摘要:

Bullous pemphigoid (BP) is an acquired bullous disorder that predominantly affects the elderly. It is rare in children but when it occurs, there is considerable clinical and histologic overlap with other acquired or congenital blistering disorders. A definitive diagnosis of childhood BP requires direct immunofluorescence and, in some cases, characterization of the target antigen. Three cases of childhood BP are presented, with their histologic and immunofluorescence findings. The first was a 5-month-old male infant who presented with erythema and bullae of the palms and soles and was found to have linear deposition of IgG and C3 along the dermoepidermal junction on direct immunofluorescence (DIF). Histopathologic examination revealed a subepidermal blister containing eosinophils. Type IV collagen was demonstrated along the floor of the blister cavity by a direct immunoperoxidase technique. The second case was an 8-month-old female infant who presented with a blistering eruption of her palms and soles that then became widespread. Direct immunofluorescence showed linear IgG and C3 at the dermoepidermal junction, with laminin deposition at the base of the blister. The third case was a 7-year-old female with bullae and erosions on the vulva and vaginal mucosa. A subepidermal blister was seen on microscopic examination whereas immunofluorescence demonstrated linear IgG and C3 deposition at the basement membrane zone (BMZ). A literature review uncovered 50 cases of childhood BP confirmed by direct or indirect immunofluorescence, or both, and often with evidence of autoantibodies against either the 180 kD or the 230 kD human bullous pemphigoid antigens (BP180 or BP230). This review was used to delineate characteristics of childhood BP, including the newly proposed subtypes: infantile BP and childhood localized vulval BP. Infantile BP presents within the first year of life and is characterized by BP-like lesions on erythematous or normal acral skin. Localized vulval BP is a self-limited, nonscarring BP-like process that involves only the vulva. Both subtypes are normally self-limited and respond well to either topical or systemic steroids, if treatment is initiated before the disease becomes widespread.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Differentiation between malignant melanomas (MMs) and benign nevi based on histologic features can sometimes be difficult. This study evaluated the diagnostic effectiveness of argyrophilic staining of nucleolar organizer regions (AgNORs) in separating benign nevi from MMs by assessing 27 compound nevi (CN), 20 dysplastic nevi (DN), 10 Spitz nevi (SN), and 24 MMs. Both AgNOR count and morphology variables were measured from the superficial, middle, and deep zones of the lesions using video image analysis. Malignant melanomas had a significantly greater AgNOR number per nucleus, mean AgNOR area per nucleus, and variation in AgNOR area per nucleus compared with all types of benign nevi (p< 0.05). In multivariate discriminant analysis using a combination of four AgNOR counts and morphometric parameters, all CN and DN, 8 of 10 SN, and 23 of 24 MMs could be correctly classified as benign or malignant. The results suggest that both AgNOR count and morphology help to separate benign and malignant melanocytic lesions and that the combination of both sets of parameters improves their discriminating ability.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Electron microscopic examination still is the gold standard for classifying epidermolysis bullosa, although it is relatively expensive, time consuming, and not readily available. Immunoreagents have been developed recently to map antigens in the basement membrane on routinely processed specimens. The current study was performed to examine the diagnostic usefulness of immunohistochemistry, as compared with electron microscopic examination, for analyzing routine formalin-fixed paraffin-embedded sections of epidermolysis bullosa. This study investigated 39 consecutively diagnosed cases of epidermolysis bullosa in which both electron microscopic examination and immunohistochemistry were used. In each case, three monoclonal antibodies were used to stain for laminin 1, collagen IV, and keratin. The immunohistochemical patterns were defined as follows: epidermolysis bullosa simplex (laminin, collagen IV, or both at the dermal floor of the blister and keratin at both the dermal floor and the epidermal roof), junctional epidermolysis bullosa (laminin, collagen IV, or both at the dermal floor of the blister and keratin only at the epidermal roof), and dystrophic epidermolysis bullosa (collagen IV, laminin, or both, and keratin all at the epidermal roof). Altogether, electron microscopic examination subclassified epidermolysis bullosa into its three major forms in 37 of the 39 cases (95%), and immunohistochemistry in 33 of the 39 cases (85%). All of the classifiable cases were concordant. Specifically, immunohistochemistry was diagnostic in 10 of 14 (71%) epidermolysis bullosa simplex cases, 14 of 14 (100%) junctional epidermolysis bullosa cases, and 9 of 11 (82%) dystrophic epidermolysis bullosa cases. The most frequent cause for inconclusive immunohistochemical results was failure in staining of the basement membrane with the antibodies to both laminin and collagen IV. In conclusion, the use of immunohistochemistry on routinely processed specimens may be useful for subclassifying epidermolysis bullosa into its major forms in the majority of the cases, although it still cannot fully replace electron microscopic examination or immunofluorescence mapping in the diagnosis of epidermolysis bullosa.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Nephrogenic fibrosing dermopathy (NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. It appears similar to scleromyxedema but with some notable exceptions, including the lack of involvement of the face and absence of plasma cells on histology, systemic involvement, and paraproteinemia. Patients can present with thickened or edematous skin with indurated papules and plaques involving the extremities and the trunk. We report the first three cases of NFD after liver transplantation successfully treated with plasmapheresis. Two patients underwent liver transplantation for hepatitis C virus–induced cirrhosis and one for hepatitis B virus–induced cirrhosis. All the patients had encephalopathy, refractory ascites, and malnutrition prior to transplantation. Like those patients with NFD, all three of our patients had renal dysfunction and required hemodialysis before and after transplantation. Two were not dependent on dialysis at the time of diagnosis, however. These patients had excellent liver allograft function, but the other patient had allograft failure secondary to recurrent hepatitis C. Immunosuppression therapy consisted of basiliximab, mycophenolate mofetil, calcineurin inhibitor, and prednisone. The patients developed “woody” skin induration of the distal extremities, erythematous papules, and contractures at 1, 2, and 120 months after transplantation. Skin biopsies resembled NFD. No paraproteinemia was evident. One to three 5-day courses of plasmapheresis resulted in moderate to marked clinical improvement. The improvement of the kidney function in two of our patients did not appear to correlate with that of the skin disorder, because the kidney function was improving at the time the diagnosis of NFD was made. In conclusion, we report the first three cases of NFD after liver transplantation. Plasmapheresis was moderately successful in resolving the skin-indurated papules, severe skin induration, and associated joint contractures. Preliminary studies (unpublished data) show that decreasing plasma levels of transforming growth factor-&bgr;1 after plasmapheresis appear to correlate with the amelioration of this clinical condition.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Eleven cases of seborrheic keratoses with desmoplastic stroma and associated nests and cords of squamous epithelium simulating infiltrating carcinoma are presented. There were 7 males and 4 females ranging in age from 44 to 88 years (mean = 62). Eight cases were on the head and neck, one each on the lower and upper extremity, and one on the pubic area. Clinical diagnoses included seborrheic keratosis, squamous and basal cell carcinoma, and dermal nevus. Histologically, the lesions were characterized by exophytic growth pattern of basaloid and squamous cells, without cytologic atypia, and well-demarcated peripheral borders, typical for that seen in seborrheic keratoses. Squamous eddy formation with parakeratosis and spongiosis was present in all cases. Located within the body of the lesions were irregular nests and cords of squamous cells extending into the surrounding dermis with associated fibroblastic dermal proliferation trapping the epithelial nests, simulating invasive carcinoma. Immunohistochemically, the epithelial nests were cytokeratin positive and HPV negative, and the dermal stromal cells were vimentin positive and factor XIIIa, cytokeratin, and CD34 negative. Adjacent changes of carcinoma, trichilemmoma, verruca, or other adnexal neoplasm were not identified. Seborrheic keratoses may demonstrate desmoplastic changes analogous to that seen in desmoplastic trichilemmomas. Awareness of these changes in seborrheic keratosis will avoid misdiagnosis and excessive therapy.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

In tissue counter analysis, complex histologic sections are overlaid with regularly distributed measuring masks of equal size and shape, and the digital contents of each mask (or tissue element) are evaluated by gray level, color, and texture parameters. In this study, the feasibility of tissue counter analysis and classification and regression trees for the quantitative evaluation of skin biopsies was assessed. From 100 randomly selected skin biopsies, a learning set of tissue elements was created, differentiating between cellular elements, collagenous elements of the reticular dermis, fatty elements and other tissue components. Classification and regression trees based on the learning set were used to automatically classify tissue elements in samples of normal skin, benign common nevi, malignant melanoma, molluscum contagiosum, seborrheic keratosis, epidermoid cysts, basal cell carcinoma, and scleroderma. The procedure yielded reproducible assessments of the relative amounts of tissue components in various diagnostic groups. Furthermore, a reliable diagnostic separation of molluscum contagiosum versus normal skin and epidermal cysts, benign common nevi versus malignant melanoma, and seborrheic keratosis versus basal cell carcinoma was possible. Tissue counter analysis combined with classification and regression trees may be a suitable approach to the fully automated analysis of histologic sections of skin biopsies.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Our series was comprised of 11 children age 10 years or younger (6 were younger than age 5) with primary cutaneous melanoma. All of the melanomas occurred de novo and all metastasized; one child died. In no instance was melanoma a clinical consideration, and in none did the histopathologist who first “signed out” the case make a diagnosis of melanoma. Despite the inability of clinicians and pathologists to diagnose correctly, with repeatability, melanomas that develop in children yet to be pubescent, those neoplasms, nonetheless, are melanomas and, therefore, criteria employed currently for diagnosis of melanoma, especially clinically, must be refined in order that they be applicable equally to melanomas in pre- and postpubescents. The vaunted ABCDs (Asymmetry, Border irregular, Color variability, Diameter >6.0mm) surely do not work for melanomas that appear in children who are prepubescent. Additionally, melanomas that occur in these children have distinctly different architectural and cytopathological features from those that arise in postpubescents, often being confused as they are by conventional microscopy with a Spitz's nevus. As a rule, melanomas in prepubescent children grow much more rapidly then those in adults but, like them, have the capability to disseminate widely and cause death.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Primary cutaneous ganglioneuroma is a rare neoplasm first described by Collins et al. in 1972. Eight cases have been reported in the English literature to date. We report two cases of solitary cutaneous ganglioneuroma, one constituting, to our knowledge, the first reported case of this entity occurring on the face. Clinically, both lesions were firm flesh-colored papules and asymptomatic, being removed primarily for cosmesis. Neither patient had any significant past medical history. Histologically, both neoplasms consisted of relatively well-circumscribed spindle cell proliferations of axons and Schwann cells with interspersed mature ganglion cells. There was also focal myxoid change. The spindle cell component stained positively for S-100 protein, and the ganglion cells stained positively for glial fibrillary acidic protein and neurofilaments by routine immunohistochemistry.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Human papillomavirus (HPV) types 57 and 60 are associated with epidermal cysts of palmoplantar location (PPECs). Recently, there was a report of HPV 60 in a wart located in a nonpalmoplantar area as a possible precursor of a nonpalmoplantar epidermal cyst (NPPEC). There has been no study that has examined the presence and frequency of HPV in ordinary NPPECs. We reviewed 63 cases of epidermal cysts, including 59 NPPECs and 4 PPECs. After routine histopathologic review, polymerase chain reaction (PCR) of extracted DNA, sequencing of PCR products, and, finally, a homology search were undertaken. As a result, 9 of 63 (14.3%) cysts, including 6 of 59 NPPECs (10.2%), showed a positive reaction on PCR. By homology search after PCR, all were demonstrated as HPV 60. For NPPECs, no variables, including some histopathologic features, are significantly related to the presence of HPV 60 in univariate or multivariate analysis (P> 0.05).

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

A 51-year-old white woman presented with thickening of the scalp located at the vertex and left lateral occiput without hair abnormalities or alopecia. Skin biopsies of the thickened scalp showed thickening of the subcutaneous tissue with proliferation of mature subcutaneous fat cells but no signs of inflammation or hair abnormalities. During 2.5 years of follow-up, scalp thickening progressed over the entire hair-bearing scalp and persisted without signs of further progression at 3.5 year follow-up. Lipedematous scalp is an extremely rare diagnosis. It is defined by a thickening of the subcutaneous layer of the scalp and can be distinguished from lipedematous alopecia, in which subcutaneous thickening is associated with diffuse alopecia and shortening of scalp hairs. A total of seven cases of lipedematous alopecia and two cases of lipedematous scalp have been reported. We report the third case of lipedematous scalp in a 51-year-old white woman associated with early symptoms of meningitis. Additional features described in the literature include pruritus, pain, and paresthesia of the scalp as well as associated medical problems such as hyperelasticity of skin and laxity of joints, renal failure, and diabetes mellitus. This sporadic disorder is predominantly located at the vertex and occiput. The etiology and pathogenesis of lipedematous scalp and alopecia remain unclear. The treatment is symptomatic.

ISSN:0193-1091    

出版商:OVID    

年代:2003

数据来源: OVID