A 44-year-old woman developed a photo-distributed eczematous eruption which did not respond to topical steroids or hydroxychloroquine. Histologic examination showed eczematous dermatitis. T-cell marker analysis showed a majority of CD8 cells in the infiltrate. The patient was found to be HIV-positive and CD4 counts were markedly reduced to 190/mm3 while CD8 counts were increased to 1260/mm3. Antiretroviral tritherapy cured the photosensitivity. Cure was apparently related to increased CD4 lymphocyte and normalized CD8 lymphocyte counts. DISCUSSION: Photosensitivity was the presenting disorder of HIV infection in this case. Cure of the photosensitivity with antiretroviral tritherapy has not been reported previously. CD8 T-cell infiltration and very low CD4/CD8 ratio would appear to play a key role in the pathogenesis of photosensitivity in these patients.

Adult↗

[Interstitial granulomatous dermatitis and paraneoplastic rheumatoid polyarthritis disclosing cancer of the lung].

INTRODUCTION: Interstitial granulomatous dermatitis is a histological entity usually associated with systemic auto-immune conditions or malignant lymphoproliferation. Its spontaneous regression after surgical treatment of a lung cancer suggests an eventual paraneoplasic variant. OBSERVATION: A 62 year old male patient, a smoker, was hospitalized for weakness, pruritus and symmetrical arthritis of small and large joints. Numerous rheumatoid nodules were located on the extensor aspects of the involved joints. During hospitalization, he developed an inflammatory plaque located on a thigh, which showed histologic features consistent with the diagnosis of interstitial granulomatous dermatitis. He had elevated E.S.R., blood eosiniphilia, and the search for antinuclear antibodies and antineutrophilic leukocyte cytoplasm antibodies (ANCA), of the p-ANCA type, was positive. A lung C.T. scan showed a cavitary tumor. Surgical removal of this tumor evidenced a bronchial squamous cell carcinoma. Four week after surgery, clinical signs and eosinophilia disappeared. After 6 months, ANCA became undetectable. DISCUSSION: Parallelism between the evolution of the cutaneous and articular symptomatology and of the cancer are diagnosis of paraneoplastic rheumatoid arthritis and paraneoplastic interstitial granulomatous dermatitis. Paraneoplastic rheumatoid arthritis is very unusual and this is the first reported case of simultaneously occurring paraneoplastic interstitial granulomatous dermatitis.

Antibodies, Antineutrophil Cytoplasmic↗

[Histiocytic cytophagic panniculitis].

A HISTOPATHOLOGICAL DIAGNOSIS: Histiocytic cytophagic panniculitis is the specific skin finding in the reactive hemophagocytic syndrome. It is a feverish nodular eruption which accompanies the other clinical and biological features of the reactive hemophagocytic syndrome. Histopathologic changes are diagnostic and consist in a lobular panniculitis characterized by the presence of a histiocytic infiltration of the fat. Histiocytes are benign in appearance and show variable degree of phagocytosis. INFECTION AND IMMUNE ANOMALIES: In more than 50% of the cases, the disease is triggered by an infection (mainly a viral infection from the herpes-virus family) in a patient with altered immune function (immunodeficiency, autoimmune disease, hematological disease). Search for subcutaneous T-cell lymphoma is mandatory, but is a diagnostic challenge. Such lymphomas are associated with histologic features of histiocytic cytophagic panniculitis. PATHOPHYSIOLOGY: Histiocytes in the histiocytic cytophagic panniculitis are activated by an abnormal cytokine secretion (interferon gamma, granulocyte/macrophage colony stimulating factor, phagocytosis inducing factor, ...) from neoplastic or reactive T cells. CLINICAL TRIALS: Untreated, the course of the disease is usually lethal. Etiologic treatment should be provided whenever possible. Symptomatic treatment consists in transfusion and corticosteroid therapy. Etoposide, and sometimes cyclosporin, have been reported to be efficient. Some authors recommend that histiocytic cytophagic panniculitis should always be treated with a CHOP-like polychemotherapy. More recently, good results were obtained with high dose intravenous immunoglobulin and this treatment therefore deserves further evaluation.

Diagnosis, Differential↗

Molecular basis of a new type of C1q-deficiency associated with a non-functional low molecular weight (LMW) C1q: parallels and differences to other known genetic C1q-defects.

Analysis of an abnormal C1q molecule of individuals of a Moroccan family by ultracentrifugation in sucrose gradients revealed a low molecular weight C1q (LMW-C1q). We investigated the molecular basis of this defect by sequencing all six exons of the three C1q genes. One point mutation in the codon for Gly at position 15 (GGT) of the B chain was found resulting in an amino acid substitution to Asp (GAT). The exchange not only leads to an interruption of the collagen-like motif Gly-X-Y, but also introduces one negatively charged residue per B chain which results in two additional charges per structural subunit (A-B, C-C, A-B). The mutation which has been identified by DNA-sequencing in the C1q-deficient younger brother of the propositus was confirmed by PCR-EcoRV-RFLP in the sister and the propositus himself. This mutation is very similar to a mutation previously described in another case of functional C1q deficiency where Gly at position 6 of the C chain was substituted by a large positively charged residue (Arg). Again, a LMW-C1q was demonstrated. These point mutations that lead to amino acid substitutions result in the production of a LMW-C1q where the formation of functionally active 11S C1q consisting of three structural subunits appears to be inhibited by the introduction of six additional charges, one per B or C chain, respectively, in the collagenous region of the molecule.

Adolescent↗

Expression pattern of human hair keratin basic 1 (hHb1) in hair follicle and pilomatricoma.

Using in situ hybridization, human hair keratin basic 1 (hHb1) gene expression was investigated in human normal scalp. hHb1 transcripts were specifically detected in the cortical cells of hair shaft but neither in the outer and inner root sheaths, nor in the hair cuticle or the medulla. hHb1 expression was detected strongly in cortical cells located from the beginning of the keratogenous zone up to the isthmus. These data specify the localization of hHb1 expression. Furthermore, neoplasms with follicular differentiation, including trichoblastoma, trichoepithelioma, pilomatricoma, pilar carcinoma and basal-cell carcinoma, were analysed for hHb1 gene expression. One of the 4 pilomatricoma specimens examined exhibited a very high level of hHb1 transcripts. Interestingly, this labeling was specifically associated to a transitional cell layer en route to trichocytic differentiation, providing evidence that in pilomatricoma, epithelial germ cells can differentiate towards hair shaft keratinocytes before evolving in ghost cells.

Basal Cell Carcinoma↗

Topical application of a platelet-activating factor (PAF) antagonist in atopic dermatitis.

Platelet-activating factor (PAF acether) is a lipid mediator with a potent proinflammatory activity. Results derived both from in vitro and in vivo studies suggest a possible role of this substance in the pathophysiology of atopic dermatitis. A double-blind, randomized, multi-center, within-patient study was performed to evaluate the efficacy of a topically applied PAF antagonist (RO-24-0238) in 36 patients with atopic dermatitis. Over a period of 28 days, 0.25 ml of the PAF antagonist and the vehicle (placebo) were applied twice daily on opposite sites of symmetrical lesions (measuring 10 to 20 cm2 each). The overall assessment of the therapeutic efficacy did not demonstrate a superior effect of the PAF antagonist in comparison to placebo, and this was the same with the individual study parameters erythema, scaling, induration and exudation. For reducing pruritus, as assessed by the patient using a visual analogue scale, a statistically significant action was documented during the first 2 weeks of the study (p < 0.04; Wilcoxon rank sum test), with a continued, yet not statistically significant efficacy after weeks 3 and 4. The exact role of the pathological events of atopic dermatitis that might be influenced by a PAF antagonist remains to be determined, but the anti-pruritic component of this substance especially deserves further scientific interest.

Administration, Topical↗

Malignant melanoma and granulomatosis.

Sarcoidosis or granulomatous reactions have rarely been reported in association with malignant melanoma (MM). We describe seven patients who presented with both granulomatous disease and MM, and discuss the physiopathological and prognostic significance of this association. In three patients, the granulomatosis was diagnosed as true sarcoidosis and in one patient, as tumour-associated granuloma. In three cases, designated here as atypical tumour-associated granulomatoses, the presence of clear-cut pulmonary granulomatous nodules was typical neither for sarcoidosis nor for tumour-associated granuloma and was highly suggestive of melanoma metastases. Mediastinal lymphadenopathy was present in every patient. In all seven patients, the question of mediastinal or pulmonary involvement or relapse of the MM was raised, but could be confirmed in only one patient. MM can be associated with granulomatous disease. Knowledge of this association has implications in the management of patients with MM.

Adult↗

Nail abnormalities in rheumatoid arthritis.

Many nail abnormalities have traditionally been described in association with rheumatoid arthritis (RA), but their specificity has never been assessed in a controlled study. Our purpose was to evaluate the frequency and the specificity of nail changes associated with RA in a case-controlled study including 50 patients suffering from RA and 50 controls. For each patient, a general skin examination was performed and the 20 nails were examined. The nail features were noted and classified. A chi 2 test or a Fisher test was used to compare the two groups. The only nail abnormalities significantly associated with RA were longitudinal ridging on nine or 10 finger nails (29 patients in the RA group vs. three in the controls, chi 2: P < 0.001) and clubbing on at least one nail (24 patients vs. 10, chi 2: P < 0.01). Other nail changes were noticed but were not frequent enough to be significant. The presence of longitudinal ridging on the finger nails was significantly associated with RA.

Adolescent↗

[Lymph node and cutaneous syndrome associated with bone plasmacytoma].

INTRODUCTION: We report two patients in whom a slowly growing erythematous thoracic lesion led to the diagnosis of an underlying plasmocytoma. After the treatment of the latter, the cutaneous lesions disappeared, strongly suggesting a link between the two manifestations. CASE REPORTS: The two male patients, aged respectively 66 and 73 years old, had erythematous thoracic plaques. In both cases, extensive laboratory work-up and a histological examination of a cutaneous biopsy did not allow a precise diagnosis. The two patients had a solitary bony plasmocytoma located beneath the cutaneous plaques. The plasmocytomas were discovered respectively 2 and 4 years after the first cutaneous manifestations and were associated to histological non-specific lymph node hyperplasia. Treatment of the plasmocytoma led to the disappearance of skin lesions in both patients. DISCUSSION: In both cases, an erythematous scleroderma-like plaque associated to palpable peripheral and mediastinal lymph nodes, was located over a solitary bony plasmocytoma. Treatment of the plasmocytoma led to the disappearance of the cutaneous lesions, strongly suggesting a link between the two manifestations. Such an association has not been previously reported. We think this is a new entity, characterised by a scleroderma-like cutaneous plaque overlying a solitary bony plasmocytoma and associated to superficial and deep lymph node hyperplasia. We suggest to call it "plasmocytoma associated cutaneous lymph node syndrome". Its relationship to the POEMS syndrome and scleromyxedema are discussed. The pathophysiology remains completely unknown; the syndrome regresses after the treatment of the plasmocytoma.

Aged↗

[Peri-sudoral lipoma].

INTRODUCTION: We identified in our files 11 cases of a new variant of the superficial lipoma recently described by Hitchcock, Hurt and Santa Cruz, characterized by the presence of eccrine sweat glands and denominated "adenolipoma of the skin". PATIENTS AND METHOD: We examined 1,742 skin lesions registered in the Laboratory for Skin Histopathology from January 1989 to August 1996. These lesions were 397 lipomas, 1,325 skin tags (acrochordons) of which 120 with a fatty stroma and 20 connective tissue hamartomas of which 7 superficial lipomatous hamartomas. Among these lesions we looked for those corresponding to the prime description of the cutaneous adenolipoma. RESULTS: We identified 11 cases of adenolipoma, i.e. a frequency of 0.65 p. 100 for the whole examined lesions and 2 p.100 among the 524 lesions with a fatty component (lipomas, lipomatous hamartomas and fibrolipomas). The mean age of the patients was 50 years and the sex-ratio F/M 1.75. The lesions were localized on the lower limbs (7 cases), especially on the thighs (4 cases), on the trunk (3 cases) and on the shoulder (1 case). DISCUSSION: The adenolipoma is quite different from the common cutaneous lipomas. It develops within the dermis or the fatty layer. It is a solitary lipoma most often localized on the proximal parts of the limbs, especially on the thighs. Histologically it presents as a lobulated and capsulated tumor where eccrine sweat glands and ducts are present inside the fatty lobules. Apparently the adenolipoma originates from the fat pad around the sweat coils without proliferation of the sweat glands or ducts; this entity should therefore be denominated "perisudoral lipoma" rather than adenolipoma. It has to be differentiated from the skin tags with a fatty stroma (fibrolipomas) and from the superficial lipomatous hamartomas.

Adult↗

[Rapid diagnosis of cutaneous leishmaniasis and histoplasmosis by direct microscopic tests].

BACKGROUND: Cutaneous leishmaniasis and disseminated histoplasmosis can be diagnosed by direct examination of pathology specimens after Giemsa staining. CASE REPORT: An HIV-infected man developed cutaneous leishmaniasis and disseminated histoplasmosis with buccal lesions concomitantly. Smears stained with the RAL 555 kit provided the diagnosis of these two diseases. DISCUSSION: This case illustrates how direct microscopic examinations can provide inexpensive, rapid and safe diagnosis of infectious diseases. Direct microscopic examinations are routine practice in a tropical dermatology unit.

Cytodiagnosis↗

[Collagenolysis above eccrine spiradenoma].

BACKGROUND: The casual observation of a peritumoral edema with collagenolysis in a case of eccrine spiradenoma led us to search for similar lesions in a retrospective series of 50 spiradenomas. MATERIAL AND METHODS: Among 50 cases of eccrine spiradenomas, 36 excised with the overlying epidermis and the surrounding dermis were finally available for a conventional histologie study. RESULTS: In 14 cases, i.e. 39p. 100, we observed an edema above the tumour with thin and sparse collagen fibers and less altered elastic fibers. The clinical presentation of these 14 cases was not different from the general presentation of eccrine spiradenomas, but no precise photographical or clinical data were available. DISCUSSION: The cause of this epitumoral collagenolysis is unknown. It seems unrelated to the clear perivascular spaces often observed within the tumour lobules. It is of no help for diagnosis and may be considered as an unexplained curiosity like the elastolysis and the anetoderma seen above some pilomatricomas.

Adenoma, Sweat Gland↗