Mycoplasma Jackie Hsieh, MD, Ali Yalcindag, MD, Daniel T. Coghlin, MD

A Sweet Case of abstract

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory disorder marked by fever and swelling of the skin that can be very painful. It is especiallyMycoplasma rare in the pediatric population. Infection is a well-known trigger for Sweet syndrome, but this entity has, to our knowledge, neverMycoplasma been described after infection. Herein, we describe the first pediatric case of febrile neutrophilic dermatosis associated with infection.

A 6-year-old boy presented with he was switched from clindamycin left leg pain and swelling, as well as to vancomycin; yet, he continued decreased ability to bear weight. He to have fevers and then developed was in his usual state of health until a migratory swelling in 1 area of the few hours before presentation. He was body that would then resolve 24 sitting on the ground playing video to 48 hours later. These swellings games when he started complaining were edematous, erythematous, and of leg pain and inability to walk. The severely painful for the patient (‍Fig 1). parents noted some swelling on the Regions included his right posterior ’ medial aspect of his left leg as well as head, left face and eye, right abdomen, Hasbro Children’s Hospital, Warren Alpert Medical School at Brown University, Providence, Rhode Island a rash characterized by red, raised left arm, and both legs. The patient s lesions on both legs. The rash was not papular rash also appeared on the Dr Hsieh obtained data for the case and drafted pruritic and he denied any trauma. extensor surfaces of both elbows and the initial manuscript; Drs Coghlin and Yalcindag ∼ obtained data for the case and critically reviewed Of note, the patient did have a cough knees (‍Figs 2 and 3). An MRI of his left and revised the manuscript; and all authors and sore throat 1 week before these leg showed subcutaneous and fascial approved the final manuscript as submitted and symptoms, but he never had a fever edema and enhancement involving agree to be accountable for all aspects of the work. and his upper respiratory symptoms the anterior, medial, and posterior DOI: https://​doi.​org/​10.​1542/​peds.​2016-​2762 had resolved by the time of this calf, including the soleus muscle, Accepted for publication Jan 30, 2017 presentation. suggestive of infectious myositis; Address correspondence to Daniel Coghlin, MD, however, his creatine kinase level When the patient was examined in Hasbro Children’s Hospital, 593 Eddy St, Providence, was normal. Multiple ultrasounds RI 02903. E-mail: [email protected] the emergency department, he was ° failed to demonstrate any synovitis. normotensive, tachycardic, and febrile PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, As the clinical picture became less 1098-4275). to 100.9 F. His medial left thigh had consistent with infection, antibiotics a 4- to 5-cm area that was noted to Copyright © 2017 by the American Academy of were discontinued a few days into his Pediatrics be swollen, erythematous, and warm. hospital course. There were also small erythematous FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships papules bilaterally on the dorsum of Rheumatology, Immunology, relevant to this article to disclose. ankles and hands. The patient was in Infectious Disease, and Dermatology FUNDING: No external funding. obvious discomfort, including inability were consulted. Multiple laboratory to ambulate or tolerate any palpation studies were collected, and a skin POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of of the area. The patient was initially biopsy of an elbow papule led to interest to disclose. thought to have a left leg cellulitis the final diagnosis. The biopsy and was started on clindamycin showed neutrophilic dermatosis and admitted for pain control. For and no signs of vasculitis, consistent To cite: Hsieh J, Yalcindag A, Coghlin DT. A Sweet the next day, his fevers persisted with Sweet syndrome. Given the Case of. Pediatrics. 2017;140(3):e20162762 despite antipyretics and antibiotics. association between Sweet syndrome Because of this lack of response, and malignancy, the patient had Downloaded from www.aappublications.org/news by guest on October 1, 2021 PEDIATRICS Volume 140, number 3, September 2017:e20162762 CASE REPORT FIGURE 3 FIGURE 2 Rash on both knees. Rash on the elbow.

FIGURE 1 Erythematous swelling of our patient’s left foot. Mycoplasma patient experienced. Additionally, had edematous swellings that were IgM titers were positive likely a result of the pronounced and increased on a repeat level edema in the upper dermis, another a bone marrow biopsy that was many weeks later, demonstrating9 possible presentation of Sweet unremarkable. He was started on 2 evidence of infection. Streptococcus Although syndrome. Sweet syndrome is steroid therapy and discharged from Sweet syndrome has been described uncommon, but has been more the hospital after improvement of his to be associated with prevalently described in adults and ’ pain and swelling.’ ∼ infections, in this particular case, the is rarely seen in children, with only patient s throat culture was negative On the patient s initial labwork, 70 pediatric3 cases reported in the and anti-DNase B antibodies were he was found to have positive literature. The mechanism behind negative. Despite the positive anti- anti-streptolysin O titersStreptococcus with Sweet syndrome is not completely streptolysin O, the sensitivity and negative anti-DNase B antibodies known. Existing literature posits specificity of a throat culture are and negativeMycoplasma Group A pneumoniae that it is a hypersensitivity reaction both high, including some studies throat DNA probe. He also had to a number of possible triggers, that report them to10 be 97% and positive Mycoplasma including hematologic and solid 99%, respectively. Moreover, immunoglobulin (‍Ig)M and IgG levels. malignancies, inflammatory bowel anti-DNase B antibodies should Convalescent titers disease, immunodeficiencies,2, 4,​ certain5​ have been detectable for up to 6 to ’ 5 weeksMycoplasma later showed an increase medications, and infections. ‍ There 9 months after infection. Thus, this in IgM, suggesting recent infection also have been case studies of Sweet Mycoplasmapatient s Sweet syndrome was more syndrome after vaccination in both with as the trigger for 6,7​ ∼ likely to have been associated with this case of Sweet syndrome. To our children and adults. ‍ Symptoms in infection rather than knowledge, this is the firstMycoplasma reported classic Sweet syndrome appear 1 to streptococcal infection. association between Sweet syndrome 3 weeks after infection, inflammatory bowel disease flare, or exposure to and a pediatric case of This case highlights the importance any of the aforementioned factors. infection. of recognizing Sweet syndrome The treatment of Sweet syndrome Discussion 8 as a mimicker of common causes is steroids. If Sweet syndrome is for soft tissue swelling. Initially, suspected, a biopsy of the lesions is this patient had a soft tissue important to obtain for diagnosis. Sweet syndrome, also known as acute swelling that persisted for a few Meanwhile, a bone marrow biopsy febrile neutrophilic dermatosis, was days, and appeared consistent is also recommended to rule out first described by Dr Robert Sweet with cellulitis. Areas of swelling 1 malignancy if a source of the Sweet in 1964. It presents with painful also appeared over joint spaces syndrome is not found. plaques or nodules accompanied by and were concerning for joint fever. Although there are a variety In terms of association with infection, effusions or hematogenous spread of presentations of Sweet syndrome, Sweet syndrome has been described of infection, such as in osteomyelitis. the most common clinical appearance to follow upper respiratory infection2 Additionally, these symptoms were is usually pseudovesicular in and gastrointestinal infections. accompanied by fever and elevated ∼ appearance, which our patient did In our patient, he experienced inflammatory markers, including have in some areas of his body, most cough and throatM pneumoniae discomfort 1 leukocytosis, thrombocytosis, notably his elbows and knees. A week before diagnosis with Sweet elevated C-reactive protein, and skin biopsy of these lesions revealed syndrome. infection erythrocyte sedimentation rate. the characteristic dense interstitial presents often with a nonproductive These signs and symptoms are neutrophilic infiltrate without cough and posterior pharyngitis, and certainly suspicious for infectious vasculitis. Additionally, our patient can be without pneumonia, as our etiology, in particular soft tissue Downloaded from www.aappublications.org/news by guest on October 1, 2021 e2 Hsieh et al References 1. Sweet RD. An acute febrile neutrophilic infection. Another diagnosis that may multiple broad-spectrum antibiotics, ö dermatosis. Br J Dermatol. be considered other than cellulitis is also concerning for autoimmune 1964;76(8–9):349–356 a vasculitis such as Henoch-Sch nlein or systemic inflammation. Once purpura, given the erythematous Sweet syndrome is suspected, a 2. Cohen PR. Sweet’s syndrome–a comprehensive review of an acute lesions. Some other autoimmune biopsy would confirm presence of febrile neutrophilic dermatosis. considerations that are more likely neutrophilic dermatosis without ö Orphanet J Rare Dis. 2007;2(1):34 in adults include sarcoidosis and vasculitis, after which steroid L fgren syndrome, although these therapy can be initiated. Our 3. Hospach T, von den Driesch P, Dannecker GE. Acute febrile neutrophilic are more likely to cause erythema patient improved after 1 week with dermatosis (Sweet s syndrome) in nodosum and are not associated with prednisone therapy. ’ childhood and adolescence: two new the classic pseudovesicular plaques Mycoplasma patients and review of the literature This case also demonstrates another of Sweet syndrome. It is important on associated diseases. Eur J Pediatr. post- condition that to consider Sweet syndrome in the 2009;168(1):1–9 differential diagnosis of soft tissue already includes acute disseminated é 4. Cohen PR, Kurzrock R. Sweet s encephalomyelitis, transverse ’ infections to minimize exposing a syndrome and cancer. Clin Dermatol. child to unnecessary antibiotics and myelitis, Guillain-Barr , and now 1993;11(1):149–157 pain. Although Sweet syndrome Sweet syndrome. Identifying it in 5. Hagen JW, Swoger JM, Grandinetti is characterized by major and this contextMycoplasma is important for future recognition, and may warrant LM. Cutaneous manifestations minor criteria, many of the criteria, of Crohn disease. Dermatol Clin. including fever and inflammatory testing for in suspected 2 2015;33(3):417–431 markers, are nonspecific. One cases. 6. Pedrosa AF, Morais P, Nogueira distinguishing factor that pointed Conclusions A, Pardal J, Azevedo F. Sweet’s our team away from cellulitis was Mycoplasma syndrome triggered by pneumococcal that the lesions were of abrupt vaccination. Cutan Ocul Toxicol. may trigger Sweet onset and were markedly painful. Mycoplasma 2013;32(3):260–261 Some of them were also clear and syndrome. We recommend testing 7. Jovanovi M, Poljacki M, Vujanovi L, for when evaluating ć ć edematous in appearance, which was Duran V. Acute febrile neutrophilic less consistent with cellulitis. Second, potential triggers of Sweet dermatosis (Sweet’s syndrome) after there were migrating areas of syndrome. This case also highlights influenza vaccination. J Am Acad edema located throughout the body, that not all febrile soft tissue Dermatol. 2005;52(2):367–369 pointing to a systemic and nonfocal swelling is infectious. Although 8. Cohen PR, Kurzrock R. Sweet s rare, Sweet syndrome is important ’ source of inflammation. Moreover, syndrome: a review of current for primary care providers and these areas of swelling were ’ treatment options. Am J Clin Dermatol. hospitalists to distinguish from associated with abruptly-appearing 2002;3(2):117–131 tender erythematous papules, which cellulitis, given Sweet syndrome s 9. Waris ME, Toikka P, Saarinen T, et al. severe pain, its response to steroids, are characteristic of Sweet syndrome. Diagnosis of Mycoplasma pneumoniae Another unique marker was that and its association with serious pneumonia in children. J Clin illnesses. our patient had 70% neutrophils in Microbiol. 1998;36(11):3155–3159 the setting of leukocytosis, which Abbreviation 10. Vincent MT, Celestin N, Hussain AN. is one of the criteria for Sweet 2 Pharyngitis. Am Fam Physician. syndrome (‍neutrophilia >70%). 2004;69(6):1465–1470 Finally, the patient had persistent Ig: immunoglobulin fever despite antipyretics and

Downloaded from www.aappublications.org/news by guest on October 1, 2021 PEDIATRICS Volume 140, number 3, September 2017 e3 A Sweet Case of Mycoplasma Jackie Hsieh, Ali Yalcindag and Daniel T. Coghlin Pediatrics originally published online August 18, 2017;

Updated Information & including high resolution figures, can be found at: Services http://pediatrics.aappublications.org/content/early/2017/08/16/peds.2 016-2762 References This article cites 10 articles, 1 of which you can access for free at: http://pediatrics.aappublications.org/content/early/2017/08/16/peds.2 016-2762#BIBL Subspecialty Collections This article, along with others on similar topics, appears in the following collection(s): Dermatology http://www.aappublications.org/cgi/collection/dermatology_sub Infectious Disease http://www.aappublications.org/cgi/collection/infectious_diseases_su b Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.aappublications.org/site/misc/Permissions.xhtml Reprints Information about ordering reprints can be found online: http://www.aappublications.org/site/misc/reprints.xhtml

Downloaded from www.aappublications.org/news by guest on October 1, 2021 A Sweet Case of Mycoplasma Jackie Hsieh, Ali Yalcindag and Daniel T. Coghlin Pediatrics originally published online August 18, 2017;

The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pediatrics.aappublications.org/content/early/2017/08/16/peds.2016-2762

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. Pediatrics is owned, published, and trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 2017 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

Downloaded from www.aappublications.org/news by guest on October 1, 2021