Lecture 3 Part 2
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Questions and Answers

What is the primary goal of treating mastocytosis?

  • Increase histamine release
  • Prevent mast cell degranulation
  • Relieve symptoms caused by mast cell mediators (correct)
  • Eliminate mast cells

Which of the following is NOT a common symptom of Hand-Schuller-Christian disease?

  • Petechiae (correct)
  • Exophthalmos
  • Diabetes insipidus
  • Lytic bone lesions

What mutation is primarily linked to prolonged systemic disease in mastocytosis?

  • c-kit mutation (correct)
  • NRAS mutation
  • JAK2 mutation
  • GNAS mutation

Which variant of histiocytosis presents with widespread red, brown papules that crust over?

<p>Hashimoto-Pritzker disease (C)</p> Signup and view all the answers

What are Birbeck granules primarily involved in?

<p>Antigen processing and presentation (A)</p> Signup and view all the answers

How does eosinophilic granuloma typically present?

<p>Single lytic bone lesion (A)</p> Signup and view all the answers

Which treatment option is commonly used for mastocytosis to prevent degranulation of mast cells?

<p>Oral cromolyn sodium (A)</p> Signup and view all the answers

What is a common feature of Letterer-Siwe disease?

<p>Widespread systemic involvement (A)</p> Signup and view all the answers

What characteristic is true of ash leaf macules?

<p>They are usually oval shaped and white in color. (C)</p> Signup and view all the answers

Which of the following conditions is associated with multiple ash leaf macules?

<p>Tuberous sclerosis (C)</p> Signup and view all the answers

What percentage of hemangiomas typically disappear by age 5?

<p>50% (D)</p> Signup and view all the answers

Which type of hemangioma has a pebbly surface and is typically bright red?

<p>Superficial hemangioma (C)</p> Signup and view all the answers

Which statement best describes the growth pattern of superficial hemangiomas?

<p>They grow rapidly for up to one year and then shrink. (C)</p> Signup and view all the answers

Which of the following is NOT a characteristic of ash leaf macules?

<p>They are typically dark-colored. (C)</p> Signup and view all the answers

Hemangiomas are more common in which of the following groups?

<p>Premature infants (B)</p> Signup and view all the answers

What is a distinguishing feature of plaque-type hemangiomas?

<p>They are flat with greater width than height. (A)</p> Signup and view all the answers

What is the most common age group for impetigo to occur?

<p>Early childhood (C)</p> Signup and view all the answers

What characteristic of impetigo lesions can help differentiate it from herpes simplex infections?

<p>Formation of honey-colored crusting (C)</p> Signup and view all the answers

What is the primary cause of typical impetigo?

<p>Staphylococcus aureus (C)</p> Signup and view all the answers

What type of impetigo is characterized by blister formation due to exfoliative toxin?

<p>Bullous impetigo (C)</p> Signup and view all the answers

What should be done immediately when impetigo is suspected?

<p>Initiate topical treatment and take a bacterial culture (A)</p> Signup and view all the answers

Which of the following is true regarding café-au-lait macules?

<p>Having five café-au-lait macules over 5 mm in diameter requires evaluation for neurofibromatosis. (C)</p> Signup and view all the answers

Which treatment is used for mild cases of impetigo?

<p>Topical mupirocin ointment (B)</p> Signup and view all the answers

What can happen if desmoglein type 1 protein is cleaved by the exfoliative toxin in bullous impetigo?

<p>Formation of blisters in the epidermis (A)</p> Signup and view all the answers

What is the general treatment for superficial hemangiomas that commonly ulcerate?

<p>Antibiotic ointments and non-stick dressings (B)</p> Signup and view all the answers

Which of the following accurately describes urticaria pigmentosa?

<p>Multiple lesions that may number from several to thousands (B)</p> Signup and view all the answers

What type of hemangioma is more likely to require treatment due to its location?

<p>Hemangiomas next to critical structures (D)</p> Signup and view all the answers

What is a common presentation of a solitary mastocytoma?

<p>A single brown-yellow papule or nodule (A)</p> Signup and view all the answers

What recent treatment has shown promise for hemangiomas?

<p>Propranolol (A)</p> Signup and view all the answers

Which mechanism is most well described in the context of excessive mast cell proliferation?

<p>Abnormality in the c-kit gene (A)</p> Signup and view all the answers

What is the main distinguishing factor between solitary mastocytoma and urticaria pigmentosa?

<p>Number of lesions present (C)</p> Signup and view all the answers

What symptom is associated with rubbing a mastocytoma?

<p>Release of mast cell granules and hive formation (A)</p> Signup and view all the answers

Flashcards

Impetigo

A common bacterial skin infection, often seen in young children, presenting with sticky, honey-colored crusts and typically affecting the face.

Bullous Impetigo

A type of impetigo characterized by large blisters, often caused by Staphylococcus aureus strains producing exfoliative toxins.

Exfoliative Toxin

A toxin produced by certain bacteria like Staphylococcus aureus, causing breakdown of cell-to-cell adhesion in the skin's upper layer.

Desmoglein Type 1

A protein found in desmosomes, which are structures responsible for holding skin cells together.

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Café-au-lait Macule

Flat, sharply bordered brown skin patches, often present at birth or developing in early childhood.

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Neurofibromatosis

A genetic condition characterized by excessive growth of nerve tissue, often accompanied by café-au-lait macules and other skin lesions.

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Five or More Café-au-lait Macules

The presence of five or more café-au-lait macules greater than 5 millimeters in diameter.

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Evaluation for Neurofibromatosis

When the presence of café-au-lait macules raises concerns about neurofibromatosis, a thorough evaluation should be conducted.

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What is an Ash Leaf Macule?

A white, flat, oval-shaped mark on the skin, usually measuring 1-3 cm in diameter. They are often present at birth but may develop within the first two years of life.

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What is tuberous sclerosis?

A rare skin condition characterized by the presence of multiple ash leaf macules, seizures, and developmental delays.

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What is a hemangioma?

A common benign tumor of blood vessels that often grows rapidly in infancy before slowly shrinking until it disappears. They can occur on the skin, mucous membranes, and internal organs.

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What is a superficial hemangioma?

Bright red, raised bumps on the skin with a pebbly surface. They grow rapidly for a year and then gradually shrink and disappear.

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What are deep hemangiomas?

A type of hemangioma that is usually bluish in color and can be difficult to distinguish from surrounding skin. They are located deeper in the skin.

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What is a mixed hemangioma?

A hemangioma that has both a superficial and deep component, appearing as a combination of raised red bumps and a bluish base.

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What is a plaque type hemangioma?

A type of superficial hemangioma that is flat and slightly raised, often covering a large area. They are frequently linked to other birth defects.

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What is ulceration in hemangiomas?

The process of a hemangioma becoming ulcerated, commonly occurring due to rapid growth.

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Hemangioma

A benign, vascular tumor that usually fades with time, but can cause problems if located near vital structures.

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Propranolol

A common treatment for hemangiomas that can accelerate their disappearance.

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Hemangioma Ulceration

Inflammation and potential infection of superficial hemangiomas, often causing pain.

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Mastocytosis

A rare disease characterized by an abnormal proliferation of mast cells.

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Urticaria Pigmentosa

The most common form of cutaneous mastocytosis, featuring multiple brown-yellow papules that often exhibit Darier's sign.

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Darier's Sign

The hallmark of mastocytosis, where rubbing a papule causes a red hive-like reaction.

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c-kit mutation

A genetic mutation in the KIT gene that leads to the uncontrolled growth and survival of mast cells.

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Systemic Mastocytosis

A rare, systemic form of mastocytosis where mast cells invade multiple organs, potentially leading to associated conditions.

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c-kit Gene

A gene encoding the KIT protein, a tyrosine kinase receptor, implicated in the abnormal proliferation of mast cells in mastocytosis.

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Letterer-Siwe disease

A type of histiocytosis that typically affects infants and presents with skin lesions, enlarged organs, and systemic involvement.

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Hand-Schuller-Christian disease

A type of histiocytosis that presents with diabetes insipidus, exophthalmos, and bone lesions.

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Eosinophilic granuloma

A type of histiocytosis that presents as a single, localized bone lesion.

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Hashimoto-Pritzker disease

A type of histiocytosis that affects newborns and presents with widespread skin papules and nodules that heal spontaneously.

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Langerhans cells

Specialized cells within the immune system that act as antigen-presenting cells, normally found in the skin.

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Study Notes

Impetigo

  • Impetigo is a common skin infection, typically affecting young children.
  • Characterized by superficial ulcers with honey-colored crusting, often on the face.
  • Can sometimes resemble herpes simplex, requiring a culture for accurate diagnosis.
  • Bullous impetigo presents as blisters at the site of infection, caused by a specific toxin.
  • Treatment usually involves topical antibiotics (e.g., mupirocin) for mild cases and oral antibiotics (e.g., dicloxacillin, cephalexin) for more severe cases, including Staph Scalded Skin Syndrome.
  • Bacterial culture is recommended but treatment should begin prior to results.

Café-au-Lait Macules

  • Common, usually harmless, flat, brown spots.
  • More frequent in African-Americans.
  • Can appear at birth or develop during childhood.
  • Five or more macules over 5mm in diameter in a patient should prompt evaluation for neurofibromatosis.
  • Infants with one or two macules are not at increased risk for neurofibromatosis.
  • Treatment is not typically needed.

Ash Leaf Macules

  • Uncommon, flat, white spots, typically oval-shaped.
  • Usually present at birth or develop within the first two years of life.
  • Measure 1-3 cm in diameter.
  • May be a manifestation of tuberous sclerosis.
  • Evaluation for tuberous sclerosis is warranted if multiple ash leaf macules or other associated symptoms are present.
  • No specific treatment is needed, primarily a need for observation.

Hemangioma

  • Common childhood vascular tumor.
  • Typically appears within the first few weeks of life.
  • More common in females and premature infants.
  • Two main types: superficial (red, pebbly, typically resolves by age 9) and deep (bluish, difficult to delineate borders).
  • Mixed hemangiomas are combinations of both types.
  • Most hemangiomas resolve spontaneously.
  • Treatment may be considered for hemangiomas located near critical structures or causing significant problems.
  • Methods may include intralesional steroids, systemic steroids, or propranolol.

Mastocytosis

  • Uncommon disorder, frequently presenting in children.
  • Characterized by excessive mast cell proliferation.
  • Two main forms: solitary mastocytomas (single lesion) and urticaria pigmentosa (multiple lesions).
  • Lesions appear as brown-yellow papules or nodules.
  • Diagnosis often confirmed by Darier's sign (hive-like reaction when the lesion is rubbed).
  • Treatment typically focuses on symptom relief using antihistamines and avoiding mast cell degranulators (like alcohol and opioids).

Histiocytosis

  • Rare but serious group of disorders.
  • Characterized by proliferation of abnormal histiocytes.
  • Can present at birth or later in childhood.
  • Several forms exist, including Letterer-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma, and Hashimoto-Pritzker disease.
  • Letterer-Siwe disease is often characterized by skin lesions, bone involvement, and systemic symptoms, presenting early in life.
  • Treatment varies depending on the specific type and extent of disease, and may range from topical therapy to chemotherapy.

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Lecture 3 Part 2 PDF

Description

Explore the characteristics and treatment options for common skin infections, including impetigo and café-au-lait macules. Learn about their appearance, diagnosis, and the significance of symptoms. This quiz provides insights into the management of these conditions effectively.

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