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We encountered a patient who complained of many small papules on the neck, shoulders, upper chest and upper back. Biopsy specimens showed complete loss of elastic fibers in the upper dermis including papillary dermis, whereas those of the mid dermis were intact. Electron microscopy revealed that assembly of component fibrils of elastic fibers was loose, and electron dense substance was aggregated in the spaces between these loosely bound subunit fibrils or along the periphery of abnormal fibers. Dermal phagocytes engulfed abnormal as well as normal elastic fibers. Upper dermal elastolysis is a clinical and histopathological entity different from mid-dermal elastolysis. Ultrastructural changes of the former are essentially similar to those of the latter but much more severe. It is suggested that activated elastophagocytosis of dermal phagocytes may play a role in this disease. [ABSTRACT FROM AUTHOR]

The article focuses on the completion of first thirty years of American Society of Dermatopathology (ASD). Prior to the establishment of the ASD, the major forum for the presentation and discussion of anything new in dermatopathology was in the dermatopatholgy courses and the Clinicopatholgic Conference. Dermatology and pathology have always been closely linked. Some of the earliest treaters of skin disease were pathologists who had learned skills of careful observation and analysis of morphological data that make up core of work of both specialties.

In a freshly induced blister from transient bullous dermolysis of the newborn, we found in epidermal basal cells structures similar to anchoring fibrils and lamina densa, often in combination. In the upper dermis, collagenolysis and phagocytosis of degenerated collagen fragments were observed. It is concluded that the stellate inclusions previously reported in this disease are prematurely assembled anchoring fibrils and lamina densa. A disturbance of delivery mechanism of precursor materials for anchoring fibrils and lamina densa may cause the deficiency of these structures which are essential for the integrity of dermo-epidermal adhesion. The presence of many normal anchoring fibrils and lamina densa in perilesional skin and their prompt degeneration after rupture of the inclusions previously led us to conclude that lytic enzymes are involved in the formation of blisters in this disease. The new finding of collagen phagocytosis in the lesional dermis seemed to support this contension. [ABSTRACT FROM AUTHOR]

A case of harlequin ichthyosis was studied with electron microscopy. From the basal cell to granular cell layers, keratinocytes contained giant mitochondria (860 nm) which exhibited vesicular cristae. Typical lamellar granules were absent; instead, numerous dense cored granules (DCG) and particles containing cored granules (PCG) were produced. Some of these were discharged into the intercellular spaces of granular cells, but the majority failed to be released from the cytoplasm. These retained DCG and PCG coalesced to form large vacuoles and cavities in the stratum corneum. In the hair follicle keratinized cells failed to loosen and desquamate into the hair canal; instead, they formed concentric keratin rings surrounding hair, a diagnostic features of this disease. It is suggested that the abnormal lamellar granule underlies the pathogenesis of this disease and that giant mitochondria may be related to an abnormal lipid metabolism of keratinocytes which may affect lipid composition of lamellar granules. [ABSTRACT FROM AUTHOR]

A case of transient bullous dermolysis of the newborn is reported. A healthy Hispanic newborn developed bullae during the first two days of life. From age two to four weeks, the lesions healed with milia formation. There was no residual scarring or hypopigmentation. An induced blister showed dermal-epidermal separation with the PAS-positive basement membrane in the epidermal roof. PAS-positive inclusions were present in the cytoplasm of a few basal cells. Examination by electron microscopy showed degeneration of the collagen and anchoring fibrils. There were numerous stellate inclusions in the endoplasmic reticulum of the cytoplasm of the lesional basal keratinocytes. [ABSTRACT FROM AUTHOR]

The article focuses on abstracts of papers presented at the 28th annual meeting of American Society of Dermatopathology. The paper "Lymphocyte Markers in Formalin Fixed Tissue in Jessner's Lymphocytic Infiltrate and Discord Lupus Erythematosus," by R. Akasu and H. Kahn presents a clinical and histological differentiation between Jessner's lymphocytic infiltrate and discoid lupus erythematosus. "Lysozyme in Abnormal Dernal Elastic Fibers of Cutaneous Aging, Solar Elastosis and Pseudozanthoma Elasticum," by S. Albrecht, L. From and H.J. Kahn presents a staining pattern of dermal elastic fibers in aging.

A typical case of infantile digital fibromatosis (IDF) was studied with antibodies raised against actin, vimentin, desmin and several species of cytokeratin. Strong reactions were observed for desmin, cytokeratin and CK-5, and moderate reactions for vimentin and actin. The diagnostic intracytoplasmic inclusion bodies within the tumor cells were ultrastructurally composed of aggregations of dense microfilaments. Immunoelectron microscopy showed that these filament aggregations are positively stained with anti-actin antibody. From these results, we suggest that' the large tumor cell of IDF is a myofibroblast and may originate from or differentiate toward vascular smooth muscle cells, because only this type of smooth muscle can coexpress desmin, vimentin and cytokeratin. [ABSTRACT FROM AUTHOR]

A hair matrix tumor showing an unusual tumor cell arrangement was found at the base of a solitary trichoepithelioma. Coexisting with solid epithelial islands and immature hair follicle-like structures, areas of alternating epithelial cords and hand-like stroma resembling the Verocay bodies of neurilemmoma or "ripplemarks" on waves were found. In other areas myxomatous degeneration of the stroma changed the rippling into a cribriform pattern. In some parts of the tumor there was a dense melanin pigment associated with MEL5 stained melanocytes. S-100 and CD1 (OKT6) antigen stains demonstrated Langerhans cells scattered in the parenchyma and less frequently in the stroma. The majority of tumor cells were considered immature pilar cortical cells because of the following: 1. HKN-6 was strongly positive; 2. a large number of melanocytes Were associated with tumor cells in some foci; 3. ultrastructurally immature tumor cells, which had electron-dense tonofilaments and many desmosomes, were transformed without production of trichohyalin granules into semikeratinized cells which showed nuclear degeneration and loss of electron density in tonofilaments. This tumor, however, has not attained the degree of differentiation observed in trichoblastoma (1) another example of an immature cortical cell tumor. Squamous eddy-like or horn pearl-like foci of incomplete keratinization and large keratin-filled cysts were also present within the immature parenchyma, indicating that some immature cells were differentiating toward non-cortical cells, as found in the outer sheath. We would like to designate this tumor "rippled pattern trichomatricoma", a new entity. [ABSTRACT FROM AUTHOR]

A 51-year-old immunosuppressed man presented with ulcerations of the abdominal wall. Biopsy of the ulcer margin was necessary for the diagnosis of cutaneous cryptococcosis. Since a variety of opportunistic organisms can present with non-specific cutaneous lesions, these infections must be ruled out by tissue diagnosis of all immunosuppressed patients. A review of 22 additional cases is included together with electron microscopic findings. [ABSTRACT FROM AUTHOR]

Several Moll's gland cysts from 4 different patients were studied with the light and electron microscopes. The cysts were lined with a combination of several different epithelia, such as completely keratinized epithelium, glycogen-rich, stratified squamous epithelium with incompletely keratinized luminal cells, sweat duct epithelium, and secretory cells. In one case in addition to the above components, secretory-type wall cells contained very large lamellar inclusions which were PAS positive and diastase resistant. It was concluded that Moll's gland cyst is composed of dilated duct of the Moll's gland and secretory segment; the proportion of each segment is variable but the portion showing ductal differentiation is usually predominant and typical secretory epithelium is not always seen. [ABSTRACT FROM AUTHOR]

Blood vascular and lymphatic tumors were evaluated immunohistochemically by studying a spectrum of endothelial associated antigens. (1) UEA-1 lectin reacted with the tumor cells of one patient with malignant angioendothelioma in the non-metastatic stage. However, when metastasis occurred, the binding sites of this lectin completely disappeared from the surface of the tumor cells in both original and metastatic lesions, suggesting the loss of blood group H antigen from the tumor cells could be used as an indicator of metastasis in this tumor. (2) Reaction with anti-HLA-A, B, C, intense in normal blood vessels, remained intensely positive in pyogenic granuloma and Kaposi's sarcoma, whereas it did not react with normal lymphatics and lymphangioma. This indicates that anti-HLA-A, B, C is useful in differentiating blood vascular structures from lymphatic structures in both normal and pathological conditions. (3) OKM5 reacted intensely with benign hyperplasias in pyogenic granuloma. while barely reacting with proliferating parts in Kaposi's sarcoma, suggesting the difference in staining patterns can be used to distinguish vascular proliferation or malignancy. (4) Reaction with anti-Type IV collagen and anti-laminin was intense in normal blood vessels, pyogenic granuloma and Kaposi's sarcoma, whereas reaction with these antibodies in normal lymphatics was patchy and irregular in its thickness. [ABSTRACT FROM AUTHOR]

A patient presented with a frontal nodule of the scalp. Histopathological examination revealed a myxomatous multilobulate tumor composed of epithelioid cells with variable pleomorphism. Perineurium-like structures were seen but only around isolated lobules located at the tumor periphery. Electron microscopy revealed polygonal cells and cells with elongated cytoplasmic processes. Many cells had myelinoid figures. A basement membrane-like lamina was noted around some cells. Some of the tumor cells were immunoreactive for myelin basic protein. This finding suggests that the tumor cells are of schwannian type. Neurothekeoma of Gallager and Helwig is a rare, probably benign tumor with fairly distinctive histopathologic characteristics. It appears to be a variant of dermal nerve-sheath myxoma. [ABSTRACT FROM AUTHOR]

Despite significant chemical variations, all "amyloids" have 2 common denominators, positive Congo red slain which shows greenish birefringence (dichroism) and 6-10 nm straight, non-branching filaments. In systemic primary amyloidosis patients seem to have no other diseases, whereas sonic of them eventually develop multiple myeloma, that is, myeloma-associaied amyloidosis. Hyalinosis cutis et mucosae (lipoid proteinosis) is an autosomal recessive disorder of mucopolysaccharide metabolism, possibly related to lysosomal hexuronic acid.

Serum amyloid P component (SAP) is present not only in all types of cutaneous amyloidosis, but also in the normal dermal elastic fibers as well as abnormal elastic fibers, such as elastotic degeneration of elastic fibers in actinic keratosis. It was previously postulated that the colloid substance in colloid milium is a final degeneration stage of actinic elastosis. In a lesion of colloid milium SAP was intensely positive with an indirect immunofluorescence method using rabbit antiserum to human SAP. Electron microscopy of the colloid lesion revealed components of normal elastic fibers as well as those of actinic elastoid as seen in actinic elastosis. It is concluded that the colloid substance derives, at least in major part, from elastic fibers through actinic degeneration. [ABSTRACT FROM AUTHOR]

How much fun dermatopathology really is, can be appreciated only by those who are in it. In addition to being fun, it serves a most useful purpose. This usefulness, however, has not always been fully appreciated. In Europe, dermatopathology was already appreciated early in this century. The author was eager to learn dermatopathology, but there were a few problems. The first problem was that there were no biopsy punches available in the Skin Clinic where he studied, which was understaffed with physicians and overcrowded with patients. The second problem concerned the Pathology Laboratory at Massachusetts General Hospital which was not used to receiving skin biopsy specimens other than tumors. In addition to that, article sheds light on various developments in the life of the author.

Many appendage tumors of the skin are inherited such as neurofibroma and pheochromocytoma. In familial tumors, the basic genetic abnormality is uniformly present in germinal cells and inherited. The second change is mutational and occurs in the somatic cells. The tumor could be monoclonal or multiclonal origin depending upon how many cells, genetically at risk, are actually transformed in the second stage. Benign tumors are generally multiclonal whereas malignant counterparts are monoclonal in origin. Another example of the benign polyclonal tumor is trichoepithelioma.

Epidermal keratinocytes in the basal layer divide and gradually differentiate as they migrate to the surface where they eventuate in dead, cornified cells, which protect the underlying living tissues. The basal cells contain sparse, thin tonofilaments in their cytoplasm, which gradually become thicker and condense as the cells migrate through the epidermis to form keratin filaments. Keratin filaments are a class of intermediate filament, as are vimentin, desmin, glial filaments and neurofilaments. These keratin filaments are formed from tonofilaments by ionic as well as by S-S bond formation. The S-S bond is particularly common in the substance along the cell periphery of cornified cells.

Tissues peripheral to well-developed papules of colloid milium were chosen for biopsy and electron microscopy examination. Evidence was presented that colloid is derived from elastic fibres through sequential degenerative changes. In the upper to middle dermis of the peripheral tissue, a number of abnormal masses in the vicinity of small colloid depositions were observed. Ultrastructurally, those abnormal masses were degenerating elastic fibres in which "electron-dense layers" of normal elastic fibre increased in amount, although their electron density diminished. Electron-light layers were gradually diminished in quantity. Degenerated elastic fibres finally became granulo-fibrillar and were indistinguishable from colloid. Many fibrils which closely resembled 10 nm tubular microfibrils of normal elastic fibre were observed in the granulo-fibrillar substance. Since one stage of the degeneration of elastic fibre which eventually leads to the formation of colloid milium was very similar to that of actinic elastosis, a direct role of sunlight in the formation of colloid milium is suggested. [ABSTRACT FROM AUTHOR]

A 35-year-old Japanese woman developed vitiligo with an inflammatory erythema on the face two years after the onset of bilateral uveitis and dysacusis. Histological studies of the lesions revealed the loss of pigment and dense mononuclear cell infiltration around the blood vessels. At the dermo-epidermal junction area, a few eosinophilic masses were found. Disclosed that the masses were filamentous masses (colloid bodies) and amyloid. After the oral administration of prednisolone, the erythema subsided, and the vitiligo showed some repigmentation. [ABSTRACT FROM AUTHOR]

A 14-year-old Japanese male showed numerous small cutaneous hemangiomas and severe anemia produced by internal hemorrhages from intestinal hemangiomas. Histological studies revealed ectatic vessels lined by a single layer of endothelial cells. Six hemangiomas, with fibrous walls of variable thickness, showed characteristics of vegetant intravascular hemangioendothelioma (Masson) in an organizing thrombus. Electron microscopy confirmed that the dilated cavities, were lined by a layer of flattened endothelial cells, surrounded by a few pericytes and/or smooth muscle cells. No appreciable abnormalities were found in the blood vessels located in the clinically normal skin. A few amyloid deposits in the subepidermal connective tissue were also observed. [ABSTRACT FROM AUTHOR]

Seven cases of angioma serpiginosum were examined in our clinic during a period of 15 years. A histological study performed in six of them revealed an increased number of ectatic capillaries in the dermal papillae. One of the cases, a 31-year-old woman, was studied by electron microscopy. The dilated capillaries consisted of endothelial cells, pericytes and veil cells. Walls of capillaries showed a heavy precipitation of fine fibrillar material admixed with collagen fibers. Most vessel walls were composed of two layers. The inner layer consisted of fine fibrillar material and the outer layer consisted of bundles of collagen fibers of about 40 nm in diameter. Characteristic findings in the endothelial cells were the formation of accessory lumen or slit-like protrusion of lumen into the endothelial lining. It appears that angioma serpiginosum is not a simple telangiectasia of pre-existing vessels but represents a type of capillary nevus, which has a tendency to manifest itself in the form of capillary dilation and proliferation. [ABSTRACT FROM AUTHOR]

A case of typical vibration-induced acrosclerosis was studied with the electron microscope. In addition to increased collagenization of the dermis, vascular wall and perivascular spaces, a severe vacuolization and an increased number of lysosomes in the vascular endothelial cells were observed. In many peripheral nerves, axonal degeneration, demyelination and an extensive collagenization of peri- and endoneurium were observed. These changes appeared to be the organic basis for sclerosis and neuropathies of this condition. [ABSTRACT FROM AUTHOR]

Electron dense membrane limited granules were observed in basal cells of the outer root sheath and in connective tissue sheath of the anagen sinus hair of mice. These granules were oval or round in configuration, and were 150-l80 nm in size. They were considered to represent structures related to collagenolytic activity to accelerate elongation of anagen hair. [ABSTRACT FROM AUTHOR]

Six examples of basal cell epithelioma were compared to fetal and adult skin. Five parameters were used in this study: abnormally large aggregates of melanosomes in epithelial cells; the paucity of half-desmosomes; the increase of microfilaments in the cell periphery; clustered membrane-limited granules; and desmosome-like structures of stromal fibroblasts. All of these indicators were positive in both basal cell epithelioma and fetal hair follicle; but they were negative in both fetal and adult epidermis. Therefore, it was concluded that basal cell epithelioma seems to mimic fetal primary epithelial germ. Interestingly, stromal fibroblasts of basal cell epithelioma phagocytize collagen fibers, and membrane-limited granules were found both inside and outside the tumor cells facing the digested collagen stroma. In view of the collagenolytic activity of the homogenate of basal cell epithelioma, membrane-limited granules might represent zymogen granules for collagenases. [ABSTRACT FROM AUTHOR]

Histologic, immunologic and electron microscopic studies were performed in a patient with transient acantholytic dermatosis which involved oral mucosa. Histologic and electron microscopic findings were almost identical in both cutaneous and mucous membrane lesions and these were similar to pemphigus vulgaris; suprabasilar separation with acantholytic cells. Desmosome-desmosome complexes were separated without disruption of cell membranes and cytoplasmic structures were well-preserved without dyskeratosis. Lamina lucida, however, was often separated in mucous membrane lesions, in contrast to the normal lamina lucida in cutaneous lesions of such cases or in pemphigus vulgaris. Direct and indirect immunofluorescence studies for pemphigus were repeatedly negative. This study shows that transient acantholytic dermatosis may involve mucous membrane and may resemble pemphigus vulgaris histologically and ultrastructurally except for the widened lamina lucida of the mucous membrane. [ABSTRACT FROM AUTHOR]

This article announces that the twenty-fifth annual postgraduate course in dermal pathology will be held at The Newporter Resort, Newport Beach, California, from July 31, 1983 to August 5, 1983. This course in dermal pathology is designed primarily for pathologists and dermatologists interested in cutaneous pathology. Another course on practical skin pathology approved by the American Medical Association and the American Academy of Dermatology will be given at Grossinger's, Liberty, New York, from August 28, 1983 to September 2, 1983. This is a rigorous, comprehensive, practical review and update of dermatopathology for pathologists and dermatologists.