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Questions and Answers
What is the primary role of desmosomes in the epidermis?
What is the primary role of desmosomes in the epidermis?
- Regulating blood flow in the epidermis.
- Providing adhesion between keratinocytes. (correct)
- Protecting skin from UV rays.
- Producing skin collagen.
Which antibody is primarily associated with pemphigus foliaceous?
Which antibody is primarily associated with pemphigus foliaceous?
- Desmoglein-1 antibodies. (correct)
- Desmoglein-3 antibodies.
- IgE antibodies.
- Autoantibodies against collagen.
Where does pemphigus foliaceous mainly affect the skin?
Where does pemphigus foliaceous mainly affect the skin?
- The lower epidermis.
- The upper epidermis. (correct)
- The mucosal layer.
- The dermal layer.
What is a characteristic clinical feature of pemphigus vulgaris?
What is a characteristic clinical feature of pemphigus vulgaris?
What does a positive Nikolsky sign indicate?
What does a positive Nikolsky sign indicate?
What does the Bulla Spread Sign indicate?
What does the Bulla Spread Sign indicate?
Where are common sites for lesions in pemphigus?
Where are common sites for lesions in pemphigus?
What is tested in a Tzanck smear?
What is tested in a Tzanck smear?
Which type of therapy can be classified as a novel treatment for mechanobullous disorders?
Which type of therapy can be classified as a novel treatment for mechanobullous disorders?
What type of management is essential for individuals with mechanobullous disorders?
What type of management is essential for individuals with mechanobullous disorders?
What genetic inheritance pattern is associated with Darier's Disease?
What genetic inheritance pattern is associated with Darier's Disease?
In Hailey-Hailey Disease, which gene defect is primarily responsible?
In Hailey-Hailey Disease, which gene defect is primarily responsible?
What distinguishes albinism from other skin conditions?
What distinguishes albinism from other skin conditions?
Which chromosome is associated with the genetic factors of Darier's Disease?
Which chromosome is associated with the genetic factors of Darier's Disease?
What accurately defines alopecia?
What accurately defines alopecia?
Which enzyme is primarily defective in individuals with albinism?
Which enzyme is primarily defective in individuals with albinism?
What best characterizes cicatricial alopecia?
What best characterizes cicatricial alopecia?
Which statement best describes ocular albinism?
Which statement best describes ocular albinism?
Which type of alopecia is marked by patchy hair loss?
Which type of alopecia is marked by patchy hair loss?
What is a notable feature of piebaldism?
What is a notable feature of piebaldism?
What genetic inheritance pattern is associated with piebaldism?
What genetic inheritance pattern is associated with piebaldism?
What leads to the condition known as nevus depigmentosus?
What leads to the condition known as nevus depigmentosus?
At what stage of life does nevus depigmentosus typically appear?
At what stage of life does nevus depigmentosus typically appear?
Which factor is most often associated with causing vitiligo?
Which factor is most often associated with causing vitiligo?
What is a primary characteristic of Relaxed Skin Tension Lines (RSTL)?
What is a primary characteristic of Relaxed Skin Tension Lines (RSTL)?
In which area are Relaxed Skin Tension Lines characterized as being V-shaped?
In which area are Relaxed Skin Tension Lines characterized as being V-shaped?
How should incisions be made during surgery to promote effective healing?
How should incisions be made during surgery to promote effective healing?
What is the correct definition of diascopy?
What is the correct definition of diascopy?
What does the diascopy procedure primarily involve?
What does the diascopy procedure primarily involve?
What is a primary use of diascopy in the field of dermatology?
What is a primary use of diascopy in the field of dermatology?
How is erythema defined in dermatological terms?
How is erythema defined in dermatological terms?
What indicates a non-blanching response in erythema?
What indicates a non-blanching response in erythema?
What is the mainstay treatment for Pemphigus Vulgaris?
What is the mainstay treatment for Pemphigus Vulgaris?
What additional treatment requires urine monitoring?
What additional treatment requires urine monitoring?
What is the purpose of Rituximab in treating pemphigus?
What is the purpose of Rituximab in treating pemphigus?
What differentiates Pemphigus Foliaceous from other forms?
What differentiates Pemphigus Foliaceous from other forms?
Where is Desmoglein-1 predominantly found?
Where is Desmoglein-1 predominantly found?
What type of split is associated with Desmoglein-3?
What type of split is associated with Desmoglein-3?
What does a positive Nikolsky Sign indicate?
What does a positive Nikolsky Sign indicate?
What characterizes the Pemphigoid group of disorders?
What characterizes the Pemphigoid group of disorders?
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Study Notes
Relaxed Skin Tension Lines (RSTL)
- RSTL are crucial for minimizing scarring during surgical procedures.
- They do not correspond to arteries, veins, or nerves and primarily guide incision planning.
- On the upper spine, RSTL are characterized as being V-shaped.
- Incisions should be made along or parallel to skin tension lines to promote optimal healing.
Diascopy
- Defined as the Vitro Pressure Test, used primarily to assess skin lesions.
- It involves pressing lesions with a glass slide rather than injecting dye or scraping skin.
- A key application in dermatology is evaluating skin lesions for diagnostics.
Erythema
- Refers to redness of the skin, often indicating various underlying conditions.
- The non-blanching response in erythema means redness does not fade under pressure, signaling more serious issues.
Pemphigus Disorders
- Pemphigus Foliaceous is characterized by desmoglein-1 antibodies and affects the upper epidermis.
- Pemphigus Vulgaris is identified by the presence of oral lesions in 95% of cases, and a positive Nikolsky sign indicates separation of epidermal layers with pressure.
- The Bulla Spread Sign indicates the extension of bulla margins when lateral pressure is applied.
- Common sites for pemphigus lesions include the scalp, oral mucosa, and trunk.
Tzanck Smear and Histopathology
- Tzanck smear tests for Tzanck cells in vesicles, important for diagnosing blistering diseases.
- Histopathology of Pemphigus Vulgaris shows a suprabasal split resembling a row of tombstones.
Treatment and Management
- The mainstay treatment for Pemphigus Vulgaris is vigorous systemic steroids.
- Cyclophosphamide, whose use requires urine monitoring, serves as an additional treatment option.
- Rituximab targets CD20 on B cells, aiming to modify the immune response.
Acantholysis
- Acantholysis is the rupture of desmosomes, leading to skin blister formation.
- Pemphigus Foliaceous is associated with desmoglein-1 antibodies, found primarily in the upper epidermis and oral mucosa.
Inheritance Patterns and Genetic Factors
- Darier's Disease follows an Autosomal Dominant pattern and is linked to chromosome 3.
- Hailey-Hailey Disease is associated with the ATP2A2 gene.
Alopecia Types
- Alopecia refers to hair loss and can be categorized into cicatricial (damage to stem cells) and non-cicatricial types (trichotillomania).
- Alopecia Areata presents with circular areas of complete hair loss and is classified under Non-Cicatricial Alopecia.
Albinism and Related Conditions
- Albinism is defined by a defective tyrosinase enzyme, leading to impaired melanin production.
- Ocular albinism affects only the eyes, while piebaldism is noted for the presence of a white forelock and exhibits Autosomal Dominant inheritance.
- Nevus depigmentosus results from defects in the transfer of melanosomes, often manifesting at birth.
Vitiligo
- The destruction of melanocytes by auto-antibodies is a primary cause of vitiligo.
- Segmental vitiligo features unilateral patches that do not cross the midline, differing from the bilateral nature of non-segmental vitiligo.
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