Biomedical subjects
Publications and source records attributed to H Kerl.
An immunohistochemical staining procedure using Leu 7 (HNK-1) monoclonal antibody was used to study the distribution of myelin-associated glycoprotein in granular cell tumours. Positive reactions were noted in 10 of the 13 tumours investigated. This observation supports the concept that granular cell tumours are of Schwann cell origin.
We investigated the co-distribution of lymphocyte subpopulations and non-lymphoid 'accessory' cells in 35 cases of cutaneous lymphoproliferative diseases (T-cell lymphoma, 10 cases; B-cell lymphoma, 17 cases; pseudolymphoma, 8 cases) using immunohistochemical methods. T-zone histiocytes and particularly Langerhans cells were abundant in all cutaneous T-cell lymphomas, but were also found in B-cell lymphomas. T-zone histiocytes were associated with T-lymphocytes, especially T-helper cells, but not with T-suppressor cells. Dendritic reticulum cells were essentially confined to well differentiated germinal centres. Macrophages occurred in both lymphomas and pseudolymphomas without definite relationship with either B- or T-cells. In malignant lymphomas of high grade malignancy, macrophages represented the only non-lymphoid cell type. Our results indicate that malignant lymphoid cells, like normal lymphocytes, require definite micro-environments which are, at least in part, maintained by certain non-lymphoid cells.
Inflammatory cells in 15 specimens of normal human epidermis were selectively stained by a monoclonal antibody immunoperoxidase technique. The quantitative assessment using an interactive image analysis system revealed OKT 11 positive cells (T lymphocytes) and Leu 2a positive cells (suppressor/cytotoxic cells). As there was no significant difference in the distribution of these markers, helper/inducer cells obviously are not present in considerable amounts. OKM 5 positive cells outnumbered OKM 1 positive cells, indicating the presence of a OKM 5+, OKM 1-macrophage subset. The epidermal dendritic cells clearly showed a striking heterogeneity regarding the expression of HLA-DR (62% of OKT 6-positive cells) and Leu 3a (47%), suggesting the existence of immunologically distinct subsets of human epidermal dendritic cells.
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This report draws attention to pitfalls that may be encountered in histologic diagnosis of early stages of pemphigus vulgaris. A man is described who had clinical and histologic features of bullous pemphigoid to begin with and later typical features of pemphigus vulgaris. Intercellular antibodies were demonstrable by direct immunofluorescence from the beginning.
The presence and distribution of cytokeratins, actin, neurofilament protein, neuron-specific enolase, S-100 protein, and different neuropeptides were studied immunohistochemically by the peroxidase-antiperoxidase immunoenzyme method or the avidin-biotin-peroxidase technique in 10 patients with primary cutaneous neuroendocrine carcinoma. In all cases of cutaneous neuroendocrine carcinoma, immunoreactivity for neuron-specific enolase, cytokeratin, and neurofilament was identified. No staining was found after incubation with antibodies to S-100 protein, actin, and other tested neuropeptides. The cytoplasmic cytokeratin and neurofilament immunoreactivity was particularly strong in perinuclear areas, sometimes showing an annular pattern or displaying a discoid profile. The diagnosis of cutaneous neuroendocrine carcinoma may be reliably made by the immunocytochemical demonstration of neuron-specific enolase and intermediate filaments (cytokeratin, neurofilament protein) by conventional microscopy. Cutaneous neuroendocrine carcinoma has morphological, immunological, and histogenetic similarities to carcinoid neoplasms of the gut. We favor the concept that cutaneous neuroendocrine carcinoma is derived from, or differentiates toward, dermal neuroendocrine cells.
Cutaneous B-cell lymphomas represent a considerable number of skin lymphomas (about one-third) primarily showing skin manifestation without any extranodal involvement detectable using routine and special staging procedures. Differentiation from cutaneous T-cell lymphomas can be achieved clinically; histologically, due to the distinct growth pattern in the skin; and by immuno- or enzymophenotyping of the proliferating cells. Further differentiation of subtypes of cutaneous B-cell lymphomas is based primarily on cytomorphologic phenotyping of the cells. Classification of B-cell lymphomas with "primary" cutaneous manifestation according to the TNM system discloses a more adequate basis for prognostic and therapeutic considerations than the Ann Arbor classification for nodal lymphomas.
This article summarizes the conceptual and morphologic background for the diagnosis of cutaneous B-cell lymphomas (CBCL). The architectural pattern of the infiltrate and cytomorphology can indicate the B-cell nature of a skin lymphoma. Virtually all types of B-cell lymphomas, including pre-B-cell lymphoma; Burkitt-like lymphoma; malignant lymphoma, small lymphocytic; the many forms of germinal center cell-derived lymphomas; immunocytoma; plasmacytoma; and immunoblastic lymphoma (B type)-may involve the skin and can be classified according to modern lymphoma classifications.
Among cutaneous pseudolymphomas (PL) in a strict sense, in which the etiology is largely unknown, B-cell types, simulating malignant B-cell lymphoma, and T-cell types, simulating malignant T-cell lymphoma of the skin, can be differentiated. Criteria favoring a pseudolymphomatous rather than a malignant lymphoid infiltrate of B-cell type may be clinical (solitary lesion on the head), histologic (wedge-shaped infiltrate with follicle formation and sharply demarcated germinal centers), or cytomorphologic (polymorphous infiltrate showing an admixture of great numbers of macrophages, plasma cells, and eosinophils). Additional information can be provided by enzymophenotyping of the cells, showing the typical starry-sky pattern of the macrophages in pseudolymphomatous infiltrates, and by the demonstration of the polyclonality of the lymphoid cells in pseudolymphomas with respect to surface markers and intracytoplasmatic Ig production, as shown by immunophenotyping techniques.
Monoclonal immunoglobulin-producing lymphomas (immunocytomas, plasmacytomas, and immunoblastomas) constitute 15 to 20% of all cutaneous lymphomas. Their differentiation from polymorphous lymphoplasmacytoid inflammatory or pseudolymphomatous infiltrates may be difficult if sections are stained for a single light chain only. It was the aim of the study to elucidate the ratio of the kappa- to lambda-positive cells in 10 lymphoproliferative, 5 pseudolymphomatous, and 42 inflammatory lymphoplasmacytoid cutaneous infiltrates and to characterize them by the type of Ig (alpha, gamma, mu, kappa, or lambda) synthesized intracellularly. An indirect immunoenzymatic double-labeling method (alkaline phosphatase and peroxidase) was used for the simultaneous demonstration of kappa and lambda light chains and alpha, gamma, and mu heavy chains in paraffin sections. Ig-producing lymphomas of the skin show patchy monoclonal proliferations of cells synthesizing kappa IgM in almost 50% of the cases (5 of 10). Monoclonality is claimed if the ratio of lambda- to kappa-positive cells is at least 1:10, or vice versa. In polyclonal inflammatory and pseudolymphomatous infiltrates, the lambda/kappa ratio never exceeds 1:5. The most common cell type found in these reactive infiltrates produces gamma heavy chain; some produce alpha chain, but only a few produce mu chain. In high-grade malignant lymphomas, the tumor cells may lose their capacity for Ig production.
Germinal center cell-derived (GCC) malignant lymphomas are B-cell tumors that represent neoplastic equivalents of normal lymphoid follicles. These lymphomas include, in the Kiel classification, three main types: centrocytic lymphoma, centroblastic-centrocytic lymphoma, and centroblastic lymphoma. We have diagnosed 13 cases of germinal center cell-derived cutaneous lymphomas among 178 evaluated malignant lymphomas of the skin. Some clinical, histologic, and immunologic aspects are discussed in this article.
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A case of neuroendocrine Merkel cell carcinoma of the skin in a 65-year-old female patient is reported. The definite diagnosis can be established only by ultrastructural and immunohistochemical investigations. Characteristic features are cytoplasmic neurosecretory granules and the identification of neuron-specific enolase.
In recent years dermatopathology has experienced a dynamic development and acquired highly original aspects. Even today it is possible with classical histomorphology and clinical observation to discover new diseases, to recognize etiopathogenetic relationships and to give an important impetus to modern experimental research. The application of immunohistological techniques to the diagnosis of skin diseases is one of the most remarkable trends.
On the basis of over 100 cases of histologically verified granuloma anulare, we made an attempt to classify its many clinical variants. A principal distinction must be made between clinically typical and atypical forms on the one hand, and between localized and disseminated or generalized forms on the other. The various clinical pictures of the atypical forms are systematisized and described.
A 13-year-old boy received penicillin for influenza and otitis media. Within days of taking this medication, he developed recurrent edema of the face and a generalized urticarial eruption which waxed and waned. The salient and unusual features of this person's disease were: 1) A senile appearance of his face with flaccid folds and sagging of the skin. Histopathologic findings were dense dermal inflammatory-cell infiltrates with numerous eosinophils and destruction of elastic fibers. Findings by electron microscopy were fragmentation and clumping of elastic fibers and accumulation of granular material in the dermis. 2) Dermatitis herpetiformis-like cutaneous lesions and gluten-sensitive enteropathy. 3) Elastolysis increased in time and led to further deterioration of the patient's physical appearance. Six years later, the patient developed severe internal manifestations and died. We assume that the inflammatory-cell infiltrates and prolonged edema following therapy with penicillin caused the dissolution of elastic tissue and resultant systemic elastolysis.
A new case of pilar sheath acanthoma is reported. This tumor has been identified in 1978 and shows characteristic histological features: a central sinus with keratinous material, a pore-like opening to the surface, keratinization of the epidermoid type and projecting epithelial tumor lobes containing small keratin cysts resembling incomplete pilar differentiation.
Case report of a 70-year-old woman with plasmocytoma-associated bullous "amyloid purpura" and a lambda light-chain IgA paraproteinemia. Cutaneous hemorrhage with blisters, erosions, and milia in the intertriginous areas were the main clinical features. Amyloid deposits in the walls of blood vessels, and the pericollagenous affinity of the amyloid in the dermis are likely to be responsible for the increased capillary fragility, i.e., the hemorrhagic tendency and the infrapapillary blisters. A differentiation from epidermolysis bullosa acquisita and porphyria cutanea tarda is possible by means of the histological level of the blisters.