Zinc deficiency during parenteral hyperalimentation.
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Biomedical subjects
Publications and source records attributed to D F Fretzin.
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Since Kimura's original description of an unusual subcutaneous disorder with distinctive histologic features of vascular and lymphoid proliferation, numerous descriptions of diseases with similar features have been reported. A variety of descriptive labels, including Kimura's disease, angiolymphoid hyperplasia (ALH) with eosinophilia, atypical pyogenic granuloma, papular angioplasia, and histiocytoid hemangioma, have been applied to these diseases. Although this information has broadened our understanding of the clinical and pathologic spectrum of ALH, the etiology and/or pathogenesis remains unknown. Our case report illustrates the wide range of clinical features of ALH, demonstrating extensive lesions on skin and mucosa. The extreme histologic variability with regard to eosinophils, lymphoid infiltrates, and vascular alterations is also illustrated. Dramatic response to vinblastine sulfate treatment has not been previously reported and may provide a viable treatment alternative in selected patients.
Woolly-hair is a congenital defect that produces a localized patch of lightly colored, curly scalp hair. Scanning electron microscopy of the abnormal hairs demonstrates twisting of the hair shaft and abnormal cuticle formation.
Toxic epidermal necrolysis (TEN) has been reported following bone marrow transplantation. This rare and unfortunate complication may portend fatal outcome. We report on a patient with an allogeneic bone marrow transplant and graft-versus-host disease (GVHD) in whom TEN subsequently developed. In contrast to a previously reported case, our patient survived and is well two years after bone marrow engraftment.
Three patients had epidermolysis bullosa acquisita in association with Crohn's disease. We believe that epidermolysis bullosa acquisita should be included among the extraintestinal manifestations of inflammatory bowel disease. This article describes the diagnostic criteria, treatment, and other systemic diseases associated with epidermolysis bullosa acquisita.
A case of congenital pilar and smooth muscle nevus (CPSMN) is reported. CPSMN is a distinct clinical and histologic entity characterized by prominent vellus hairs accompanying a proliferation of smooth muscle within the reticular dermis. Plaquelike elevation and mild hyperpigmentation are variable features of this unusual organoid nevus.
The primary form of cutis verticis gyrata, a rare skin lesion, is described for the first time in a neonate in whom it was associated with other congenital anomalies. The combination of a histologically normal skin biopsy from the lesion and neurologic deficit supported the diagnosis; follow-up at 7 months of age showed persistent hypotonia and significant developmental delay. Differentiation of primary and secondary forms of cutis verticis gyrata and currently recognized etiologies are also briefly reviewed.
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This case report illustrates the value of ultrastructural examination of an undifferentiated carcinoma mestastatic to skin. In this patient, ultrastructural study of a cutaneous nodule demonstrated cytoplasmic neurosecretory granules characteristic of an amino precursor-uptake and decarboxylation cells (APUD) tumor and supported the diagnosis of metastatic bronchial carcinoid. Additional nine cases of bronchial carcinoids metastatic to skin are also briefly reviewed.
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In 1967, Mehregan et al described a new and distinct clinicopathologic entity that they called reactive perforating collagenosis (RPC). This rare disease is classified with a group of disorders that involve the transepithelial elimination of histochemically altered dermal tissue. In RPC, minor trauma such as an insect bite, scratch, or pilosebaceous infection alters the collagen fibers in the papillary dermis. The histochemically altered but ultrastructurally intact collagen elicits both a dermal and epithelial response. For the case presented, it is our purpose to demonstrate transepithelial elimination of collagen fibers by ultrastructural study, thereby supporting the recognized histochemical observations.
An adolescent boy with a bizarre progressive poikiloderma had episodic acral blistering and the development of palmar and plantar keratosis suggestive of hereditary acrokeratotic poikiloderma. Growth and development had been normal. Vesicobullous lesions were said to be present at birth and during early infancy. No family members were similarly affected. Results of laboratory studies were normal. Histological sections of skin demonstrated epidermal atrophy in some areas, increased epidermal melanin fibrosis of the papillary dermis, and modest capillary proliferation.
A patient with multiple myeloma had severe cutaneous and gastrointestinal xanthomatization and symptoms characteristic of systemic sclerosis. Clinical findings included thickened sclerotic skin, dysphagia associated with an akinetic esophagus, and abnormal findings on roentgenograms of the upper and lower gastrointestinal tract. Biopsy specimens obtained from the skin and the gastrointestinal tract studied by light and electron microscopy showed thickening, hyalinization, and xanthomatization of the subepithelial connective tissue. Macrophages containing lipid vacuoles were shown to assume a perivascular orientation on ultrastructural study. Our case appears to be unique since this combination of findings has not been reported previuosly. In addition, our electron micrographs may help to define better the pathogenetic process involved in the association between myeloma and plane xanthomata.
Hypomelanosis of Ito (incontinentia pigment achromians, systematized achromic nevus) is a cutaneous abnormality consisting of bizarre, patterned, macular hypopigmentation over variable portions of the body surface. Multiple associated defects in other systems occur in a significant precentage of affected individuals. Most commonly, the central nervous system, eye, and musculoskeletal structures are involved. It is suggested that the cutaneous abnormality, which is often detectable at birth or during infancy, may forewarn pediatricians of the possible emergence of defects in other organ systems.
Through actual case illustration, I have tried to present the way biopsy of a vesicobullous lesion would be approached by the dermatopathologist. I did not intend this article to be a comprehensive review and, because of the limitation of space, have ommited the more unusual bullous dermatoses. Comprehensive reviews of the role of immunofluorescence in vesicobullous disorders can be found in the Archives of Dermatology and Progress in Dermatology.
Two children had a hyperpigmented monomorphous papular eruption of several years' duration. Biopsy specimens demonstrated cysts in the middle dermis that contained multiple fragmented vellus hair shafts. The eruption in one child involuted spontaneously. The cause of these lesions is obscure. The term eruptive vellus hair cysts is proposed for this entity.
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A 10-day-old girl was examined because of a peculiar, patterned hyperkeratosis that was noted at birth. The lesions were distributed over the trunk and limbs and consisted of whorls and streaks of thick, yellow, adherent scales. The salient histologic feature was marked hyperkeratosis with deep invagination of the dilated pilosebaceous ostia nearly to the level of the hair bulb. Additional physical abnormalities included patchy alopecia, bilateral zonular cataracts, a depressed nasal bridge, small stature, and multiple skeletal deformities. Roentgenograms demonstrated widespread stippled epiphyses characteristic of Conradi-Hünermann syndrome.