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Background: We have characterized a new variant of endemic pemphigus foliaceus in El Bagre (El Bagre-EPF) (AKA pemphigus Abreu-Manu) and surrounding municipalities. Herein, we describe nail alterations in several patients affected by this disease. In the pre-steroid era, patients with endemic pemphigus foliaceus (EPF), especially in Brazil where the disease is known as fogo selvagem (FS), were described to have some nails changes including the Viera’s sign (yellowish of the nail). In this study, we have attempted to describe a range of nail alterations in patients affected by El Bagre-EPF. Methods: A case-controlled study was conducted where 40 cases and 40 controls were evaluated for nail changes. A clinical exam was performed in the cases and in a series of control patients from the same geographic region who were matched by age, gender and work activities. Gram stains and cells cultures for fungus and bacteria were done on affected nails. Results: In 25 chronic patients (affected for more than two decades) presented with toenail alterations. These changes included change of color nail (yellowish) (Viera sign), atrophy, dystrophy, chronic paronychia, onycholysis, nail bed erosion, subungual hyperkeratosis and trachyonychia. All of these findings were over-represented in patients compared with control population (p < 0.05). Cell cultures and gram stains were negative in all study participants. Discussion: Chronic patients have nail damage, maybe due to the presence of chronic inflammatory process affecting the nail bed cell, matrix, and/or the nail fold cells junctions. [ABSTRACT FROM AUTHOR]

Background: El Bagre endemic pemphigus foliaceus (El Bagre-EPF) is a new variant of endemic pemphigus foliaceus present in the El Bagre area of Colombia, South America. Here, we investigate the presence of complement/C5-b9 in lesional skin of patients and matched controls from the endemic area. We also aim to compare the patient's autoantibody levels using indirect immunofluorescent titers (IIF) and correlate with the lesional presence of complement/C5b-9., Methods: A case-control study was carried out by testing for the presence of complement/C5b-9 in lesional skin in 43 patients affected by El Bagre-EPF, as well as 43 matched, healthy controls from the endemic area. Skin biopsies were obtained and evaluated via hematoxylin and eosin staining, and immunohistochemistry., Results: The presence of complement/C5b-9 was observed in all cases of the patients affected by El Bagre-EPF and was not observed in the controls from the endemic area (P < 0.001). The patients' autoantibody titers utilizing IIF for IgG and IgM showed correlation between higher autoantibody titers and stronger intensity of staining with complement/C5-b9 staining (P < 0.001)., Conclusion: Patients affected by El Bagre-EPF have lesional deposition of complement/C5b, which correlates with disease severity and previously established serologies., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Senear Usher syndrome is a variant of pemphigus foliaceus, confined to seborrheic sites and considered to be a clinical overlap syndrome, with features of both pemphigus foliaceus and lupus erythematosus. We recently described autoantibodies to skin eyelid meibomian glands in patients with a new variant of endemic pemphigus foliaceus (El Bagre EPF) in South America. We tested for El Bagre EPF patient sera autoreactivity to pilosebaceous units utilizing direct and indirect immunofluorescence, confocal microscopy, immunohistochemistry and immunoelectron microscopy. Hematoxylin and eosin staining of skin biopsies revealed that one third of the patients affected by El Bagre-EPF demonstrated some histologic alteration of the pilosebaceous units. By immunohistochemistry, most El Bagre EPF biopsies demonstrated evidence of an autoimmune response along the neural and vascular supply routes of the pilosebaceous units. An active immune response was seen with antibodies such as anti-human mast cell tryptase, myeloid/histoid antigen, CD8, CD20, CD68, CD117/c-kit, ZAP-70 and vimentin. Immunoelectron microscopy demonstrated autoantibodies within the hair follicle and at the basement membrane area of the sebaceous glands. El Bagre-EPF patients have autoantibodies to pilosebaceous units and to their surrounding neurovascular packages. Our results warrant further characterization and may explain the loss of hair described in severe endemic pemphigus foliaceus before the therapeutic steroid era.