“ ” John B. Darby, MD, a Gregory Valentine, MD, a Kirsty Hillier, MD, a Raegan Hunt, MD, PhD, a, b AC. Mary 3-Week-Old Healy, MD,a Valeria Smith, MD, a Wendy With Allen-Rhoades, an MDa Isolated Blueberry Muffin Rash abstract A 3-week-old boy, former 39-week term infant, presented to the emergency department with a rash. One week before presentation, he developed dark, purple papules on his forehead, which then spread to the abdomen and inguinal regions. Throughout this time, he was eating well, gaining weight, developing appropriately, and was afebrile without cough, congestion, or rhinorrhea. On presentation, the patient was well appearing with normal vital signs. His weight was 4.83 kg (86th percentile for age), his length was 56 cm (47th percentile for age), and his head circumference was 37 cm (62nd percentile for age). On skin examination, there were scattered purpuric papules and macules on the scalp, forehead, trunk, abdomen, and inguinal Departments of aPediatrics and bDermatology, Baylor region. Initial laboratory studies were remarkable only for mild anemia. “ ” College of Medicine/Texas Children’s Hospital, Houston, Our expert panel examines the case, offers a differential for a child with a Texas blueberry muffin rash, and makes diagnostic considerations. Dr Darby contributed to the design and execution of the case conference and drafted and edited the CASE HISTORY WITH SUBSPECIALTY original manuscript; Drs Valentine, Hillier, Hunt, INPUT ’ Healy, Smith, and Allen-Rhoades contributed to the or delivery. He passed his hearing design and execution of the case conference and Dr Gregory Valentine (Resident, Streptococcusscreen, and the mother s serologies reviewed the manuscript; and all authors made Pediatrics, Global Child Health) were negative, including group B revisions to the manuscript and approved the final . Additionally, both manuscript. newborn screens were normal, and DOI: https:// doi. org/ 10. 1542/ peds. 2016- 2598 A 3-week-old boy, former 39-week he received the hepatitis B vaccine at Accepted for publication Jan 31, 2017 term infant, presented to the birth. Address correspondence to John B. Darby, MD, emergency department (ED) with a The patient had 2 healthy older Department of Pediatrics, Baylor College of rash. One week before presentation, Medicine/Texas Children’s Hospital, 1102 Bates siblings who did not have a similar Street, #FC1860, Houston, TX 77030. E-mail: jbdarby@ he developed dark, purple papules rash in the first few months of their texaschildrens.org on his forehead, which then spread lives. The family had not traveled to PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, to the abdomen and inguinal regions. any other country since his birth, and 1098-4275). Over the course of a week, the rash there were no animal exposures. The Copyright © 2017 by the American Academy of continued to spread and appeared to patient was seen by his pediatrician on Pediatrics be on the extremities, abdomen, and the day of presentation, who referred FINANCIAL DISCLOSURE: The authors have head, which prompted presentation him to be evaluated in the ED. indicated they have no financial relationships to the ED. From the time of onset of relevant to this article to disclose. In the ED, the patient was well the rash to the presentation at the ED, ° appearing with a temperature of FUNDING: No external funding. he was eating well, gaining weight, 98.0 F, a pulse of 156 beats per minute, POTENTIAL CONFLICT OF INTEREST: The authors developing appropriately, and had blood pressure of 87/52 mmHg, and have indicated they no potential conflicts of interest remained afebrile without cough, to disclose. a respiratory rate of 40 breaths per congestion, or rhinorrhea. minute. His weight was 4.83 kg (86th Regarding birth history, the patient percentile for age), his length was To cite: Darby JB, Valentine G, Hillier K, et al. A was born to a gravida 3 para 3 mother 56 cm (47th percentile for age), and 3-Week-Old With an Isolated “Blueberry Muffin” who had prenatal care beginning in his head circumference was 37 cm Rash. Pediatrics. 2017;140(1):e20162598 the first trimester and no reported (62nd percentile for age). He was in no complications during pregnancy acute distress. He had no discernible Downloaded from www.aappublications.org/news by guest on September 25, 2021 PEDIATRICS Volume 140, number 1, July 2017:e20162598 DIAGNOSTIC DILEMMAS petechiae, but he had scattered, (hemangiomatosis, blue rubber bleb ecchymotic, purpuric papules and nevus syndrome). macules on the scalp, forehead, trunk, Cytomegalovirus (CMV) is the most abdomen, and inguinal region (Figs 1 common congenital infection and ’ and 2). can cause a petechial rash due to Results of the patient s laboratory thrombocytopenia. Congenital evaluation are shown in Table 1. toxoplasmosis has been described They are notable for a mild anemia with this type of rash, but it is a rare but otherwise are unremarkable. infection in the United States. Syphilis Cerebral spinal fluid studies, liver isDr another Darby possibility. function tests, chest radiograph, and coagulation studies were interpreted as normal. A head ultrasound was Dr Cabrera-Meza mentioned several significant for visualization of infectious causes of a purpuric lenticulostriate vessels, consistent FIGURE 2 rash. With TORCH infections, do with mineralizing vasculopathy, a Skin findings. you typically see a purpuric rash nonspecific finding that can presenting at birth or several weeks be seen with in utero infection. later?Dr Mary Healy (Pediatric Infectious The patient was admitted for Disease) additional evaluation and occurs because of extramedullary hematopoiesis in sites of different Drmanagement. John Darby (Moderator, Pediatric Hospital Medicine) magnitudes and extensions. Since then, the differential diagnosis In an infant with a purpuric rash, as associated with this type of rash mentioned by Dr Cabrera-Meza, you “ ” has been broadened significantly. need to think broadly because some Does this patient qualify as having The differential includes the infections presenting with this type a blueberry muffin rash and, if so, following: infectious etiologies, such of rash need to be treated promptly. Drwhat Gerardo is the differentialCabrera-Meza diagnosis? However, in this case, the infant has as toxoplasmosis, other (syphilis, (Neonatology) been perfectly well until this point, varicella-zoster, parvovirus B19), and the rash did not develop until rubella, cytomegalovirus, and herpes several weeks after birth. This makes (TORCH) infections (especially Yes, the description is consistent TORCH infections less likely, but not congenital rubella), blood with a blueberry muffin rash. impossible. dyscrasias (ABO incompatibility, The term originated in the early Rh incompatibility, minor Without the history above, the 1960s when the rubella epidemic group incompatibility, twin-to- appearance of the rash alone would occurred in this country. This rash twin transfusion syndrome), lead me to consider the blueberry malignancies (congenital leukemia, muffin rash characteristic of Langerhans cell histiocytosis [LCH], congenital rubella syndrome (CRS). TAneuroblastoma),BLE 1 ED Laboratory and Evaluation skin disorders However, CRS is rare in the United Variable Reference Range Laboratory Test Results WBC count, 103 cells/μL 9.1–34 16.46 Differential, % Segmented neutrophils 32–67 19.8 Lymphocytes 25–37 50.4 Bands 0–8 7.4 Hemoglobin, g/dL 15.0–22.0 13.0 3 Platelet count, 10 cells/μL 150–450 327 INR 0.8–1.2 1.2 PT, sec 10.4–15.0 14.1 PTT, sec 24.9–34.1 47.5 LDH, U/L 500–920 2014 Uric acid, mg/dL 2.0–6.2 2.4 FIGURE 1 INR, international normalized ratio; LDH, lactate dehydrogenase; PT, prothrombin time; PTT, partial thromboplastin time; Skin findings. WBC, white blood cell. Downloaded from www.aappublications.org/news by guest on September 25, 2021 2 DARBY et al States and in other developed later when they present with, for CMV include small for gestational countries with high uptake of example, poor visual acuity early age, jaundice, hepatosplenomegaly, childhood vaccination. Between in childhood and are found to have neurologic manifestations, including 2004 and 2012, there were only 6 chorioretinitis, or perhaps hearing microcephaly and hypotonia, and reported cases to the Centers for1 problems or developmental delay. a rash. Pinpoint petechiae are Disease Control and Prevention. The cutaneous findings may be more characteristic of the rash – Virtually all of these cases were in diverse, including a maculopapular, of congenital CMV, rather than a infants of unimmunized mothers or blue, or petechial rash2 secondary to blueberry muffin type appearance. were imported cases, meaning that thrombocytopenia. It is noteworthy Also, thrombocytopenia is associated they were from people that were not that this infant had a normal platelet with this rash. immunized and came to the United count. ’ Although unlikely, congenital States from another country. Congenital syphilis could also present CMV should also be ruled out In this case, the infant s mother has with a rash at this age. However, the because neonates with symptomatic had 3 children all born within 5 to rash is typically different. Our patient congenital infection, with or 6 years of each other and has had did not have any rash on the palms without CNS involvement, medically attended deliveries in US or soles, and congenital syphilis does have improved hearing and hospitals each time with multiple not give you the typical blueberry neurodevelopmental