Jain Chapter 1 - Basic Science Flashcards
What has developed by 3-4 weeks gestational age?
Single layer of ectoderm
What has developed by 6 weeks gestational age?
Outer flattened periderm and inner, cuboidal germinal (basal) layer
Germinal layer in contact w/ underlying mesenchyme (germinal layer produces entire epidermis)
What has developed by 7 weeks gestational age?
*Fetal basement membrane
Tooth primordia
What has developed by 8-12 weeks gestational age?
Epidermal stratification begins ~8 weeks (Completed by 2nd trimester)
*Appearance of Melanocytes, Langerhans cells, Merkel cells
Dermal-subcutaneous boundary distinct
What has developed by 9-12 weeks gestational age?
Appearance of anchoring filaments/hemidesmosomes
Hair follicle and nail primordia seen
What has developed by 12 weeks gestational age?
- Formation of dermoepidermal junction (DEJ)
- Nail bed starts to keratinize, proximal nail fold forms
Type III collagen appears
What has developed by 12-14 weeks gestational age?
Parallel ectodermal ridges (fingerprints)
Eccrine and sebaceous gland primordia seen
Fibroblasts actively synthesizing collagen and elastin in dermis
What has developed by 12-24 weeks gestational age?
Melanin production (12–16 wks), melanosome transfer (20 wks)
Hair follicles differentiate during 2 nd trimester (7 concentric layers present)
What has developed by 15-20 weeks gestational age?
Periderm is shed (periderm is part of vernix caseosa) [20–21 weeks]
*Follicular keratinization, nail plate completely covers nail bed
Papillary/reticular boundary distinct, dermal ridges appear
What has developed by 22 weeks gestational age?
Trunk eccrine gland primordia
Elastic fiber seen
What has developed by 22-24 weeks gestational age?
*Mature epidermis complete (w/ interfollicular keratinization)
Adipocytes appear under dermis
How thick is:
- eyelid skin
- palmoplantar skin
eyelid = 0.04 mm
palmoplantar skin = 1.5
Keratinocytes comprise approximately ___ of epidermal cells
80–85%
Total epidermal turnover time
Average 45–60 days (30–50 days from stratum basale to stratum corneum and approximately 14 days from stratum corneum to desquamation)
Epidermal self-renewal maintained via stem cells in basal layer of ___ epithelium and the bulge region of hair follicles (latter location only activated with epidermal injury)
interfollicular
Keratinocytes produce ___
keratin filaments (syn: intermediate filaments or tonofilaments), which form the cell’s cytoskeletal network; this provides resilience, structural integrity, along with serving as a marker for differentiation (ie. basal layer: K5/14)
Type I (acidic) epithelial keratins
K9–28, chromosome 17
Type I (acidic) hair keratins
K31–40 (old nomenclature: hHa1–hHa8, Ka35, Ka36)
Type II (basic) epithelial keratins
K1–8 and K71–80, chromosome 12
Type II (basic) hair keratins
K81–86 (old nomenclature: hHb1–hHb6)
Location of expression and disease: 1 & 10
Suprabasal keratinocytes
Epidermolytic hyperkeratosis, UnnaThost PPK
Location of expression and disease: 1 & 9
Palmoplantar suprabasal keratinocytes
Vorner PPK
Location of expression and disease: 2e & 10
Granular and upper spinous layer
Ichthyosis bullosa of Siemens
Location of expression and disease: 3 & 12
Cornea
Meesman corneal dystrophy
Location of expression and disease: 4 & 13
Mucosal epithelium
White sponge nevus
Location of expression and disease: 5 & 14
Basal keratinocytes
Dowling-Degos disease, EBS
(Do not confuse Dowling-Degos with Degos disease: Dowling-Degos: AD, reticulated pigmentation over skin folds Degos (malignant atrophic papulosis): occlusion + tissue infarction)
Location of expression and disease: 6a & 16
Outer root sheath
Pachyonychia congenita I
Location of expression and disease: 6b & 17
Nail bed
Pachyonychia congenita II
Location of expression and disease: 8 & 18
Simple epithelium
Cryptogenic cirrhosis
Location of expression and disease: K81 K86
Hair
Monilethrix
Location of expression and disease: 19
Stem cells
Stratum Basale (Germinativum)
- What % are stem cells?
- Expression of ___, which is a marker for proliferative activity
- De novo expression of ___ occurs, forming keratin filaments which insert into both desmosomes and hemidesmosomes and form keratinocyte cytoskeleton
- 10%
- ornithine decarboxylase (ODC)
- K5/14
Ornithine decarboxylase (ODC)
- Stimulated by?
- Blocked by?
- stimulated by UVB
- partially blocked by retinoic acid/corticosteroid/vitamin D3
Stratum Spinosum
- New synthesis of ___; K5/14 still present (not de novo)
- Cells contain ___
- K1/K10
- lamellar granules (lamellated bodies or odland bodies)
Desmosomes
- Transmembrane proteins
- Desmosomal plaque proteins
- Transmembrane proteins: desmoglein 1/3, desmocollin 1/2 (desmosomal cadherins)
- Desmosomal plaque proteins: plakoglobin (γ-catenin), desmoplakin 1/2, keratocalmin, desmoyokin, band 6 protein, envoplakin
Adherens junctions
- What kind of cell junction?
- Linked to?
- classical cadherins (namely E and P)
- linked to actin cytoskeleton via cytoplasmic plaque proteins (α, β, γ-catenin)
Gap junctions
- transmembrane channels formed by six ___ monomers
- connexin
Tight junctions
- major constituents are?
- claudins and occludins
Gap junction - Connexin 26 diseases
- Vohwinkel syndrome
- PPK + deafness
- KID syndrome
Gap junction - Connexin 30 diseases
Hidrotic ectodermal dysplasia (HED)
Gap junction - Connexin 31/30.3 diseases
Erythrokeratoderma variabilis (EKV)
Adherens junction diseases - b-catenin
Muscular dystrophy
Hemidesmosome diseases - a6b4
- Junctional EB with pyloric atresia
- Occular CP
Hemidesmosome diseases - Plectin
- EB simplex with muscular dystrophy
- PNP
Hemidesmosome diseases - BPAG1 (BP 230)
- Bullous pemphigoid (BP)
- PNP
Hemidesmosome diseases - BPAG2 NC16A (BP 180)
- BP
- Pemphigoid gestationis
- LABD
Hemidesmosome diseases - BPAG2 COOH (BP 180)
CP
Desmosome diseases - Plakoglobin
Naxos syndrome
Desmosome diseases - Desmoplakin
Carvajal syndrome
Desmosome diseases - Plakophilin
Ectodermal dysplasia (ED) + with skin fragility
Desmosome diseases - Desmocollin
Subcorneal pustular dermatosis (SCPD)
Desmosome diseases - Desmoglein 1
- Pemphigus foliaceous, (PF)
- striate PPK
- PNP
- also target for SSSS
Desmosome diseases - Desmoglein 3
- Pemphigus vulgaris (PV)
- PNP
Hemidesmosome diseases - K5&14
- EBS
Hemidesmosome diseases - 322
- JEB intermediate and severe
- anti-epiligrin
- CP
Hemidesmosome diseases - collagen 7
- Bullous SLE
- DEB
- EBA