Psoriasis after cord blood stem cell transplantation.
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Child↗
Biomedical subjects
S Lepreux
Publications and source records attributed to S Lepreux.
Skin ultrastructure in senile lentigo.
Senile lentigo is a common component of photoaged skin. It is characterized by hyperpigmented macules which affect chronically irradiated skin mostly after the age of 50. This study was undertaken to assess the morphology of senile lentigo on the dorsum of the hands. A systematic comparison between lesional and perilesional skin using histology and transmission electron microscopy was done to determine whether melanocytes or keratinocytes are affected in the evolution of lesions and which tissue structure is modified. The histology study showed that lesional skin is characterized by a hyperpigmented basal layer and an elongation of the rete ridges, which seem to drive deeply into the dermis. The epidermis contained clusters of keratinocytes, which retained and accumulated the melanin pigment. Electron microscopy studies showed important modifications in the lesional skin ultrastructure in comparison with perilesional skin. In melanocytes from perilesional and lesional skin, we observed normal size melanosomes at all stages of maturation in the cytoplasm and in migration within dendrites. No pigment accumulation was observed. However, the morphology of melanocytes in lesional skin revealed an activated status with numerous mitochondria and a well-developed endoplasmic reticulum, which could reflect intense protein synthesis. In basal keratinocytes from lesional skin, we observed numerous melanosome complexes called polymelanosomes, which formed massive caps on the nuclei. Observations in colored semi-thin sections also revealed perturbed structures in the basal layer region, which could explain the skin perturbation. Indeed, we observed keratinocytes that presented important microinvaginations and pendulum melanocytes, which sank into the dermis, beneath the basal layer of keratinocytes. These cell modifications seemed to be due to a perturbation of the dermal-epidermal junction, which appeared disorganized and disrupted and could directly disturb the basal support of the cells.
Aged↗
Lymphomatoid annular erythema: a new form of juvenile mycosis fungoides.
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Child↗
Hereditary mucoepithelial dysplasia: clinical, ultrastructural and genetic study of eight patients and literature review.
BACKGROUND: Hereditary mucoepithelial dysplasia is a dominantly inherited disease, mainly characterized by chronic mucosal lesions associated with keratitis, non-scarring alopecia, keratosis pilaris and perineal intertrigo. Since the original report by Witkop, this condition has been considered to be a disorder of desmosome/gap junction formation, but there has been no ex vivo investigation of these components using genetic and immunolabelling techniques. OBJECTIVES: To perform light and immunoelectron microscopic studies, and partial genetic analysis on five patients in a family and three sporadic cases and to point out similarities of this rare disorder with chronic mucocutaneous candidiasis and other follicular keratosis syndromes, i.e. ichthyosis follicularis-alopecia-photophobia (IFAP), keratitis-ichthyosis-deafness (KID) and Siemens syndromes. METHODS: Biopsies from the involved oral mucosa and armpit skin of patient 1 were prepared for standard histopathology, electron microscopy and immunocytochemistry. Microsatellite genotyping was performed in three affected family members. Direct sequencing after polymerase chain reaction amplification of the entire coding region was performed. RESULTS: A 14-year-old male had recurrent keratitis, widespread keratosis pilaris, perineal intertrigo, hypotrichosis and oral mucosal involvement. A similar phenotype was noted in four members of his family and in three sporadic cases. Histological examination of oral mucosa and skin samples showed a psoriasiform pattern, dyskeratotic features and cytoplasmic vacuoles. Expression of connexins (Cx), desmosomal, adherens junction and cytoskeleton proteins (Cx 26, 32 and 43, desmogleins 1 and 2, plakoglobin, desmoplakins I-II, plakophilin 1, beta-catenin, E-cadherin, keratins, beta-tubulin, vimentin and actin) was normal. Ultrastructural studies showed a reduced number of desmosomes. Dyskeratotic cells exhibited internalized gap junctions, long filamentous inclusions reactive with antikeratin antibodies, and bundles of perinuclear fibres resembling clear tonofilaments. Genetic analysis in the studied family excluded the desmosomal cadherins in chromosome 18q12 as candidate genes. CONCLUSIONS: A diagnosis of hereditary mucoepithelial dysplasia should be strongly suggested by the triad of non-scarring alopecia, well-demarcated erythema of oral mucosa and psoriasiform perineal rash, after exclusion of the clinically related follicular keratosis syndromes. Defective expression of cytoskeleton elements and/or a modification of mechanisms regulating junction-cytoskeleton assembly may be primarily responsible for impaired epithelial cohesion.
Adolescent↗
Mucosal pigmentation after oral lichen planus treatment with topical tacrolimus.
Erosive oral lichen planus (OLP) is a painful chronic inflammatory disease that is sometimes resistant to systemic or topical therapies. Topical steroids remain the mainstay of therapy, but topical tacrolimus has recently been used to treat OLP resistant to topical corticosteroids. Topical tacrolimus appears as an effective and safe treatment of symptomatic OLP. We report the first histopathologically documented case of oral mucosa pigmentation after OLP treatment with topical tacrolimus. The relation between tacrolimus treatment and staining was suggested by the appearance of pigmentation during topical tacrolimus treatment and its clinical disappearance when treatment was stopped. Histopathology showed an increase in melanocyte numbers and melanogenesis.
Administration, Cutaneous↗
[Eccrine squamous syringometaplasia and cytomegalovirus infection].
BACKGROUND: Eccrine squamous syringometaplasia has been reported in some cases as an Herpeviridae complication. We report a case of eccrine squamous syringometaplasia associated with a severe cytomegalovirus infection in an immunocompromised patient, and we discuss about potential viral or drug triggering factors. METHODS: A 22 years-old man was hospitalized in an intensive care unit for rejection of a renal graft associated with a disseminated cytomegalovirus infection. A papular and papulopustular eruption appeared on the trunk and the limbs. RESULTS: Histological examination of a skin sample showed eccrine squamous syringometaplasia, with evidence of cytomegalovirus genomic sequences using PCR. Two weeks later, the patient developed toxic epidermal necrolysis, with fatal issue. CONCLUSIONS: Eccrine squamous syringometaplasia is a rare condition, without specific clinical features. Numerous local affections have been reported to induce eccrine syringometaplasia (ulcer, scar, pyoderma gangrenosum, drug injection.), drugs (cytotoxic agents, non steroidal anti inflammatory therapies) and in cases of infection due to cytomegalovirus or herpes simplex virus. The potential implication of cytomegalovirus or foscarnet as triggering factors in our case is discussed. This observation and other similar reported cases lead to the conclusion that eccrine squamous syringometaplasia may be an underestimated complication of cytomegalovirus infections in immunocompromised patients.
Adult↗
Melanocyte detachment after skin friction in non lesional skin of patients with generalized vitiligo.
BACKGROUND: In vitiligo, melanocytes are gradually lost in depigmented macules of the skin. The disappearance of melanocytes has, however, not been clearly observed and consequently the aetiology of the disease (autoimmune, neural, cytotoxic) is still elusive. The starting point of vitiligo macules is frequently determined by local conditions such as wounds and excoriations, but may also follow minor traumas such as pressure or repeated friction. This prominent feature is often neglected. OBJECTIVES: To clarify the biological consequences of repeated friction on the attachment and survival of melanocytes in non lesional vitiligo skin. METHODS: Light reproducible skin friction was performed for 4 min on the volar forearm of 18 patients with extensive vitiligo and five controls with normal healthy skin. Biopsies from the test area and control skin were taken at 1, 4, 24 and 48 h following friction. Serial sections were examined with standard light microscopy, transmission electron microscopy, histochemistry and immunohistochemistry (dihydroxyphenylalanine, HMB-45, E-cadherin and an early apoptosis marker, M30 cytoDEATH antibody). RESULTS: The observation of sections at 1 and 48 h after friction on vitiligo skin and at all time points in controls revealed no changes. In contrast, in vitiligo skin at 4 and 24 h after friction, several melanocytes had undergone detachment and were found in various suprabasal positions, including the stratum spinosum, granular layer, and within and outside the stratum corneum. CONCLUSIONS: Detachment and transepidermal elimination of melanocytes following minor mechanical trauma in non lesional vitiligo skin is probably the cause of depigmentation occurring in the isomorphic response (Koebner phenomenon). We propose that transepidermal elimination of melanocytes in vitiligo should be regarded as a possible mechanism of chronic loss of pigment cells, perhaps previously damaged by another process.
Adult↗
[Kawasaki's disease with eruptive pustular and guttate psoriasis].
BACKGROUND: Kawasaki's disease may have numerous atypical forms and these must be recognized in order to avoid delay of treatment. We report a case of psoriasis, first pustular and then guttate, occurring during Kawasaki's disease, and discuss a common pathophysiological mechanism. CASE-REPORT: A 3 year-old boy was seen for a febrile exanthema suggestive of Kawasaki disease (bilateral conjunctivitis, red and fissured lips, palmoplantar erythema, scarlet fever-like rash and perineal desquamation) associated with pustular lesions. A biopsy specimen of a pustular area showed histological features consistent with the diagnosis of pustular psoriasis. No coronary abnormality was found. The child was treated with intravenous immunoglobulins (2 g/kg) and oral aspirin (60 mg/kg/d). All the symptoms disappeared and immediate follow-up was marked by the appearance of guttate psoriasis. DISCUSSION: Onset of psoriatic lesions during Kawasaki disease has been reported in 12 cases, either in acute phase or in immediate follow-up. Coronary complications have been found in 4 of 5 cases with acute psoriasis, suggesting a severe prognosis for this association. The hypothesis of a common pathophysiological mechanism is discussed with the intervention of a bacterial toxin acting as a superantigen and resulting in an strong activation of T-cells that leads to keratinocyte activation. The psoriatic lesions could hence be considered as a form of Köbner's phenomenon.
Anti-Inflammatory Agents, Non-Steroidal↗
[Cocaine-related localized necrotic livedo].
INTRODUCTION: The cutaneous signs of toxicomania are better known since the last two decades. We describe an original case of segmental necrotic and chronic livedo of the right arm associated with cocaine use. OBSERVATION: A 31 year-old intravenous cocaine user presented a necrotic and painful livedo of the right arm. Two years before referral, localized recurrent edema was noted without general involvement. No local (infectious, vascular) or general (dysimmunity, coagulation abnormalities) aetiologies could be found. An amorphic substance composed of mineral bodies was found in the dermis, without damage to the vessels. Dramatic improvement was obtained with simple supportive care, while intravenous injections were stopped. DISCUSSION: The vasoconstrictive properties of cocaine have been demonstrated in Raynaud's phenomenon, ischemia, focal necrosis of the extremities, and also in liver and kidney disorders. This drug has pharmacological effects on endothelial cells and coagulation, explaining the arterial and venous thrombosis reported. The foreign bodies found in our patient probably came from an intraarterial injection. The progressive and chronic evolution, without any evidence of thrombosis at the time of examination, are suggestive of the vasoconstrictive action of chronic dermal deposits.
Adult↗
[Multiple and painful nodules of the chest].
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Acrospiroma↗
Expression and cellular localization of fibrillin-1 in normal and pathological human liver.
BACKGROUND: The expression and the distribution of fibrillin-1 and elastin were studied in normal and pathological human liver samples. METHODS: As controls, histologically normal/subnormal liver samples (n = 24) were used. Pathological samples corresponded to seven cirrhosis and eight hepatocellular carcinomas (HCC) developed on cirrhotic (four) or noncirrhotic (four) liver. RESULTS: In normal liver, fibrillin-1 and elastin co-localized in vessel walls and portal tract connective tissue. Fibrillin-1 alone was detected along sinusoids and in portal spaces at the interface with the limiting hepatocytic plates and close to the basement membrane of bile ducts. By transmission electron microscopy, typical bundles of microfibrils were detected both in Disse space and in portal zones. Cirrhotic nodules were usually rich in fibrillin-1 along sinusoids; fibrillin-1 and elastin were co-localized in fibrotic septa surrounding nodules. In HCC, fibrillin-1 was present between tumoral hepatocytes; stromal reaction around the tumors contained both fibrillin-1 and elastin. CONCLUSIONS: Fibrillin-1 was associated with elastin in portal mesenchyme and vessel walls of normal liver, in fibrotic septa around cirrhotic nodules and stromal reaction around HCC, but was expressed alone in the perisinusoidal space. The functional roles for fibrillin-1 in non-elastic tissues, such as the liver, remain to be elucidated.
Adult↗
Can hepatic stellate cells express alpha-smooth muscle actin in normal human liver?
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Actins↗
[Adenoma or focal nodular hyperplasia of the liver? A difficult pathological diagnosis].
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Adenoma↗
Activation of cultured rat hepatic stellate cells by tumoral hepatocytes.
Hepatocellular carcinoma (HCC) is the main type of primary liver cancer, and it develops from hepatocytes. The stroma of HCC is infiltrated by myofibroblasts. In other settings, such as liver fibrosis, myofibroblasts are derived mainly from the activation of hepatic stellate cells (HSC). In this study, we investigated whether tumoral hepatocytes were able to activate HSC. HSC were isolated from normal rats and were plated in dishes coated with Matrigel, to prevent their spontaneous activation. HSC were exposed to conditioned medium (CM) from the rat HCC lines Fao and H5. Tumor cell CM elicited major morphologic changes, such as spreading and generation of cytoplasmic processes. Fao and H5 CM increased HSC proliferation to 1.60 and 1.76 times control values, respectively. The expression of alpha-smooth muscle actin was low or undetectable in control cells and was markedly increased by both tumor cell CM but not by normal rat hepatocyte CM. Desmin expression was also enhanced. Gelatinase A secretion was significantly increased 1.20-fold by Fao CM and 1.55-fold by H5 CM. Expression of beta-type platelet-derived growth factor receptor mRNA was increased 5.8-fold by H5 CM but was decreased to 13% of control levels by Fao CM. HSC activation by tumor cell CM was not prevented by urokinase or matrix metalloproteinase inhibitors, suggesting that Matrigel degradation was not central to the activation process. Finally, a blocking antibody to transforming growth factor-beta1 did not impede Fao CM-induced activation but significantly blocked the increase in matrix metalloproteinase-2 expression induced by H5 CM. Our results show that tumoral rat hepatocyte CM is able to induce the activation of rat HSC in culture. The lack of induction of beta-type platelet-derived growth factor receptor mRNA by Fao CM indicates that, in some cases, tumor-induced activation differs from classic fibrosis-type activation. Our data thus suggest that HSC recruitment and activation in HCC could be under the control of tumor cells.
Animals↗
[Herpetic-like worsening of an epidermolysis bullosa simplex during pregnancy].
INTRODUCTION: Episodes of hereditary epidermolysis bullosa simplex are usually provoked by repeated cutaneous traumas or exposure to heat. We report a case of hereditary epidermolysis bullosa simplex worsened during pregnancy. OBSERVATION: A 21 year-old woman suffering from hereditary epidermolysis bullosa simplex presented with unusual symptoms in the form of an extensive vesicular-bullous eruption at two months of her first pregnancy. Standard histological examination revealed an eosinophilic infiltrate in the bullae. The global results of the initial examinations suggested a herpetic-like episode of epidermolysis bullosa as is observed during the Dowling-Meara form of the disease. DISCUSSION: Various physiopathological hypotheses can be advanced to explain the worsening of the eruption during pregnancy: a mechanical origin with scratching of pruritus, enhanced fragility of the inter-keratinocyte bridges secondary to edema provoked by salt-water retention or a loss in antigenic tolerance to muted keratins 5 and 14, as in pemphigoid gestationis. The presence of eosinophils in the dermis and epidermis may also participate directly in the formation of bullae due to the release of cationic proteins.
Adult↗