Skin Lesions Flashcards

1
Q

An obese 47-year-old man comes to the office for pain and drainage in the perineal region. A photograph is shown - hidradenitis. Medical history includes hypertension, hypercholesterolemia, and diabetes. Which of the following is the most important factor in reducing the risk of recurrence after surgery?

A) Adjuvant radiation
B) Antibiotic therapy
C) Extent of resection
D) Intralesional corticosteroid injection
E) Method of closure
A

The correct response is Option C.

This patient has hidradenitis suppurative (HS). There are various surgical treatments available to these patients with varying risk of recurrence. Surgical options include incision and drainage, surgical deroofing, local excision, and radical resection of all involved tissue. Reconstructive and closure techniques include secondary healing, primary closure, skin grafting and locoregional pedicled flaps. Numerous studies have looked at the outcomes with various closure techniques and they show that risk of recurrence is likely influenced more by the extent of disease rather than the method of definitive closure. Because of the recurrent nature of this disease, surgery has been considered as the only effective curative therapy for HS. Inadvertent compromise in the margin of resection may diminish the probability of successful healing. Radical resection of all hair-bearing skin with a 1- to 2-cm clear margin of normal tissue is the gold standard and the most important factor in reducing risk of recurrence. Recurrence rates tend to be higher after excision in regions where functional and aesthetic outcomes take priority, and often limit the extent of resection and compromise the ability to obtain clear margins (ie, axilla, perineum, breast). Historically, low-voltage radiation was used as a treatment modality for HS and it is thought to cause complete follicular destruction. Current studies on radiotherapy as treatment for HS are very limited because of concern regarding malignancies arising in radiation fields. Radiation should be reserved for individuals with severely recalcitrant disease and used with extreme caution in younger individuals. Bacterial burden may also diminish the probability of successful wound healing. Topical and systemic antibiotics are still the mainstay treatment for mild HS. Despite their widespread use, few studies have shown their efficacy. In addition, antibiotics do offer relief by reducing the burden of abscesses and pustules in some individuals, but recurrence in these people is frequent. Standard practice of managing acute flares with intra-lesional steroid injections lacks clinical evidence. It has been shown in a series of patients to reduce erythema, edema, size and pain, but no effect on recurrence of disease has been shown.

2018

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2
Q

A 50-year-old woman is evaluated for multiple firm, nodular, pink-colored lesions of the scalp, ranging in size from 2 to 4 mm. Examination of a specimen obtained on biopsy shows benign cylindroma. Which of the following is the most appropriate management of these lesions?

A) Cryotherapy
B) Electrodessication and curettage
C) Imiquimod therapy
D) Radiotherapy
E) Surgical excision
A

The correct response is Option E.

Cylindromas are benign adnexal tumors showing an eccrine and an apocrine differentiation. They are found most commonly on the scalp and face, and are more common in women. Solitary cylindromas are generally sporadic in nature. Multiple cylindromas can be seen in patients with Brooke-Spiegler syndrome as an autosomal dominant trait with variable penetrance.

Cylindromas may undergo malignant transformation, and therefore surgical excision is typically recommended, with close postoperative follow-up given high recurrence rates.

Cryotherapy, electrodessication and curettage, and imiquimod are not treatments for cylindromas.

Radiotherapy has been used to treat malignant cylindromas (also known as cylindrocarcinoma or adenoid cystic carcinoma), but not benign cylindromas.

2018

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3
Q

A 56-year-old woman presents with a 1-cm primary superficial basal cell carcinoma on the left volar mid-forearm. Medical history includes renal transplantation, carcinoma of the right breast managed with lumpectomy and radiation, and treatment for a gunshot wound to the left forearm. The basal cell carcinoma is located within the previous traumatic scar. Which of the following clinical features is an indication for Mohs micrographic surgery in this patient?

A) Basal cell carcinoma arising in traumatic scar
B) History of radiation
C) Immunocompromised status of patient
D) Size of basal cell carcinoma
E) Superficial basal cell carcinoma
A

The correct response is Option A.

The clinical feature in this particular patient that fulfills the criteria to get Mohs micrographic surgery is that the basal cell carcinoma is arising in a traumatic scar. There are many clear indications for Mohs micrographic surgery for basal cell carcinoma: certain size, histology, and anatomic location, all recurrent basal cell carcinomas, and the occurrence of basal cell carcinoma in irradiated skin, traumatic scars, areas with osteomyelitis/chronic ulceration/inflammation, and/or patients with genetic syndromes. Almost all primary basal cell carcinomas in the H and M zones, regardless of pathology (i.e. superficial, nodular, or aggressive), size, or health status of the patient, are candidates for Mohs micrographic surgery. In the L zone, most basal cell carcinomas are also candidates for Mohs micrographic surgery (except all superficial subtypes [irrespective of health of patient], or those less than 1 cm size in immunocompromised patients or nodular subtypes).

Area H: “Mask areas” of face (central face, eyelids [including inner/outer canthi], eyebrows, nose, lips [cutaneous/mucosal/vermilion], chin, ear and periauricular skin /sulci, temple), genitalia (including perineal and perianal), hands, feet, nail units, ankles, and nipples/areola.

Area M: Cheeks, forehead, scalp, neck, jawline, and pretibial surface.

Area L: Trunk and extremities (excluding pretibial surface, hands, feet, nail units and ankles).

In this clinical case, the patient has a basal cell carcinoma that has a favorable pathology (i.e. superficial subtype) in the L zone, not an indication for Mohs micrographic surgery, irrespective of tumor size. Additionally, with a 1-cm tumor of this pathology subtype, her immunocompromised state is not an indication for Mohs micrographic surgery, either. The radiation was remote from the area she developed her basal cell carcinoma, so it is not an indication, either.

2018

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4
Q

A 60-year-old man presents with a 6-mm lesion of the forehead. The patient states that it has enlarged over a period of 2 years. Examination of a biopsy specimen shows squamous cell carcinoma (adenoid subtype) with a 1.5-mm depth of involvement. Which of the following is the most likely risk factor for recurrence of this patient’s lesion after surgical excision?

A) Anatomic location
B) Depth of involvement
C) Growth rate
D) Histologic subtype
E) Size
A

The correct response is Option D.

A number of risk factors for recurrence have been identified for squamous cell carcinomas.

Histologic subtype is one such factor. Adenoid, adenosquamous, and desmoplastic subtypes are considered high risk for recurrence.

Anatomic location is another such factor and is typically considered in combination with the size of the lesion.

High-risk areas include the “mask” areas of the face (eyelids, eyebrows, periorbital, nose, lips, chin, temple, ear), as well as genitalia, hands, and feet. Lesions greater than 6 mm in these areas indicate a high risk for recurrence.

Mid-risk areas include the cheeks, forehead, scalp, and neck, with lesions greater than 10 mm indicating a high risk for recurrence.

Low-risk areas include the trunk and extremities, with lesions greater than 20 mm indicating a high risk for recurrence. Based on this, this patient with a 6-mm forehead lesion would not be considered at high risk for recurrence.

Rapid growth is a risk factor for recurrence. However, this patient’s lesion grew gradually over a period of 2 years and not rapidly.

Depth of involvement greater than 2 mm indicates a high risk for recurrence.

Other risk factors for recurrence include poorly defined borders, immunosuppression, prior irradiation, site of inflammatory process, neurologic symptoms, moderate/poor differentiation, and perineural/vascular involvement.

2018

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5
Q

Which of the following is the most appropriate surgical treatment recommendation for a 4-cm round sebaceous nevus of the scalp in a child?

A) Excision, skin grafting, and delayed tissue expansion
B) Serial excision without tissue expansion
C) Serial monitoring and selective excision
D) Tissue expansion and excision
E) Tissue expansion, excision, and rotational flaps

A

The correct response is Option C.

Historically, there has been a 10 to 15% reported malignant degeneration in nevus sebaceous in children and the recommendation had been for all lesions to be removed before puberty. More recent studies have shown malignant transformation of these lesions to be less than 1%. Therefore, the decrease in malignant change prompted a more conservative approach to nevus sebaceous based on serial monitoring and excision of lesions that are a source of irritation, difficult to monitor clinically, or aesthetically displeasing. Tissue expansion is an option in patients with giant nevus sebaceous and usually requires one to two rounds of expansion and serial excisions. However, this is not a first line treatment for a small 4-cm lesion. Although serial excision is an alternative reconstructive option, it is recommended only for larger lesions that can be excised in three stages or less. If that is not possible, then tissue expansion is the preferred procedure. Reconstruction with rotational flaps should be planned in consideration of natural facial aesthetic units and an effort should be made to maintain natural brow and hairline position and symmetry and avoid creating tension in the perioral, periorbital, and periauricular regions.

2018

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6
Q

Which of the following peripheral nerve tumors is most commonly associated with von Recklinghausen disease?

A) Astrocytoma
B) Glioblastoma
C) Neurilemoma
D) Neurofibroma
E) Schwannoma
A

The correct response is Option D.

A neurofibroma is a lesion of the peripheral nervous system, which is derived from Schwann cells, other perineural cell lines, and fibroblasts. Neurofibromas may arise sporadically, or in association with von Recklinghausen disease (neurofibromatosis 1 or NF1). A neurofibroma may arise at any point along a peripheral nerve, and comes in two varieties.

The plexiform neurofibromas are larger tumors that develop inside the body and tend to intimately involve the nerves, blood vessels, and other structures in the body. They can reside deep inside the body or closer to the skin. Plexiform neurofibromas can cause pain, numbness, weakness, and disfigurement. These tumors do have a small chance of becoming cancerous. Plexiform neurofibromas may also be asymptomatic.

Dermal (subcutaneous) neurofibromas are small, nodule-like tumors that grow on or just under the surface of the skin. They can be painful, itchy, disfiguring, or tender when touched, but they have no known potential to become cancerous. Dermal neurofibromas may also be asymptomatic.

Schwannomas are peripheral nerve sheath tumors that can be seen with NF1, but are more commonly associated with neurofibromatosis 2. The major distinction between a schwannoma and a solitary neurofibroma is that a schwannoma can be resected while sparing the underlying nerve, whereas resection of a neurofibroma requires the sacrifice of the underlying nerve. A neurilemoma is another name for a schwannoma.

Astrocytomas and glioblastomas are tumors of the central nervous system. Astrocytomas and optic gliomas can be seen in association with NF1.

2018

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7
Q

A 13-year-old girl is brought for evaluation because of a 4-month history of severe pain of the tip of the right index finger. There is no history of trauma. On examination, the finger appears normal with no visible swelling or discoloration. The pain is exacerbated by local pressure when the patient writes and during her weekly swimming lessons. MRI (T2-weighted) image is shown. Which of the following is the most likely diagnosis?

A) Digital fibroma
B) Giant cell tumor
C) Glomus tumor
D) Neuroma
E) Venous malformation
A

The correct response is Option C.

This lesion is a glomus tumor. Glomus tumors are benign hamartomas originating from the glomus body, a structure comprised of vascular and neural elements that is responsible for thermoregulation in the skin. These often inconspicuous tumors present with pain, point tenderness, and sensitivity to cold. X-ray studies may show cortical erosion of the bone adjacent to the lesion, and ultrasonography can provide confirmation. MRI is the most accurate imaging modality and the tumor appears as a bright, discrete mass on T2-weighted images.

Digital fibroma is a cutaneous fibroblastic proliferation and would be visible. It rarely causes pain and would not enhance on T2-weighted MRI imaging. Neuroma can cause focal pain as described in the vignette, but the enhancing focal lesion on the T2-weighted MRI effectively rules out solid masses such as neuroma or giant cell tumor (which is common but rarely causes pain or cold intolerance). Venous malformation would enhance on T2-weighted MRI, but would typically present with swelling and would not be as well circumscribed as the lesion shown here.

2018

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8
Q

A 72-year-old man comes to the office for evaluation of a 2-cm growth over the lower half of his nose that has been growing slowly during the past 15 years. A photograph is shown. Medical history includes acne rosacea. Physical examination shows a broad, exophytic mass. Which of the following is the most appropriate management?

A) Application of 5-fluorouracil
B) Direct excision and coverage with a forehead flap
C) Direct excision and coverage with a skin graft
D) Direct excision and healing by secondary intention
E) Observation only

A

The correct response is Option D.

Rhinophyma occurs on the far end of progressive acne rosacea involving the sebaceous glandular overgrowth of the skin. It most commonly occurs on the nose but phymas can occur elsewhere on the face.

In early stages, rhinophyma can be treated with isoretinoin and antibiotics; however, in advanced states it needs to be resected. Ablation modalities include dermabrasion, carbon dioxide laser excision, scalpel excision, or a combination of techniques. Since the procedure resects the skin to mid-dermis, skin grafting or other coverage is unnecessary and can lead to unsatisfactory aesthetic results. The best option is excision with secondary epithelialization.

2018

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9
Q

An 85-year-old man who takes an anticoagulant medication comes to the office for evaluation of a recurrent 1-cm nodular basal cell carcinoma at his nasal tip that has started to bleed intermittently. Medical history includes placement of a cardiac stent 1 month ago, after myocardial infarction. Which of the following is the most appropriate treatment for this patient?

A) Electrodessication
B) Excision with forehead flap
C) Pembrolizumab therapy
D) Superficial radiation therapy
E) Topical application of 5-fluorouracil
A

The correct response is Option D.

With a 5-year recurrence rate of about 3% for nodular basal cell carcinomas (BCC), superficial radiation therapy has become a viable alternative to Mohs micrographic surgery, which remains the gold standard for treatment of nonmelanomatous skin cancers.

Electrodessication has a high recurrence rate and would subject this anti-coagulated patient to the risk of bleeding after surgery or thrombosis should his anticoagulation be discontinued.

Excision with forehead flap would not be safe for a patient with a recent myocardial infarction.

Topical 5-fluorouracil is not indicated in the management of nodular BCC.

Pembrolizumab therapy is indicated for Stage IV melanoma.

2018

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10
Q

A 34-year-old woman comes to the office because of a 6 × 7-cm subcutaneous mass below the left scapula. Biopsy confirms dermatofibrosarcoma protuberans. To minimize recurrence yet maximize the chances of primary closure, which of the following is the most appropriate margin when planning wide local excision?

A) 5 mm
B) 10 mm
C) 20 mm
D) 40 mm
E) 50 mm
A

The correct response is Option C.

Several recent studies have confirmed that a surgical margin of 15 to 20 mm is associated with high rates of recurrence-free survival and primary closure when wide local excision is performed. Marginal excision is associated with higher rates of recurrence, and larger wide local excisions (>20 mm) are associated with similar recurrence-free survival but a much higher need for reconstructive surgery. Mohs micrographic surgery has shown promise, with higher initial clearance rates using smaller margins, but the question specifically addressed surgical margins when planning wide local excision.

2017

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11
Q

A 12-year-old girl develops a 12-mm nodule on her right cheek that grows slowly over 2 months. It is firm to the touch, mildly tender, and slightly bluish. There is no redness, ulceration, or visible punctum. Which of the following is the most likely diagnosis?

A) Hemangioma
B) Keratinous cyst
C) Pilomatricoma
D) Sebaceous nevus
E) Spitz nevus
A

The correct response is Option C.

Pilomatricoma (also known as pilomatrixoma or calcifying epithelioma of Malherbe) is a common, benign calcifying tumor of the hair appendages that mostly occur under the age of 20. Most occur in the head and neck, but the extremities and trunk are also affected. Surgical excision is the treatment of choice. Malignancy is very rare. Intraoperative findings show a calcific, friable mass adherent to the undersurface of the skin. Unlike a keratinous or sebaceous cyst, there is no discrete capsule or punctum (plugged pore). Recurrence is reported in the 1 to 2% range. This benign growth is related to a somatic (non-inherited) gene mutation CTNNB-1, that is involved in cell replication of the hair matrix.

Sebaceous nevus presents as a waxy textured skin patch, often present at birth. The scalp is a common site, although it may present elsewhere. Lesions are slow-growing and benign, but over the course of one’s lifetime, they have up to a 50% transformation rate to basal cell carcinoma, with squamous cell carcinoma less likely.

Hemangiomas are cutaneous blood vessel proliferations that are bright red or purple in color and are typically present at birth. While they are also common in childhood, they are more superficial in location and have a very different appearance versus pilomatricomas, which are subepithelial.

Spitz nevi are melanocytic lesions that can occur in children and adults. They can mimic melanoma though they are benign spindle cell lesions. Malignant transformation is not common, though atypical variants exist, therefore excision is recommended. They appear as dark brown or black macules on the skin.

2017

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12
Q

A 58-year-old Caucasian farmer comes to the office because of several pink scaly macules on his cheeks and nose. Biopsy of one of the lesions shows pleomorphic keratinocytes within the basal layer of the epidermis and hyperkeratosis, consistent with actinic keratosis. If left untreated, which of the following is the likelihood that these lesions will become malignant?

A) 0%
B) 10%
C) 30%
D) 50%
E) 70%
A

The correct response is Option B.

The patient has actinic keratoses (AKs). AKs are common in in people with significant sun exposure and are a response to ultraviolet radiation. The likelihood of malignant transformation to squamous cell carcinoma (SCC) is approximately 10%.

There are various treatment modalities for AKs, including cryotherapy, 5-flurouracil (5-FU), photodynamic therapy, superficial glycolic peels, and imiquimod. Cryotherapy with liquid nitrogen is commonly used for isolated lesions, while the other therapies are more commonly used for diffuse disease.

2017

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13
Q

A 7-year-old boy is evaluated because of new nodular lesions on his skin. Patient history includes a jaw cyst, pits in the hands and feet, and a treated meduloblastoma. Biopsy is planned. Which of the following is the most likely diagnosis?

A) Basal cell carcinoma
B) Melanoma
C) Merkel cell carcinoma
D) Sebaceous adenocarcinoma
E) Squamous cell carcinoma
A

The correct response is Option A.

The patient has basal cell nevus (Gorlin) syndrome. It is an autosomal dominant genetic condition affecting 1 in 56,000. Males and females are equally affected. It is caused by a mutation in the PTCH1 gene. Clinical characteristics include multiple basal cell carcinomas, odontogenic cysts of the mandible, facial dysmorphism, and skeletal abnormalities of the vertebrae, skull, and ribs. 5 to 10% of patients will develop medulloblastoma. The other malignancies are not associated with Gorlin syndrome.

2017

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14
Q

A 50-year-old woman with a history of scleroderma is evaluated because of a 1.5-cm lesion on her right cheek. Patient history includes basal cell carcinoma excision at the same site 3 years ago. A punch biopsy shows basal cell carcinoma (micronodular subtype). Which of the following is the most appropriate indication for Mohs micrographic surgery in this patient?

A) Histologic subtype
B) History of scleroderma
C) Location of lesion
D) Recurrence of lesion
E) Size of lesion
A

The correct response is Option D.

Mohs micrographic surgery is a surgical technique in which tumor excision and microscopic examination of tissue margins are performed by the same surgeon. Use of a beveled excision and careful mapping of the peripheral and deep margins of horizontal frozen sections allow for comprehensive examination of all the borders of the excised tissue, resulting in excellent cure rates. In addition to the high cure rate, Mohs surgery is a tissue-sparing procedure that is an important advantage in cosmetically and functionally sensitive areas and contrasts with traditional approaches in which a set margin of excision is performed.

Indications for Mohs surgery include recurrent basal cell carcinomas (BCC) and squamous cell carcinomas (SCC), locations prone to recurrence (“H-zone” of the face: inner canthus, nasolabial fold, nose, periorbital, temple, upper lip and periauricular regions, retroauricular, and chin), at/near critical structures (e.g., eye, lip), large tumors (>2 cm), ill-defined tumor margins, aggressive histology (BCC - morpheaform infiltrative, basosquamous, perineural; SCC - poorly differentiated, invasive, perineural), and special hosts (immunosuppressed, basal cell nevus syndrome, xeroderma pigmentosum). Therefore, in this patient, the primary indication for Mohs surgery would be the recurrent nature of her BCC.

2017

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15
Q

A 25-year-old obese man is evaluated because of new onset of inflamed nodules involving the bilateral axillae. A diagnosis of hidradenitis suppurativa is made. Which of the following is the most appropriate initial medication for treatment of this patient’s condition?

A) Botulinum toxin type A
B) Clindamycin
C) Cyclosporine
D) Etanercept
E) Prednisone
A

The correct response is Option B.

Hidradenitis suppurativa is a chronic inflammatory skin disease. Also known as acne inversa, it is characterized by recurrent nodules and abscesses, typically of apocrine gland–bearing skin.

This patient has mild hidradenitis, with an initial presentation of a few abscesses without sinus tracts or cicatrization/scarring.

Clindamycin applied topically is often used as a first-line therapy for mild hidradenitis. In a randomized, placebo-controlled trial, patients treated with twice-daily topical application of 1% clindamycin solution were found to have significantly less disease burden, and the treatment was well tolerated with few side effects.

Although there have been reports of the use of botulinum toxin in the treatment of hidradenitis, its role and efficacy in this setting are currently unclear.

Etanercept is a TNF-alpha inhibitor. Although some TNF-alpha inhibitors, particularly infliximab, have shown efficacy in patients with moderate-to-severe hidradenitis, data are conflicting with regard to Etanercept.

Prednisone is occasionally used to calm the inflammatory process in severe hidradenitis. However, it does not prevent formation of new lesions and is rarely used for long-term therapy in patients with hidradenitis because of possible adverse effects.

A few case reports have described improvement with cyclosporine in refractory cases of hidradenitis. However, it is typically not used for initial medical treatment of hidradenitis, and duration of use is often limited by adverse effects.

2017

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16
Q

A 75-year-old woman is evaluated because of a new skin lesion on the right upper eyelid. Examination of the specimen obtained on biopsy shows a 1-cm Merkel cell carcinoma. In addition to regional node sampling, which of the following is the most appropriate excision and adjuvant management in this patient?

A) 1-cm margins and chemotherapy
B) 2-cm margins and chemotherapy
C) 1-cm margins and postoperative radiation therapy
D) 2-cm margins and postoperative radiation therapy
E) 5-mm margins and postoperative radiation therapy

A

The correct response is Option C.

Merkel cell carcinoma, an aggressive neuroendocrine tumor, is most likely. It presents in older, immunocompromised women in sun-exposed areas. About 80% of Merkel cell carcinomas are secondary to polyomavirus infection. Treatment of the primary tumor should be wide local excision or Mohs micrographic surgery. For wide local excision of tumors smaller than 2 cm, the recommended surgical margin should be 1 cm. As there is a high rate of occult nodal metastasis, and nodal status is associated with mortality rates, biopsy of the sentinel node is recommended for all cases regardless of primary tumor size. Merkel cell carcinoma is a radiosensitive tumor, and postoperative adjuvant radiation therapy has been shown to decrease local recurrence. Chemotherapy is only currently indicated for palliation and distant metastasis.

2017

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17
Q

A 62-year-old man is evaluated because of a new skin lesion in his back. Excision of the lesion with administration of a local anesthetic agent is planned. Medical history shows hypertension and coronary artery disease treated with coronary balloon angioplasty 10 years ago. He takes 81 mg of aspirin daily. Preoperatively, which of the following is the most appropriate aspirin regimen for this patient?

A) Maintaining current dosage
B) Withholding for 1 day
C) Withholding for 2 days
D) Withholding for 7 days
E) Withholding for 14 days
A

The correct response is Option A.

For this patient with increased risk of cardiovascular events, the safest approach is not to withhold aspirin prior to dermatologic surgery.

Aspirin inhibits platelet aggregation by irreversibly binding to the cell’s cyclooxygenase enzyme stores, blocking the production of thromboxane. Its use at lower doses in long-term primary and secondary prevention of cerebrovascular and cardiovascular thrombotic events is well established.

Withholding of daily aspirin in patients with known cardiovascular disease can lead to a platelet rebound phenomenon featuring increased thromboxane production, decreased fibrinolysis, and a prothrombotic state. Discontinuation of oral antiaggregants has been found to be an independent predictor of both death and major ischemic events.

Several studies revealed no significant increase in the risk of bleeding complications after cutaneous surgery in patients who continued taking aspirin perioperatively.

2017

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18
Q

A 20-year-old man comes to the office for definitive treatment of a punch biopsy–proven dermatofibrosarcoma protuberans (DFSP) of the scalp. Which of the following is the most appropriate treatment plan?

A) Cryoablation
B) Radiation
C) Referral for Mohs micrographic surgery
D) Resection with 0.5-cm margin
E) Resection with 1-cm margin
A

The correct response is Option C.

Dermatofibrosarcoma protuberans (DFSP) is relatively uncommon, soft-tissue-only tumor that is locally aggressive. Since it can extend along connective tissues along the deep layers, margins required for DFSP need to be no less than 2 cm for an acceptable non-recurrence rate, and even then it is quite high. Predicted rate of recurrence is anywhere from 11 to 20% with a 3-cm margin.

Mohs micrographic surgery has demonstrated to have a much lower recurrence rate, with multiple studies demonstrating less than 10%. Subsequently, Mohs micrographic surgery is the best initial treatment plan for complete resection of DFSP.

Radiation therapy is contraindicated for DFSP. Cryoablation is employed for precancerous skin lesions.

2017

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19
Q

Which of the following patients’ skin lesions is most suggestive of malignancy on the basis of its clinical features?

A) A 14-year-old boy with a 5-mm, round, brown macule present since birth on the distal aspect of the thigh
B) A 22-year-old woman with a 6-mm friable, pedunculated papule present for 6 weeks on the cheek
C) A 55-year-old man with a 5-mm pink, pearly papule present for 6 months on the lateral nasal sidewall
D) A 70-year-old man with a 1.5-cm waxy, yellow and brown, scaly plaque present for several years on the left temple

A

The correct response is Option C.

Once an individual’s personal and family histories are considered, the history and appearance of a suspicious skin lesion will provide important information and provide clues about a diagnosis.

The “ABCD” (Asymmetry, Border irregularity, Color variegation, Diameter >6 mm) criteria are important for assessing potential malignancy of pigmented lesions. A flat, unchanging pigmented lesion in a young patient is unlikely to be malignant.

A pyogenic granuloma classically presents after local trauma and is characterized by a friable papule that bleeds easily.

Basal cell carcinoma and squamous cell carcinoma are the most common cutaneous skin malignancies. They may present as discrete, slowly growing lesions that have a history of bleeding and ulceration. Basal cell malignancies have a characteristic appearance of round, oval nodules with a shiny, pearly appearance and overlying telangiectasias.

Seborrheic keratoses are common benign skin lesions found in old age. They are a proliferation of immature keratinocytes and have a characteristic appearance of being well circumscribed, scaly, and have a “stuck on” appearance. While unsightly, these lesions have no malignant potential.

2017

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20
Q

A 62-year-old woman with biopsy-proven basal cell carcinoma of left mid cheek presents for consultation for surgical treatment. On physical examination, the lesion is 0.6 cm in diameter and has indistinct borders. Which of the following criteria is the most likely indication for Mohs micrographic surgery in this patient?

A) Anatomical location of the cancer
B) Diagnosis of basal cell carcinoma
C) Indistinct borders
D) Patient age
E) Size of the carcinoma
A

The correct response is Option C.

In the case presented above, the strongest indication for Mohs micrographic surgery comes from the anatomic finding of indistinct borders. Other findings are not necessarily indications for Mohs. Other proven indications are recurrent cancer, high risk zones of the face, morpheaform basal cell cancer, or evidence of neurovascular involvement. By some studies, larger sized basal cell cancers (>2 cm) may be indications.

By definition, Mohs micrographic surgery is a technique for treatment of complex or ill-defined skin cancers with examination of 100% of tissue. One physician performs the procedure, acting in two distinct capacities: surgeon (excising the cancer) and pathologist (reading the slides). Usually, the final pathologic clearance is given on the same day as the resection. This is in comparison with a wide local excision technique, which is performed by two different physicians: a surgeon who removes the cancer and a pathologist who reads it separately. In the latter scenario, although a “wet” read can be done on the same day, the final pathologic evaluation has to await the permanent preparation of slides and a final read. Also, using the routine pathologic evaluation, only 2% of the margins are looked at (using the common “bread loafing” technique), compared with the 100% of tissues evaluated using the Mohs technique.

The vast majority of Mohs resections is done with one or two excisions. Although at times there is a need for multiple excisions, the need for excisions is driven by the positive margins detected pathologically. In Mohs technique, the amount of healthy tissue taken to obtain “clear” margins is the minimum needed to do so, thus preserving the healthy tissue, which can be critically useful in the ensuing reconstruction. These margins are definitively assessed on that same day of surgery, so once the patient leaves the Mohs surgeon’s office, he or she knows the cancer has been completely removed. This is why the margin control is superior in Mohs technique compared with other surgical techniques.

2016

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21
Q

A 75-year-old man comes to the office because of a 5-year history of a pruritic lesion on the right groin that has been enlarging gradually in size. A photograph is shown. Examination of a specimen obtained on biopsy shows Paget disease. Which of the following is the most appropriate next step in management?

A) Oral miltefosine
B) Topical hydrocortisone
C) Topical miconazole
D) Wide excision
E) Observation
A

The correct response is Option D.

This patient described has extramammary Paget disease and the treatment is wide excision. Paget disease of the breast also presents with eczematous skin changes and is associated with breast cancer. Extramammary Paget disease, however, is an intraepithelial carcinoma that commonly involves the vulvar, perianal, perineal, scrotal, and penile regions. It presents as well-defined, moist, erythematous plaques associated with pruritis. Histopathologic examination shows epidermal acanthosis and elongated rete ridges. Paget cells are large intraepidermal cells with a large nucleus and abundant pale cytoplasm. There is a 7 to 40% rate of associated malignancy. Wide excision is the standard of care, and recent reports have shown that Mohs micrographic surgery can improve evaluation of resection margins.

Observation is not appropriate for extramammary Paget disease given the potential risk for malignancy. Topical treatment with steroids or antifungals is also not appropriate. Oral miltefosine is a treatment for leishmaniasis, an infectious disease that can involve the skin, mucous membranes, and internal organs. Although cutaneous leishmaniasis can present with ulcerating lesions or a dense dermal infiltrate, the histology is predominantly histiocytes, lymphocytes, and plasma cells.

2016

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22
Q

An otherwise healthy 65-year-old man comes to the clinic because of a 3-cm ulcerated lesion of the scalp. There are no palpable regional lymph nodes. Chest x-ray study shows no abnormalities. A punch biopsy is performed and a diagnosis of well-differentiated squamous cell carcinoma is made. Wide local excision of the lesion is planned. Which of the following is the recommended minimum surgical margin in this patient?

A) 1 mm
B) 2 mm
C) 4 mm
D) 6 mm
E) 12 mm
A

The correct response is Option D.

The most appropriate surgical margin recommended for the clinical scenario described is 6 to 10 mm.

Cutaneous squamous cell carcinoma (cSCC) is the second most common skin cancer after basal cell carcinomas. They are broadly categorized into low- and high-risk lesions depending on size, location, depth of invasion, recurrence, and patient factors such as immunosuppression. A 4-mm margin of healthy tissue is recommended for lower-risk lesions. This category includes well-differentiated tumors smaller than 2 cm in diameter that do not occur on the scalp, ears, eyelids, lips, or nose, and do not involve subcutaneous fat. Therefore, simple excision is most valuable in the treatment of small primary squamous cell carcinomas on the trunk, extremities, or neck, where tissue sparing is less essential. The recurrence rate after the excision of low-risk lesions ranges from 5 to 8%.

A 6-mm margin of healthy tissue is recommended for lesions that are larger than 2 cm, invasive to fat, or in high-risk locations (i.e., central face, ears, scalp, genitalia, hands, feet). Given the cosmetic and functional impact of these wider margins, tumors in this latter category are often removed via Mohs micrographic surgery to achieve high cure rates while sparing healthy tissue. The depth of an excision should always include a portion of the subcutaneous fat.

Cure rates following simple excision of well-defined T1 lesions may be as high as 95 to 99%. The generally accepted 5-year cure rate for primary tumors treated with standard excision is 92%; this rate drops to 77% for recurrent cSCC. No large randomized studies have addressed the issue of appropriate margin size in cSCC, as has been done for melanoma. The recommendations for margin size should be taken only as rough guidelines, with the understanding that large, aggressive lesions frequently have substantial extension beyond the apparent superficial boundary. Therefore, a surgeon’s experience and judgment when planning surgical margins is paramount to the successful treatment of cSCC.

2015

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23
Q

A 24-year-old woman is evaluated because of a slow-growing subcutaneous mass of the scalp. An excisional biopsy is performed, and pathologic examination shows keratin and its breakdown products. Which of the following is the most likely origin of this lesion?

A) Adipocyte
B) Capillary
C) Hair follicle
D) Mechanoreceptor
E) Sebaceous gland
A

The correct response is Option C.

Pilar cysts, also known as trichilemmal cysts, originate from the outer root sheath of the hair shaft. They present as firm, slow-growing subcutaneous nodules, and may be difficult to differentiate clinically from epidermoid cysts. They are commonly found on the scalp where they are the most common cutaneous cyst. They are lined by stratified squamous epithelium, which undergoes keratinization. In some cases, these lesions can demonstrate aggressive biologic behavior (proliferating trichilemmal tumors, malignant proliferating trichilemmal tumors), in which case they should be completely excised.

Lesions that originate from adipocytes include lipomas and angiolipomas.

Lesions of vascular origin include cherry angiomas and pyogenic granulomas.

2015

24
Q

A 45-year-old woman is evaluated because of a 1-year history of skin abnormalities. Physical examination shows areas of thickened skin involving the forearms and hands. Telangiectasias are seen on the face and oral cavity. A review of systems discloses symptoms of heartburn and dysphagia. Which of the following is the most likely diagnosis?

A) Amyloidosis
B) Dermatomyositis
C) Hypothyroidism
D) Scleroderma
E) Systemic lupus erythematosus
A

The correct response is Option D.

The scleroderma spectrum of disorders includes localized scleroderma and systemic sclerosis, both of which are characterized by thickened sclerotic skin. Localized scleroderma involves only the skin, whereas systemic sclerosis is associated with extracutaneous involvement and is subcategorized into limited and diffuse forms. Limited cutaneous systemic sclerosis is restricted to the hands, distal forearm, face, and neck. Limited scleroderma is sometimes referred to as CREST syndrome, which is an acronym for the following features: calcinosis, Raynaud syndrome, esophageal dysmotility, sclerodactyly, and telangiectasia. Diffuse cutaneous systemic sclerosis includes truncal involvement (chest, abdomen, upper arms, shoulders).

In most patients with systemic sclerosis, there is gastrointestinal involvement. Symptoms are present in more than half of patients and most commonly are related to gastroesophageal reflux disease, resulting in symptoms such as heartburn and dysphagia. Other findings in systemic sclerosis include: diarrhea, mucocutaneous telangiectasia of the face/lips/oral cavity/hands, dyspnea on exertion/interstitial pulmonary disease, and digital infarctions/pitting.

Amyloid infiltration of the skin may produce thickening and stiffness. Telangiectasias are not a feature of amyloidosis. Gastrointestinal involvement with amyloid typically does not cause esophageal reflux or heartburn.

Dermatomyositis is an idiopathic inflammatory myopathy characterized by skin and muscle abnormalities. Cutaneous involvement manifests in the form of distinct rashes, such as of the upper eyelids (heliotrope rash), hands (Gottron sign), and chest and shoulders (shawl sign). However, thickened skin of the trunk and limbs, and telangiectasias are not features of dermatomyositis.

Hypothyroidism can result in cutaneous and dermal edema (myxedema). Other findings include fatigue, cold intolerance, weight gain, constipation, dry skin, myalgia, and menstrual irregularities. It is not associated with telangiectasias.

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that can affect the skin, joints, kidneys, lungs, nervous system, serous membranes, and/or other organs of the body. Mucocutaneous changes include butterfly rash, erythematous plaques (discoid lesions), and ulcers. SLE is not associated with telangiectasias.

2015

25
A 55-year-old woman comes to the office because of a 3-year history of a raised, dark brown lesion on her back. The lesion is not painful and does not bleed. A photograph is shown. An excisional biopsy is performed. Pathologic examination shows proliferation of cells from the basal layer of the epidermis with cystic inclusions. The lesion exhibits hyperkeratosis, acanthosis, and papillomatosis. Which of the following is the most likely diagnosis? ``` A) Basal cell carcinoma B) Malignant melanoma C) Nevus sebaceous D) Seborrheic keratosis E) Verrucous carcinoma ```
The correct response is Option D. The lesion is a seborrheic keratosis, also known as verruca senilis or pigmented papilloma. Seborrheic keratoses are common benign lesions that may begin to appear during the fifth decade of life. They arise from the basal layer of the epidermis and are composed of well-differentiated basal cells. They often contain cystic “inclusions” of keratinous material called “horn cysts.” Lesions exhibit hyperkeratosis (thickening of the stratum corneum), acanthosis (diffuse epidermal hyperplasia and thickening of the skin), and papillomatosis (skin surface elevation). The growth and depth of pigmentation vary directly with exposure to sunlight. If left untreated, they will enlarge gradually and increase in thickness. Seborrheic keratoses typically do not involute spontaneously. They may appear on the head, neck, and trunk after age 50 years and are often distinctly marked and have a waxy, stuck-on appearance. The surface is soft and oily to the touch. Sizes can range from 1 mm to 5 cm. No treatment is necessary, but these lesions are cosmetically unappealing, and for that reason alone, patients may request to have them removed. It is extremely rare for cutaneous malignancies to develop within seborrheic keratoses. A verrucous carcinoma is a variant of squamous cell carcinoma. As such, it requires wide local excision with negative margins for treatment. Nevus sebaceous (Jadassohn nevus) may be described as cerebriform, nodular, or verrucous. It is hairless and can appear on the scalp, face, or neck. When present at birth, it persists throughout life and tends to become more verrucous and nodular during the growth phase associated with puberty. Over time, these lesions are associated with a risk of basal cell carcinoma, which occurs in approximately 15 to 20%. Keratoacanthoma and squamous cell carcinoma may also develop, although with much less frequency than basal cell carcinoma. Because of the risk of malignant transformation, complete excision is generally recommended. Melanoma results from malignant transformation of the melanocyte; the pigment-producing cell of the body can occur anywhere melanocytes are present, including skin, eye, and the mucous membranes of the upper digestive tract, sinuses, anus, and vagina. The incidence of cutaneous melanoma in the United States has increased steadily over the past 50 years and is now 15 per 100,000. Worldwide, the incidence of melanoma is generally reflective of variation in genetic, phenotypic, and ultraviolet (UV) exposure risk factors. Major risk factors include exposure to UV radiation and genetic predisposition. The exposure risk primarily involves intermittent, damaging exposure to the sun such that history of a severe sunburn (blistering or pain for more than 2 days), even in youth, confers an approximately twofold increase in risk. Patients who have fair skin, blue eyes, red hair, and are prone to freckling are at increased risk for melanoma. Mutations in two genes are associated with hereditary melanoma predisposition. 2015
26
A 60-year-old woman comes to the office for evaluation of a firm, violaceous, 3-cm lesion of the left cheek. An incisional biopsy shows a Merkel cell tumor. The residual margins are positive. Which of the following is the most appropriate management? ``` A) Chemotherapy B) Laser ablation C) Mohs micrographic surgery D) Radiation therapy and chemotherapy E) Wide local excision and radiation therapy ```
The correct response is Option E. Merkel cell tumor is an unusual and highly aggressive skin cancer. More than 50% of Merkel cell tumors occur in the head and neck region. Risk factors for Merkel cell tumors are exposure to sun and ultraviolet light, and immunosuppression. There is a recent association with Merkel cell polyomavirus. Merkel cell generally presents as a firm, painless nodule (up to 2 cm in diameter) or as a mass (>2 cm in diameter). Although classically described as red in color, it may be flesh-colored or blue. It often enlarges rapidly. The standard of management is surgical excision combined with radiation therapy. Radiation therapy decreases local recurrence rates. Node-negative patients with no distant metastasis treated with surgery and radiation have 5-year survival rates of approximately 90%. Mohs micrographic surgery and wide local excision are both accepted modalities of surgical resection. It is well known that surgery alone is insufficient to cure or control Merkel cell tumors. Consideration should be given to evaluation of the lymph nodes. Sentinel node biopsy is a common modality. 2015
27
A 14-year-old girl with Fitzpatrick Type V skin comes to the office for evaluation of a nevus on the right side of the face. Physical examination shows a macular, bluish grey, irregular area of hyperpigmentation involving the right infrapalpebral region, nasolabial fold, and zygomatic region. Pigmentation of the right sclera is noted. Which of the following is the most appropriate treatment for this lesion? ``` A) Camouflage therapy B) Cryotherapy C) Dermabrasion D) Mohs micrographic excision E) Q-switched ruby laser ```
The correct response is Option E. This patient has the acquired form of nevus of Ota, also known as nevus fuscoceruleus ophthalmomaxillaris or oculodermal melanocytosis, a dermal melanocytic hamartoma that demonstrates bluish hyperpigmentation along the ophthalmic and maxillary divisions of the trigeminal nerve. The failure of complete embryonic migration of melanocytes from the neural crest to the epidermis results in dermal nesting with the resultant dermal melanin causing the Tyndall effect. This disorder primarily affects darker-pigmented individuals and is more prevalent in females. It has a bimodal age incidence, with a peak at 1 year of age and a second around puberty. The lesion tends to become increasingly prominent with age, puberty, and postmenopausal state. Most patients have no family history. Ophthalmologic examination is recommended because of a reported 10% association with ipsilateral glaucoma. Malignant degeneration to melanoma occurs in approximately 4% of reported cases and is more frequent in lighter-skinned individuals. Diagnosis is mainly clinical with confirmatory biopsy indicated when the diagnosis is uncertain or in rapidly expanding or nodular lesions suggestive of malignancy. The most effective treatment option is laser therapy, particularly with a Q-switched laser with ruby (694 nm), alexandrite (755 nm), or neodymium: yttrium-aluminum-garnet (1064 nm). The wavelength, pulse duration, and energy densities inherent in the Q-switched laser provide the desired parameters for melanin photothermolysis. Dyspigmentation is a possible complication, although it is mostly transient. Before the advent of laser therapy, treatment options were suboptimal. Makeup or camouflage therapy offered only temporary improvement. Dermabrasion followed by cryotherapy had the potential for dermal scarring and atrophy and was ineffective for those lesions with deep dermal melanocytes. Surgical excision options were also associated with scarring. Mohs micrographic excision has not been described for excision of these lesions. 2014
28
An 87-year-old Caucasian man comes to the office with multiple 5- to 6-mm lesions on the forehead. He has a long history of sun exposure. Physical examination shows the lesions are erythematous, rough, and scaly. Which of the following is the most appropriate treatment? ``` A) Dexamethasone B) Docosanol C) Imiquimod D) Isotretinoin E) Observation with 1-month follow up ```
The correct response is Option C. This patient’s lesions are most consistent with actinic keratoses. Actinic keratoses are most commonly seen in fair-skinned individuals in areas that have had long-term sun exposure. They are the most common skin lesions to demonstrate malignant potential and may progress to squamous cell carcinoma. Given the propensity of actinic keratoses to malignant transformation, treatment is generally recommended over observation. For multiple lesions, topical agents are generally effective and well tolerated. Imiquimod is thought to exert its effects by inducing a local immune response as well as apoptotic pathways. Other effective treatments include photodynamic therapy, cryotherapy, 5-fluorouracil, and diclofenac gel. Dexamethasone is a corticosteroid typically used for inflammatory or autoimmune skin conditions. Isotretinoin is used to treat cystic acne. Docosanol is an antiviral medication used for herpes simplex. 2014
29
A 55-year-old woman is evaluated for a biopsy-proven squamous cell carcinoma of the right preauricular area measuring 2.1 cm in diameter. She is otherwise healthy. Which of the following is the most appropriate next step in management? A) Electrodessication of the lesion B) Excision of the lesion with frozen sections C) Excision with a 2-mm margin D) Excision with a 4-mm margin E) Topical application of 5% fluorouracil
The correct response is Option B. Successful local treatment of squamous cell carcinoma of the skin depends significantly on whether the tumors are at high or low risk for the complications of recurrence and metastasis. The external ear, lips, nose, and scalp appear to be high-risk locations for squamous cell carcinoma of the skin. Squamous cell carcinomas of the skin larger than 2 cm are twice as likely to recur locally and three times as likely to metastasize than tumors that are less than 2 cm in diameter. Frozen intraoperative examination of specimen edges can be used to judge thoroughness of excision before closure. Frozen sections of margins are recommended for high-risk squamous cell carcinoma and basal cell carcinoma in high-risk areas, lesions more than 2 cm, and any morpheaform basal cell carcinoma. Electrodessication has excellent cure rates in small, low-risk squamous cell carcinoma of the skin. Topical application of 5% fluorouracil has a role in the treatment of diffuse actinic keratoses of the face. Surgical excision is subdivided into excision with standard margins, excision with frozen-section margin evaluation, and Mohs micrographic surgery. For low-risk non-melanoma skin cancers extending into the dermis only, excision with standard margins (4 mm for basal cell carcinoma) is the usual treatment. Adequate margins of 4 mm for low-risk squamous cell carcinoma and 6 mm for high-risk squamous cell carcinoma have been demonstrated by direct tumor extension from the clinical margin but are not necessarily an estimate of cure rate. Squamous cell carcinomas are slower to invade deeper tissue than are cutaneous malignant melanomas. 2014
30
A 55-year-old woman with a BMI of 32 kg/m2 comes to the office with advanced hidradenitis suppurativa of the groin, lower abdomen, and upper thigh. Which of the following treatments is most likely to have the greatest likelihood of success in this patient? ``` A) Antibiotics and excision B) Antibiotics and percutaneous drainage C) Clindamycin irrigation D) Intralesional injection of a corticosteroid E) Sclerotherapy ```
The correct response is Option A. Percutaneous drainage of hidradenitis suppurativa pustule and fistula tracts, although a plausible short-term fix to address the immediate symptoms, does little to ensure long-term resolution of this very difficult clinical entity with marked impact on quality of life. Addition of topical antibiotic washes or oral antibiotics to percutaneous drainage had no significant effect on long-term recurrence rates. Hidradenitis suppurativa is a recurrent inflammatory disease of the apocrine glands. It initially develops from follicular occlusion with subsequent abscess, inflammation, fistulas, sinus tracts, and scarring. The sites most commonly affected are the intertrigonal regions such as the axilla, groin, and genital/anal region; although, it can also affect the breasts, hips, and thighs. Women are affected three times as often as men. Initial treatment involves local wound care and antibiotic therapy. For advanced disease, this may be followed by excision of the area of high-density apocrine glands with minimal undermining and direct closure at the site of the hidradenitis wound. At the site of inadequate resection of an area of infected glands, or if there is a recurrence, radical resection yields the best long-term result. Skin grafting and fasciocutaneous and musculocutaneous flaps have been described to cover the excisional defect. The musculocutaneous flap has been reported to be a valid option for managing infected lesions because of the abundant blood supply. Delayed secondary wound closure, with or without vacuum-assisted closure or skin substitutes, has also shown plausible outcomes. Sclerotherapy has no role in treatment of hidradenitis suppurativa. 2014
31
A 54-year-old man comes to the office because of a lesion on his back that has enlarged gradually for the past 2 years. He says it occasionally expresses white material when he squeezes it. Physical examination shows a mobile, firm, 2 × 1.5-cm nodule with a central opening and white plug on the mid back. No tenderness is noted on palpation. Which of the following is the most appropriate initial step in management? A) Excision B) Incision and drainage C) Punch biopsy D) Wide local resection
The correct response is Option A. The patient described has an epidermal inclusion cyst or sebaceous cyst. Cysts can occur anywhere on the body, including the face, back, and chest. These cysts are benign and occur as a result of proliferation of epidermal cells within the dermis. They are usually well circumscribed by a cyst wall made of stratified squamous epithelium. They communicate with the surface through a small opening, which may contain a keratinous plug or blackhead. Epidermal inclusion cysts grow slowly and occasionally can become inflamed and infected. Manual squeezing of the cysts may produce white keratinous material, which is often foul-smelling. Treatment is excision of the cyst in its entirety with care to leave no epithelial remnants in the wound that could develop into a recurrent cyst. Wide local resection is performed for malignant skin lesions with appropriate negative margins that can be evaluated by frozen-section pathology. 2013
32
A 67-year-old Caucasian man comes to the office because of a 3-month history of a lesion on his forehead that he says has enlarged gradually and sometimes bleeds. Examination shows a nontender, soft, flat, purple lesion on the anterior hairline that is 4 cm in its largest dimension. Results of punch biopsy are consistent with angiosarcoma. Which of the following is the most appropriate treatment? ``` A) High-dose chemotherapy B) Radiation therapy C) Vascular embolization D) Wide local excision E) Observation Only ```
The correct response is Option D. The patient has cutaneous angiosarcoma of the scalp that requires wide local excision with reconstruction. Angiosarcoma is a rare (2% of all soft-tissue sarcomas) but highly aggressive tumor that is most commonly found in the face and scalp in older Caucasian men. Fifty percent of all cutaneous angiosarcomas are found in the head and neck, and they are also commonly found in the breast and extremities, particularly in patients with a history of lymphedema or radiation therapy. It appears initially as a purple plaque which is often confused with a bruise or cellulitis, which can delay diagnosis. It is frequently multifocal, and local recurrences are common, so wide local excision is recommended, frequently combined with radiation therapy. Preoperative serial punch biopsies of the surrounding tissue can help in determining appropriate resection margins. The tumor can microscopically infiltrate normal tissues for some distance away from the obvious tumor, so wide local excision is necessary. Observation or embolization is not indicated for an aggressive cancer like angiosarcoma. Although radiation is frequently used postoperatively, its role as a neoadjuvant therapy is not well established. There is no current standard of care for chemotherapy in angiosarcoma patients, and most trials of chemotherapy have shown no survival benefit. There may be a role for taxanes in the treatment in metastatic angiosarcoma. 2013
33
A 39-year-old woman is evaluated because of a 6-month history of a growth on the face. A biopsy is planned. Which of the following findings on pathology can be safely treated with observation only in this patient? A) Cylindroma B) Eccrine poroma C) Nevus sebaceous D) Trichoepithelioma
The correct response is Option D. Trichoepitheliomas are neoplasms of follicular differentiation. Trichoepithelioma usually presents as multiple, yellowish-pink, translucent papules distributed symmetrically on the cheeks, eyelids, and nasolabial area. The lesions are more frequently seen in women. Lesions are benign but can be confused with basal cell carcinomas clinically and histologically. As they are benign, no further measures should be taken. However, in cases of desmoplastic trichoepithelioma, complete excision or Mohs surgery may be needed to clearly differentiate this entity from a carcinoma. Eccrine poroma occurs as a solitary lesion usually on the sole of the foot or the palm of the hand in persons older than 40 years. It may also occur on the chest, the neck, or other locations. Eccrine poromas are seen as firm papules less than 2 cm in size. Lesions may occasionally be pedunculated and have a normal or erythematous color and a firm consistency. In rare instances, malignant eccrine poroma or porocarcinoma develops either spontaneously or from long-standing benign eccrine poroma. Treatment is surgical excision. Verrucous nevus consists of closely set verrucous papules that may coalesce to form well-demarcated plaques. They may be skin colored, brown, or gray-brown. A linear configuration is common, especially for lesions on the limb. Such lesions may appear to follow skin tension lines. On histologic evaluation, there is hyperkeratosis, acanthosis, and papillomatosis. The histologic appearance is essentially that of a benign papilloma. Excision is the most reliable treatment. This may not be practical or advisable if the lesion is extensive or at sites not amenable to simple surgery. The excision should extend to the deep dermis; otherwise, the lesion may recur. Alternative treatments have included laser cryotherapy and electrodesiccation dermabrasion. Cylindroma presents as either solitary or multiple lesions. Nodules may also be present on the face and rarely on the extremities. The lesion appears in adulthood. The surface is smooth and may be telangiectatic. Cylindromas are usually benign, but malignant changes have been reported. For solitary lesions, treatment is by excision or electrosurgery. For small cylindromas, the carbon dioxide laser may be used. Multiple cylindromas usually require extensive plastic surgery that may be obviated by progressively excising a group of nodules in multiple procedures. Nevus sebaceous is a distinctive growth most commonly found on the scalp, followed by the forehead and retroauricular region. A nevus of epithelial and nonepithelial skin components, nevus sebaceous sustains age-related modifications in morphologic appearance. The nevus occurs singly and is asymptomatic. Two thirds are present at birth; the remaining third develop in infancy or early childhood. Male and female infants are equally affected. The three-stage evolution of the nevoid condition (newborn, puberty, and adult) parallels the natural histologic differentiation of normal sebaceous glands. In approximately 20% of patients, a third phase of evolution involves the development of secondary neoplasia in the mass of the nevus. A number of benign and malignant “nevoid tumors” may occur, the most common of which is the basal cell epithelioma. The malignant degenerations are relatively low grade; only a few instances of metastasis have been reported. Surgical excision of a nevus sebaceous is recommended because of the high potential for development of basal cell carcinoma and other tumors. The lesion should preferably be excised before puberty because it may enlarge, and the risk of malignant transformation increases after puberty. 2013
34
A 47-year-old man comes to the office because of an asymptomatic lesion of the anterior abdominal wall that has been enlarging gradually for the past 10 years. The lesion has accelerated in growth during the past several months and recently ulcerated. Examination shows a 6-cm, raised, indurated, and irregularly shaped violaceous plaque consisting of firm, irregular nodules. Examination of a specimen obtained on incisional biopsy shows a soft-tissue malignancy arising from mesenchymal cells in the dermis. Which of the following is the most appropriate treatment? A) Wide local incision and molecular targeted therapy B) Wide local excision and sentinel node biopsy C) Wide local excision, molecular targeted therapy, and radiation therapy D) Wide local excision only E) Wide local excision, sentinel node biopsy, and chemotherapy
The correct response is Option D. The diagnosis of the described lesion is dermatofibrosarcoma protuberans (DFSP). It accounts for less than 0.1% of all malignant neoplasms and approximately 1% of all soft-tissue sarcomas, but is the most common type of cutaneous sarcoma. It is a malignant mesenchymal tumor that arises in the dermis and is characterized by latency in its initial detection, slow infiltrative growth, and local recurrence if not adequately treated. Distant metastasis is rare and generally occurs as a late sequela after repeated local recurrences. DFSP is most commonly found on the trunk followed by the proximal extremities, and rarely in the head and neck. These tumors have irregular shapes, frequent finger-like extensions, and an infiltrating growth pattern extending beyond clinical margins that result in incomplete removal and a propensity for local recurrence. Treatment primarily consists of wide surgical excision to include margins of 2 to 3 cm beyond the clinical tumor border if possible. Mohs micrographic surgery has been used with good outcomes in aesthetically sensitive areas such as the head and neck where tissue sparing is important. Reconstruction with tissue rearrangement or flaps should be performed after negative margins are confirmed. Most recurrences occur within 3 years of the primary excision, and close follow-up is indicated. Conventional chemotherapy is rarely used. Radiation therapy is used as an adjunct to surgery for close or positive margins in areas where adequate wide resection alone may result in major cosmetic or functional deficits. Molecular targeted therapy such as imatinib mesylate (Gleevec) is indicated for unresectable, recurrent, or metastatic DFSP. Sentinel node biopsy is not indicated in the treatment for DFSP. 2013
35
A 47-year-old man with hypertension and ulcerative colitis comes to the office because of a painful lesion of the right lower leg. He says that he dropped a typewriter on his leg 1 year ago and noticed a small wound that slowly began to grow larger despite treatment with bandages and topical antibacterial ointment. Examination of a specimen obtained on incisional biopsy shows neutrophilic dermatosis. Cultures grow Staphylococcus aureus. Which of the following is the most likely diagnosis? ``` A) Leishmaniasis B) Marjolin ulcer C) Necrotizing fasciitis D) Pyoderma gangrenosum E) Scleroderma ```
The correct response is Option D. The patient has pyoderma gangrenosum, which is often associated with ulcerative colitis, Crohn disease, or rheumatoid arthritis. The lesion may begin as a small erythematous plaque or have purplish discoloration, and it commonly occurs on the lower extremities after minor trauma. The lesions may rapidly become painful and appear as a necrotizing ulcer. The exact cause of pyoderma gangrenosum is not entirely understood. It is believed the disease is immune-related, such that initial treatments may be primarily medical, including systemic steroids or immunosuppression. Generally speaking, surgery is reserved as a last resort as it is believed that further surgery may exacerbate the condition. Furthermore, recurrent, ulcerative lesions after surgery are not uncommon. Marjolin ulcer is typically a squamous cell carcinoma arising from a long-standing wound, chronically inflamed tissue, or previous burn scar. The latency period may range from 5 to 30 years from the time of initial injury before developing into a Marjolin ulcer. Treatment generally involves radical resection, as Marjolin ulcers tend to be aggressive. Leishmaniasis is a disease caused by a protozoan parasite (Leishmania), which is typically transmitted by the bite of a sand fly. In the area of the bite, patients develop ulcerations of the skin. Diagnosis can be made by obtaining a history of an insect bite, a history of travel and exposure to where sand flies reside (Central America, South America, West Asia, or the Middle East), and by obtaining scrapings from the ulcers and looking for the organisms under the microscope. Scleroderma is a chronic autoimmune disease that is characterized by fibrosis of the skin. The underlying cause is not entirely understood. Patients may present with skin ulcers. Scleroderma may be categorized as systemic or limited. Patients with limited disease may present with Raynaud phenomenon as an early sign. The hands and fingers may be affected with distal tip ulceration and thin, taut skin over joints. Patients with systemic disease may have additional manifestations including gastrointestinal, pulmonary, renal, and cardiac fibrosis. Necrotizing fasciitis is a rapidly spreading soft-tissue infection involving the skin and subcutaneous tissues. It typically travels along fascial planes and can result in tissue necrosis, sepsis, and death. It can be mono- or polymicrobial, with typical offending pathogens including Group A Streptococcus, Staphylococcus aureus, Bacteroides fragilis, and Clostridium. Treatment includes high-dose intravenous antibiotic therapy and timely surgical debridement. Patients may require repeat debridement frequently in the early stages of treatment to limit progression of the disease. 2013
36
An otherwise healthy 17-year-old boy is brought to the office because of a 3-month history of a bleeding, 8 × 4-mm, red lesion of the left temple that has enlarged rapidly. A photograph is shown. Which of the following is the most likely diagnosis? ``` A ) Congenital hemangioma B ) Infantile hemangioma C ) Kaposiform hemangioendothelioma D ) Pyogenic granuloma E ) Venous malformation ```
The correct response is Option D. The most likely diagnosis is pyogenic granuloma. Pyogenic granuloma usually appears in childhood as a rapidly growing, red lesion. Lesions are commonly small (less than 1 cm) and complicated by bleeding. Definitive treatment is excision. Congenital hemangioma is fully grown at birth. There are two types: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). RICH rapidly involutes postnatally and is usually fully regressed by age 12 months. Treatment is rarely necessary. NICH does not undergo involution; it remains the same size over the course of the patient’s lifetime. NICH is rarely problematic, but may be resected if it causes a significant deformity. Infantile hemangioma is usually noted 2 weeks after birth and enlarges rapidly over the first few months of life; bleeding is rare. By age 1 year, the tumor begins to regress. Problematic lesions are treated with intralesional corticosteroid, oral prednisolone, or resection. Kaposiform hemangioendothelioma (KHE) is usually present at birth and does not increase in size. It is often large, superficial, and diffuse. It typically involves the trunk and extremities. Patients commonly have Kasabach-Merritt phenomenon (thrombocytopenia, bruising, bleeding). First-line treatment is vincristine. Venous malformation is a congenital lesion that is present at birth and enlarges slowly over time. It is typically blue, and bleeding is uncommon. Treatment involves either sclerotherapy or resection. 2012
37
A 56-year-old man comes to the office because of a lesion on the tip of his nose. He has a 10-year history of coronary artery disease, skin cancers, excisions of the face, and poorly controlled type 2 diabetes mellitus. He has smoked one pack of cigarettes daily for the past 30 years. Medications include 81 mg of aspirin daily. Physical examination shows a 1-cm nodule with irregular borders. Examination of a specimen obtained on biopsy shows morpheaform basal cell carcinoma. Which of the following is the most appropriate next step in management? ``` A ) Cryotherapy B ) External beam radiation therapy C ) Intralesional injection of 5-fluorouracil D ) Mohs micrographic surgery E ) Wide local excision ```
The correct response is Option D. Morpheaform basal cell carcinoma is notorious for having "finger-like" extensions that are not always apparent on gross visual examination and can only be appreciated histologically. The morpheaform subtype of basal cell carcinomas tends to have a higher recurrence rate when simply excised. Mohs micrographic surgery is a technique that uses tangential excisions and frozen section pathology, which more accurately assess the margins of excision. Mohs micrographic surgery may also help minimize the extent of resection. Mohs micrographic surgery is particularly useful in morpheaform basal cell carcinoma, where obtaining negative surgical margins is critical to minimizing the risk of recurrence. Cryotherapy and external beam radiation therapy are recognized treatments for basal cell carcinomas only when surgical excision is not feasible. In the patient described with no absolute contraindications to surgery, surgical management would be considered the mainstay of therapy. 5-Fluorouracil is an antineoplastic agent and can be used topically for basal cell carcinomas when surgical therapy is not an option. Intralesional injection for this purpose is infrequently performed and would be considered an off-label use of the product. It has a low cure rate. Wide local excision in the area of the tip of the nose would be inappropriate because this would create a larger defect than may otherwise be required to obtain clear margins. Gross margins of 2 to 3 mm are generally sufficient for non–morpheaform-type basal cell carcinomas. 2012
38
A 2-year-old boy is brought to the emergency department because of a 2-day history of lethargy, fever, and a reddish purple rash on his arms and legs. Temperature is 103.0°F (39.4°C). Physical examination shows a petechial rash on the upper and lower extremities and trunk. Broad-spectrum antibiotics are initiated. After 6 hours, the rash begins to hemorrhage and blister and his digits become ischemic. Which of the following is the most appropriate management? ``` A ) Activated protein C B ) Amputation of the ischemic digits C ) Avoidance of inotropic support D ) Debridement of skin E ) Thrombolytics ```
The correct response is Option A. Purpura fulminans is a rapidly evolving autoimmune syndrome of septic shock and hemorrhagic bullae that can result in massive desquamation and is frequently fatal. Management includes prompt recognition of the infection (which is usually due to Neisseria meningitidis), initiation of broad-spectrum antibiotics, mechanical ventilation, and aggressive fluid resuscitation with inotropic support. Patients develop disseminated intravascular coagulopathy and appear to benefit from replacement of activated protein C. A recently published, multicenter retrospective review of 70 patients documented an amputation rate of 90% and suggested the need for early fasciotomy to improve limb salvage. It is very difficult to determine tissue viability during the resuscitation period; therefore, amputation, debridement, and coverage are delayed until demarcation has occurred. Thrombolytics are not used in this situation because the patient has a hemorrhagic disorder. 2012
39
A 75-year-old man has a biopsy-proven basal cell carcinoma of the eyelid that measures 5 mm in diameter. After a discussion about the patient's options of resection, he declines Mohs micrographic surgery. Which of the following is the smallest clinical margin beyond the edge of the tumor that will yield at least a 95% chance of cure? A ) 1 mm B ) 3 mm C ) 5 mm D ) 7 mm
The correct response is Option B. Many studies have had various observations as to the least margin required to cure a basal cell carcinoma. Almost all are retrospective, and conclusions have ranged greatly. Most articles recommend a 4-mm margin. However, in aesthetically sensitive areas like the eyelid, knowing the minimum margin of resection is crucial. Of course, Mohs micrographic surgery is an alternative, but it is not always feasible because of cost, availability, and the difficulty in coordinating two physicians’ schedules. A recent meta-analysis of the literature concluded that for basal cell carcinomas 2 cm or less, a 3-mm margin is sufficient to achieve at least a 95% chance of cure. A surgical margin difference of 1 mm can mean the difference in flap requirements, aesthetic outcome, and in some cases, functionality of the affected area. 2012
40
A 67-year-old man comes to the office with biopsy-proven Merkel cell carcinoma of the forehead. In addition to wide resection, which of the following is the optimal treatment? A) Administration of interferon B) Injection of 5-fluorouracil C) Neoadjuvant chemotherapy D) Radiation therapy
The correct response is Option D. Merkel cell carcinoma is a rare tumor that usually consists of smooth, painless, indurated, solitary dermal nodules approximately 2 to 4 mm in size. It occurs more frequently in patients older than age 65 years. Merkel cell carcinoma appears most often at sun-exposed sites on white skin; 50% occur on the head and neck, and 40% on the trunk. Merkel cell carcinoma is an aggressive tumor; metastases to regional lymph nodes are noted on initial diagnosis in 12 to 15% of patients. Regional metastasis eventually occurs in one half to two thirds of patients. Local recurrence following primary excision develops in 24 to 44% of patients. Time from diagnosis of the primary tumor to clinically apparent regional nodal metastases is approximately 7 to 8 months. Distant metastases occur ultimately in one third of patients; in order of frequency, metastases occur in the lymph, liver, bone, brain, lung, and skin. The mean time from diagnosis to systemic involvement is 18 months, with death occurring 6 months later. The 5-year survival rate has been reported as 30 to 64%. Two thirds or more of patients with local or regionally recurrent disease ultimately die. Surgical excision is the treatment of choice for primary tumors. The prevailing opinion regarding Merkel cell cancers is that they should be excised with margins similar to those for melanoma. Sentinel lymph node biopsy is used in clinically node-negative patients with Merkel cell carcinoma. Radiation should be considered for all patients with Merkel cell carcinoma, as it is radiosensitive. Injection of 5-fluorouracil and administration of interferon have never been shown to be effective in Merkel cell treatment. In Merkel cell carcinoma, tumor width dictates surgical excision, not tumor depth of invasion (as in melanoma). Mohs micrographic surgery has been advocated for resection of Merkel cell carcinoma, not only to obtain clear margins, but also to preserve the most amount of tissue. 2011
41
A 42-year-old woman comes to the office because of a 1-year history of a mass in the upper abdominal wall that has enlarged gradually. Examination of a specimen obtained on excision biopsy shows a desmoid tumor. Which of the following is the most appropriate next step in management? ``` A) Cryoablation B) Enucleation C) Excision with a 1-mm margin D) Excision with a 1-cm margin E) Observation ```
The correct response is Option D. Desmoid tumors are relatively rare, technically benign fibrous tumors that may arise in the musculoaponeurotic abdominal wall. These lesions exhibit local invasion and a high rate of recurrence. Wide local excision is regarded as the most effective treatment for these lesions, and intraoperative frozen section is often helpful. Observation will likely result in recurrence, and cryoablation has not been reported as a treatment option for desmoid tumors. Enucleation is not appropriate because the tumor will recur. Excision with a 1-mm margin is not appropriate because wide margins are necessary. 2011
42
A 79-year-old man has a rapidly growing lesion on the left side of his forehead. Physical examination shows a 2-cm, raised, fungating lesion of the left temple with intact facial nerve function and no lymphadenopathy. Examination of a specimen obtained on biopsy is suspicious for squamous cell carcinoma. Four weeks later, the lesion has disappeared, leaving a small circular scar. Excision is performed, and pathologic study shows no evidence of malignancy. Which of the following is the most likely diagnosis? ``` A) Amelanotic melanoma B) Cutaneous horn C) Keratoacanthoma D) Merkel cell carcinoma E) Squamous cell carcinoma ```
The correct response is Option C. The most likely diagnosis is keratoacanthoma, a low-grade malignancy that resembles squamous cell carcinoma both clinically and pathologically. The most common natural course of the disease is that of rapid growth followed by spontaneous regression over several months, which is not seen in squamous cell carcinoma. Keratoacanthoma can progress to squamous cell carcinoma with metastasis. Amelanotic melanoma is an uncommon (less than 5%) form of melanoma characterized by nonpigmented lesions that appear pink or tan and can mimic basal cell or squamous cell carcinoma. It can also occur in the context of cutaneous metastatic melanoma, when cells lack the differentiation required to synthesize melanin. It does not usually ulcerate or regress. A cutaneous horn is a conical projection of hyperkeratosis overlying a hyperproliferative skin lesion such as a seborrheic keratosis or actinic keratosis. Less commonly, it can form from a squamous cell carcinoma or other skin cancer. Ulceration and regression are not characteristic. Merkel cell carcinoma is a rare and aggressive form of skin cancer that shows neuroendocrine features and is prone to metastasis if left untreated. 2011
43
A 65-year-old woman is evaluated because of multiple ulcerative, nonhealing wounds on the left shoulder 8 years after undergoing left modified radical mastectomy and subsequent radiation therapy to the chest wall. She has a 5-year history of chronic lymphedema. Which of the following is the most appropriate next step? ``` A) Brachytherapy B) Hyperbaric oxygen therapy C) Incisional biopsy D) Isolated limb perfusion with chemotherapy E) Skin resection only with 5-mm margins ```
The correct response is Option C. Stewart-Treves syndrome is an aggressive but rare upper extremity lymphangiosarcoma that occurs in postmastectomy patients. Although plastic surgeons typically do not manage these patients, plastic surgeons should be familiar with their diagnostic workup. These patients may be referred to a plastic surgeon for management of a chronic wound, which can be confused with radionecrosis. Initial tissue diagnosis, via incisional biopsy, is the critical first step in management. Despite a historical 5-year survival of less than 1%, cure rates may be improving, caused in part by refinement of the resection techniques and adjuvant therapies used to treat the original breast cancer. Therapeutic options for lymphangiosarcoma are not standardized but include radical ablative surgery and isolated limb perfusion with tumor necrosis factor and melphalan. Hyperbaric oxygen may accelerate growth and is contraindicated. Excision of skin only may be indicated, but this approach would require margins greater than or equal to 1 cm. 2011
44
A 40-year-old man comes to the office because of a 12-year history of recurrent painful nodules in his groin, buttocks, and perineum. Physical examination shows deep subcutaneous abscesses. Some have ruptured and formed multiple discharging sinus tracts. In addition to meticulous hygiene, which of the following is the most appropriate management? ``` A) Antiandrogen therapy B) Anti-tumor necrosis factor-a therapy C) Excision D) Radiotherapy E) Regular chlorhexidine baths ```
The correct response is Option C. The patient has hidradenitis suppurativa. The disease presents with tender subcutaneous nodules beginning around puberty. The nodules may spontaneously rupture or coalesce, forming deep, painful dermal abscesses. Eventually, fibrosis and the formation of extensive sinus tracts result. The location of the lesions may lead to social embarrassment. Due to the multiple interconnected sinus tracts and abscesses throughout an entire region, the patient described has such a debilitating disease that only surgery can adequately address his symptoms. Wide excision of all affected tissue and the underlying sinus tracts is the most effective treatment. It is also advisable to stage the process, preferably with the use of allograft. For patients with abscesses but no cicatrization or sinuses, hygienic measures and antibiotics are an appropriate first-line therapy. The disease primarily involves the follicular epithelium, which is colonized secondarily and infected by bacteria. Clindamycin and tetracycline have shown benefit in clinical trials. Smaller studies using dapsone and minocycline have shown useful short-term benefit. Several trials using antiandrogen therapy have been conducted. In a randomized trial comparing ethinyl estradiol 50 mg/cyproterone acetate 50 mg to ethinyl estradiol 50 mg/norgestrel 500 mg in 24 women, both regimens produced improvement in disease activity. The tumor necrosis factor-a inhibitors infliximab and etanercept have also produced favorable outcomes. Limited lesions can be injected with corticosteroids, and flares can be addressed with short courses of oral or intramuscular corticosteroids. Patients with one or more widely separated, recurrent abscesses with sinus tract formation and scars or patients who have failed the first-line therapies may need more aggressive treatment than those with early stage lesions. Treatments that carry more risk may be worth trying depending on the severity of the patient's disease course. Long-term immunosuppressive therapy or surgical therapies, such as limited excisions or the laying open of sinus tracts, may be helpful. Radiation therapy for hidradenitis suppurativa was used extensively in the past. Techniques and responses have varied widely, but poor tissue healing was noted. 2011
45
A 58-year-old man comes to the office because of a 3-month history of multiple light-red, scaly lesions of the scalp and forehead. Physical examination shows extensive sun damage to the face and scalp, including multiple flat lesions measuring between 3 and 10 mm in diameter. Examination of a specimen obtained on shave biopsy shows actinic keratosis without invasive malignancy. In addition to daily application of sunscreen and wearing protective garments, which of the following is the most appropriate management? ``` A) Application of topical 5-fluorouracil B) Excision of the lesions with 2-mm margins C) Repeat biopsy in 6 months D) Shave excision of the lesions E) Observation only ```
The correct response is Option A. Actinic keratosis is a common, premalignant lesion that is a direct result of sun damage. These lesions typically occur in fair-skinned patients who have an extensive history of solar injury. Lesions are flat or slightly raised, red, and scaly. They can be isolated or diffuse. Over time, they can progress to squamous cell carcinoma or other precancer lesions, such as Bowen disease (in situ squamous cell), cutaneous horns, and keratoacanthomas. All patients must be counseled on sun protection measures, such as daily sunscreen application and wearing protective garments. Observation alone may be applicable for very small lesions or in certain infirm or elderly patients, but it is not appropriate in most circumstances because of the potential for malignancy. The rate of transformation is not clear but has been reported to be 1 to 25% per year per lesion. Ideal treatment involves topical destructive measures, such as application of 5-fluorouracil cream. Typically, it is applied twice daily for 2 to 4 weeks. Other common treatments include imiquimod cream (Aldara), cryotherapy with liquid nitrogen, and photodynamic therapy with 5-aminolevulinic acid (Levulan). These topical treatments are preferable to excision of the superficial lesions described because they can typically treat multiple and diffuse lesions without extensive scarring. Local redness and irritation does occur and resolves over a period of weeks. Subclinical lesions can also be treated, preventing growth of new lesions. Excision or repeat biopsy should be reserved for isolated lesions that are refractory to less invasive measures. Chemical peels and laser resurfacing have also been described as treatment options. 2011
46
A 64-year-old Hispanic woman comes for evaluation of a biopsy-proven basal cell carcinoma on her right cheek. She says that the lesion has been present for 8 months and has grown rapidly during the past 3 months. A photograph is shown. Which of the following is the most appropriate treatment? ``` A ) Cryotherapy B ) Electrodesiccation and curettage C ) Mohs micrographic surgery D ) Radiation therapy E ) Wide local excision ```
The correct response is Option C. The patient described has a basal cell carcinoma (BCC), which is the most common type of cancer worldwide affecting approximately 1 million people per year. These types of cancers were once more common in people over 40 years of age, but they now are often diagnosed in younger people (85% of lifetime sun exposure occurs before age 18). BCC starts in the epidermis and grows slowly and painlessly. It most often appears on areas of skin that are regularly exposed to sunlight or other ultraviolet radiation, such as the face, scalp, ears, chest, back, and legs. These tumors can have several different forms. The most common appearance of BCC is that of a small dome-shaped bump that has a pearly white color. Telangiectasias may be seen on the surface. BCC can also appear as a pimple-like growth that heals, only to come back again and again. A common sign of BCC is a sore that bleeds and heals up, only to recur again; oozing or crusting spots within the sore are also common. While there are many ways to treat BCC, Mohs micrographic surgery is the most appropriate option in the scenario described, as the lesion demonstrated has indistinct borders, is located in both a high-risk and cosmetically sensitive area, and is growing rapidly. Wide local excision would be indicated for BCC with discrete, distinct borders. Radiation therapy is not a primary option for BCC. Cryotherapy and electrodesiccation and curettage have lower cure rates and higher recurrence rates compared with Mohs micrographic surgery. Generally accepted indications for Mohs micrographic surgery include the following: 1. Recurrent or incompletely excised BCC or squamous cell carcinoma (SCC) 2. Primary BCC or SCC with indistinct borders 3. Lesions located in high-risk areas (ie, eyelids, nose, ear, nasolabial folds, upper lip, vermillion border, columella, periorbital, temples, preauricular and postauricular areas, scalp) 4. Cosmetically and functionally important areas, including genital, anal, perianal, hand, foot, and nail units 5. Tumors with aggressive clinical behavior (ie, rapidly growing, greater than 2 cm in diameter) 6. Tumors with an aggressive histologic subtype (ie, morpheaform BCC) and/or those with perivascular invasion 7. SCCs ranging from undifferentiated to poorly differentiated, and SCCs that are adenoid (acantholytic), adenosquamous, desmoplastic, infiltrative, perineural, periadnexal, or perivascular 8. Tumors arising in sites of previous radiation therapy 9. Tumors arising in immunosuppressed patients 10. Basal cell nevus syndrome patients Mohs micrographic surgery has the highest 5-year cure rates for primary and recurrent BCC. Five-year recurrence rates for primary BCC: 1. Mohs micrographic surgery 1% 2. Surgical excision 10.1% 3. Curettage and desiccation 7.7% 4. Radiation therapy 8.7% 5. Cryotherapy 7.5% Five-year recurrence rates for recurrent BCC: 1. Mohs micrographic surgery 5.6% 2. Surgical excision 17.4% 3. Curettage and desiccation 40% 4. Radiation therapy 9.8% 5. Cryotherapy 13% (< a 5-year period) 2010
47
A 44-year-old woman has a biopsy-proven basal cell carcinoma of the cheek. An indication for resection using conventional excision, over Mohs micrographic surgery, includes which of the following? ``` A ) Diameter of 1.2 cm B ) Location next to the alar rim C ) Morpheaform subtype D ) Preexisting burn scar surrounding the lesion E ) Recurrent tumor ```
The correct response is Option A. Indications for Mohs micrographic resection of a basal cell carcinoma include morpheaform or other aggressive subtypes, recurrence of a tumor, a previous scar, or locations where the preservation of tissue is critical for final reconstruction. A basal cell carcinoma of the cheek, measuring 1.2 cm in diameter, would require a several millimeter margin circumferentially. It could be resected and closed without difficulty using a variety of tissue rearrangements, such as a rhomboid flap or a slide-swing flap. 2010
48
A 22-year-old woman comes for evaluation of a lesion on her right anterior thigh (shown). She says that the lesion has been present for a number of years and that she frequently nicks it when shaving. She has no other similar lesions, and no family history of similar lesions. Physical examination shows a single, firm lesion. The nodule contracts with lateral compression. No other abnormalities are noted. Which of the following is the most appropriate next step in management? ``` A ) Cryotherapy B ) Excisional biopsy C ) Mohs micrographic surgery D ) Shave biopsy E ) Observation ```
The correct response is Option B. The lesion is a dermatofibroma, which is a benign nodule derived from mesodermal and dermal cells. They can be found anywhere on the body but most commonly appear on the anterior surface of the lower legs. Studies have not definitely determined whether these are true neoplasms or whether they are fibrous reactions to minor trauma, insect bites, viral infections, ruptured cysts, or folliculitis. Dermatofibromas are asymptomatic, firm, raised papules, plaques, or nodules that can vary in size from 3 to 10 mm in diameter. Coloration ranges from brown to purple, red, yellow, and pink. They are diagnosed based on physical examination and commonly display a Fitzpatrick sign, which is the dimpling or retraction of the lesion beneath the skin with lateral compression. Underlying autoimmune disorders, such as systemic lupus erythematosus, may be suspected if multiple dermatofibromas (ie, > 15) are found on a patient. Treatment is generally performed for cosmetic reasons or histologic diagnosis in case there is any question about the clinical diagnosis. Excisional biopsy is the procedure of choice to ensure clear histology, as well as complete removal of the lesion, as it exists in the dermal plane; however, the drawback to this technique is scar formation. Cryosurgery is an option for a less invasive treatment, but it will not completely destroy the lesion. Mohs micrographic surgery is not indicated for this benign lesion. Observation is not advised in this particular case, as the patient described notes a significant nuisance that motivated her to seek treatment. Shave biopsy would not completely eliminate the lesion, as it frequently does not penetrate deep enough into the dermis to clear the pathology. 2010
49
A 38-year-old woman has severe hidradenitis suppurativa of the groin and axillae. Which of the following dermal appendages are located in these areas and implicated in the disease process? ``` A ) Apocrine glands B ) Eccrine glands C ) Glomus bodies D ) Hair follicles E ) Sebaceous glands ```
The correct response is Option A. Apocrine glands are uniquely located in the axillae, groin, and perineum, and they secrete a viscid, milky fluid that becomes malodorous with bacterial colonization. Occlusion of the glands causes inflammation and subcutaneous abscess formation with pain, drainage, and foul odor. This crippling disease can be medically managed with chronic suppressive topical and systemic antibiotics but often requires intermittent incision and drainage, or even surgical resection. Eccrine glands are found throughout the skin, secreting a thin, clear, hypotonic fluid ( sweat). Glomus bodies are located in tissues exposed to the cold, such as the fingertips and ears. They form a thickening in the arterial wall before naturally occurring thermoregulatory arteriovenous shunts, and they are thought to control the flow through these shunts. Although some recent data suggest that hair follicle occlusion and folliculitis lead to apocrine gland occlusion and subsequent hidradenitis suppurativa, hair follicles are located all over the body and do not form the abscesses responsible for the clinical disease. Sebaceous glands secrete sebum, an oily substance that lubricates hair follicles and surrounding skin. They are found throughout the skin, except for the palms and soles. They are found in abundance in the face and scalp. 2010
50
A 72-year-old man is being evaluated because of a 3-month history of lesions on the nasal dorsum and cheek (shown). He is a poor surgical candidate and is treated with imiquimod (Aldara). Which of the following is the most likely mechanism of action of this treatment? A ) Inhibition of the cyclooxygenase pathway B ) Inhibition of DNA synthesis C ) Modification of gene transcription D ) Modulation of cell differentiation E ) Stimulation of proinflammatory cytokine production
The correct response is Option E. Imiquimod (Aldara) is a new immune response enhancer that stimulates host cytokine production and induces apoptosis of tumor cells. It has been used to treat actinic keratoses, viral warts, and nonmelanoma skin malignancy. Topical 5-fluorouracil is a topical chemotherapeutic agent that directly inhibits DNA synthesis. Retinoids prevent new skin cancer development by regulating cell differentiation. Topical diclofenac is an anti-inflammatory drug that inhibits the cyclooxygenase pathway and has been found useful in the treatment of actinic keratoses. Interferons control cell differentiation by modification of gene transcription and have been used in combination with retinoids for advanced squamous cell cancers. 2010
51
An 86-year-old woman comes to the office because of an 18-month history of the isolated, painful lesion shown - calcinosis cutis. History includes CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome. Which of the following is the most appropriate initial management? ``` A ) Debridement B ) Hyperbaric oxygen therapy C ) Injection of corticosteroids D ) Measurement of serum calcium level E ) Measurement of serum uric acid level ```
The correct response is Option A. CREST syndrome is a member of the heterogeneous sclerodermas. Calcinosis cutis is the pathologic calcification of soft tissues and skin. The calcific deposits, when symptomatic, can be tender and painful. They can ulcerate, drain a white chalky substance, and become secondarily infected. In scleroderma, calcific deposits are found predominantly in the extremities and around joints and bony prominences. Deposits are typically found in the flexor surfaces of the hands and the extensor surfaces of the forearms and knees. The deposits rest in the dermis but can also be found in deeper periarticular tissues. Serum calcium, phosphorus, and alkaline phosphatase levels typically are normal; serum measurement does not contribute to management. CREST is not associated with gout, so measurement of serum uric acid is noncontributory. Autografting prior to wound debridement and removal of calcific deposits is not recommended. Hyperbaric oxygen therapy may follow debridement. No consistently reliable pharmacologic treatment, including injection of corticosteroids, has been shown to prevent or eliminate calcinosis. However, surgical excision of localized, painful large deposits can relieve symptoms; recurrence is rare. If calcinosis is diffuse, recurrence is more common. Successful palliation and significant remission of calcinosis using a carbon dioxide laser for debridement was shown in two case reports with a total of seven patients. Calcinosis cutis is distinct from calciphylaxis, which is a poorly understood and highly morbid syndrome of vascular calcification and skin necrosis typically seen in 1 to 4% of patients with end-stage renal disease. Wound debridement in this setting is controversial and may cause exacerbation of skin necrosis. 2010
52
Which of the following is the most important management for patients with xeroderma pigmentosum? ``` A ) Cryotherapy B ) Fluorouracil C ) Isotretinoin D ) Minimization of sun exposure E ) Skin grafting ```
The correct response is Option D. Xeroderma pigmentosum (XP) is an autosomal recessive disorder demonstrating defective DNA repair. The ability to repair damage caused by ultraviolet (UV) light is deficient. In severe cases, it is necessary to avoid sunlight completely. The most common defect in XP is a genetic defect whereby nucleotide excision repair (NER) enzymes are mutated, leading to a reduction in or elimination of NER. Unrepaired damage can lead to mutations, altering the information of the DNA in individual cells. If mutations affect important genes, like tumor suppressor genes or proto-oncogenes, then this disorder may lead to cancer. Patients with XP exhibit elevated risk of developing cancer. Multiple basaliomas and other skin malignancies, such as squamous cell carcinoma, occur as early as 8 years of age. Patients with XP are usually diagnosed at 1 to 2 years of age. The sunburn usually occurs during a child's first sun exposure. An unusually severe sunburn that may last for several weeks even after a short sun exposure course is common. Other symptoms include development of many freckles at an early age, irregular dark spots on the skin, thin skin, excessive dryness of skin, rough-surfaced growths (solar keratoses), and skin cancers; eyes that are painfully sensitive to the sun and may easily become irritated, bloodshot, and clouded; blistering or freckling on minimum sun exposure; premature aging of skin, lips, eyes, mouth, and tongue; skin crusting; spidery blood vessels; scaly skin; and oozing raw skin surface. The most important management is minimizing sun exposure. The number of keratoses can be reduced with isotretinoin (though there are significant side effects). Existing keratoses can be treated using cryotherapy or fluorouracil. Skin cancers are detected at approximately 8 years of age. Frequent skin cancer surgical excisions may be necessary for local control. 2010
53
A 52-year-old man is scheduled to undergo a midline laparotomy for colon cancer. History includes cutis laxa. Examination shows hypoelastic skin that does not spring back when stretched. This patient is at increased risk for which of the following complications? ``` A ) Anastomotic dehiscence B ) Hypertrophic scarring C ) Thrombosis D ) Ventral hernia E ) Wound dehiscence ```
The correct response is Option D. Cutis laxa patients are at a higher risk for ventral hernia formation. Cutis laxa is a rare condition with hypoelasticity of the skin from a defect in elastin fibers. The skin does not spring back in place immediately upon being stretched, as compared to Ehlers-Danlos syndrome, where the skin springs back without wrinkling. These conditions can be congenital (autosomal dominant, recessive, X-linked recessive) and acquired (drug ingestion, paraneoplastic, postinflammatory). There is an increased risk of hernia formation. Scar formation and healing appear normal; however, procedures to reduce skin redundancy, such as rhytidectomy and blepharoplasty, may require serial procedures as progression of skin laxity continues. Ehlers-Danlos syndrome (cutis hyperelastica) is characterized by skin hyperextensibility, joint laxity, and tissue friability. It is one of the most frequently inherited collagen disorders and is frequently underdiagnosed. Patients can dehisce their wounds or anastomoses at 1 to 2 weeks. Wound failure is common. Ventral hernias are common and may require closure techniques, such as components separation. Scar formation is wide and extremely thin, and it has been described as papyraceous and cigarette-paper. Homocystinuria is associated with an increased risk of thrombosis. 2010
54
An otherwise healthy 30-year-old woman is diagnosed with dermatofibrosarcoma protuberans (DFSP) of the upper back. A wide excision is performed, and a local flap is used to reconstruct her back. On follow-up evaluation, CT scan shows multiple pulmonary metastases. Which of the following is the most appropriate next step in management? ``` A) Chemotherapy B) Hormone therapy C) Immunotherapy D) Radiation therapy E) Surgical excision ```
The correct response is Option A. The most appropriate next step in management is chemotherapy. Patients with inoperable, recurrent, or metastatic disease may benefit from imatinib which is a tyrosine kinase inhibitor and acts as a molecularly targeted drug. It acts by inhibiting the platelet-derived growth factor receptor tyrosine kinase. Dermatofibrosarcoma protuberans (DFSP) is characterized by chromosomal rearrangements resulting in the production of platelet-derived growth factor B, eventually leading to autocrine growth stimulation of DFSP. Imatinib functions as an inhibitor of platelet-derived growth factor receptors, thus blocking this autocrine stimulation. Therefore, imatinib can be used as an adjuvant therapy for cases in which obtaining sufficient surgical margins is impossible. Neoadjuvant imatinib has also been used for locally advanced primary tumors. Radiation therapy may improve local control and reduce the risk of recurrence postoperatively in patients with DFSP. There is no described role of it in pulmonary metastases. Resection of the multiple lesions in the lung, and hormonal or immune therapy are not recommended for metastatic DFSP. 2019
55
An otherwise healthy 20-year-old woman develops a keloid on her right earlobe after an ear piercing. Excision and radiation therapy are planned. Which of the following is the ideal time after excision for the initiation of radiation therapy? ``` A) 1 day B) 7 days C) 2 weeks D) 4 weeks E) 6 weeks ```
The correct response is Option A. The ideal time to give radiation therapy in this case is on postoperative day one. Earlobes and the helix of the auricle are common sites for keloid formation, usually after trauma or ear piercing, with an incidence of approximately 2.5%. There are numerous adjuvant therapies (eg, radiation therapy), medical therapies, (eg, intralesional steroids, 5-fluorouracil interferon, and topical silicone), and physical approaches (eg, pressure) that can be used in addition to excision of the keloid to help reduce its recurrence. However, the treatment options for such lesions are still controversial, because there are numerous challenges, and no single best treatment or best combination of treatments has been proved to manage these conditions effectively. Radiation therapy has long been known to be effective in the early phase of wound healing. More specifically, radiation therapy is sensitive to endothelial vascular buds and decreases proliferation of new fibroblasts. As such, radiation therapy after keloid excision should be performed as soon as possible. Usually, better results are reported when radiation therapy is performed within 1 to 3 days after surgery. The most commonly administered doses are between 10 and 15 Gy over a period of 2 or 3 days. The other options are incorrect as they are outside the ideal time frame. 2019
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A 65-year-old man comes to the office with a 2-cm basal cell carcinoma involving the left nasal ala extending across the nasofacial junction onto the cheek. A photograph is shown. Medical history includes previous melanoma. Which of the following is an indication for Mohs micrographic surgery over conventional excision in this patient? ``` A) Cancer size B) Diagnosis of basal cell carcinoma C) History of previous melanoma D) Location of the lesion E) Patient age ```
The correct response is Option D. Mohs micrographic surgical technique has demonstrated cure rates of 99% for primary basal cell carcinomas and up to 95% for recurrent basal cell carcinomas. In this particular patient, the strongest indication for use of the Mohs technique is the anatomical location. The nose is considered a high-risk location in the classically described “H-zone.” This patient underwent Mohs excision with multi-stage forehead flap reconstruction, as shown in the photographs. Patient age, history of previous melanoma, and tumor size 2 cm or less are not standard indications for Mohs excision. Other indications for Mohs technique include the following: Recurrent basal cell/squamous cell carcinomas, Locations prone to recurrence- “H-zone” of the face: periorbital, periauricular, temple, upper lip, nose/nasolabial fold, and chin, Tumors involving critical structures such as the eyelid or lip, Functionally important areas such as the genitals, perianal location, hands, and feet, Tumors arising in sites of previous irradiation therapy, Large tumors (> 2 cm) Lesions with ill-defined tumor margins, Histologic aggressive subtype (morpheaform, basosquamous, perineural, and invasive/poorly differentiated squamous cell carcinoma) Tumors arising in immunosuppressed patients such as transplant recipients or patients with genetic predisposition (basal cell nevus syndrome, xeroderma pigmentosum) 2019
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A 76-year-old man comes to the office for follow-up after undergoing Mohs resection of a basal cell carcinoma (BCC) of the left central cheek. He smokes a half pack of cigarettes daily and has no intention of quitting. Final margins have been confirmed to be free of residual tumor. The defect is full-thickness skin and measures 2 × 2 cm. Which of the following is the most appropriate treatment option for this patient? ``` A) Cervicofacial flap B) Direct primary closure C) Full-thickness skin grafting D) Healing by secondary intention E) Rhomboid transposition flap ```
The correct response is Option B. Direct primary closure is the best option for this patient. The cheek skin has ample laxity and allows for closure of small- to medium-sized cheek defects. In general, closure should be aligned with the relaxed skin tension lines, and aggressive management of dog ears should be performed. Direct linear closure also allows for simple monitoring for recurrence without any distortion of the anatomy. Secondary intention is better utilized in areas of concavity, like the medial canthus. Full-thickness skin grafting, rhomboid flaps and cervicofacial flaps will have a higher complication rate in an active smoker, while likely providing a lesser aesthetic outcome given the poor orientation of scars. 2019