Superimposed linear psoriasis: differential therapeutic response of linear and nonlinear lesions

Linear psoriasis is a very unusual clinical variation of psoriasis. Typical clinical features include early onset of erythematosquamous lesions along Blaschko's lines, ability to elicit psoriatic features, absence of pruritus and positive family history for psoriasis. Recently, the term 'superimposed linear psoriasis' was coined for cases with development of nonlinear psoriatic lesions at predilection sites in later life. We report a 19-year-old woman meeting all criteria for the diagnosis of superimposed linear psoriasis including typical histological features. Remarkably, treatment with topical steroids and dithranol cleared the psoriatic lesions on predilection sites whereas the linear lesions were resistant to topical therapy. Linear psoriatic lesions are believed to be caused by genetic alterations in early embryogenesis leading to loss of heterozygosity at a gene locus involved in the pathogenesis of psoriasis. Comparison of mosaic keratinocytes derived from linear lesions with wild-type keratinocytes from the same person may therefore allow identification of key regulatory genes.     

银屑病患者皮损局部朗格汉斯细胞数量异常机制的研究

目的：探讨银屑病患者皮损局部朗格汉斯细胞(LCs)数量异常的原因。方法：培养银屑病患者皮损处角质形成细胞，通过微孔小室实验检测其上清液对单核细胞的趋化功能；通过酶联免疫吸附法(ELISA)检测上清液中单核细胞趋化蛋白—1(MCP-1)的表达。结果：银屑病患者皮损处角质形成细胞分泌上清液对单核细胞的趋化能力明显强于正常对照组；其分泌的MCP-1水平也高于正常人。结论：银屑病角质形成细胞表达的趋化因子趋化了更多数量的单核细胞至皮损局部，因此，银屑病患者皮损局部LCs的数量理应是增多的。但由于银屑病角质形成细胞表达的其它一些因子也促使了单核细胞衍生的LCs的活化，活化的LCs会迁移至淋巴结或活化后凋亡，又导致其数量减少。因此，银屑病患者皮损局部朗格汉斯细胞数量是一动态变化过程。     

Ultrastructural features of ichthyosis hystrix strongly resembling Lambert type

Ichthyosis hystrix (IH) is characterized by spiny hyperkeratotic scale, and includes Brocq type, Lambert type, Curth–Macklin type, Rheydt type and Bäfverstedt type. The first documented cases of familial IH were of Lambert type. However, the ultrastructural features of IH Lambert type have not been reported. Three patients in two generations of a family from north China were observed. The patients showed widespread verrucose lesions without blister formation. The face, palms and soles were unaffected. This presentation strongly resembled IH Lambert type. The lesions faded dramatically in summer, without treatment. Light microscopic examinations showed binuclear cells and shell formation in the granular and upper spinous layers in all specimens, with similar findings in winter, when lesions were prominent, and in summer, when lesions had subsided. Electron microscopic examination revealed binuclear keratinocytes and concentric, thin to thick, unbroken shells of tonofilaments surrounding the nuclei, and segregation of cytoplasmic components. This family is the first with familial IH strongly resembling Lambert type to be reported in China. Binuclear cells and tonofilaments shells surrounding the nucleus in upper keratinocytes were characteristic features, which were similar to those reported in IH Curth–Macklin type. The basic histopathological defects were not changed despite significant clinical improvement of the lesion.     

PIXE analysis in different stages of psoriatic skin

Elemental distribution in psoriatic skin varies with the functional state of the keratinocytes, e.g., electrolytes influence cell metabolism and cell proliferation, and trace elements play a crucial role in a great number of enzymes. Elemental distribution in pinpoint lesions, old plaques, and uninvolved skin of 5 psoriatic patients and 4 healthy controls was studied by means of PIXE (proton-induced x-ray emission) analysis. This technique allows the simultaneous detection of elements with an atomic number greater than or equal to 14 along the epidermis and dermis in freeze-dried skin biopsies. Trace elements such as Fe, Cu, and Zn were determined down to a level of 1 ppm. In comparison with uninvolved skin, concentrations of P and K were elevated in psoriatic epidermis. In addition, increased levels of K were correlated with the stage of the psoriatic lesion. Zinc concentrations were significantly elevated in pinpoint lesions. The Zn concentration profiles within the epidermis and upper dermis showed high correlation to the P concentration profiles. Iron levels were decreased in old psoriatic plaques, whereas Cu concentrations varied considerably. In comparison to the controls, Cl concentrations were markedly decreased in the dermis of involved and uninvolved psoriatic skin, whereas epidermal Cl levels were unaffected. As high K levels prevent the Ca-induced differentiation of keratinocytes, high K levels may be the cause of the high cell differentiation in psoriatic skin. Elevated DNA- and RNA-polymerases might be the cause of elevated Zn levels in pinpoint lesions.     

Intraepithelial mast cells in gingival lichen planus: an ultrastructural study.

Intraepithelial mast cells, identified by ultrastructural criteria, were seen in lesions of gingival lichen planus. The mast cells were found either singly, interspersed among keratinocytes, or in combination with other mononuclear cell types, especially lymphocytes. The mast cells were seen in regions with relatively normal intercellular spaces, as well as in regions of more severely disrupted keratinocytes. They had cytologic features indicative of active synthesis and release of granules. Moreover, the finding of centrioles in several intraepithelial mast cells, in combination with certain other cytologic features, suggested that these cells might be in an early stage of differentiation. It is speculated that intraepithelial mast cells have a role in the pathogenesis of gingival lichen planus.     

他克莫司软膏对银屑病皮损角质形成细胞增殖分化的影响

目的探讨他克莫司软膏对银屑病皮损处角质形成细胞增殖分化的影响。方法采用免疫组化的方法检测10例0.1%他克莫司软膏治疗前后寻常性银屑病(斑块型)患者的皮损组织及10例正常健康人的皮肤组织中Ki-67,CK10的水平,并进行比较。结果 0.1%他克莫司软膏治疗后银屑病皮损的表皮Ki-67的水平明显升高,CK10的水平明显降低,差异均有统计学意义(P均<0.05)。结论他克莫司软膏对银屑病皮损处角质形成细胞分化有显著的抑制作用,对角质形成细胞增殖有促进作用。     

慢性光化性皮炎真皮浸润细胞免疫表型及角朊细胞Fas抗原的表达

目的：研究慢性光化性皮炎（ＣＡＤ）不同时期皮损内浸润细胞免疫表型及Ｆａｓ抗原在角朊名的表达。方法：应用免疫组化技术分别检测了８例患者的早期皮损及９例患者的后期皮损内的真皮浸润细胞免疫表型；同时检测了表皮角朊细胞Ｆａｓ的表达。结果：早期及后期皮损内浸润细胞主要是Ｔ淋巴细胞戌后期皮损内浸润Ｔ细胞的亚群分布不同，早期以Ｔｈ／ｉ细胞为主，而后期则是Ｔｃ／ｓ细胞占多数。早、后期皮损角朊细胞均可表达Ｆａｓ抗原     

Immunohistopathologic studies in the development of psoriatic lesion influenced by gamma-interferon and the producing cells

Immunological studies on psoriasis have been done using monoclonal antibodies (MoAbs) to elucidate the relation between gamma-interferon (IFN-gamma) secreted from inflammatory infiltrate, and the expression of HLA-DR molecule on epidermal keratinocytes in the lesion. In highly inflamed psoriatic lesions, IFN-gamma producing cells were found in the inflammatory infiltrate of the dermal papillae, while keratinocytes located near IFN-gamma+ cells expressed HLA-DR molecules on the surface. In fully developed psoriatic lesions, HLA-DR+ keratinocytes were mainly found in the thin epidermis over the elongated dermal papillae where IFN-gamma deposited not only at infiltrates but also in teh dermal components. The IFN-gamma+ cells were activated T cells which exhibited HLA-DR and interleukin 2 receptor. Munro's microabscess under the horny layer also included IFN-gamma producing cells. A radioimmunoassay by the sandwich method with anti-human IFN-gamma and anti-recombinant IFN-gamma MoAbs found that extracts from psoriatic scales contained IFN-gamma. The results suggest that HLA-DR+ keratinocytes are due to the effect of IFN-gamma secreted by activated T cells in psoriatic lesions. The proliferation of keratinocytes over the dermal papillae in fully developed lesions seemed to be inhibited by IFN-gamma from the inflammatory infiltrate. We consider that cell-mediated immune reaction plays an important role in the development of psoriatic eruption.     

Dermoscopic features of actinic keratosis and follow up with dermoscopy: A pilot study

Actinic keratosis (AK) is a common precursor of sun‐related squamous cell carcinoma. AK is difficult to be differentiated from other malignancies with the naked eyes. Dermoscopic features of AK were previously described in some studies, but not extensively investigated. We investigated the dermoscopic features of AK in Asians and assessed dermoscopy as a post‐treatment monitoring tool of AK. We retrospectively examined 34 AK lesions which had been diagnosed by histology. The changes of dermoscopic features and histopathological findings were assessed in all these lesions before and after treatment. Before treatment, 18 lesions were pigmented and 16 lesions were non‐pigmented AK dermoscopically. The frequent dermoscopic features of AK were keratin/scales (79.4%), red pseudonetwork (73.5%), targetoid‐like appearance (55.9%), rosette sign (38.2%) and absent fissures/ridges, crypts and milia‐like cysts. All the lesions had been treated with either photodynamic therapy, cryotherapy or 5% imiquimod cream. After treatment, dermoscopic features of 33 AK lesions were decreased or disappeared, and skin biopsies confirmed that atypical keratinocytes disappeared. One lesion showed accentuated and new dermoscopic features after treatment, and skin biopsy also showed progressing squamous cell carcinoma. In conclusion, scales, red pseudonetwork, targetoid‐like appearance and rosette sign were common dermoscopic findings of AK in Asians. In most cases, the treatment response correlated with the changes in dermoscopic features. These findings suggest that dermoscopy is a useful tool to monitor AK.     

BRAF V599E Mutation is Not Age Dependent: It is Present in Common Melanocytic Nevi in Both Children and Adults

Pigmented dermatofibrosarcoma protuberans (Bednar tumor) constitute 5–10% of all cases of dermatofibrosarcoma protuberans, and are usually considered mimics of melanocytic proliferations rather than fibrous lesions. We report two cases of pigmented fibrous proliferations that demonstrate features of both dermatofibromas and DFSP. The first case is a 19‐year‐old man with a three year history of a slowly growing pigmented lesion on the right arm. On clinical exam the lesion was a 7 mm firm pigmented papulonodular lesion. The second case is a 31‐year‐old woman with a 4–5 year history of a slowly enlarging, asymptomatic 'dark area' on the right buttock. On clinical exam the lesion is a 2 cm darkly pigmented flat nodule. Morphologically both lesions are primarily dermal proliferations of spindled cells admixed with pigmented dendritic melanocytes. The lesional cells trap collagen fibers at the periphery and there is basal cell hyperpigmentation. Adnexal structures are effaced but significant trapping of subcutaneous fat is not present. By immunohistochemistry both lesions show focal CD34 positivity but are negative for Factor XIIIa and melanocytic markers. Although overlap between dermatofibromas and DFSP is well documented in the literature, pigmented fibrous lesions with features of both entities are not well described.     

Fas抗原及Fas配体在红斑狼疮患者皮损中的表达

目的 研究Ｆａｓ抗原及Ｆａｓ配体（Ｆａｓ－Ｌ）在红斑狼疮皮损中的表达情况。方法 应用免疫组化技术检测２５例系统性红斑狼疮（ＳＬＥ）及１４例盘状红斑狼疮（ＤＬＥ）不同病程的皮损中Ｆａｓ抗原及Ｆａｓ－Ｌ的表达。结果 ＳＬＥ及ＤＬＥ早期皮损朊细胞Ｆａｓ抗原表达强度显著高于正常皮肤（Ｐ〈０．０１）,与病程呈负相关（Ｐ〈０．０５）,且真皮中单一核细胞亦有Ｆａｓ抗原表达;红斑狼疮患者皮损及正常皮肤的角朊细胞均     

Pagetoid dyskeratosis of the lips

Pagetoid dyskeratosis is an incidental finding in a variety of lesions of the skin and squamous mucosa. The lesion is considered a selective keratinocytic response in which a small part of the normal population of keratinocytes is induced to proliferate in response to friction. As far as we know, pagetoid dyskeratosis has not been reported in the lips. In this article, we describe the location of the lesion in the lips and its incidence in a group of 90 unselected patients who underwent biopsy or were surgically treated for diverse labial lesions. Histochemical staining and immunohistochemical studies were performed in selected cases. Pagetoid dyskeratosis was found in 38 cases (42.2%) but only in 6 cases (6.7%) the lesion was conspicuous. There was no significant difference between the upper and the lower lip in terms of incidence of the lesion. Labial pagetoid dyskeratosis was more frequent in younger patients (46.7 +/- 25.0 versus 58.5 +/- 20.5; p < 0.05) and in women (chi(2) = 3.89; p < 0.05). Pagetoid cells were more common in suprabasal location and in the labial mucosa. These cells showed positivity for high-molecular weight cytokeratin and negative reaction for low-molecular weight cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, and human papilloma virus. The immunohistochemical profile is different from the surrounding keratinocytes, indicating premature keratinization. The main differential diagnoses include white sponge nevus, leukoedema, oral koilocytoses, hairy leukoplakia, pagetoid squamous cell carcinoma in situ, and extramammary Paget's disease of the oral mucosa. The morphologic features of dyskeratotic pagetoid cells are distinctive and easily recognized as an incidental finding, thus preventing confusion with other important entities including an intraepidermal tumor.     

Richner-Hanhart's syndrome. Electron microscopic study of the skin lesion

The plantar hyperkeratotic skin lesion in a case of Richner-Hanhart's syndrome was investigated using ultrastructural examination. Light microscopic examination showed remarkable hyperkeratosis and some aberrant keratinocytes with multiple nuclei. On ultrastructural examination, some abnormal structures were seen in the affected keratinocytes: aggregations of tonofilaments and intracytoplasmic inclusions. The inclusions were needle shaped and were considered to be "crystal ghosts," presumably of tyrosine. The formation of tyrosine crystalline inclusions seems to be an important factor in the pathogenesis of the cutaneous lesions in Richner-Hanhart's syndrome.     

An atypical melanocytic lesion without genomic abnormalities shows locoregional metastasis

A subset of difficult melanocytic lesions exists with histopathologic features that evade diagnostic consensus from even expert dermatopathologists. Comparative genomic hybridization (CGH) has emerged as a useful diagnostic tool to categorize these lesions, by identifying known chromosomal aberrations in malignant melanoma or the lack thereof in melanocytic nevi. However, determining a lesion's biological behavior primarily on CGH is limited by a relatively small series of corroborative cases without long term follow up. We present a case of a pigmented lesion on the right cheek of a 4 year old boy. The lesion had features of a deep penetrating nevus, but the presence of frequent mitoses, tumor infiltrating lymphocytes, and microscopic foci of tumor necrosis were concerning for an unusual melanoma. We termed this lesion a melanocytic tumor of uncertain potential (MELTUMP) for these reasons. High-resolution array-CGH performed elsewhere on the lesion demonstrated no melanoma-associated genomic abnormalities. A sentinel lymph node biopsy of this patient later revealed multiple small tumor deposits. Although the presence of nodal involvement in similar lesions often do not lead to progressive and fatal disease, this case illustrates that atypical melanocytic lesions with nodal involvement may not demonstrate genomic abnormalities by CGH, and that histopathologic assessment remains paramount in defining these difficult melanocytic lesions. Further comprehensive study of these lesions is needed.     

Ear melanoma: a four-case series

External ear melanoma is rare, and early diagnosis and treatment are paramount for the patient's survival. Four clinical cases are reported, emphasizing the importance of the routine clinical examination of the ears in the dermatological consultation. The study included male and female patients, aged 60 to 81 years old, with melanocytic lesions in the outer ear, evaluated with detailed physical and dermoscopic examination, leading to the identification of lesions suggestive of melanoma. The cases were treated surgically with excision of the lesion, and the diagnoses were confirmed by histopathological study. The therapeutic approach was instituted early as most cases were diagnosed at an early stage, which directly impacted global survival.     

Detection of the interferon-gamma-induced protein 10 in psoriasiform dermatitis of acquired immunodeficiency syndrome

Interferon-gamma-induced protein 10 is a 10-kd protein produced by human keratinocytes following an exposure to interferon gamma. Keratinocytes within psoriatic plaques and within delayed-type hypersensitivity reactions have been shown to stain strongly with an affinity-purified rabbit antibody prepared against interferon-gamma-induced protein 10, suggesting a possible role for interferon gamma in the production of the lesions. A psoriasiform eruption has been seen in patients with acquired immunodeficiency syndrome (AIDS). Its severity appears to correlate with the degree of immunodeficiency in the early stages of AIDS. We stained 10 lesions of psoriasiform dermatitis of AIDS with the anti-interferon-gamma-induced protein 10 antibody using immunoperoxidase techniques. As controls, we studied 10 lesions of non-AIDS psoriasis, six lesions of seborrheic dermatitis with psoriasiform hyperplasia, one lesion of lichen simplex chronicus, and four biopsy specimens of normal skin from patients with AIDS. In addition, normal skin specimens taken from patients with AIDS and human immunodeficiency virus-negative patients at time of autopsy were examined. An identical, strong and diffuse staining pattern was seen in all cases of psoriasiform dermatitis of AIDS, non-AIDS psoriasis, seborrheic dermatitis, and lichen simplex chronicus. The specimens of normal skin showed only weak basal layer staining with anti-interferon-gamma-induced protein 10. Thus, the presence of interferon-gamma-induced protein 10 in keratinocytes was associated with psoriasiform hyperplasia and could be detected in both AIDS-associated and classic psoriasis.     

Differential proliferation of endothelial cells and keratinocytes in psoriasis and spongiotic dermatitis

This study used MIB-1 monoclonal antibody to quantify the proliferating keratinocytes and endothelial cells and their proliferation fractions in cases of normal skin, acute and established plaque psoriasis, and acute and chronic spongiotic dermatitis. The number and the proliferation fraction of MIB-1 positive cells were higher in psoriatic and chronic spongiotic lesions than in normal skin (p < 0.05). Established plaque psoriasis had a higher number of proliferating keratinocytes and a higher keratinocytic proliferation fraction than did acute psoriasis (p < 0.05). The number of proliferating endothelial cells decreased as acute psoriatic lesion became chronic, but the number in acute spongiotic lesion increased as it became chronic. The endothelial proliferation fraction was higher in acute psoriasis than in established plaque psoriasis (p < 0.05). The ratio of keratinocytic proliferation fraction to endothelial cell proliferation fraction of the psoriatic and spongiotic lesions suggested the presence of different reaction patterns to inflammation in psoriasis and spongiotic dermatitis.     

Solitary lichenoid benign keratosis: a clinicopathological investigation and comparison to lichen planus

The dermatopathologist is sometimes confronted with a single lesion biopsy showing the histopathology of a lichen planus (LP) taken from a patient having no further clinical signs of LP. This entity represents the 'solitary lichenoid benign keratosis' (SLBK). We report about 202 patients with 204 SLBK lesions which were diagnosed between 1981 and 1987. The mean age of these patients was 59 years, 62.4% were females. Only 2 of the 202 patients showed multiple lesions. In 30.6% the SLBK was located in the face and neck area. In the order of frequency the following clinical diagnoses were made: basal cell carcinoma, senile keratosis, Bowen's disease or seborrheic wart among others. We were able to distinguish three clinical types: (1) an erythematous type, (2) a papulokeratotic type and (3) a plaque-like type. The histopathological difference between SLBK and LP is primarily quantitative. The SLBK shows the following pronounced criteria: parakeratosis, vacuolized keratinocytes in the basal layer, a spongiotic epidermis in the center of the lesion with exocytosis of lymphoid cells, edema in the papillary layer, elastosis and plasma cells. Regularly one can find 'lentigo-senilis'-like lesions at the edge of an SLBK. Immunohistopathology showed no characteristic features in SLBK. The pathogenesis of this distinct entity is not yet clear.     

Self-healing juvenile cutaneous mucinosis

The clinical evolution and histological features of skin lesions in a 14-year-old boy were characteristic of self-healing juvenile cutaneous mucinosis. They were (a) early age of onset with fever, (b) plaque lesion over the nape of the neck and thigh, (c) nodular lesions over the scalp, face and periarticular regions and (d) spontaneous resolution.