Biomedical subjects
Publications and source records attributed to D M Bronson.
Cutaneous mucinosis is rarely associated with systemic lupus erythematosus. A 13-year-old girl had a two-month history of numerous cutaneous mucinous papules and the new onset of systemic lupus erythematosus. The differential diagnosis and pathogenesis of this variant of cutaneous mucinosis are discussed. This is the first report of a child with this unusual entity.
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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.
A case of acute hemorrhagic edema in a 3-year-old boy is presented. Its cause, differential diagnosis, and relation to Schönlein-Henoch purpura are discussed. Acute hemorrhagic edema is an unusual variant of leukocytoclastic vasculitis.
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A 56-year-old black man developed a pemphigus foliaceus-like bullous eruption as the initial presentation of chronic T cell lymphocytic leukemia. Histologic examination disclosed superficial acantholysis consistent with pemphigus foliaceus and an infiltrate of atypical lymphoid cells in the papillary dermis. Repeated direct and indirect immunofluorescence studies yielded negative results. Acantholysis could be reproduced in vitro by incubation of normal human skin with the patient's serum and plasma but not with blister fluid. In the following months, the course of the bullous eruption paralleled that of the leukemia.
A case of acanthosis nigricans coexistent with mycosis fungoides, the second report in the literature of such an association, is described.
Scleromyxedema (Arndt-Gottron syndrome) is a rare cutaneous disease in which hyaluronic acid is deposited in the dermis. The authors describe a patient with scleromyxedema and corneal deposits. A corneal biopsy demonstrated hyaluronic acid deposition in the corneal stroma and amyloid P component in Bowman's membrane. This is the first report of scleromyxedema involving the cornea. It is also the first report of amyloid P component deposition in the cornea occurring independent of corneal amyloid deposits.
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A 20-month-old boy with anhidrotic ectodermal dysplasia developed fever and papulopustular skin lesions. A skin biopsy showed the histologic changes of acute miliary tuberculosis. Anhidrotic ectodermal dysplasia is characterized by an absence of seromucous glands in the oropharynx and tracheobronchial tree, making children with this disease prone to viral and bacterial respiratory infections. Mild defects in their immunologic system have also been reported. We suggest that these abnormalities may have predisposed the patient to this rare form of cutaneous tuberculosis.
Acquired ichthyosis and cutaneous metastases occurred in a 59-year-old black woman with adenocarcinoma of the breast. Acquired ichthyosis has been associated with "solid" tumors such as carcinomas of the lung, colon, and breast in only a few cases. Systemic diseases, medications, and malignancies associated with acquired ichthyosis are reviewed, and the need for a thorough clinical investigation of patients with acquired ichthyosis is stressed.
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A 4-year-old Hispanic boy had an asymptomatic eruption of red-brown macules and papules on the face, neck, trunk, and upper extremities. Examination of the skin lesions by light microscopy revealed a diffuse infiltrate of histiocytes in the upper dermis. Examination by electron microscopy showed wormlike inclusion bodies within the histiocytes. A diagnosis of benign cephalic histiocytosis was made on the basis of these distinct clinical, histologic, and ultrastructural features.
Zosteriform connective-tissue nevus, because of its distribution and histopathologic characteristics, is considered to be a separate entity. The only previous case of zosteriform connective-tissue nevus to be reported in the American literature was in 1944. We report and discuss a similar case.
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.