Date post: | 06-Apr-2018 |
Category: |
Documents |
Upload: | emily-eresuma |
View: | 217 times |
Download: | 0 times |
of 24
8/3/2019 Newborn Skin? 12.12.2011
1/24
Mariposa Wolford MD
12/12/2011
AM Report
8/3/2019 Newborn Skin? 12.12.2011
2/24
RG, 53 day old male Transferred from Las Vegas due to severe dermatitis
and FTT
PMHx: born at 39 weeks, via C/S (breech). Nuchalcord. Birth wt 3.35kg.
Stayed in NICU since respiratory distress and
meconium aspiration, had ROS work-up and wasd/cd after 15 days (was intubated and treated forpna/possible sepsis with amp and gent. Also got rashon his chest)
8/3/2019 Newborn Skin? 12.12.2011
3/24
OSH course Started as erythematous area on chest, then to trunk
and extremities.
Extensive work up at OSH. Seen by GI, Cardiology,
and ophtho.
Also had fever and LP was done and not c/wmeningitis
Treated with Rocephin and Keflex for MSSA bloodculture. Later switched to amikacin and cefepime.
8/3/2019 Newborn Skin? 12.12.2011
4/24
Course prior to PCMC Seen by dermatology in Las Vegas. Biopsy
thought to be spongiotic dermatitis.
Started on 2.5% hydrocortisone.
Also , slightly high eosinophil count
Still FTT & poor feeding. Admitted again for this.
8/3/2019 Newborn Skin? 12.12.2011
5/24
Evidence of infection Positive blood cultures on Jan 12 and 14 GPC
Treated with Keflex and Rocephin. Had been
afebrile. Unsure of how long trtmt course was.
Negative bld Cx on Jan 16 and 18, then positiveagain on Jan 20
Skin: switched to triamcinolone x2weeks for rash
8/3/2019 Newborn Skin? 12.12.2011
6/24
Continued course OSH Left subclavian line placed Jan 21st
First fever on Jan 24th
PCR positive for influenza. Started on Tamiflu andRocephin.
Blood culture from Jan 24th with GNB. Central lineremoved. Echo done, unremarkable.
8/3/2019 Newborn Skin? 12.12.2011
7/24
Suspected immune problem Normal serum immunoglobulins: IgA 64, IgG 369,
IgM 79.
Jan 25th plts 639K. Mean plt volume enlgd.
Jan 27
th
plts 81K. Seen by ophtho and not thought to have corneal
keratinization.
Cultures came back as MSSA bld and MRSA
from wound. Ear culture grew Citrobacter koseri from Jan 20th
C diff negative Jan 25th. HIV negative.
8/3/2019 Newborn Skin? 12.12.2011
8/24
Additional w/u prior to PCMC Seen by GI for feeding issues. Poor wt gain
attributed to metabolic demand fromconstant skin sloughing w/ insensiblelosses due to poor barrier of his skin.
Fecal leukocytes, FOBT and fecal elastasewere all nl. CMP wnl. Zinc level wnl.
Feeds supplemented with 20% IL and formulachanged to Elecare 24kcal. Pt still did notgain wt on this regimen.
8/3/2019 Newborn Skin? 12.12.2011
9/24
At time of admission to PCMC Transferred to PCMC Jan 29th.
Mom stating infant improving with feedings.
On amikacin, cefepime and ofloxacin otic drops.
For skin, off triamcinolone since had skin breakdown.Using vaseline.
Afebrile at first, then temp to 38.2 early am 1/29.
Dermatology consulted here.
8/3/2019 Newborn Skin? 12.12.2011
10/24
Physical exam on admission Wt 3.49kg (1st%ile) Lngth 53cm (3rd%ile) T 38.2 HR 160 RR 40 BP 93/38 92% RA
GEN: lying supine, upset w/ exam, but calms
HEENT: NCAT, AFOSF, some swelling of eyelids.Yellow purulent discharge in medial aspects ofeyes bilaterally. RR present BL. PERRL. EOMI.Normal appearing sclera. TMs not visualized dueto sloughing skin in ears. MMM. Lips without
scaling, but thickened appearance, non-erythematous skin, no mucosal lesions. Nopharyngeal erythema/exudate.
8/3/2019 Newborn Skin? 12.12.2011
11/24
NECK: supple and no lymphadenopathy. CV: RRR. Normal S1, S2. gradeI-II/VI systolic
murmur heard best at LSB. No radiation to axillaeor back. CRT 2s centrally.
CHEST: some brth holding, then shallowbreathing. Lungs CTAB with some transmittedupper airway sounds.
ABD: S/NT/ND and no HSM or masses. BS nl
EXTR: No clubbing, cyanosis or edema. GU: nl male, testes descended BL and
circumcised.
8/3/2019 Newborn Skin? 12.12.2011
12/24
More exam NEURO: CN II-XII grossly intact. Grossly normal
muscle tone & strength. No apparent focal motoror sensory deficits.
SKIN: Scaling, erythematous rash diffusely overentire body. Face and chest most affected w/ Lg
scales of skin flaking off these areas. Extremitieswith blanching erythema. No visible open lesionsor areas of serous weeping.
8/3/2019 Newborn Skin? 12.12.2011
13/24
Course here at PCMC Tachycardia on admit. NS bolus x1.
LP done. CBC, CRP, VRP and Syphilis sent.
Derm, ID, Optho and Rheumatology consulted.
Elecare feeds increased to 27kcal. Ical, Mg, phoschecked
Switched to Vanc, Gent and Meropenem.
Needed NP suctioning overnight.
8/3/2019 Newborn Skin? 12.12.2011
14/24
Labs Mg 2.2, phos 5.4, ical 1.4, Zinc 50L, prealbumin
10, Vanc trough 16.7
CMP notable for Na 151H, Cl 111H, CO2 28H,BUN 25H, prot 5.0L, tbili
8/3/2019 Newborn Skin? 12.12.2011
15/24
Labs WBC 10.6, Hgb 7.2, Hct 23.4, Plts 107
Diff: 6B/20N/67L/5mono/2eos
HSV PCR qns
CRP 8.4 Biotinidase activity normal
Cortisol 27.3H (2-23)
ACTH normal at 37
Repeat Na 147
C5 normal at 11
8/3/2019 Newborn Skin? 12.12.2011
16/24
Differential Diagnosis: Nutritional deficiency dermatitis, from zinc, vitamin B2,
B3 or B6 deficiency or biotinidase deficiency or CF
Ichthyosis (specifically bullous congenitalichthyosiform erythroderma aka epidermolytichyperkeratosis)
Recessive X-linked ichthyosis
Nethertons syndrome
Lamellar ichthyosis
Dermatitis: contact vs irritant with id reaction Seborrheic dermatitis with id reaction
Atopic dermatitis that is secondarily infected
8/3/2019 Newborn Skin? 12.12.2011
17/24
Differential diagnosis ctd. Organic acidemias
Multiple carboxylase deficiency
Epidermolytic hyperkeratosis associated with an
immune issue such as Ommens syndrome
Leiners disease
Leukocyte adhesion deficiency type 1
Staph scalded skin syndrome
Wiskott-Aldrich syndrome
8/3/2019 Newborn Skin? 12.12.2011
18/24
Nethertons syndrome High IgE level. Pts usually develop allergies to
peanuts, eggs and fish.
Rare autosomal recessive genodermatosis withcongenital ichthyosiform erythroderma,
trichorrhexis invaginata (bamboo hair), and atopicdiathesis as well as FTT.
Chronic skin inflammation can cause scaling andexfoliation.
Patients are pre-disposed to life-threateninginfections, sepsis and dehydration.
DNA testing of SPINK5 gene
8/3/2019 Newborn Skin? 12.12.2011
19/24
Nethertons syndrome kiddo
8/3/2019 Newborn Skin? 12.12.2011
20/24
Ichthyosis Scaly skin, comes from Gr, fish
Genetic coding error in keratin causes differentphenotypes from epidermolysis bullosa toichthyosis
Defects in intracellular enzymes lead to diseaseslike X-linked ichthyosis or congenitalichthyosiform erythroderma.
Defects in cells themselves from abnl keratin cancause epidermolytic hyperkeratosis or llamellarichthyosis
8/3/2019 Newborn Skin? 12.12.2011
21/24
X linked ichthyosis Uncommon, 1:2,000 to 1:6,000 males
Worldwide and no ethnic or racial predominance.
Desquamation or pronounced peeling seen w/in afew wks or mos after birth persistent, darkbrown scaling on arms, legs, trunk, and sides ofneck & lower abd.
Palms, soles, hair, nails, teeth and mucosa not
involved.
8/3/2019 Newborn Skin? 12.12.2011
22/24
What are these diseases? Ommens syndrome; auto. rec SCID with
desquamation
RAG1/RAG2 mutations
LAD1; failure of lymphocytes to traffic/APC issues
Wiskott Aldrich: recurrent infections
Leiner disease: recurrent diarrhea, wasting andseborrheic dermatitis. Defect in complement 3,4
or 5.
8/3/2019 Newborn Skin? 12.12.2011
23/24
Other & Lab findings X linkedichthyosis
Cryptorchidism in as many as 25% of pts About 50% of adults exhibit asymptomatic
punctate corneal opacities
Will have decreased steroid sulfatase
Can measure this by 1) enzyme assays, 2)cholesterol sulfate accumulation in varioustissues 3) FISH
8/3/2019 Newborn Skin? 12.12.2011
24/24
Our patient Had CT of head to look for abscessnone found.
Bone scan also unrevealing.
Skin biopsy
Trichogram from eyebrow hairs for Netherton's:No trichorrhexis invaginata, nodosa, ortrichothiodystrophy noted. Hairs appear normal.
Serum sulfatase normal
Discharged and went back home withoutdefinitive diagnosis, but was doing much better.