Scleroderma is an autoimmune disorder characterized by fibrosis in the skin and is divided into two types: systemic sclerosis and localized scleroderma. The etiology and pathogenesis of scleroderma are still unclear. Autoantibodies have been useful diagnostic tools and their clinical significance is reviewed in this article. Furthermore, recent studies have revived the idea that autoantibodies and B lymphocytes play principal roles in systemic autoimmune diseases. In the tight skin (Tsk/+) mouse, a genetic model of systemic sclerosis, B cells exhibit a hyper-responsive phenotype due to the disruption of the CD22/SHP-1 negative regulatory pathway. This inhibitory pathway is regulated by CD19, and the loss of CD19 expression significantly reduces skin fibrosis and autoantibody production in Tsk/+ mice. Two features of scleroderma, fibrosis and autoimmunity, thus affect each other, which may provide a clue to comprehending the pathogenesis of the disease.
Desmoglein enzyme-linked immunosorbent assays (Dsg ELISAs) are highly sensitive and specific as the diagnostic tools for pemphigus. The purpose of this study was to evaluate the diagnostic and therapeutic values of Dsg ELISAs in pemphigus showing several clinical phenotypes. The patients enrolled in this study were as follows; pemphigus vulgaris with dominant mucosal lesion (n=7), mucocutaneous lesion (n=11), and typical pemphigus foliaceus (n=15). We analyzed 364 serum samples from the 33 patients at multiple-time points during 12 years of follow up in our hospitals. Dsg ELISA scores of each patient correlated well with their disease severities, as well as the clinical phenotypes : Dsg1 and Dsg3 scores were sensitive for skin and mucous lesions, respectively. Of particular interest, these scores increased immediately before the active lesions recurred, suggesting a predictive marker for exacerbation of pemphigus. By sequential examination of ELISA at 1～2 month-intervals, some clinical information could be obtained (eg. the disease activity, therapeutic intensity which needed, and transition of the clinical phenotypes). We concluded that Dsg ELISAs could be comprehensive tools not only for diagnosis of pemphigus but also for management of the treatment.
We examined 531 HE stained specimens of melanocytic nevi on the face, which were histopathologically diagnosed in our laboratory from April 1990 to March 2000. First, these specimens were divided into two groups, non-elevated and elevated lesions. Two hundred and five cases were non-elevated, and 326 cases were elevated. We think that non-elevated lesions are earlier lesions than elevated ones. Second, based on the location of the tumor cells, these specimens were classified into three groups: junctional, compound, and dermal. Fifteen cases were junctional, 134 cases were compound, and 382 cases were dermal. We clinicopathologically recognized four types of lesions; Unna’s, Miescher’s, Spitz’s, and Clark’s nevus, as advocated by Ackerman. There were 3 cases of Unna’s nevus, 511 cases of Mieschers’s nevus, 1 case of Spitz’s nevus, and 16 cases of Clark’s nevus in our study. We determined two characteristics that the melanocytic nevus of the face has; one is that the junctional type is rare, and the other is that Miescher’s nevus is the most common.
We examined 271 HE stained specimens of melanocytic nevi of the sole. We classified them into 3 patterns according to the location of the tumor cells: junctional, compound and dermal. We classified the junctional type of melanocytic nevi into two patterns according to the existence of the tumor cells, that is nest type and single cell type (without nest). Then we classified each of the nest and single cell types into two patterns based on the distribution of the tumor cells: confined and scattered (pagetoid) type. The characteristics of the melanocytic nevus of the sole are that; most of the lesions are junctional type (247/271=91.1%) and that the scattered type is not rare (58/271=21.4%).
We examined 201HE stained specimen of melanocytic nevi on trunk and extremities excepting the axilla, inguinal area, genital area, hand, and foot. These were histopathologically diagnosed in my laboratory from April 1990 to March 2000. We classified these melanocytic nevi into three groups according to the location of the tumor cells; junctional, compound, and dermal. We measured the length of each lesion under a microscope. We classified the melanocytic nevi according to the clinico-pathological classification advocated by Ackerman that is Unna’s Miesher’s, Spitz’s, and Clark’s nevus. The characteristics of the melanocytic nevus of the trunk and extremities are that the dermal type and the Unna’s type are the most common. We believe that Unna’s nevus has the character of congenital melanocytic nevus.
We examined HE stained specimens of 6 cases of trichoepithelioma. In 2001, Ackerman advocated that trichoepithelioma was the cribriform type of trichoblastoma because both tumors consisted of follicular germinative cells. We checked the existence of the histopathological findings said to be commonly seen in trichoepithelioma and trichoblastoma. We think that the characteristics of the tumor cells and stroma that comprise the trichoepithelioma and trichoblastoma are the same and we agree with the hypothesis that trichoepithelioma is one type of trichoblastoma.
We describe a 31-year-old-male case of primary cutaneous blastic NK cell lymphoma. He had a red tumor and an indurated erythema plaque on his back. Skin and lymph node biopsies revealed diffuse proliferation of medium-to large-sized lymphoblastic cells that were positive for CD4, CD45, CD45RA, CD56 and HLA-DR, and negative for CD2, CD3 (surface and cytoplasmic), CD5, CD30, CD57, and CD68. In situ hybridization for EB virus and Southern blot analysis of T cell and B cell receptor gene rearrangement were negative. Based on these results, we diagnosed this case as blastic NK cell lymphoma. After several courses of combination chemotherapy and electron beam irradiation followed by allogeneic peripheral blood stem cell transplantation, this case went into complete remission. We review previously published reports of blastic NK cell lymphoma.
A 44-year-old woman developed an asymptomatic subcutaneous tumor measuring 3×5 cm in size on her right labium majorum. CT and MRI showed a well-circumscribed lesion. On T1-weighted MR images, the tumor was isointense in relation to muscle, and, on T2-weighted images, it showed hyperintensity. Histopathologically, the tumor was composed of a hypocellular area in loosely edematous stroma and a hypercellular area. Spindle— or oval― shaped tumor cells were arranged with numerous thin-walled vessels. Immunohistochemical examination revealed that the tumor cells were positive for vimentin and desmin and negative for CD34, S-100, α-smooth muscle actin, and myoglobin. A part of the tumor expressed estrogen receptors and progesterone receptors. We diagnosed the tumor as an angiomyofibroblastoma of the vulva.