ABSTRACT
Paraneoplastic pemphigus (PNP) is a rare autoimmune skin disease closely related to tumors, characterized by a maculopapular rash with mucosal pain, bronchiole occlusion, and respiratory failure may occur over time, even resulting in death. We report a rare case of a child with autoimmune PNP misdiagnosed as juvenile dermatomyositis (JDM), and summarize the key points of differentiation of clinical manifestations and auxiliary examinations of PNP and JDM. When the diagnosis is not clear because the patient has features not typical of JDM, then skin biopsy and other diagnostic studies should be considered prior to any immunosuppressive therapy, as this could potentially obscure and delay the diagnosis of malignancy.
Subject(s)
Autoimmune Diseases , Dermatomyositis , Paraneoplastic Syndromes , Pemphigus , Child , Humans , Pemphigus/diagnosis , Pemphigus/drug therapy , Dermatomyositis/diagnosis , Dermatomyositis/drug therapy , Dermatomyositis/complications , Autoimmune Diseases/complications , Skin/pathology , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/drug therapy , Paraneoplastic Syndromes/etiology , Diagnostic Errors/adverse effectsABSTRACT
Juvenile idiopathic Arthritis (JIA) is a common rheumatic disorder in children that can cause multiple systems to be affected simultaneously, leading to severe clinical symptoms and a high mortality rate in those with pulmonary involvement. Pleurisy is the most common manifestation of pulmonary involvement. At the same time, other conditions, such as pneumonia, interstitial lung disease, occlusive bronchiectasis, and alveolar protein deposition, have been increasingly reported in recent years. This review aims to provide an overview of the clinical manifestations of JIA lung damage and the current treatment options to assist in identifying and treating JIA lung involvement.
