In-Service Exam
Skin Lesions - 2002

Aesthetic surgical procedures can be performed in patients who have which of the following disorders of excess skin laxity?

(A) Cutis laxa
(B) Ehlers-Danlos syndrome
(C) Elastoderma
(D) Hutchinson-Gilford syndrome
(E) Werner's syndrome

The correct response is Option A.

Aesthetic surgical procedures can be performed in patients who have cutis laxa, a genetic disorder with variable expressivity that results from a nonfunctioning elastase inhibitor or premature degeneration of elastin fibers. Affected patients have coarsely textured, drooping skin. Because this condition is not associated with wound healing problems, rhytidectomy may be considered if the patient is otherwise healthy.

Ehlers-Danlos syndrome, or cutis hyperelastica, is an inherited disorder of abnormal molecular collagen cross-linking, and is thought to result in a deficiency of the lysyl oxidase enzyme. It is characterized by hyperextensibility and excessive thinness of the skin and hypermobility of the joints. Cosmetic surgery is contraindicated in patients with Ehlers-Danlos syndrome because wound healing is poor and subcutaneous hemorrhages are common.

Elastoderma is a disorder of unknown cause characterized by pendulous skin laxity initially involving the trunk and extremities that progresses to encompass the entire body. Elective surgery is contraindicated because wound-healing mechanisms are unknown.

Progeria, also known as Hutchinson-Gilford syndrome when seen in adults, is an autosomal recessive disorder of unknown cause and is characterized by growth retardation, craniofacial malformations, cardiac disease, and auricular abnormalities, as well as skin laxity, loss of subcutaneous fat, and findings similar to premature aging. Aesthetic surgery is contraindicated because wound healing is poor; in addition, premature death is associated.

Werner's syndrome is a rare autosomal recessive disorder of unknown cause. Associated features include premature aging, cataracts, pigmentary abnormalities, and diabetes mellitus. Because these patients have arteriosclerosis and microangiopathy, cosmetic procedures should not be performed.
References
1. Rae V, Falanga V. Wrinkling due to middermal elastolysis: report of a case and review of the literature. Arch Dermatol. 1989;125:950.
2. Thomas WO, Moses MH, Craver RD, et al. Congenital cutis laxa: a case report and review of loose skin syndromes. Ann Plast Surg. 1993;30:252.

A neonate has a 4 * 4-cm congenital defect of the scalp and underlying skull. The brain is visible beneath a gray membrane. Which of the following is the most appropriate initial management?

(A) Frequent application of silver sulfadiazine ointment
(B) Wet-to-dry dressing changes twice daily
(C) Biopsy of the wound margins
(D) Tissue expansion and coverage with scalp flaps

The correct response is Option A.

This neonate has cutis aplasia, or congenital absence of the layers of the skin and scalp that can also expand to include the skull. Conservative management is most appropriate; frequent application of silver sulfadiazine ointment and coverage with occlusive dressings will allow for wound healing. Some surgeons also advocate skin grafting over the exposed areas to prevent wound desiccation.

Use of dry dressings can actually result in desiccation of the dura and rupture of the sagittal sinus, a fatal complication. In patients with cutis aplasia, the wound must be kept moist at all times. Biopsy of the wound margins is not indicated because this patient does not have a malignant or premalignant condition. Tissue expansion is not required for this small wound, which will heal by secondary intention without reconstruction.

References
1. Elliot LF, Jurkiewicz MJ. Scalp and calvarium. In: Jurkiewicz MJ, Mathes SJ, Krizek TJ, et al, eds. Plastic Surgery: Principles and Practice. Saint Louis, Mo: CV Mosby Co; 1990:419-440.
2. Wexler A, Harris M, Lesavoy M. Conservative treatment of cutis aplasia. Plast Reconstr Surg. 1990;86:1066.

A 45-year-old man has a painful lesion in the left conchal bowl. A biopsy specimen of the lesion shows chondrodermatitis nodularis helicis. Which of the following is the most appropriate management?

(A) Topical administration of retinoic acid
(B) Administration of acyclovir
(C) Administration of fluorouracil
(D) Administration of interferon
(E) Surgical excision of the lesion

The correct response is Option E.

Surgical excision is the most appropriate management of chondrodermatitis nodularis helicis, a painful erythematous nodule found on the helix, antihelix, or antitragus in persons older than 40 years. Although the cause of chondrodermatitis nodularis helicis is unknown, trauma, sun damage, and cold exposure may be mitigating factors. Men are more frequently affected. Because these lesions can become ulcerated, resulting in exposure of underlying cartilage, early surgical excision is recommended. Adjunctive removal of the underlying cartilage lessens the risk for recurrence, which is common at the borders of the resection. Other forms of therapy such as topical retinoic acid, acyclovir, fluorouracil, and interferon have not been proven effective. Intralesional injection of a corticosteroid has been helpful in some patients.

References
1. Graham GF. Cryosurgery. Clin Plast Surg. 1993;20:131-147.
2. Morganroth GS, Leffell DJ. Nonexcisional treatment of benign and premalignant cutaneous lesions. Clin Plast Surg. 1993;20:91-104.

In a patient who has a halo nevus, which of the following is the primary indication for surgical excision?

(A) Elimination of circulating antibodies
(B) Premalignant potential
(C) Prevention of leukoderma
(D) Relief of pain
(E) Resemblance to melanoma

The correct response is Option E.

Halo nevi, so called because of the distinct "halo" area of depigmentation surrounding the benign nevus, are typically seen on the trunk in teenagers and young adults. Histologic examination will show nevus cells surrounded by a band-like infiltrate of lymphocytes that may completely obliterate the lesion. Halo nevi have no known premalignant potential; however, because melanomas can also develop an irregular, incomplete halo, excisional biopsy should be performed for any halo nevus that becomes enlarged, asymmetric, or ulcerated or displays other characteristics similar to melanoma. The depigmentation around the nevus, known as leukoderma, is typically not painful. Although circulating antibodies to melanoma have been found in patients with halo nevi, this discovery alone is not an indication for nevus excision.

References
1. Lazova R, McNiff JM, Glusac EJ. Under the microscope: surgeons, pathologists, and melanocytic nevi. Clin Plast Surg. 2000;27:323-329.
2. Popkin GL. Tumors of the skin: a dermatologist's viewpoint. In: McCarthy JG, ed. Plastic Surgery. Philadelphia, Pa: WB Saunders Co; 1990;5:3560-3613.

A 50-year-old man has a keratoacanthoma on the left arm. He underwent removal of skin tumors on three previous occasions. His brother and father have had similar findings; the father subsequently died of a malignant tumor. Which of the following is the most appropriate diagnostic test?

(A) Bone scan
(B) Colonoscopy
(C) CT scan of the chest
(D) Panoramic radiograph
(E) Plain radiographs of the long bones

The correct response is Option B.

This patient has findings consistent with Muir-Torre syndrome, an autosomal dominant disorder that typically has its onset between the ages of 50 and 70 years. Multiple skin malignancies (eg, keratoacanthomas, basal and squamous cell carcinomas, adenocarcinomas) are characteristic. Because malignancies of the colon, kidneys, bladder, ovary, pancreas, and breast are also associated and may even be present prior to the appearance of skin lesions, diagnostic testing (such as colonoscopy) should be performed in any patient with multiple keratoacanthomas, especially in this patient who has a family history of similar findings. The other diagnostic tests listed above would not be effective in detecting malignancies associated with Muir-Torre syndrome.

References
1. Gherardini G, Bhatia N, Stal S. Congenital syndromes associated with nonmelanoma skin cancer. Clin Plast Surg. 1997;24:649-661.
2. Harris AO, Levy ML, Goldberg LH, et al. Nonepidermal and appendageal skin tumors. Clin Plast Surg. 1993;20:115-130.

A 54-year-old woman undergoes excisional biopsy of a 6-mm papule from the right alar crease followed by direct closure of the wound. Histologic examination of a biopsy specimen of the lesion shows sclerosing basal cell carcinoma with positive deep margins.

Which of the following is the most appropriate next step in management?

(A) Observation and follow-up examination every two months
(B) Postoperative radiation therapy
(C) Reexcision with 5-mm margins and immediate reconstruction
(D) Reexcision with 1-cm margins and immediate reconstruction
(E) Mohs' micrographic surgery and immediate reconstruction

The correct response is Option E.

This patient has many risk factors for recurrence of her basal cell carcinoma, indicating the need for surgical removal. The lesion is located within the "H" zone of the face (defined as the upper lip, nose, periocular regions, and temporal and preauricular areas) which is associated with an increased risk for local recurrence. In addition, because the sclerosing and morpheaform types of basal cell carcinoma are more prone to recur, pathologic evaluation of the margins is indicated prior to reconstruction, especially in this patient who has positive deep margins. Therefore, Mohs' micrographic surgery is recommended to excise this high-risk carcinoma and provide tissue for immediate horizontal frozen section analysis. This technique is associated with a high cure rate in patients with recurrent or large tumors, poorly differentiated or aggressive histologic-type lesions, and tumors with positive margins and/or perineural invasion. Standard frozen section analysis may also be considered in this patient.

Although observation has been advocated in the past for the less aggressive variants of basal cell carcinoma (ie, not sclerosing and morpheaform variants), excision is now advocated because the recurrence rate has been shown to be high. Radiation therapy is appropriate only adjuvantly or as a first-line treatment in patients who are not surgical candidates.

References
1. Friedman HI, Williams T, Zamora S, et al. Recurrent basal cell carcinoma in margin-positive tumors. Ann Plast Surg. 1997;38:232-235.
2. Johnson TM, Nelson BR. Mohs' surgery for cutaneous basal cell and squamous cell carcinoma. In: Weber RS, Miller MJ, Goepfert H, eds. Basal and Squamous Cell Cancers of the Head and Neck. Baltimore, Md: Williams & Wilkins; 1996:147-155.
3. Weber RS. Surgical principles. In: Weber RS, Miller MJ, Goepfert H, eds. Basal and Squamous Cell Cancers of the Head and Neck. Baltimore, Md: Williams & Wilkins; 1996:115-132.

Which of the following lesions is most likely to contain malignant cells?

(A) Acrochordons
(B) Cutaneous horn
(C) Dermatofibroma
(D) Molluscum contagiosum
(E) Seborrheic keratosis

The correct response is Option B.

Cutaneous horns are most likely to contain malignant cells. These hard, cone-shaped cutaneous projections are typically caused by excessive epidermal growth and retention of keratin. Although cutaneous horns are most frequently associated with benign irregularities, approximately 20% are associated with premalignant lesions, and nearly 15% are associated with squamous cell carcinoma. Therefore, in patients with cutaneous horns, shave biopsy should be performed initially to exclude any underlying malignancy.

Acrochordons are simple skin tags not linked to malignancy. Dermatofibromas are benign, localized, fibrous tumors contained within the dermis that can develop following trauma. Molluscum contagiosum are virally induced papules commonly seen in children and young adults and may be associated with sexual transmission. Seborrheic keratoses are benign keratinocytic tumors seen in patients older than 30 years of age. Although these lesions often affect sun-damaged areas, they do not contain malignant cells.

References
1. Morganroth GS, Leffell DJ. Nonexcisional treatment of benign and premalignant cutaneous lesions. Clin Plast Surg. 1993;20:91-104.
2. Schaffer JV, Bolognia JL. The clinical spectrum of pigmented lesions. Clin Plast Surg. 2000;27:391-408.

In children, Spitz nevi are most frequently found at which of the following sites?

(A) Head and neck
(B) Upper extremities
(C) Trunk
(D) Genitalia
(E) Lower extremities

The correct response is Option A.

The Spitz nevus was first described in 1948 when one researcher found that several children who had been previously diagnosed with melanoma were still alive at follow-up examinations as long as 13 years later. As a result, these lesions became known as benign melanomas. Although the diagnosis of Spitz nevus may be difficult to determine both clinically and pathologically, these are most commonly considered benign lesions. They are usually small, often less than 6 cm in diameter, and nodular, and may be multiple. In children, Spitz nevi are most commonly located in the head and neck region and first appear between ages 5 and 10 years. In adults, these lesions are most frequently found on the extremities.

References
1. Cramer SF. The melanocyte differentiation pathway in spitz nevi. Am J Dermatopathol. 1998;20:555-570.
2. Helm KF, Schwartz RA, Janniger CK. Juvenile melanoma (Spitz nevus). Cutis. 1996;58:35-39.

Administration of which of the following reverses the potential for development of premalignant lesions?

(A) Oxybenzone
(B) Padimate
(C) Para-aminobenzoic acid
(D) Retinoids
(E) Zinc oxide

The correct response is Option D.

Retinoids, which are natural derivatives of vitamin A, have been shown to reverse the formation and growth of actinic keratoses. Because approximately 5% of these lesions will undergo transformation into nonmelanotic malignancies, topical application of retinoids is recommended to reverse cutaneous actinic damage, resulting in a return to normal skin in the affected areas.

Para-aminobenzoic acid, padimate, oxybenzone, and zinc oxide are sunscreen ingredients that limit the effects of ultraviolet-A and/or ultraviolet-B light but do not treat potentially premalignant lesions.

References
1. Farmer KL, Goller M, Lippman SM. Prevention of nonmelanoma skin cancer: standard and investigative approaches. Clin Plast Surg. 1997;24:663-671.
2. Green HA, Drake L. Aging, sun damage, and sunscreens. Clin Plast Surg. 1993;20:1-8.

Which of the following is most characteristic of an amateur tattoo?

(A) Irregular penetration of the dye in the superficial dermis
(B) Large pigment particles (50 _m)
(C) Organometallic pigments
(D) Penetration of the dye confined to the epidermis
(E) Uniform penetration of the dye in the deep dermis

The correct response is Option A.

Amateur tattoos are most frequently characterized by irregular penetration of the ink within the superficial dermis. This type of tattoo is popular because it is inexpensive and easily created using ink and a needle. Blue and black dyes are generally used. Pigment particles within the tattoo are small, typically 5 _m or less.

Professional tattoos are more likely to have brightly colored dyes and large pigment granules (greater than 50 _m). There is a consistently uniform depth of penetration within the deep dermis.

References
1. Alster TS. Q-switched alexandrite laser treatment (755 nm) of professional and amateur tattoos. J Am Acad Dermatol. 1995;33:69-73.
2. van der Velden EM, van der Walle HB, Groote AD. Tattoo removal: tannic acid method of Variot. Int J Dermatol. 1993;32:376-380.

16?