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Sarah E. Stewart, MD,a​ Jody L. Lin, MD,​b Jennifer L. Everhart, MD,b​ Trung H. Pham, MD, PhD,​c Well-AppearingAnn L. Marqueling, MD,​d,​e Kerri E. Rieger, MD, PhD, ​d,Newborn​f Sarah L. Hilgenberg, MDb With a Vesiculobullous Rash at Birth abstract A term, appropriate-for-gestational-age, male infant born via normal spontaneous vaginal delivery presented at birth with a full-body erythematous, vesiculobullous rash. He was well-appearing with normal vital signs and hypoglycemia that quickly resolved. His father had a history Divisions of aGeneral Pediatrics, bPediatric Hospital c of herpes labialis. His mother had an episode of herpes zoster during Medicine, and Pediatric Infectious Diseases, Department of Pediatrics, Lucile Packard Children’s Hospital Stanford, and a prolonged rupture of membranes that was adequately Palo Alto, California; and Departments of dDermatology, e f treated. The patient underwent a sepsis workup, including 2‍ attempted Pediatrics, and Pathology, School of Medicine, Stanford University, Stanford, California but unsuccessful lumbar punctures, and was started on broad-spectrum and acyclovir, given concerns about bacterial or viral infection. Dr Stewart contributed to the conception and design of the case presentation, drafted the The rash evolved over the course of several days. Subsequent workup, with initial manuscript, and reviewed and revised the particular attention to his history and presentation, led to his diagnosis. manuscript; Drs Lin and Everhart contributed to the conception and design of the case presentation and Case History With Subspecialty reviewed and revised the manuscript; Drs Pham, Marqueling, and Reiger drafted their sections of Input ’ tests were negative for infection, the initial manuscript and reviewed and revised Dr Sarah Stewart (Pediatrics, First- the patient s mother received 3 the manuscript; Dr Hilgenberg contributed to the Year Resident) conception and design of the case presentation, dosesStreptococcus of clindamycin because of a drafted portions of the initial manuscript, and history of previous neonatal group ’ reviewed and revised the manuscript; and B (‍GBS) infection. The all authors approved the final manuscript as ’ The patient was a term, appropriate- patient s father had a history of herpes submitted. for-gestational-age, male infant born labialis. The patient s mother denied DOI: https://​doi.​org/​10.​1542/​peds.​2017-​0236 via normal spontaneous vaginal any history of herpes labialis or herpes Accepted for publication Jul 17, 2017 delivery who presented at birth with genitalis. ’ Address correspondence to Sarah E. Stewart, a full-body rash. The rash appeared MD, Division of General Pediatrics, Department The patient s initial laboratory results as diffuse discrete erythematous µ of Pediatrics, Lucile Packard Children’s Hospital, at 2‍ hours of life included a white 725 Welch Rd, Mail Code 5906, Palo Alto, CA 94304. papules and plaques with occasional – µ blood cell count of 2‍4.4 K/ L (‍normal: E-mail: [email protected] overlying fluid-filled vesicles and – 7.5 30.0 K/ L) with 74% neutrophils PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, bullae, most prominent on the face and – (‍normal: 32‍.0% 68.0%), 2‍0% 1098-4275). scalp but sparing mucous membranes, – lymphocytes (‍normal: 2‍4.0% 36.0%), Copyright 2018 by the American Academy of palms, and soles (‍Figs 1 and 2‍). He – © 4% monocytes (‍normal: 0.0% 0.9%), Pediatrics was well-appearing with normal vital – signs, Apgar scores of 8 and 9, a birth 1% eosinophils (‍normal: 0.0% 2‍.0%), FINANCIAL DISCLOSURE: Dr Lin received support weight of 3768 g, and an examination and 0% basophils (‍normal: 0.0% from the KL2 Mentored Career Development Award ’ – with results that were otherwise 1.0%); a C-reactive protein (‍CRP) level of the Stanford Clinical and Translational Science Award to Spectrum (NIH KL2 TR 001083, UL1 TR of 0.03 mg/dL (‍normal: 0.00 0.99 normal. His mother s pregnancy was – 001085) and the Clinical Excellence Research remarkable for presumed herpes mg/dL); a blood glucose level of 32‍ Center; the other authors have indicated they have mg/dL (‍normal: 45 110 mg/dL); an no financial relationships relevant to this article to zoster at 31 weeks that resolved – with valacyclovir. She had a history aspartate aminotransferase level of disclose. 68 IU/L (‍normal: 15 41 IU/L); an FUNDING: No external funding. of primary varicella-zoster at 2‍ years – alanine transaminase level of 8 IU/L of age and 2‍ episodes of herpes – zoster before this pregnancy. Labor (‍normal: 11 63 IU/L); a total bilirubin To cite: Stewart SE, Lin JL, Everhart JL, et al. Well- level of 3.1 mg/dL (‍normal: 2‍.0 6.0 Appearing Newborn With a Vesiculobullous Rash and delivery were complicated by a – prolonged rupture of membranes (‍2‍0 mg/dL); and an alkaline phosphatase at Birth. Pediatrics. 2018;141(3):e20170236 hours) and meconium-stained fluid. level of 110 IU/L (‍normal: 30 300 Though maternal prenatal laboratory IU/L). The remaining results of the Downloaded from www.aappublications.org/news by guest on September 25, 2021 PEDIATRICS Volume 141, number 3, March 2018:e20170236 Diagnostic Dilemmas FIGURE 1 Patient on first day of life. Coalescing erythematous vesicles are seen on the face, and dull gray and erythematous patches and plaques are seen on the body and extremities. The mucous membranes, hands, and the soles of the feet are spared.

complete blood cell (‍CBC) count and comprehensive metabolic panel were unremarkable. Dr Hilgenberg, as a pediatric hospitalist assuming care of this infant after birth, what is your differentialDr Hilgenberg diagnosis? (Pediatric Hospitalist) FIGURE 2 A, Lesions on the first day of life. B, New lesions on fingers on the fourth day of life. C, New lesions on ’ cheeks on the fourth day of life. D, Lesions on the fifth day of life. The forehead vesicles became less raised and less erythematous. A few new lesions appeared on the scalp. This infant s presentation is most “ ” concerning for one of the following: (‍1) a congenital TORCH infection, which is an infection caused by hepatosplenomegaly, intracranial cerebrospinal fluid pleocytosis, toxoplasmosis, other pathogens calcifications, and rash, as well as or elevated cerebrospinal fluid (‍varicella-zoster virus [VZV], syphilis, laboratory abnormalities. Despite protein. The incidence of congenital enteroviruses, or parvovirus B19), these common features, some clinical toxoplasmosis in the United rubella, cytomegalovirus, or herpes 1 ’ manifestations are more suggestive States is 1 4in 1000 to 1 in 10 000 simplex virus (‍HSV) ; (‍2‍) bacterial of particular infections in the newborns. sepsis, given the mother s previous neonate. neonatal GBS infection with her 2‍ For instance, congenital Congenital syphilis is characterized last pregnancy ; and (‍3) a primary toxoplasmosis has 4 subtypes: as either early, presenting before dermatologic condition such as subclinical, severe neonatal disease, 2‍ years of age but typically within epidermolysisDr Stewart bullosa (‍EB). mild or severe disease in the first the first 5 weeks of life, or late, few months of life, and sequelae presenting after 2‍ years of age. or relapse of an undiagnosed, Differentiating findings among How and when do TORCH infections typically ocular, infection later infants with early congenital typically present? What, if any, are in childhood. The minority of syphilis include nasal discharge, their dermatologic manifestations, ’ patients who are symptomatic maculopapular rash (‍particularly on given that skin lesions are our at birth may present with the the palms, soles, and diaper area), Drpatient Hilgenbergs only sign of illness? classic triad of chorioretinitis, generalized lymphadenopathy, and hydrocephalus, and intracranial skeletal abnormalities. Patients with calcifications. Dermatologic late congenital syphilis present with

Interestingly, the majority of live- features include a maculopapular,1,3​ physical anomalies, such as teeth born infants with TORCH infections3 petechial, or purpuric rash. ‍ or bony abnormalities, but rarely are asymptomatic at birth. When Additional signs and symptoms present with rash. The Centers for symptomatic, common features can include fever, seizures, Disease Control and Prevention include microcephaly, intrauterine generalized lymphadenopathy, reports that the incidence of growth restriction, jaundice, thrombocytopenia, mononuclear congenital syphilis in the United Downloaded from www.aappublications.org/news by guest on September 25, 2021 2 Stewart et al

States is 11.65 cases per 100000 cortical atrophy or seizures. If stable, meningitis is a potentially newborns. present, the rash in both HSV and devastating consequence of TORCH Congenital rubella syndrome can VZV is vesicular in nature. The infections and bacterial sepsis, include sensorineural hearing Centers for Disease Control and so lumbar puncture should be loss, ocular disease (‍cataracts, Prevention reports that the incidence considered. I would be inclined congenital glaucoma), cardiac of congenital varicella in the United to start empirical treatment with defects, bone disease, hemolytic States is 0.4% to 2‍.0% of newborns broad-spectrum antibiotics for “ ” anemia, thrombocytopenia, born to mothers with varicella in the possible bacterial sepsis and blueberry muffin rash,​ or first or second trimester. Neonatal antiviral agents for possible HSV varicella, a much more severe and or VZV. Lastly, I would consider petechiae and purpura caused3 by dermal erythropoiesis. After often life-threatening condition, can a dermatology consultation, the development of the rubella occur when the mother develops depending on how the clinical vaccine in 1969 and widespread varicella in the time period 5 days Drcourse Stewart evolves. use shortly thereafter, the incidence before the delivery10 through 2‍ days after delivery. of congenital rubella in the6 United States is <1 case per year. Parvovirus B19 can present with The patient underwent a sepsis workup. Lumbar puncture was Cytomegalovirus, the most ocular anomalies, hydrocephalus, attempted and unsuccessful. common congenital viral infection musculoskeletal problems, A lesion bacterial culture and serum in the United States, with an hepatocellular damage, heart defects, and lesion HSV/VZV PCRs were incidence of 0.5% to 1.0% in all and subcutaneous3 edema, as well as obtained. The patient was started on newborns, causes symptoms at Drpetechiae Stewart. 7 intravenous ampicillin, , birth in 10% of infected infants. and acyclovir. He was feeding, Differentiating clinical features urinating, and stooling normally may include sensorineural hearing How does the rest of your differential ’ with a continued overall well loss, periventricular intracranial diagnosis inform the next steps in appearance. calcifications, chorioretinitis, Drthis Hilgenberg infant s management? seizures, thrombocytopenia, and rash Dr Pham, as a pediatric infectious disease specialist, what are you with a petechial, morbilliform, or ’ widespread erythematous, macular Bacterial sepsis, most commonly most concerned about, given this appearance. caused by GBS in the neonatal period, patient s presentation and history? typically presents within the first 2‍4 Are there other studies you would Congenital HSV infection is to 48 hours of life and is associated Drrecommend? Pham (Pediatric Infectious relatively rare, with an incidence of with low birth weight, hypothermia, Disease) 1 in 3000 to 1 in 2‍0 000 newborns, lethargy, poor feeding, and an and infants may exhibit the elevation of CRP levels. Respiratory characteristic triad of skin findings distress and cardiac symptoms may The patient has been well- (‍scarring, active lesions, hypo- and be present if these organ systems are appearing and neurologically hyperpigmentation, aplasia cutis, involved. Cutaneous manifestations appropriate with normal vital signs and/or an erythematous macular are less11 common, but they can despite the extensive rash. His exanthem), eye damage, and severe occur. CBC panel, CRP test, liver function test (‍LFT), and blood culture central nervous8, system9​ (‍CNS) The fact that our patient is overall manifestations. ‍ Most newborns well-appearing and well-developed results are reassuring to date. with perinatally acquired HSV with normal vital signs and without In terms of infectious etiologies, appear normal at birth and then significant laboratory abnormalities the differential diagnosis for a present within the first 1 to 6 weeks argues against a congenital TORCH diffuse vesiculobullous rash in of life with infection symptoms infection or bacterial sepsis. His newborns, as seen in this patient, localized to the skin, eyes, and rash is not typical of those seen in would include viral infections such mouth (‍SEM), involving the CNS, or toxoplasmosis, syphilis, rubella, as HSV and VZV, staphylococcal disseminated. cytomegalovirus, or parvovirus and streptococcal skin infections, listeriosis, congenital candidiasis, Congenital varicella may present B19. Therefore, I would send blood 12‍ and congenital syphilis. with cicatricial skin lesions, which and lesion cultures to test for may be pigmented or depressed in bacteria and HSV/VZV polymerase Neonatal HSV infection seems a dermatomal distribution, ocular chain reactions (‍PCRs). Though the unlikely, given how early the lesions disease, limb abnormalities, and patient appears hemodynamically appeared (‍at birth) and given his lack Downloaded from www.aappublications.org/news by guest on September 25, 2021 PEDIATRICS Volume 141, number 3, March 2018 3 Dr Stewart of systemic symptoms with this level VZV reactivation has only been of cutaneous severity. Approximately reported on rare occasions in Dr Pham, would you recommend 85% of HSV infections in newborns immunocompromised individuals starting varicella-zoster immune are transmitted intrapartum, and who have disseminated disease. 13 globulin (‍VariZIG) for possible 10% are transmitted postnatally. Evidence linking zoster during Drvaricella? Pham Congenital or intrauterine HSV pregnancy with congenital varicella ∼ 19,2‍​ 0 infection is very rare, accounting is lacking. ‍ for only 5% of neonatal cases, and Staphylococcal and streptococcal is often accompanied by congenital We would not recommend VariZIG at skin infection, listeriosis, and malformations, which our patient this point. Based on maternal history 13,14​ congenital candidiasis are possible; does not appear to have. ‍ As and timing of clinical presentation, however, given the extensive skin Dr Hilgenberg pointed out, clinical our suspicion of primary varicella manifestation, it would be unusual manifestations of neonatal HSV in this infant is low. Required to have such a well-appearing infant infections usually fall into 3 disease criteria for VariZIG administration with normal vital signs and benign types: SEM disease, CNS disease, are significant exposure to laboratory results. Lastly, congenital and disseminated disease. SEM varicella (‍such as being an exposed syphilis is also less likely, because disease accounts for 45% of cases newborn) and inclusion in one – the maternal syphilis screen during and typically presents at 10 to 14 of the following populations: (‍1) 15 17 pregnancy had negative results days of life. ‍ Skin manifestations immunocompromised children, (‍2‍) and because the infant, apart from typically include small clustered pregnant women without evidence the rash, has no other clinical vesicles with surrounding erythema of immunity, (‍3) newborns whose manifestations suggestive of that mature into pustules and then mothers had an onset of chickenpox syphilis. erosions with overlying eschar between 5 days before delivery and formation over 1 to 3 days. Lesions In terms of management, empirical 48 hours after delivery (‍VariZIG is not indicated if the mother has begin at the site of inoculation, coverage with acyclovir, principally ≥ ’ most commonly at sites of skin for HSV at this point, is prudent zoster), (‍4) hospitalized premature infants of 2‍8 weeks gestation injury or prolonged contact with while the patient is being9 worked whose mothers lack immunity the cervix, and have the potential to up for neonatal HSV. However, our ’ spread locally or disseminate more suspicion of HSV, particularly of against VZV, and (‍5) hospitalized broadly. In contrast, congenital HSV CNS and disseminated disease, is premature infants of <2‍8 weeks commonly presents with vesicles at low. Thus, we do not recommend gestation or with a birth weight of birth. Cutaneous scars often are also 1000 g or less,18,2‍​ 1 regardless of maternal 18 ∼ repeating lumbar puncture at this present. CNS disease represents point. We agree with obtaining HSV Drimmunity Stewart. ‍ 30% of cases, with onset at 16 and VZV PCRs from blood and HSV to 19 days. Disseminated disease, PCRs from the conjunctivae, nares, accounting for the remaining 2‍5%, oropharynx, and rectum. In addition, A pediatric ophthalmologist was usually presents at 7 to17 12‍ days, with we recommend that HSV/VZV PCRs, consulted and found no evidence of many cases at <7 days. However, a Gram-stain, a bacterial culture, a conjunctivitis, keratitis, cataracts, newborns with disseminated potassium hydroxide stain, and a optic nerve swelling, chorioretinitis, HSV infections are often critically fungal culture be obtained from the or uveitis. Over the next 72‍ hours, ’ ill, and 60% to13, 75%17​ have CNS lesions. An ophthalmology exam to new similar lesions appeared on the involvement. complete the workup for TORCH infant s face, scalp, hands, and feet. ’ infections, including neonatal HSV The older lesions became dull and VZV is also unlikely because the disease, should also be performed. flat. A pediatric dermatologist was patient s mother has a history of The patient has been on ampicillin consulted. primary varicella in early childhood, and gentamicin, empirically, for corroborated by her history of bacterial sepsis. Given the lack of Dr Marqueling, as a pediatric subsequent episodes of zoster. signs and symptoms suggestive of dermatologist, what is your Thus, her dermatomal eruption sepsis, it would be reasonable to Drdifferential Marqueling diagnosis (Pediatric for this rash? during pregnancy should reflect a discontinue empirical antibiotics Dermatology) localized reactivation of VZV rather if the blood culture is negative for than a primary varicella infection, bacteria at 48 hours. Lastly, it is which would have put the infant important to consider noninfectious In addition to the infectious disease at risk for congenital or neonatal etiologies for this rash, such as etiologies, bullous diseases presenting VZV transmission. Viremia from primary dermatologic conditions. in the neonatal period should be Downloaded from www.aappublications.org/news by guest on September 25, 2021 4 Stewart et al considered. In particular, I am this possible finding have for our predictive of systemic mastocytosis, concerned about bullous mastocytosis, patient? although this has not been studied Dr Marqueling 2‍5 given the vesicles and small bullae in the pediatric population. with overlying erythematous and Regarding the other diagnoses on ’ ’ edematous papules and plaques and the differential, no viral cytopathic the questionably present Darier s Darier s sign is said to be present changes were identified, and an sign on my examination. Bullous when the skin urticates, becoming immunostain for HSV had negative lesions in cutaneous mastocytosis edematous and red, similar to a results, providing no support for a usually develop within erythematous hive, after stroking it with a blunt herpetic infection. No neutrophilic papules or plaques on involved skin. object. It is caused by the release of infiltrate or subepidermal split were Other conditions I would include in histamine from mast cells and is seen identified to support chronic bullous my differential diagnosis, though Drin conditionsStewart such as mastocytosis. disease of childhood. No blister was extremely uncommon, are chronic identified to support an inherited bullous disease of childhood (‍linear blistering disease. Thus, overall, this immunoglobulin A disease), EB Do you have additional skin biopsy was compatible with (‍including bullous dermolysis of recommendations regarding cutaneous mastocytosis. the newborn), and epidermolytic diagnostic workup, given your ichthyosis. Chronic bullous disease Dr Marqueling, what is mastocytosis? Drconcerns Marqueling and exam findings? Dr Marqueling of childhood is a rare autoimmune bullous disease that typically presents at 1 to 6 years of age with widespread, I would follow-up on blood and tense, bullae-forming rosettes. Rare The mastocytoses are disorders lesion cultures and PCRs and reports of neonatal presentation with characterized by mast cell 2‍2‍ continue acyclovir until viral PCR bullae at birth have been reported. accumulation in tissues throughout results returned. Given my concern EB is a group of inherited disorders the body. They are caused by a for possible bullous mastocytosis characterized by skin fragility. in the c-tyrosine-kinase or a neonatal blistering disorder, I Although severity later in life varies by gene. It is estimated that <2‍00000 Drrecommend Stewart a skin biopsy. type of EB, neonatal presentation may people total 2‍in6 the United States be similar in all types, presenting with are affected. Severity ranges 2‍from7 generalized mechanically-induced cutaneous to systemic disease. ∼ blisters and erosions. Additional After 48 hours, all cultures and PCRs Cutaneous mastocytosis accounts features may include absent or were negative for infection, so anti- for 50% of cases, occurs primarily dystrophic nails and cutaneous infectives were discontinued. A skin in children, and presents most often localized absences of skin (‍large biopsy was performed. Dr Reiger, can shortly after birth, with 80% of cases Dryou R describeieger (Dermatopathologist) your findings? ulcers on the lower extremities, each occurring before age 2‍ and 50%2‍ 6,of2‍​ 8 with well-demarcated edges and cases resolving by adolescence. ‍ a red, shiny base). Epidermolytic The most common form is a solitary ichthyosis may present in ways A skin biopsy showed an infiltrate mastocytoma, presenting as a single that are nearly indistinguishable of monomorphic cuboidal and tan to brown papule or small plaque. from EB, with spontaneous or fusiform cells in the upper third Infants with multiple lesions, ranging mechanically-induced bullae as the of the dermis (‍Fig 3). There was from a few to a couple hundred, are most prominent feature. Features a mild superficial dermal edema, diagnosed with urticaria pigmentosa. that may help to differentiate the 2‍ but there was no evidence of a In the most severe form, diffuse conditions include a thickened, red, subepidermal split. The cells in cutaneouspeau mastocytosis, d’orange patients and macerated base with bullae or the infiltrate contained small have nearly confluent erythema with raw denuded areas. Incontinentia central nuclei and lightly granular a rough texture. When pigmenti also crossed my mind, but amphophilic cytoplasm, and they mast cells are stimulated, they may as this is X-linked dominant trait, it stained positive for CD117 and degranulate and release histamine, is usually not2‍ compatible3,2‍​ 4 with life in the mast cell tryptase, confirming causing lesions of mastocytosis to male fetuses. ‍ the diagnosis of cutaneous Dr Stewart become raised, or in some cases, to mastocytosis. The mast cells did blister. A definitive diagnosis is made not express CD2‍5. The expression by confirming the presence of an ’ ’ of CD2‍5 on cutaneous mast cells accumulation2‍8 of mast cells on skin You mention Darier s sign. What is from adult patients with urticaria biopsy. Darier s sign,2‍7 as described this, and what implication does pigmentosa has been shown to be above, is common. Serum tryptase, Downloaded from www.aappublications.org/news by guest on September 25, 2021 PEDIATRICS Volume 141, number 3, March 2018 5 marrow biopsy would not change the treatment of this patient at this time because he currently has no signs of systemic involvement, so I recommend deferring it. Treatment α of systemic mastocytosis includes biologics such as interferon- -2‍b and tyrosine-kinase inhibitors and

should be started30 at signs of systemic Drinvolvement Stewart .

’ What do you recommend for our patient s management now that we Drhave Marqueling a diagnosis?

In terms of treatment, I recommend FIGURE 3 starting the histamine receptor A punch biopsy showed an infiltrate filling the upper third of the dermis. The infiltrate was composed blockers cetirizine (‍an oral of monomorphic spindled and cuboidal cells with small central nuclei and lightly granular histamine 1 receptor antagonist amphophilic cytoplasm. A, Hematoxylin and eosin stain (original magnification× 4). B, Hematoxylin with a long track record of safety and eosin stain (original magnification 40). C, Mast cell marker CD117 (original magnification 10). × × in infants) and ranitidine (‍an oral D, Mast cell tryptase (original magnification× 10). histamine 2‍ receptor antagonist, which also protects against gastric hypersecretion because of the

increased 31number of mast cells in the a marker of overall mast cell burden, Although the patient has had stomach). For topical treatment, is often elevated in more widespread no diarrhea and no evidence I recommend topical cromolyn, cases. of hepatosplenomegaly, a liver Dr Stewart a , for existing ultrasound would be prudent. I also closed lesions to try to prevent recommend a CBC count to evaluate ’ formation of urticarial lesions and for bone marrow involvement. bullae. I also recommend Given the extent of our patient s Finally, I would obtain a serum 2‍% ointment, a topical , cutaneous involvement, are tryptase level, which is more likely to for open and exposed lesions, and you concerned about systemic be elevated in patients with systemic 0.1% ointment, a involvement? If so, what studies and disease. If lymphadenopathy, topical , to decrease hepatosplenomegaly, or ill- Drtreatment Marqueling might be indicated? skin inflammation. Patients should appearance are present, evaluation have ready access to injectable for systemic mastocytosis should epinephrine because of the increased include a CBC panel, LFTs, and, risk of anaphylaxis; parents should Although the bullous presentation is potentially, an abdominal ultrasound, be educated on when and how striking, the number and relatively a bone scan, and a bone marrow to use it. discrete nature of his plaques places biopsy. A CBC panel, LFTs, and him into the category of urticaria abdominal ultrasound were It is common for mastocytosis to pigmentosa, as opposed to the conducted for this patient and involute in childhood, but some yielded normal results. The authors cases persist until puberty. We more severe diffuse cutaneous ’ mastocytosis. With the more common of a 2‍0-year retrospective study discussed this entity at length with forms of childhood mastocytosis, have suggested that the results of a the patient s parents, and discussed solitary mastocytoma and urticaria bone marrow biopsy, if normal, were the potential triggers and/or mast pigmentosa, there is rarely systemic predictive of resolution of pediatric cell degranulators that should be involvement. Unlike adult-onset mastocytosis if presenting before avoided, such as physical stimuli mastocytosis, internal organ the age of 2‍ and, if abnormal, might (‍friction, pressure, and scratching involvement is rare but can be severe predict progression to ongoing2‍9 lesions), medications (‍nonsteroidal when present. systemic mastocytosis. A bone anti-inflammatory drugs, opiates, Downloaded from www.aappublications.org/news by guest on September 25, 2021 6 Stewart et al Abbreviations codeine, and polymyxin), and foods secondary to gastric hypersecretion. (‍citrus, strawberries, tomatoes, Bleeding resolved with omeprazole, spinach, specific cheeses, egg whites, and he is now maintained on ranitidine CBC: complete blood cell CNS: central nervous system Drand S certaintewart fish). and cromolyn sodium. His lesions are now primarily hyperpigmented CRP: C-reactive Streptococcus protein ’ with almost complete resolution EB: epidermolysis bullosa of blistering. For the lesions, he GBS: group B Dr Marqueling s treatment HSV: herpes simplex virus recommendations were initiated, and receives topical cromolyn daily and topical steroids as needed. He has an LFT: liver function test the patient was discharged from the PCR: polymerase chain reaction Dr Hilgenberg epinephrine autoinjector with him hospital. How is the patient doing now? SEM: skin, eyes, and mouth at all times but has never needed to TORCH: toxoplasmosis, other, use it. Because his laboratory work rubella, cytomegalovi- At 2‍ years of age, he is doing well. remains normal, indicating there is no rus, or herpes He had 1 visit to the emergency systemic involvement, he has not had VariZIG: varicella-zoster immune department at 5 months of age for a bone marrow biopsy to evaluate for globulin hematemesis and melena presumed hematologic involvement. He continues VZV: varicella-zoster virus to be caused by Mallory Weiss tears to grow and develop normally.

POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

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Downloaded from www.aappublications.org/news by guest on September 25, 2021 8 Stewart et al Well-Appearing Newborn With a Vesiculobullous Rash at Birth Sarah E. Stewart, Jody L. Lin, Jennifer L. Everhart, Trung H. Pham, Ann L. Marqueling, Kerri E. Rieger and Sarah L. Hilgenberg Pediatrics 2018;141; DOI: 10.1542/peds.2017-0236 originally published online February 6, 2018;

Updated Information & including high resolution figures, can be found at: Services http://pediatrics.aappublications.org/content/141/3/e20170236 References This article cites 19 articles, 4 of which you can access for free at: http://pediatrics.aappublications.org/content/141/3/e20170236#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 September 25, 2021 Well-Appearing Newborn With a Vesiculobullous Rash at Birth Sarah E. Stewart, Jody L. Lin, Jennifer L. Everhart, Trung H. Pham, Ann L. Marqueling, Kerri E. Rieger and Sarah L. Hilgenberg Pediatrics 2018;141; DOI: 10.1542/peds.2017-0236 originally published online February 6, 2018;

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