1.
INTEGUMENTARY EMBRYOLOGY
Melanocytes are found in which epidermal layer?
Correct Answer
A. Stratum basale
Explanation
Melanocytes are found in the stratum basale, the deepest layer of the epidermis, at the dermoepidermal junction.
2.
A young black girl shows isolated patches of skin and hair that lack melanin pigment. In
addition, other skin lesions are observed that look suspiciously like a malignant melanoma.
What is the most likely diagnosis?
Correct Answer
C. Piebaldism
Explanation
Piebaldism is an autosomal dominant disorder and is basically a localized albinism.
3.
A young infant shows extremely stretchable and fragile skin, hypermobile joints, and cigarette-paper scars over the knees. What is the most likely diagnosis?
Correct Answer
A. Ehlers-Danlos syndrome
Explanation
Ehlers-Danlos syndrome is an autosomal dominant disorder involving the gene for peptidyl
lysine hydroxylase.
4.
A young infant shows skin blisters over the entire body with generalized skin erosion.
Pathology indicates a cleft between the epidermis and dermis. What is the most likely diagnosis?
Correct Answer
B. Junctional epidermolysis bullosa
Explanation
Junctional epidermolysis bullosa refers to a group of autosomal recessive disorders caused
by a mutation in the gene for laminin 5.
5.
The administration of which of the following agents may result in discoloration of both
deciduous and permanent teeth?
Correct Answer
E. Tetracycline
Explanation
Tetracyclines are bound to calcium in newly formed teeth both in utero and in young children. They may cause discoloration and enamel dysplasia
6.
Which of the following cell types is normally not found in the epidermis?
Correct Answer
E. Dendrocyte
Explanation
Dendrocytes are found in the dermis
7.
Bullus pemphigoid is an autoimmune disorder that attacks the basement membrane between the epidermis and the dermis via IgG antibodies. Between what two layers would the IgG be deposited?
Correct Answer
B. Stratum basale and papillary dermis
Explanation
The correct answer is "stratum basale and papillary dermis". Bullous pemphigoid is an autoimmune disorder that targets the basement membrane between the epidermis and the dermis. In this condition, IgG antibodies are deposited between the stratum basale (the deepest layer of the epidermis) and the papillary dermis (the upper layer of the dermis). This deposition leads to the formation of blisters and bullae in the affected area.
8.
A patient presents with a 3 month history of progressive weakness. On physical examination, you find bilateral decreases in strength in the legs and back. In addition, she has a rash surrounding both eyes. Laboratory values are significant for elevated LDH and CPK, and a positive ANA. What is the diagnosis?
Correct Answer
E. Dermatomyositis
Explanation
These are typical findings for the CD8+ T cell mediated injury to myofibers in dermatomyositis. If there were no rash, polymyositis would be likely. Additional tests to diagnose include EMG of the muscles, and biopsy.
9.
A 70 year old man presents with discomfort in his thigh for 2 months. X ray revealed a radiolucent mass without any calcification. Biopsy of the tissue showed a pinwheel pattern of fibroblasts. Which soft tissue tumor does he most likely have?
Correct Answer
C. Malignant fibrous histiocytoma
Explanation
The real tipoff here is that the biopsy has a pinwheel patten. Dr. Hunter describes it as 'storiform'. Malignant fibrous hisiocytoma is the most common sarcoma of the elderly
(60s & 70s). Not only is it common, but it's deadly since it metastasizes up to 50% of the time.
10.
Rhabdomyosarcoma is the most common soft tissue tumor of which age group?
Correct Answer
A. 0-15
Explanation
Rhabdomyosarcoma (malignant skeletal muscle tumor) is a small blue cell tumor and is the most common soft tissue sarcoma in children. It comes in three types, perhaps the most important is embryonal. It may present as a grape-like mass protruding from the vagina. Knowing this rather distinct presentation should be easy points on a test.
11.
A 50 year old man has had a painless, slowly enlarging mass in his arm for the past 2 years. A biopsy reveals a herringbone pattern. What is the most likely diagnosis?
Correct Answer
B. Fibrosarcoma
Explanation
Like question 33, the real tipoff is knowing that cellular pattern on the biopsy. Fibrosarcoma looks like a herrigbone patten. This tumor commonly presents as a slow growing painless mass in the upper extremity. Here's what the biopsy would look like. Below it is what a herringbone pattern looks like
12.
Small vessel vasculitic myopathy best describes which of the following?
Correct Answer
E. Nemaline myopathy
Explanation
Dermatomyositis is a complement mediated, small vessel vasculitic myopathy that results in perifascicular atrophy. Dr. Brumback made a point to repeat that statement. Polymyositis differs grossly in that it does not involve a rash and microscopically in that lymphocytes invade the muscle fibers. Both polymyositis and dermatomyositis result in proximal muscle weakness (ie trouble getting out of a chair) and elevated plasma muscle enzymes (CKP, myoglobin). Kearns-Sayre syndome is one of many mitochondrial myopathies. This presents as eye muscle paralysis. Leber's optic atrophy results in blindness.
13.
You are looking at a biopsy specimen from a suspicious skin lesion. The tissue consists mainly of spindle cells in a storiform pattern, and it stains with CD34. What is the most appropriate statement to tell the patient?
Correct Answer
B. This is characteristic of dermatofibrosarcoma, a rare but treatable skin cancer.
Explanation
The storiform patten of spindle cells is most characteristic of dermatofibrosarcoma. The other lesions tend to involve keratinocytes. Another reasonable answer for this biopsy would be Kaposi sarcoma, especially if we knew that the patient was immunosuppressed.
14.
A 45 year old patient presents to you with dozens of velvety pigmented papules on his trunk and extremities that appear stuck on. He is concerned since he had very few nevi before 3 months ago. Which of the following aspects of his history is most relevant to his current lesions:
Correct Answer
B. He has a 40 pack/year history of smoking
Explanation
He most likely has many seborrhei keratoses. These can develop as an explosion of growth, which is associated with paraneoplastic syndrome. This is called the Leser-Trelat sign. His age and smoking history put him at high risk for lung cancer
15.
After walking in the woods while wearing sandals, a 20 year old man develops a weeping erythematous rash with vesicles. Which type of cell is least likely to be involved in this reaction?
Correct Answer
D. CD8+ T cell
Explanation
The delayed type IV hypersensitivity reaction begins when Langerhans cells are exposed to an antigen (such as poison ivy). The antigen is then presented to CD4+ T cells. These T cells are responsible for producing the eczema upon re-exposure. They produce cytokines which attract inflammatory cells such as neutrophils.
16.
Erythema multiforme is a hypersensitivity reaction in the skin characterized by targetoid vesicles. It can be triggered by infection, drugs, and malignancy, among others. How is Stevens-Johnson syndrome different from erythema multiforme?
Correct Answer
A. Stevens-Johnson syndrome involves mucosal membranes
Explanation
Stevens-Johnson syndrome is a more extensive form of erythema multiform that involves both the skin and the mucosal membranes. Both are type II hypersensitivity reactions.
17.
The H&E of a skin biopsy seen below was most likely taken from a patient with:
Correct Answer
C. Basal cell carcinoma
Explanation
This is an example of palasading cells. They form a ring around the tumor islands. This is evident in cancers resulting from the basal cell layer in the epidermis.
18.
How would you treat a patient with the lesion seen in this skin biopsy?
Correct Answer
D. Steroids
Explanation
This is bullous pemphigoid, a disease caused by IgG antibodies against the basement membrane between the epidermis and dermis. In the picture, the epidermis and dermis have separated. In pemphigus vulgaris, the antibodies are directed against desmosomes within the epidermis. In either case, immunosuppression by steroids is the most effective treatment.
19.
This tissue section came from a skin lesion that is best described as:
Correct Answer
C. Common, benign
Explanation
This is seborrheic keratosis. It is a common, benign lesion which exhibits epidermal hyperplasia and horn cysts
20.
Your patient has an erythematous, weeping lesion on her face. If you treat this lesion with steroids, which of the following preparations is most appropriate?
Correct Answer
C. Non-fluorinated lotion
Explanation
Don't use a potent steroid on warm, wet areas such as the face, axilla, and groin. Potent steroids tend to be fluorinated. Since the lesion is 'wet' (weeping), it would be better to use a lotion. Lotions, powders, and wet dressing are better for acute inflammation which tends to be wet. Chronic inflammation should be treated with an ointment. For example, an area of severe scaling lichenification would be treated with a fluorinated ointment.