Hyperkeratosis lenticularis perstans (Flegel's disease). Ultrastructural study of lesional and perilesional skin and therapeutic trial of topical tretinoin versus 5-fluorouracil.

Lesional and perilesional skin samples from a 57-year-old man who had hyperkeratosis lenticularis perstans (HLP) (Flegel's disease) were studied by light and electron microscopic examination. Keratohyalin granules were diminished at the center of a fully-developed lesion. In contrast, keratohyalin appeared normal and membrane-coating granules were found in reduced numbers at the edges of the HLP lesion and were easily detected in normal numbers in clinically normal, perilesional skin. The inflammatory infiltrate in the HLP lesion was composed of small lymphocytes, which often displayed nuclei with deep infoldings resembling Sézary cells, and larger histiocytic cells, many of which were in close contact with the lymphocytes. Peripheral blood mononuclear cells did not show an abnormal ultrastructural appearance. Treatment with topical 5-fluorouracil cream led to the disappearance of the HLP lesions, whereas topical tretinoin was ineffective.

Drug eruptions from mesna. After cyclophosphamide treatment of patients with systemic lupus erythematosus and dermatomyositis.

BACKGROUND: Mesna is used to abolish urotoxicity of cyclophosphamide and related compounds in immunosuppressive and antineoplastic treatment schedules. Adverse reactions to this drug have been reported only rarely. OBSERVATIONS: Drug eruptions to mesna have developed in seven of 15 patients with autoimmune disorders treated with monthly pulses of intravenous cyclophosphamide. Two different types of drug eruptions were observed: five patients had development of a macular and partly papular or urticarial rash and angioedema and two patients had a generalized fixed drug eruption, primarily and predominantly at the sites of previous skin lesions of their underlying condition. The results of prick, patch, and intradermal tests were similar in both types of rash; however, the two patients with fixed drug eruption had developed a generalized eruption upon prick testing with mesna. CONCLUSIONS: Two distinct eruptions to mesna have been induced in these patients during cyclophosphamide/corticosteroid therapy; these eruptions are not thought to share a common pathogenic mechanism. The results of skin and challenge tests do not support the hypothesis that a type 1 or a type 4 immune reaction may be responsible for these eruptions. The unusually high incidence (about 50%) of these reactions and their clinical presentation make it important to distinguish them from an exacerbation of the preexistent autoimmune disorder.

Localized vs generalized pyogenic granuloma. A clinicopathologic study.

We herein report the unusual case of a previously healthy young man who had spontaneous development of multiple lobular capillary hemangiomata disseminated over the integument. Based on the observation that the single lesions exhibited (immuno)pathologic and ultrastructural features similar, if not identical, to those of late-stage pyogenic granulomas, we propose to nosologically include our patient's eruption within the disease spectrum of pyogenic granuloma. As opposed to the occurrence of localized forms of pyogenic granuloma, the disseminated eruption seen in our patient and in other patients whose cases are reported in the literature cannot be ascribed to physical trauma. As some of these latter patients suffered from an underlying malignant neoplasm, it is tempting to speculate that both exogenous (eg, trauma) and endogenous (eg, tumor cells) factors can lead to the release of mediators promoting the development of these vascular neoplasms.

Endemic Kaposi's sarcoma in human immunodeficiency virus type 1-seronegative persons: demonstration of retrovirus-like particles in cutaneous lesions.

In 1984, Greek physicians reported on the clustering of cases of Kaposi's sarcoma (KS) on the Peloponnesus peninsula. To gain more insight into its pathogenesis, we studied the seroepidemiologic and clinicopathologic characteristics of 12 Greek KS patients (eight male/four female) five of whom were residents of an endemic area on the Peloponnesus. These patients were in good general health with ages ranging from 48 to 80 years, had no clinical signs of immunodeficiency, and combined the features of both classic and epidemic KS in that they displayed not only involvement of acral areas but also widespread mucocutaneous lesions. Routine laboratory data were within normal limits; no patient had HTLV-1 and HIV-1/2 antibodies, but all patients had antibodies to several herpesviruses. The histopathology was characteristic of KS with the peculiar feature of a dense infiltrate composed predominantly of CD4+ T lymphocytes. Immunoenzymatic/morphologic studies of the KS cells were consistent with their origin from lymphatic endothelium. Outstanding ultrastructural findings were tubuloreticular structures and cylindrical confronting cisternae, structures that are indicative of an ongoing viral infection. Indeed, extensive electronmicroscopic studies resulted in the detection of retrovirus-like particles in close association to KS cells in five of 12 patients. This in situ observation opens the possibility that this retro-virus contributes to KS development.

[Post-zoster-specific skin infiltrates in chronic lymphatic leukemia]. / Postzosterische spezifische Hautinfiltrate bei chronischer lymphatischer Leukämie.

Eight weeks after suffering from herpes zoster of the right 5th cranial nerve, an 80-year-old woman with chronic lymphocytic leukaemia developed zoster-like pseudovesicular lesions in the same nerve segment. Histological and immunohistological investigations revealed specific infiltrates of chronic lymphocytic leukaemia. Chemotherapy for the underlying disease was intensified, and the cutaneous infiltrates disappeared quickly. The patient died shortly afterwards due to exacerbation of the lymphoma.

Immunogold staining of intermediate-sized filaments of the vimentin type in human skin: a postembedding immunoelectron microscopic study.

We have studied the localization of vimentin in normal human skin at the ultrastructural level using a monoclonal mouse anti-vimentin antibody and a postembedding immunogold-staining technique on thin sections of Lowicryl K4M embedded biopsies. Selective immunogold labeling of intermediate-sized filaments (IF) of epidermal Langerhans cells and melanocytes and of dermal fibroblasts was demonstrated. The IF of fibroblasts were more intensely stained than those of the epidermal dendritic cells; cell processes and dendrites of all three cell populations exhibited a greater number of IF and more pronounced immunogold-labeling than the perinuclear cytoplasm, relating IF of the vimentin type to dendrite formation and/or function. The method described is an appropriate tool for immunoelectron microscopic studies of IF of the vimentin type in situ.

[Necrobiotic xanthogranuloma in paraproteinemia]. / Nekrobiotisches Xanthogranulom bei Paraproteinämie.

Necrobiotic xanthogranuloma is a rare generalized dermatosis occurring exclusively in association with paraproteinemia. Clinically it is characterized by large, ulcerating, nodular and plaque-like tumors of the dermis and subcutis. Histologically the lesions are composed of a nonspecific, lymphocytic and plasmacellular infiltrate and highly characteristic granulomas with giant cells of the Touton and foreign body type. Ultrastructurally, tubular cystic organelles with a central lamella and a few ribosomes at their outer membranes are found. By means of histochemical studies we excluded the skin infiltrates as the site of the paraprotein release. The pathogenesis of the disease remains unclear; it is suggested that the paraprotein has functional features of a lipoprotein, which may bind to lipoprotein receptors of the histiocytes, thereby inducing xanthoma formation.