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In two cases of elastosis perforans serpiginosa (EPS) new clinical and laboratory data are described and discussed. In Case 1, EPS was triggered by penicillamine-D within an unusually short period (about one year). In Case 2, EPS was apparently primarily triggered by a vena puncture. In both cases the light and electron microscopic findings were strictly compatible with EPS. These findings are summarized. In the active lesions of both cases increased numbers of helper T-cells and Langerhans cells were shown, while cytotoxic-suppressor T-cells were nearly completely absent. Leu 3a[sup+] cells and Leu 6[sup+] cells were also present in the inactive central area of the lesions. The data presented may cast doubt on the relationship of immunological findings to pathogenetic events in the disease. [ABSTRACT FROM AUTHOR]

The pathologic findings in the skin in a new case of toxic shock syndrome (TSS) are detailed and are discussed in view of previously reported data on TSS in the literature. Necrolysis of keratinocytes at different levels of the epidermis in combination with a predominantly perivascular lymphocytic infiltrate were characteristic features in lesional skin biopsy specimens obtained 5 days after the onset of acute illness and during the development of erythroderma with purpura and diffuse intravasal coagulation. Prominent vascular changes in the skin, which have not been reported before in TSS, were excited endothelial cells in combination with the presence of thrombi in the superficial capillaries. Histopathologic investigations of the dermatitis in TSS can be helpful in differentiating this, syndrome from other acute toxic skin eruptions. [ABSTRACT FROM AUTHOR]

We report a case of primary cutaneous adenoid cystic carcinoma in a 75-year-old man. Electron microscopy revealed similar features to those previously described in adenoid cystic carcinomas of salivary gland origin: wide intercellular spaces, pseudocysts containing replicated basement membrane-like material and true lumina lined by cytoplasmic membranes with numerous microvilli. Immunohistochemistry using antibodies to several cytoskeletal proteins (keratins and actin) indicated the presence of two epithelial tumour cell populations, one with the phenotype of myoepithehal cells, lining the pseudocysts and the periphery of the tumour fields, and another with the phenotype of acinar cells of the secretory coil of sweat glands. In addition, the tumour showed immunoreactivity for epithelial membrane antigen, but not carcinoembryonic antigen. A review of the literature on other cases of primary cutaneous adenoid cystic carcinoma showed that this tumour generally affects older patients with a female to male ratio of 4:1. The most common tumour site appears to be the scalp (40%), and the recurrence rate is 50%. Surgical treatment with extensive resection margins is recommended. [ABSTRACT FROM AUTHOR]

A child with segmental neurofibromatosis is reported. The clinical, histopathological and ultrastructural characteristics of this rare condition arc described. This entity is considered to be a localized form of von Recklinghausen's disease. The aetiology is not clear, but somatic mutation in early embryonic development may be important. [ABSTRACT FROM AUTHOR]

We assessed the efficacy of diphencyprone (DPCP) treatment in a total of 26 children with alopecia areata (AA). Sixteen children had alopecia areata totalis (AAT) and 10 had alopecia areata localis (AAL), DPCP is an absolute contact sensitizer. Twenty-five children could be sensitized with a 2% DPCP solution, whereas one child could not be sensitized. Patients were treated, once a week for at least 3 months, for up to 1 year. Twenty-one of the 25 (84%) children showed hair regrowth to a greater or lesser extent after DPCP treatment. Eight of the 25 (32%) children showed cosmetically acceptable hair regrowth. Cosmetically acceptable regrowth at the end of the study was seen in four of the 15 (27%) children with AAT and in four of the 10 (40%) children with AAL. These results are comparable with those reported in an earlier study in children with AA. Our opinion is that DPCP is a beneficial therapeutic agent in children with severe AAT and AAL showing no spontaneous remission. [ABSTRACT FROM AUTHOR]

We report a case of a 35-year-old female with a persistent pruritic acantholytic and dyskeratotic eruption on the chest and vulva. The light and electron microscopic studies showed suprabasal epidermal clefting with acatitholysis and dyskeratotic cells. We suggest that the most appropriate term for this case is that of benign persistent papular acantholytic and dyskeratotic eruption. [ABSTRACT FROM AUTHOR]

Tissue specimens were taken from three regions in a patient with oral progressive lupus erythematosus (LE): the tongue, the buccal mucosa and clinically uninvolved cutaneous tissue. They were subjected to immunocytochemical studies by use of markers. HLA-DR antigen expression on mucosal epithelial cells was found in association with a predominance of activated helper T-Cells in the lesional tissue. Langerhans cells (OKT-6) and B-cells (Leu-IO, B1 and B2) were nearly absent in the mucosal lesions. The presence of populations of macrophages (MY-4) in the lesional epithelium might suggest an important role for these cells, rather than for Langerhans cells, in the afferent limb of cellular immunity in this case of LE. [ABSTRACT FROM AUTHOR]

A 59-year-old man developed red, swollen and warm feet accompanied by intermittent burning pain during treatment for cardiac failure and arrhythmias with several drugs including verapamil. The condition gradually worsened until there was persistent disabling burning pain and severe erythema and swelling of the feet. Aspirin and other analgesics were ineffective in relieving the discomfort. Histopathology of punch biopsies showed a mild perivascular mononuclear infiltrate and moderate perivascular oedema. Within 2 weeks of stopping verapamil the burning pain, erythema, and swelling of the feet had resolved. The clinical features and subsequent course are consistent with a diagnosis of erythermalgia secondary to verapamil. [ABSTRACT FROM AUTHOR]

A patient developed an exanthem after inhalation of mercury vapors. Clinically and histopathologically the exanthem resembled an erythema exudativum multiformelike drug eruption. [ABSTRACT FROM AUTHOR]

This article presents a case study of a 2-year-old boy, who is the first child of non-consanguineous parents. He developed exfoliative erythroderma covering approximately 25% of the body surface soon after birth. In addition, large soft bullae filled with clear fluid also developed, particularly at sites of friction. Nikolsky's sign was positive. The mucous membranes and nails were normal. The erythroderma resolved rapidly. However, new bullae continued to develop, and desquamation persisted, especially at sites of friction.

This article presents a case study of a 34-year-old man, who had suffered from ulcerative colitis since 1978. In 1985, treatment with an oral iron preparation was stopped because of itching. Persistent liver enzyme abnormalities since 1986 were eventually attributed to chronic active hepatitis associated with the ulcerative colitis. There was a solitary, small erosion on the hard palate. Grouped, quite sharply edged erosions of various sizes were present on the upper right side of the trunk, and there were some erosions and scars on the lower arms.

The article discusses a case study of a patient suffering from netherton's syndrome. After an uncomplicated pregnancy the boy was born as the first child of healthy Turkish consanguineous parents. Immediately after birth a red and scaly skin was noted. The epidermis showed parakeratosis and acanthosis. The granular layer was virtually absent. There was considerable oedema in the epidermis. On electron microscopy desmosomes were irregularly developed and locally absent. There were small numbers of tonofilaments and intercellular oedema.

The article discusses some of the case reports of patients suffering from cutis verticis gyrata. Cutis verticis gyrata is a morphological description and not a disease entity. The lesion is mainly localized to the hairy scalp, particularly in the occipital region, and comprises skin folds and grooves resembling the gyri of the human cerebrum. The condition is usually asymptomatic but accumulation of material in the grooves may cause complaints about a foetid odour, burning sensation, pruritus or irritation.

The article presents information on patients of Yaws who were examined at six health centres situated at a distance from the capital city of Sumatra Barat, Padang, a total of 214 individuals were examined in November 1988. Patients with yaws were diagnosed clinically and serologically. Patients with yaws who had been previously treated patients with clinical yaws without serological data. Some of the cases that were examined included some who had yaws in the past; sero-positive individuals with a negative yaws history; sero-positive individuals in whom a different diagnosis was made.

Basal cell carcinoma (BCC) of the skin is a locally invasive, rarely metastasizing epithelial tumor. In the current study, the expression of E-cadherin, alpha- and beta-catenin and CD44V6 in normal epidermis and on BCC cells were investigated. A significantly reduced expression of alpha-catenin and CD44V6 and a slightly reduced expression of E-cadherin on BCC cells were observed compared with the overlying epidermis. Immunoelectron microscopy was used to investigate whether the decreased expression of E-cadherin and CD44V6 was due to either an absence or downregulation of specific membrane structures or due to an overall downregulation of these adhesion molecules in all membrane structures in BCC. E-cadherin and CD44V6 were expressed in adherens junctions, desmosomes, and complex interdigitating membrane structures both in normal epidermis and in BCC. A quantitative analysis showed that only a percentage of desmosomes was stained. In addition, the effect of pro-inflammatory cytokines, such as interferon-gamma (IFN-gamma) and tumor necrosis factor-alpha (TNF-alpha), was investigated in biopsy specimens of normal skin and BCC, using a biopsy culture system and immunohistochemistry. The expression of E-cadherin and CD44V6 was not significantly decreased after culturing BCC or normal skin biopsy specimens for 48 hours with or without recombinant human (rHu)IFN-gamma or rHuTNF-alpha. It may be concluded that the decreased expression of both E-cadherin and CD44V6, observed in light microscopy, was not attributable to the absence of specific specialized structures in BCC and most likely also not caused by downregulation by local cytokines, but rather by generic downregulation of both of these adhesion molecules during malignant transformation.

Basal cell carcinomas (BCCs) of the skin show varying degrees of peritumoural inflammatory infiltrate consisting mainly of T cells, but lack an effective T-cell-mediated immune response. This may be caused by the absence of the major histocompatibility complex (MHC) class I and II antigens, intercellular adhesion molecule-1 (ICAM-1), CD40 and CD80 (B7.1). Interferon-gamma (IFN-gamma) is known to induce or up-regulate their expression on epithelial cells, whereas interleukin-10 (IL-10) down-regulates their expression. The induction and up-regulation of HLA-ABC, HLA-DR, ICAM-1, CD40, and CD80 in BCC and normal skin from BCC patients were investigated in a culture system using recombinant human IFN-gamma (rHuIFN-gamma). The levels of IL-10 were determined in the supernatants after culture. The results showed that only ICAM-1 expression was significantly up-regulated on BCC cells. However, in the normal epidermis of BCC patients and in the epidermis overlying the tumour nests, significant up-regulation of ICAM-1, and CD40, and CD80 and slight up-regulation of HLA-DR were observed. No changes in HLA-ABC expression were observed in either normal skin or BCC. High levels of IL-10 were present in the supernatants of BCC biopsies after culture. It may be concluded that it is highly likely that the presence of IL-10 in BCC is directly or indirectly responsible for the complete lack of expression of HLA-DR, ICAM-1, CD40 and CD80 and the inconsistent expression of HLA-ABC on BCC cells in situ and may be a way of escaping immune survillance.

Background: Systemic hyalinoses are genetic generalized fibromatoses characterized by an accumulation of hyalin in the dermis. Two distinctive syndromes are recognized in the literature: infantile systemic hyalinosis (ISH) and juvenile hyaline fibromatosis (JHF). ISH and JHF are sometimes difficult to separate since they show significant overlap., Observations: We report on 3 children from two unrelated families suffering from JHF. The first child is severely handicapped by joint contracture, massive hyperplasia of the gingivae, diffuse skin papules and subcutaneous nodules occupying the scalp, face, perianal area, palms, soles and chest. At the same age, the second child only shows pearly skin papules on the face, groin and perianal area and gingival hyperplasia without joint stiffness or any other subjective complaint. The third patient, a brother of the second child, developed mild skin abnormalities by the end of the first year. The occurrence in siblings and consanguinity in the second family suggests autosomal recessive inheritance. Histological skin examination in the 3 cases showed hyaline deposition in the dermis and abnormal ultrastructure of fibroblasts. Biochemical findings showed mucopolysaccharide abnormalities in both families., Conclusion: Our patients do not only illustrate the different expressions of JHF but also show some overlap with ISH, suggesting a common cause for both disorders. Genetic studies will finally answer this question.