The presence of mucocutaneous manifestations has clinical significance, as it may be a part of the initial presentation or activation stage of both autoimmune and autoinflammatory rheumatic diseases. The cutaneous signs may display a particular morphological and topographic distribution according to taxonomy, whereas heterogeneity is likely observed among the individuals. The review aims to cluster and systematically approach the mucocutaneous manifestations met in autoimmune and autoinflammatory rheumatic diseases of childhood. The search strategy involved a comprehensive inquiry on Web of Science, PubMed, MEDLINE, and Embase databases using relevant search terms such as "dermatologic, cutaneous, mucocutaneous, skin, rash" for each disease and category. The awareness of the distinctive mucocutaneous manifestations and their correlation with rheumatic diseases provides a convenient definition, well-timed control of the underlying condition, and prevention of cosmetic issues. In the management of rheumatic diseases, planning the pertinent differential diagnosis and determining the requirement of histopathological assessment are essential with a multidisciplinary approach to rheumatology, dermatology, and allergy-immunology specialties.
© 2022 the International Society of Dermatology.