==== Front J Postgrad MedJ Postgrad MedJPGMJournal of Postgraduate Medicine0022-38590972-2823Medknow Publications & Media Pvt Ltd India 29992915JPGM-64-19010.4103/jpgm.JPGM_201_18Case SnippetAtypical Kawasaki disease: Diagnosis underneath diapers Tripathy SK Aggarwal B Mandal A Bagri NK Department of Pediatrics, All India Institute of Medical Sciences (AIIMS), New Delhi, IndiaAddress for correspondence: Dr. Bagri NK, E-mail: drnarendrabagri@yahoo.co.inJul-Sep 2018 64 3 190 191 Copyright: © 2018 Journal of Postgraduate Medicine2018This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. ==== Body We report a 5-month-old baby, who presented with fever, irritability with rash for a total duration of 10 days, cough, and loose motions for 2 days. He was being treated as sepsis with broad-spectrum antibiotics before being referred to us. There were no history of erythema of tongue, conjuctival congestion, breathing difficulty, altered sensorium, or convulsion. Past history and perinatal history were unremarkable. On admission, the patient was febrile, irritable, and vitals were stable. Anthropometric measures including weight (=6 kg) and length (=63cm) of the baby were within normal range as per WHO standards. General physical examination revealed presence of urticarial rash, unilateral cervical lymphadenopathy, and edema of hands and feet but there were no oral or eye changes nor any periungal desqamation. On further examination and unfolding the diapers, we noted scrotal edema with perineal maculopapular rash which led us to consider Kawasaki disease (KD) as a possibility [Figure 1]. Laboratory evaluation revealed raised erythrocyte sedimentation rate (ESR) and C-reactive protein (50 mm/1st h and 34 mg/dL, respectively), anemia (Hb 5.8 g/dL), neutrophilic leukocytosis (20,200/mm3), and thrombocytosis (platelet count: 6,00,000/mm3). Ultrasonography of scrotum was suggestive of bilateral hydrocele. Echocardiography did not show any abnormalities consistent with KD. As our case did not met all the criteria for KD, a label of incomplete (or atypical) KD was considered as per American Heart Association (AHA) guidelines[1] and patient was started with intravenous immunoglobulin (IVIG) (2 g/kg over 12 h) and aspirin (75 mg/kg/day) on day 1 of hospitalization. After 24 h of IVIG therapy, the patient became afebrile, irritability reduced, and scrotal edema resolved in 48 h [Figure 2]. Repeat inflammatory markers (ESR, CRP, leucocyte count) were normal after 72 h although thrombocytosis (500,000/mm3) continued. Blood culture was sterile. The patient was discharged on antithrombotic doses of aspirin. At 2 months follow-up, the child is doing fine and afebrile with normal echocardiography and inflammatory markers were normal. Figure 1 Presence of scrotal edema and perineal rash Figure 2 Scrotal edema has resolved in 48 h after IVIG therapy KD is a self-limited vasculitis of unknown etiology often preceded by symptoms of upper respiratory or gastrointestinal illness like in our case.[2] Our patient had both upper respiratory infection and gastroenteritis at presentation. In the absence of classical features, these children often get misdiagnosed as sepsis and receive unwanted antibiotics without any benefit. The persistence of fever, presence of skin rash, edema of hands and feet and the scrotal edema pointed toward underlying vasculitis in our case. Presence of scrotal swelling and pain due to testicular inflammation is characteristic of vasculitis including polyarteritis nodosa (PAN) (6%), Henoch-Schönlein Purpura (2%–38%) and KD.[34] Presence of rash especially over perineal area is seen in KD in initial few days of illness.[5] The appearance of scrotal edema in KD ranges from 4 to 18 days.[5] It is important for the physicians to know about this finding in KD to avoid unnecessary surgical exploration. Diagnosis of KD remains a challenge, mainly when a child presents with incomplete or atypical features. A meticulous clinical examination can reveal clues such as scrotal edema and perineal rash to enable early diagnosis and timely initiation of therapy to prevent long-term complications. Declaration of patient consent The authors certify that appropriate patient consent was obtained. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. ==== Refs 1 McCrindle BW Rowley AH Newburger JW Burns JC Bolger AF Gewitz M Diagnosis, treatment and long term management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association Circulation 2017 135 927 99 2 Baker AL Lu M Minich LL Atz AM Klein GL Korsin R Associated symptoms in the ten days before diagnosis of Kawasaki disease J Pediatr 2009 154 592 5 19038400 3 Eleftheriou D Dillon MJ Tullus K Marks SD Pilkington CA Roebuck DJ Systemic polyarteritis nodosa in the young: A single centre experience over 32 years Arthritis Rheum 2013 65 2476 85 23754739 4 Ioannides AS Turnock R An audit of the management of the acute scrotum in children with Henoch-Schonlein Purpura J R Coll Surg Edinb 2001 46 98 9 11329751 5 Isidori C Sebastiani L Cardellini MC Di Cara G Rigante D Esposito S Early desquamating perineal erythema in a febrile infant: A characteristic clinical feature of Kawasaki disease Int J Environ Res Public Health 2017 14 710