The Rare Case of Pemphigus Vegetans in Association with Malnutrition Children in the Multidisciplinary Management

Abstract

Pemphigus vegetans is a clinical variant of pemphigus vulgaris, accounting for 1–2% of all very rare pemphigus cases in children. The involvement of the oral mucosa in this disease is usually accompanied by severe pain that aggravates the patient’s malnourished condition. Conversely, malnutrition may also reduce vulnerability towards autoimmune diseases. Although pemphigus vegetans has never been reported to develop in a child with marasmus before, we encountered a case of pemphigus vegetans in a severely malnourished patient. A 12-year-old boy in marasmic condition presented with painful, clear, fluid-filled blisters, accompanied by erosions, crusts, and vegetative lesions on almost all parts of the body. Histopathological examination of the lesions revealed a suprabasal cleft, and direct immunofluorescence staining showed deposits of immunoglobulin G in the epidermal intracellular spaces. The patient was treated with a multidisciplinary approach, and intravenous corticosteroid was administered for 2 weeks with an appropriate diet. There were significant improvements in the skin lesions and his nutritional status. Although pemphigus vegetans may occur in children with malnutrition, the underlying mechanism for the development of autoimmune diseases in malnutrition remains unclear.

Extensive pemphigus vegetans in a Filipino female

“…Our case involved extensive involvement of Pveg of the face, ears, scalp, trunk, back, genital, and gluteal area. A similar report of a 30-year old female patient with extensive involvement of the mucosa, trunk, extremities, verrucous paronychia, and fissure tongue was published in 2019 ( Suwarsa et al., 2017 ), albeit with less extensive involvement compared with our patient.…”

“…Few case reports involved unusual areas, such as the nails, arm, trunk, scalp, and esophageal sites ( Messersmith and Krauland, 2021 ). In some areas, features of Pveg may be specific, such as the cerebriform tongue (sulci and gyri pattern on the dorsum of the tongue), cobblestoning of the lips, and verrucous paronychia of the nails ( Suwarsa et al, 2017 ). Our case involved extensive involvement of Pveg of the face, ears, scalp, trunk, back, genital, and gluteal area.…”

Pemphigus vegetans possible association with enalapril

“…Other immunosuppresants used for treatment of pemphigus are: cyclophosphamide, cyclosporin, mofetil mycophenolate, chlorambucil and methotrexate, and antiinflammatory drugs -gold salts, dapson and tetracyclin derivatives, including nicotinamide. Immunomodulating techniques are plasmapheresis, extracorporeal photopheresis and intravenous immunoglobulins [25].…”

“…Pozostałe leki immunosupresyjne stosowane w leczeniu pęcherzycy to: cyklofosfamid, cyklosporyna, mykofenolan mofetylu, chlorambucyl i metotreksat oraz preparaty przeciwzapalne -sole złota, dapson i pochodne tetracyklin wraz z nikotynamidem. Do technik immunomodulujących należą plazmafereza, pozaustrojowa fotoforeza i immunoglobuliny stosowane dożylnie [25].…”

Pemphigus Vegetans of Neumann Type on Lip: A Case Report

“…It is caused by autoantibodies directed transmembrane glycoproteins and results in loss of intercellular adhesion between intact keratinocytes, which results in acantholysis. This disease occurs mainly on intertriginous areas, scalp, and face but may occur anywhere on the skin surface (7)(8)(9). The oral, gastrointestinal, respiratory, genital, and conjunctival mucosa can also be affected.…”