@article{6d2a3f1fd47548b99f6d5e3cd4bb84d5,
title = "Brazilian pemphogus foliaceus autoantibodies are pathogenic to BALB/c mice by passive transfer",
abstract = "Brazilian pemphigus foliaceus (fogo selvagem) is a cutaneous blistering disease endemic to certain areas of South America that has distictive epidemiologic features suggestive of an infectious disease transmitted by an insect vector. Patients with the disease have antiepithelial autoantibodies, both circulating in the serum and bound to lesional epidermis. In order to examine the possible pathogenic role of these autoantibodies, IgG from the sera of these patients was purified and injected into the peritoneum of neonatal BALB/c mice. Thirty-four of 46 mice (74%) receiving parenteral IgG fractions from these patients developed cutaneous lesions that were identical to the human disease by clinical, histologic, immunologic, and ultrastructural criteria. High-titer Brazilian pemphigus foliaceus sera produced lesions more consistently and rapidly than low-titer sera. When injections were discontinued, new lesions ceased to appear and old lesions resolved. The extent of disease correlated with the titer of human antiepithelial antibodies detected in the mouse serum (z < 0.01). Similar concentrations of IgG fractions obtained from sera of unaffected Brazilians living in endemic areas and from American donors did not induce disease when injected into littermates. These results establish that the antiepithelial autoantibodies play an important role in the pathogenesis of the cutaneous lesions in Brazilian pemphigus foliaceus.",
author = "Roscoe, {J. T.} and L. Diaz and Sampaio, {S. A.P.} and Castro, {Raymundo M.} and Labib, {Ramzy S.} and Yuzo Takahashi and Harish Patel and Anhalt, {Grant J.}",
note = "Funding Information: Brazilian pemphigus foliaceus (BPF) is a chronic blistering disease of the skin, of which there are approximately 10,000 registered active cases [1). The disease is endemic in areas of South America, particularly central Brazil, where it is known as fogo selvagem. It occurs most frequently in rural areas where the jungle is being cleared for settlement, but becomes rare as these regions become urbanized. The onset of the disease is frequently abrupt, with malaise and fever; it occurs predomi- Manuscript received February 19, 1985; accepted for publication July 17, 1985. Supported in part by U.S. Public Health Services Grants R01-AM32599, R01-AM32081, R23-AM32490, R23-AM32079, and T32-AM07324 from the National Institutes of Health, and a gift from the Estee Lauder Corporation. Dr. Anhalt is supported by the Andrew W. Mellon Foundation and Dr. Roscoe is supported by the Syntex Corporation through the Dermatology Foundation. Reprint requests to: Justin T. Roscoe, M.D., Department of Dermatology, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, Maryland 21205. Abbreviations: BPF: Brazilian pemphigus foliaceus EM: electron microscopy FITC: fluorescein isothiocyanate ICS: intercellular spaces IF: immunofluorescence immuno-EM: immunoelectron microscopy PV: pemphigus vulgaris nantly in children and young adults and often affects several members of the same household (2]. These epidemiologic features suggest an infectious etiology, but a virus or other infectious agent has not yet been identified (3]. A consistent feature of the disease is the presence of high-titer antiepidermal cell surface autoantibodies, demonstrable in lesional skin and circulating in the serum by immunofluorescence (IF) [4] and immunoelectron microscopy (immuno-EM) techniques [5,6]. These autoantibodies are detected in all epidermal layers, bound diffusely to the keratinocyte cell surface, without preferential binding to desmosomes (6]. Ultrastructural studies (6] have clemonstrated that the epidermal cell-cell detachment (acantholysis) in lesional epidermis of BPF is localized in the granular cell layer of the epidermis, with preservation of the normal morphology of the basal and spinous cell layer. This process begins with widening of the intercellular spaces (ICS) between desmosomes, and progresses until the desmosomal units separate. When acantholysis is complete, the split desmosomes cannot be visualized and the tonofilaments retract to the perinuclear area.",
year = "1985",
doi = "10.1111/1523-1747.ep12277362",
language = "English (US)",
volume = "85",
pages = "538--541",
journal = "Journal of Investigative Dermatology",
issn = "0022-202X",
publisher = "Nature Publishing Group",
number = "6",
}