Recurrent Peeling of Skin Following Post-Streptococcal Atypical Kawasaki Disease: A Case Report and Literature Review

Abstract

Kawasaki disease (KD) is a systemic vasculitis occurring due to collective immune mechanisms in a genetically susceptible individual, triggered by infections, though the actual cause is yet to be determined. KD is the most common cause of coronary heart disease in children. Typical diagnostic criteria are fever more than 4 days plus four of clinical criteria of cervical lymphadenopathy, skin rashes, mucus membrane redness and lip changes, non-purulent conjunctivitis, peripheral changes in palms and feet. In atypical cases three criteria with fever are enough to commence treatment and echocardiograph early. An often-reported association with KD is streptococcal infections. Skin peeling occurs in both conditions, however in streptococcal infections it occurs early while late during convalescence stage in KD except peeling of the genitocrural area that may occurs early. Skin peeling in the streptococcal infection is related to exotoxin while the cause of the peeling in KD is not well known; but it is likely related to the immunological pathogenesis. Another type of skin peeling that occurs recurrently following the initial attack of KD in many months is of an unknown reason. The presentation in this report is exceptional; it is for the first time reporting association of atypical KD presented with post streptococcal complications and vitamin D deficiency followed by the skin re-peeling 6 months after the patients fully resolved. Moreover, it is for the first time reporting nail changes, painful micturition due to urethral involvement and mucus membrane erythema that all resolved without treatment. The cause possibly is related to innate immune mechanism post infections and possible role for vitamin D deficiency paving the way for the post-group A streptococcal (GAS) vasculitis which is an immune response that later acted to trigger the skin peeling. Patient initially presented with significant peeling of streptococcal infections and features of KD responding to monoclonal antibody. This case highlights that clinicians need to know about recurrent peeling following KD in many months and to make the parents aware about its harmless course. It is the first report of association of post streptococcal atypical KD vasculitis, vitamin D deficiency, skin harmless post-KD skin peeling, nail changes, mucus membrane erythema and painful micturition due to urethral involvement. Int J Clin Pediatr. 2019;8(1):12-18 doi: https://doi.org/10.14740/ijcp319