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Embryology for the Rest of Us

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Embryology for the Rest of Us Embryology For the Rest of Us Derrick Adams, DO, FAOCD Private Practice Red Bluff, CA Conflicts of Interests No conflicts My Id is in conflict with my Ego Ectoderm Follicular Units CNS – brain/spinal chord Keratinocytes Eye Merkel Cells Eye lid glands Melanocytes Parotid gland Eccrine glands Lacrimal gland Apocrine glands Lens Sebaceous glands Cornea Nerves Ear bones Teeth Facial Cartilage Are you looking…? Anterior 2/3 of Tongue? Parotid Duct & Gland? Teeth? Distal Urethra of Penis? Lower 1/3 of Anal Canal? Hard Palate? Buccal Mucosa? Mammary Gland & Ducts? Basic Germ Layers Ectoderm Mesoderm Endoderm Gastrulation Selective Affinity Dr. Heinz Christian Pander “Founder of Embryology” Trilaminar membrane Neurulation refers to the folding process in vertebrate embryos, which includes the transformation of the neural plate into the neural tube. Neural Plate Surface Ectoderm (Periderm & Epidermis) Neural Tube (Neural Crest & CNS) Question? What happens to the notochord at the termination of embryological development? Let’s Build an Epidermis! PERIDERM Plato movie? Periderm Prevents Adhesions Transport Antimicrobial Antioxidant Electrically neutral? Form Vernix after sloughed Periderm Absent Periderm? Peridermopathies Popliteal Pytergium Syndrome Cocoon Syndrome Intraoral epithelial fusions in murine models Keeps developing intermediate keratinocytes from fusing “Teflon Coat” no stick surface Pytergium syndromes Vernix Caseosa Vernix Lanugo hair, periderm, and sebum Can be absent preterm Protection? Moisturization? Antibacterial? Lubrication? Birth Canal Potentially swallowed Not vernix Collodion Membrane col·lo·di·ona syrupy solution of nitrocellulose in a mixture of alcohol and ether, used for coating things, chiefly in surgery and in a former photographic process. Word usage peaked in 1850 Spinous Kertinocytes (stratified) P63, P73-LIKE Allows epi to stratify and proliferate Adult (acro-dermatoungual-lacrimal-tooth) Ectrodactyly ectodermal dysplasia-cleft lip/palate Ankyloblepharon-ectodermal dysplasia clefing syndrome (AEC, “Hay-Wells) Corneocytes (s. corneum) Interconnected by histidine rich protein called filaggrin which is derived from components of the keratohyalin. Through friction or degradation of desmosomes and fillagrin they eventually shed (1300 cells/cm2/hour on forearm) Desmoglein 1 alpha Desmocollin Dust in your house! Progression of keratinization# of keratohyalin and lamellar body granules increases Increase in # of organelle-depleted cornified cells Neonate’s skin barrier not completely mature until a few weeks after birth Full barrier function 3 wks of age Defects of Epidermal Maturation X-linked ichthyosis Steroid sulfatase Lamellar ichthyosis TGase 1 Ectodermal Dysplasia Ectodermal dysplasia (almost 180 and counting) Hypohidrotic Ectodermal hypoplasia (peg teeth, sparse hair, poor sweating, xerosis) Pachonychia Congenita and its variants (dystrophic nails, natal teeth, keratoderma) EEC: Ectrodactyly Ectodermal Dysplasia Cleft palate synderome (lobster claw, hair, deafness, dry skin, nails dystrophy, cleft palate) All these are Ectodermal with some mesodermal related structures. Let’s Build Our Dermis! MESODERM What is the difference between a carcinoma and a sarcoma embryologically? JAOCD: 2015 Vol 33. page 43-44 Team Mesoderm Langerhans cells Adipocytes Fibrocytes Macrophages Blood and lymph vessels Muscles Somites Building blocks of the vertebrate body plan Template for nervous system Bone & musculature development Segmentation Somities “Induce” Into… sclerotome (cartilage) syndotome (tendons) myotome (skeletal muscle), dermatome (dermis) endothelial cells Somitogenesis Clock & Wave NOTCH Signaling Somities “Induce” Into… sclerotome (cartilage) syndotome (tendons) myotome (skeletal muscle), dermatome (dermis) endothelial cells Dermatomes Dermatomes EXITING MESODERM We now have our epidermis and dermis Induction Dermis controls epidermis Neural Plate Surface Ectoderm (Periderm & Epidermis) Neural Tube (Neural Crest & CNS) NEURAL CREST Neural Crest What comes from neural crest? Common origin of diseases/syndromes Doctor why do you look at my teeth and eyes? I’m here for a skin problem! Neurocristopathies Cleft lip/palate neurofibromatosis phakomatosis pigmentovascularis Dermal melanocytosis Waardenburg syndrome Piebaldism Many, many more! Waardenburg Syndrome Pax3 -- migration from neural crest & activation of melanocyte proliferation Which types of Waardenburg Syndrome involve Pax3 mutations? Piebaldism KIT (c-KIT) Belongs to a family of proteins called receptor tyrosine kinases Growth, proliferation, and migration of melanoblasts depend upon Steel Factor binding Nonfunctional KIT results in Piebaldism Melanocytes Skin Hair Iris Inner ear Nervous system Heart Blood vessels Leptomeninges What do Melanocytes Do? We make about 1 gram of it Evolutionary function of feathers Barrier function repair for basal keratinocyte Upregulates IL-6 and IL-8 Role in control of adipose cells Calcium pumps of keratinocytes (confetti hypopigmentation of Darier’s dz) Antibiotic (mycosis in darker skin) Anti inflammatory (prevent feather breakdown) Sympathetic fight or flight (changing skin colors in reptiles) Detoxification of free radicals Provide dopamine to aid in synapse formation in early nerve devolpement Arianayagam S, Ryan TJ. Human pigmentation: A side effect adapted from a primitive organism's survival, acting through cell attachment with an affinity for the keratinocyte and for elastin: Part I. Indian Dermatol Online J 2014;5:201-9 Arianayagam S, Ryan TJ. Human pigmentation: A side effect adapted from a primitive organism's survival, acting through cell attachment with an affinity for the keratinocyte and for elastin: Part I. Indian Dermatol Online J 2014;5:201-9 “The melanocyte is part of a caring community of cells surviving in a threatening environment; a nurse maid that uses the electromagnetic spectrum and mechanical forces and evolves over 500 million years to a point at which its original behavior is forgotten and skin color and its social influence dominates the literature read by dermatologists. “ Melanocyte Timeline Present in epidermis in 1st trimester Fully functional in 2nd Melanin production begins at 3-4 months Transfer to keratinocytes at 5th month **Melanoblasts travel with peripheral nerves Melanocytes Melanoblasts arise from neural crest along dorsal neural tube (more to follow…) Migration & Differentiation into melanocytes Middle of 1st Trimester in epidermis Melanin genesis and transfer How do melanocytes populate the skin? *Dorso-lateral migration (remember piebaldism example) * Ventral neural crest (Schwann Cells) Melanocyte Puberty Melanobasts multiply as they travel MITF crucial Tyrosinase appears (c-kit) tyrosinase kinase receptor SOX10, Pax 3, TYRP-2 Wnt/Frizzled protein B-catenin signaling pathway Notch and MAPK Melanoblasts Reservoirs in adult skin? Bulge area Dermis Cutaneous nerve stem cells Schwann Cells Dorso-lateral migration Melanocyte Melanoblast Dorso-lateral migration Melanocyte Melanoblast Migrating Neural Crest Cells Ventro-medial migration Dorso-lateral migration Melanocyte Melanoblast Migrating Neural Crest Cells SCP Schwann Cell Precursor Ventro-medial migration Dorso-lateral migration Melanocyte Melanoblast Melanoblast Migrating Neural Crest Cells SCP Schwann Cell Immature Precursor Schwann cell Ventro-medial migration Dorso-lateral migration Melanocyte Melanoblast Melanoblast Melanocyte Migrating Neural Crest Cells SCP Schwann Cell Immature Myelinating Schwann Cell Precursor Schwann cell Ventro-medial migration Dorso-lateral migration Melanocyte Melanoblast Melanoblast Melanocyte Migrating Neural Crest Cells SCP Schwann Cell Immature Myelinating Schwann Cell Precursor Schwann cell Ventro-medial migration Dorso-lateral migration Melanocyte Melanoblast Melanoblast Melanocyte Migrating Neural Crest Cells SCP Schwann Cell Immature Myelinating Schwann Cell Precursor Schwann cell Ventro-medial migration Lymphatic Pump=Metastatic Pump? Melanocytes accumulate around elastin Electron microscopy shows dermal melanocytes encircling elastin fibers 4 “Elastin guide wire” Tangential arrangement in upper dermis Horizontal in lower dermis (My obligatory Osteopathic slide) Intruders Dermal Melanosis & Dermal Melanocytomas What is the difference between a blue nevus, nevus of Ito/Ota and a Mongolian spot? By Week 20 all dermal melanocytes should be absent (by conventional thinking)* Fibrous extracellular sheath on EM Dermal Melanosis Mongolian Spots Nevus of Ito Nevus of Ota Ota Segmental pattern in cranial nerves V &VII Hint at relationship with Schwann cells So what pathway would these have migrated through? Why do Mongolian Spots resolve and Ito/Ota do not? Unique phenomenon Extracellular protective fibrous sheath starts to degrade in womb Early childhood sees most intense destruction Mongolian Spots Inborn errors of metabolism Hurler’s disease & GM1 gangliosidosis are most common When to suspect? Part of the Phakomatosis spectrum Other genodermatosis Merkel Cell Not Neural Crest origin in mammals (including here for traditional reasons)7,8 Epidermal origin Probably Merkel cells and “Merkel-like” cells Mammalian Merkel cells are descended from the epidermal lineage. Dev Biol. 2009 Dec 1;336(1):76-83 Langer Lines Not really an embryology issue Cadaver lines Never meant to guide surgery Borges's and Kraissl's
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