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?

Hashimoto-Pritzker disease Signup and view all the answers

What are Birbeck granules primarily involved in?

Antigen processing and presentation Signup and view all the answers

How does eosinophilic granuloma typically present?

Single lytic bone lesion Signup and view all the answers

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

Oral cromolyn sodium Signup and view all the answers

What is a common feature of Letterer-Siwe disease?

Widespread systemic involvement Signup and view all the answers

What characteristic is true of ash leaf macules?

They are usually oval shaped and white in color. Signup and view all the answers

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

Tuberous sclerosis Signup and view all the answers

What percentage of hemangiomas typically disappear by age 5?

50% Signup and view all the answers

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

Superficial hemangioma Signup and view all the answers

Which statement best describes the growth pattern of superficial hemangiomas?

They grow rapidly for up to one year and then shrink. Signup and view all the answers

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

They are typically dark-colored. Signup and view all the answers

Hemangiomas are more common in which of the following groups?

Premature infants Signup and view all the answers

What is a distinguishing feature of plaque-type hemangiomas?

They are flat with greater width than height. Signup and view all the answers

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

Early childhood Signup and view all the answers

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

Formation of honey-colored crusting Signup and view all the answers

What is the primary cause of typical impetigo?

Staphylococcus aureus Signup and view all the answers

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

Bullous impetigo Signup and view all the answers

What should be done immediately when impetigo is suspected?

Initiate topical treatment and take a bacterial culture Signup and view all the answers

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

Having five café-au-lait macules over 5 mm in diameter requires evaluation for neurofibromatosis. Signup and view all the answers

Which treatment is used for mild cases of impetigo?

Topical mupirocin ointment Signup and view all the answers

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

Formation of blisters in the epidermis Signup and view all the answers

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

Antibiotic ointments and non-stick dressings Signup and view all the answers

Which of the following accurately describes urticaria pigmentosa?

Multiple lesions that may number from several to thousands Signup and view all the answers

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

Hemangiomas next to critical structures Signup and view all the answers

What is a common presentation of a solitary mastocytoma?

A single brown-yellow papule or nodule Signup and view all the answers

What recent treatment has shown promise for hemangiomas?

Propranolol Signup and view all the answers

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

Abnormality in the c-kit gene Signup and view all the answers

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

Number of lesions present Signup and view all the answers

What symptom is associated with rubbing a mastocytoma?

Release of mast cell granules and hive formation Signup and view all the answers

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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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.