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Onset at birt or during the neonatal period Later onset

Most likely
congenital Pustules
Prominenr Blistering and/or Reccurent
ichtyhosiform Preceding or Proceding Perioficial and
generalized otosions infectinos
erythodern coexistent exanthenalous acrral
(see Ch. 57) lesions eruption accentuation of
characteristic of scaly and/or
History of primary erosive
Common dermatosis darmatitis
Rare membrane

Asses for Asses for  Involvement  Diarrhea  Histologic  Exposure in

of palms &  FTT confirmation medication
sales (if required)  Improvement
 Maternal within 2-6
vaginal weeks upon
 Trichorrhexis  Perioficial & Keratinocyte
intertriginous candidiasis necrosis no discontinuation
invaginata Netherton
of offending
 Elevated lgE syndrome accentuation skin biopsy
 Temperature agent
 FTT, electrolyte Staphylococcal
abnormalities instability/fever
scalded skin
 Irritability syndrome
 Extracularieous
 “Tiger tail” Trichothiody  Staphylococcus
hairs strophy aureus infection

 Vacuolated Neutral lipid  Hyperkeratosis Epidermolylic  Cutaneous  Relatively thin

leukocytes storage (+/-) ichthyosis induration epidermis,
disease  EHK on skin (bullous CIE) alopecia macropage >
biopsy  Prominent T-cell infiltrate
LAD & HSM on skin biopsy
eaks & swirls of Condradi  Marked Primary
athery scale hunnerman peripheral dermtosis e.g.
ppled epiphyses happle esinophillia, atopic
ilateral cataracts sydrome  Leathery Diffuse elevated lgE dermatitis,
cutaneous cutaneous  Acanthosis, psoriasis (see
induration maslocytosis parakeratosis table 10.2)
 Perloweal Sjogren  + Darlet sign & primarily T-
glistening Larsson  Increased on cell infiltrate
white dots syndrome skin biopsy on skin biopsy
 spasticly
other forms of
ency (see
 Sensorineural KID table 10.2 and
deatness syndrome  Cleft lip & AEC syndrome Omenn Ch. 60)
 Keratitis (+/-)  Ankyloblephero syndrome
 Later n
discretehyper  Prominent scalp
keratotieplaq involvement
Congenital GVHD in the “Nutritional
ues, PPK 
cutaneous setting of atermatitis (see
candidiasis SCID1 Drug eruption Ch. 51)
 Severe CNS Gaucher
disease, HSM disease
 WBC acid B-