1/10. Bullous amyloidosis: the mechanism of blister formation revealed by electron microscopy.BACKGROUND: Few electron microscopic studies of blister lesions in bullous amyloidosis have been reported, and the mechanism of blister formation remains to be elucidated. This study was designed to examine the nature of bullous amyloidosis ultrastructurally, and clarify the pathogenesis of blister formation. methods: We examined a 47-year-old woman with IgD-lambda type myeloma, suffering from bullous lesions on her hands and feet caused by trauma or rubbing. light and electron microscopic studies were performed. RESULT: Ultrastructurally, amyloid deposits aggregated under the lamina densa. Keratinocyte protrusions penetrated the dermis through the gap in the lamina densa and enfolded amyloid deposits. Amyloid globules were found in enlarged intercellular spaces of keratinocytes. desmosomes were sparsely distributed in some areas of the epidermis. CONCLUSION: These results indicate that keratinocytes enfold the amyloid globules and take them in the intercellular space of epidermis, and that the breakdown of the lamina densa and widening of the intercellular space between keratinocytes induce skin fragility. Trauma or rubbing of her hands and feet appears to act as the localized precipitating factor of blister formation in bullous amyloidosis.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
2/10. Blistering distal dactylitis: a case series in children under nine months of age.Blistering distal dactylitis (BDD) is a distinct clinical entity that is infrequently reported in the literature. Characteristically, blistering distal dactylitis is described as a localized infection involving the volar fat pad of the distal phalanx of the digits, and it usually presents as a fluid-filled blister. The usual causative organism is group A beta-hemolytic streptococcus, but less commonly, Staphyloccous aureus and Staphyloccoccus epidermis are present. The normal age range is reported to be 2 to 16 years old, but there are case reports of this infection in adults. Only one case has been reported in the literature in a child younger than 24 months of age. In this report we describe three cases in children younger than 9 months old. These three cases indicate that BDD can and does occur in children younger than 2 years of age.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
3/10. Early congenital syphilis and erythema multiforme-like bullous targetoid lesions in a 1-day-old newborn: detection of treponema pallidum genomic DNA from the targetoid plaque using nested polymerase chain reaction.A 1-day-old male newborn was born with respiratory distress, low birth weight, hepatosplenomegaly, and bullous targetoid skin lesions over the face, back, buttocks, and extremities. A diagnosis of early congenital syphilis was made based on a treponemal serologic test. Pathologic examination of the skin lesion showed scattered dyskeratotic cells in the epidermis and interface dermatitis consistent with erythema multiforme. No spirochete could be found in the skin sections staining with Warthin-Starry stain. Using nested polymerase chain reaction, treponemal genomic DNA fragments encoding dna polymerase i were detected.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
4/10. Peeling skin syndrome: 11 cases from saudi arabia.BACKGROUND: Peeling skin syndrome (PSS) is a rare genodermatoses of probable autosomal recessive inheritance. In saudi arabia, consanguinity of parents is common and consequently the occurrence of familial disease, including that of the skin, is not uncommon. methods: To characterize the clinical and pathological features of PSS in saudi arabia, we reviewed the medical records and clinical photographs of patients with recurring blistering diseases and conducted a histopathologic evaluation of skin biopsies to identify the site of cleavage. RESULTS: Eleven patients with PSS were seen at King Khalid National Guard Hospital in Jeddah between the years 1986 and 2005. Ages ranged between 2 and 15 years and there were 9 males (81.8%) and 2 females (18.2%). The most common presentation in the majority of patients was localized spontaneous peeling of the skin. Eight patients (72.7%) had a history of vesicles that were small, dry and peeled away. Trauma did not play a role in blister formation. All patients were local from Bedouin tribes where a family history of a similar complaint was documented in 8 cases (72.7%) and consanguinity of marriage was evident in 6 patients (54.5%). Histological examination of skin biopsies showed either intracorneal or superficial subcorneal cleavage above the granular layer in all biopsied patients. CONCLUSION: Although rare, PSS occurs in saudi arabia and is most likely related to consanguinity of marriages. This disease is generally mild and is characterized by intracorneal cleavage within the superficial epidermis. The disease should be recognized and not confused with other vesicobullous disease.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
5/10. IgA pemphigus foliaceus. Report of two cases and a review of the literature.The cases of two patients with vesiculobullous lesions were diagnosed clinically and histopathologically as pemphigus foliaceus; unexpectedly, both revealed intercellular IgA, but not IgG, in the upper epidermis by direct immunofluorescence. Such histologic and immunofluorescence findings have been reported in eight other cases. In our cases no circulating IgA or IgG intercellular antibodies could be detected; in four of eight other reported cases IgA antibodies showed intercellular staining like that of pemphigus antibodies. Subcorneal acantholytic lesions occurred in both our cases; of the other cases reported, five had essentially identical histopathologic findings. The clinical and histopathologic features of pemphigus, as well as the recent findings of circulating IgA intercellular antibodies alone or with IgG antibodies, appear to place this disease into the spectrum of pemphigus. The 10 IgA pemphigus cases reported to date fall into one of two groups, the IgA pemphigus foliaceus (including our two cases) and IgA pemphigus of the intraepidermal neutrophilic type, which seems to be less common.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
6/10. Intracorneal nuclear dust aggregates in dermatitis herpetiformis. A clue to diagnosis.dermatitis herpetiformis has a characteristic histologic pattern consisting of subepidermal blisters often containing fibrin, infiltrates of neutrophils and nuclear dust at tips of dermal papillae, and papillary dermal edema. These are features of early and evolving lesions. We present two cases of clinically typical dermatitis herpetiformis with previously unreported histologic features that may provide a significant diagnostic clue. In each of these cases there were focal collections of nuclear dust in the cornified layer of the epidermis, a finding that may represent a resolving phase of dermatitis herpetiformis, beyond the usual papillary dermal neutrophilic microabscesses seen in early lesions. biopsy material was available for immunofluorescent studies in one of the cases presented. In addition to the granular pattern of IgA positivity at the dermal-epidermal junction, which is diagnostic of dermatitis herpetiformis, this biopsy also showed similar IgA positivity in the intracorneal nuclear dust aggregates. In the second case, initial sections showed only intracorneal nuclear dust, but at deeper levels there were more typical diagnostic microabscesses at the tips of dermal papillae.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
7/10. lichen planus presenting with erythema-multiforme-like bullous lesions in a patient with systemic scleroderma.A patient with systemic scleroderma is described who developed a cutaneous eruption of papules and vesicobullae. Over time, the latter turned into papules. The histopathological and immunofluorescence features of the papular lesions were unequivocally those of lichen planus, while those of the bullous lesions reminded of erythema multiforme. Histologically, erythema multiforme shares common features with lichenoid reactions, such as necrotic keratinocytes. Our case suggests that erythema multiforme and lichen planus may coexist or succeed each other as different stereotype immune reactions against the same antigen(s) within the epidermis.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
8/10. blister formation over a soft fibroma of the nipple.We report a case of soft fibroma on the nipple which was accompanied by a blister. Histologically, a subepidermal blister overlying the tumor and the degeneration of the lower part of the epidermis were observed. The causes of blister formation are briefly discussed.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
9/10. Three neonatal cases of epidermolysis bullosa herpetiformis (Dowling-Meara type) with severe erosive skin lesions.We describe three neonates who had large eroded areas of skin on their extremities. The clinical course and ultrastructural findings were consistent with a diagnosis of epidermolysis bullosa herpetiformis (Dowling-Meara type). In each case blisters developed around eroded areas after birth and enlarged centrifugally in a herpetiform fashion. One patient died of sepsis at 8 days of age. In the two survivors blister formation subsided gradually within 1 year. Ultrastructural studies confirmed intraepidermal blister formation associated with spheric aggregates of tonofilaments in the lower epidermis. Spheric aggregates were also found in clinically uninvolved skin.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
10/10. Bullous lesions in Kaposi's sarcoma: case report.Bullous lesions have been only rarely described in Kaposi's sarcoma (KS), and their histopathologic features have never been described in detail. We report a case of bullous lesions of KS in an 82-year-old Italian woman. The patient had typical smooth pale reddish-grey slightly-raised KS plaques on the legs, present for at least 10 years. Several dull grayish-pink blisters (0.5 to 2 cm in diameter) affected both dorsa of her feet and ankles symmetrically. Two punch biopsies were taken, one from an infiltrated KS plaque on the right buttock and the other from a bullous lesion on the right foot. Histopathologically, the late KS plaque on the buttock showed typical features of KS, with an increased number of spindle cells arranged in short bundles and extravasation of erythrocytes. The bullous lesion on the foot showed a full-thickness vascular neoplasm involving the upper and lower dermis and the subcutaneous fat. The upper portion of the lesion contained many newly formed, highly-dilated blood vessels, touching the overlying epidermis and separated from it by a narrow band of collagen and endothelial cells; wide, empty spaces characterized the superficial dermis, in which preexisting venules and bands of collagen associated with non-atypical endothelial cells floated. All these findings would suggest a lymphangiomatous lesion, if the presence of specific diagnostic criteria of KS were not recognizable at a deeper level of the lesion. Various criteria actually suggest that the bullous lesion may be regarded as an epiphenomenon of a KS plaque lesion: (a) full-thickness involvement of the reticular dermis and, in this case, also of the subcutaneous fat; (b) dense and patchy lymphoplasmocytic infiltrate typical of plaque lesions and, much less frequently, of patch lesions; (c) presence of ectatic blood vessels, filled with plasma and erythrocytes (pseudoangiomatous findings), a nonpathognomonic but highly characteristic finding of the plaque lesion; and (d) as in the KS plaque lesions, in the bullous lesion as well the reticular dermis was characterized by an increased number of anastomosing bizarrely shaped vascular spaces lined by non-atypical endothelial cells. We hypothesize that the prevalence of lymphangiomatous differentiation in the upper dermis represents one of the many features of KS lesions. When present, it may correlate with the clinical feature of a blister.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
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