answers for self-assessment examination of the american academy of dermatology

3
aAD? -~5 1938 0 IOURNAL O[ the AmemcaN AcaDemy OF DerMaTOLOGY Answers for Self-Assessment examination of the American Academy of Dermatology IdentificationNo. 886-210 October, 1986, issue of the JOURNAL OFTHEAMERICAN ACAD- EMY OF DERMATOLOGY DISCUSSION OF QUESTIONS 1-3 Nevus anemicus is a rare congenital process oc- curring more commonly in female individuals. Le- sions are typically on covered portions of the trunk, but facial and extremity lesions have been reported. The well-defined hypopigmented lesions do not respond to heat, friction, or cold with the expected erythema. Under Wood's lamp exami- nation, lesions are no more clearly defined. Dias- copy results in obliteration of the lesion. The histology is normal, as are vascular struc- tures as seen by the electron microscope. The le- sion is currently thought to be secondary to a defect at the motor end plate or at the smooth muscle effector cells of the blood vessels. Fleisher and Zeligman noted that intralesional injection of ace- tylcholine, pilocarpine, histamine, or serotonin failed to produce vasodilatation in the affected areas. They postulated an increased stimulation of alpha adrenergic (vasoconstrictor) fibers or an in- hibition of fibers controlling vasodilatation of ar- terioles. Autotransplant studies have showed do- nor site dominance. This process may be seen in association with tuberous sclerosis (epiloia) and neurofibromatosis but can be easily distinguished from caf6 au lait spots and ash leaf spots. Lesions are typically on covered areas and do not require therapy. This is fortunate because no effective therapeutic modal- ity is known. For this series, the recommended choices are: 1, d; 2, d; 3, a, c. Ira H. Gouterman, M.D., Newark, NJ REFERENCES Daniel RH, et al: Nevus anemicus. Arch Dermatol 113:53- 56, 1977. Fleisher TL, Zetigmau I: Nevus anemicus. Arch Dermatol 100:750-755, 1969. Greaves MW, et al: Nevus anemieus. Arch Dermato1102:172- 176, 1970. DISCUSSION OF QUESTIONS 4-7 Case presented by W. Clark Lambert, M.D., Ph.D., Newark, NJ Multiple (i.e., more than 15) dermatofibromas have been reported in only a handful of patients; there is a well-documented association with sys- temic lupus erythematosus. Since Lin et al found three cases, all associated with systemic lupus er- ythematosus, in a single university consultation rheumatology service, however, this syndrome may be more common than has been appreciated. In some but not all cases there has been an asso- ciation with systemic steroid therapy for the sys- temic lupus erythematosus. The cause of this phenomenon is unknown. The lesions are identical in appearance to other der- matofibromas, with fibroblasts and newly formed fibrous tissue present (Figs. 3 and 4). Study of an outbreak of multiple dermatofibromas in rhesus monkeys in Nigeria led to the conclusion that a viral cause was likely, but this has not been proved. For this series, the recommended choices are: 4, d; 5, b; 6, a; 7, c. W. Clark Lambert, M.D., Ph.D., Newark, NJ REFERENCE Lin RY, Landsman L, Krey PR, et al: Multiple dermatofi- bromas and systernie lupus erythematosus. Cutis 37:45- 49, 1986. DISCUSSION OF QUESTIONS 8-10 Case presented by William Abramovits, MD, Caracas, Venezuela Dermatofibrosarcoma protuberans is so named because of its marked tendency to form exophytic nodules that extend far above the skin surface. Initially, however, the lesions are depressed, not 37A

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aAD? -~5 1938 0

IOURNAL O[ t h e

A m e m c a N A c a D e m y OF

D e r M a T O L O G Y

Answers for Self-Assessment examination of the American Academy of Dermatology

Identification No. 886-210

October, 1986, issue of the JOURNAL OFTHE AMERICAN ACAD- EMY OF DERMATOLOGY

DISCUSSION OF QUESTIONS 1-3

Nevus anemicus is a rare congenital process oc- curring more commonly in female individuals. Le- sions are typically on covered portions of the trunk, but facial and extremity lesions have been reported. The well-defined hypopigmented lesions do not respond to heat, friction, or cold with the expected erythema. Under Wood's lamp exami- nation, lesions are no more clearly defined. Dias- copy results in obliteration of the lesion.

The histology is normal, as are vascular struc- tures as seen by the electron microscope. The le- sion is currently thought to be secondary to a defect at the motor end plate or at the smooth muscle effector cells of the blood vessels. Fleisher and Zeligman noted that intralesional injection of ace- tylcholine, pilocarpine, histamine, or serotonin failed to produce vasodilatation in the affected areas. They postulated an increased stimulation of alpha adrenergic (vasoconstrictor) fibers or an in- hibition of fibers controlling vasodilatation of ar- terioles. Autotransplant studies have showed do- nor site dominance.

This process may be seen in association with tuberous sclerosis (epiloia) and neurofibromatosis but can be easily distinguished from caf6 au lait spots and ash leaf spots. Lesions are typically on covered areas and do not require therapy. This is fortunate because no effective therapeutic modal- ity is known.

For this series, the recommended choices are: 1, d; 2, d; 3, a, c.

Ira H. Gouterman, M.D., Newark, NJ

REFERENCES

Daniel RH, et al: Nevus anemicus. Arch Dermatol 113:53- 56, 1977.

Fleisher TL, Zetigmau I: Nevus anemicus. Arch Dermatol 100:750-755, 1969.

Greaves MW, et al: Nevus anemieus. Arch Dermato1102:172- 176, 1970.

DISCUSSION OF QUESTIONS 4-7

Case presented by W. Clark Lambert, M.D., Ph.D., Newark, NJ

Multiple (i.e., more than 15) dermatofibromas have been reported in only a handful of patients; there is a well-documented association with sys- temic lupus erythematosus. Since Lin et al found three cases, all associated with systemic lupus er- ythematosus, in a single university consultation rheumatology service, however, this syndrome may be more common than has been appreciated. In some but not all cases there has been an asso- ciation with systemic steroid therapy for the sys- temic lupus erythematosus.

The cause of this phenomenon is unknown. The lesions are identical in appearance to other der- matofibromas, with fibroblasts and newly formed fibrous tissue present (Figs. 3 and 4). Study of an outbreak of multiple dermatofibromas in rhesus monkeys in Nigeria led to the conclusion that a viral cause was likely, but this has not been proved.

For this series, the recommended choices are: 4, d; 5, b; 6, a; 7, c.

W. Clark Lambert, M.D., Ph.D., Newark, NJ

REFERENCE

Lin RY, Landsman L, Krey PR, et al: Multiple dermatofi- bromas and systernie lupus erythematosus. Cutis 37:45- 49, 1986.

DISCUSSION OF QUESTIONS 8-10

Case presented by William Abramovits, MD, Caracas, Venezuela

Dermatofibrosarcoma protuberans is so named because of its marked tendency to form exophytic nodules that extend far above the skin surface. Initially, however, the lesions are depressed, not

37A

40A Self-Assessment examination answers Volume 15 Number 4, Part 1 October, 1986

protuberant, and, in a significant proportion of cases, may remain as depressed, fibrotic lesions. Lambert and others, have given this process the name dermatofibrosarcoma non-protuberans. It is important to recognize this variant, which, histo- logically and in all other respects, resembles other dermatofibrosarcomas, because the differential di- agnosis includes such entities as desmoplastic mel- anoma and because, left untreated or inadequately excised, it is very destructive.

Histologically, a small proportion of dermato- fibrosarcomas of both types show, in addition to the storiform pattern of large spindle cells, a small number of dark-staining dendritic cells that have the histochemical properties of melanocytes (Fig. 8). The origin of these cells is unknown. Such dermatofibrosarcomas are sometimes called Bed- nar tumors. This is only one of a number of benign or malignant processes that show dendritic cells of this type.

For this series, the recommended choices are: 8, d; 9, d; 10, e.

William Abramovits, M.D., and W. Clark Lambert, M.D.

Caracas, Venezuela, and Newark, NJ

REFERENCES Lambert WC, Abrarnovits W, Gonzalez-Serva A, et al: Der-

matofibrosarcoma non-protuberans. J Surg Oncol 28:7- 11, 1985.

Lambert MW, Lambert WC, Schwartz RA, et al: Colonization of nonmelanocytic cutaneous lesions by dendritic melano- cytic cells. J Surg Oncol 28:12-18, 1985.

DISCUSSION OF QUESTIONS 11-24

The type of skin suture and the duration that cutaneous sutures remain in place are important from both the healing and the cosmetic aspects. Surgical scars on the face heal rapidly; thus, a smaller suture should be used after adequate sub- cutaneous closure. In the facial area, most sutures can be removed on the third postoperative day, provided the wound edges are supported with ster- ile tape. Sutures in the eyelids should be removed in 2 to 3 days.

There are many types of suture material. They may be absorbable or nonabsorbable, monofila-

ment or braided, natural or synthetic, and clear or colored. As a general rule, tissue reaction to syn- thetic sutures such as nylon is less than that to silk or absorbable sutures. Catgut, both plain and chromic, is absorbed by macrophages; Dexon and Vicryl are absorbed by water hydrolysis in alkaline medium. Silk is slowly absorbed by foreign body reaction. Nylon, polyester fiber, and polypropyl- ene are all nonabsorbable. Dacron and Mersiline are both polyester fibers, the former being Teflon- coated. In a potentially or confirmed contaminated wound, monofilament sutures such as plain catgut should be used because it dissolves faster. Dexon is a polyglycolic acid that is a homopolyrner o f glycolide. Vicryl is polyglactin 910, a synthetic propolymer of lactide with glycolide. Both suture materials are absorbable and have minimal tissue reaction compared with catgut.

Reverse cutting precision point needles with swaged-on sutures appear to be the best for most cutaneous surgery. The reverse cutting point has two opposing cutting edges and a third cutting edge on the other curvature of the needle, with only the edges near the tip being sharp. This allows close approximation of wound edges with minimal trauma as the needle passes through skin.

For this series, the recommended choices are: 11, a; 12, c; 13, b, c, e; 14, b; 15, a; 16, b; 17, a; 18, b; 19, b; 20, b; 21, b; 22, b; 23, a; 24, e. Victor Mellul, M.D., andlra H. Gouterman, M.D.,

Newark, NJ

REFERENCES Aston S J: The choice of suture material for skin closure. J

Derrnatol Surg 2:57-61, 1976. Epstein E, Epstein E Jr: Skin surgery, ed. 5, Springfield, IL,

1977, Charles C Thomas, Publisher. Holt RG, Holt JE: Suture materials and techniques. Ear Nose

Throat J 60:12-28, 1981.

DISCUSSION OF QUESTIONS 25-31

The yellow nail syndrome consists of the triad of yellow nails, lymphatic abnormalities, and pul- monary disease. Hyperconvexity of the nail plate occurs, it becomes yellow to green in color, opaque, and thickened. The nails grow at a slower rate and may show transverse ridging. Bronchitis,

Volume 15 Number 4, Part 1 October, 1986

Self-Assessment examination answers 43A

bronchiectasis, pleural effusion, and sinusitis often accompany this nail dystrophy. Edema of the legs and face represents the third leg of the triangle. The nail changes often precede the other manifes- tations, although Milroy's hereditary lymphedema may be the first sign. Although thyroid abnor- malities have been reported, they are not regarded as a consistent component of this triad.

Candida infection of the nails may produce yel- lowish discoloration, but it is almost always as- sociated with paronychia and onycholysis and my- cologic studies would be positive. Nail biopsy pre- sents no diagnostic histologic picture.

Treatment of the nails in yellow nail syndrome is consistently ineffective. Although intralesional steroids and vitamins C and E have been reported to help, they are not reliable. Therefore, obser- vation is the best choice; 30% of cases resolve spontaneously.

Carotenemia may result in a yellow discolor- ation of the nail bed similar to its effects on the skin. The nail plate, however, is normal both in color and absence of dystrophy. Tetracycline may produce a photoonycholysis with a yellowish flu- orescence under Wood's light, but there is not a prominent gross yellowing of the nail plate itself. Nail enamel may stain the nail plate yellow but this is very unusual with present modern-day prod-

ucts. In any event, there would be no nail dystro- phy and associated medical findings would be un- likely to be seen.

Molded plastic workers coming in contact with 4,4-methylene dianiline developed yellow staining of the skin, nails, and hair. This is an important sign to watch for because this product is both hep- atotoxic and carcinogenic. Several patients with acquired immunodeficiency disease have been re- ported to have a yellow discoloration of the nails. It remains to be shown whether or not the patients have true yellow nail syndrome.

For this series, the recommended choices are: 25, c; 26, a, b; 27, e; 28, b, d; 29, a, b, c, d, e; 30, d; 31, b, d.

Richard K. Scher, M.D., Providence, R1

REFERENCES

Chernosky ME, Finley VK: Yellow nail syndrome in patients with acquired irnmunodeficiency disease. J AM ACAD DnaMA'rOL 13:731-736, 1985.

Cohen SH: Yellow staining caused by 4,4 methylenedianiline exposure. Arch Dermatol 121"1022-1027, 1985.

Pavlidakey GP, Hashimoto K, Blum D: Yellow nail syn- drome. 1 AM ACAD DERMATOL 11:509-512, 1984.

Samman PD, White WF: The yellow nail syndrome. Br J Dermatol 76:153-157, 1964.

Venencie PY, Dicken CH: Yellow nail syndrome: Report of five eases. J AM AcAo DEaMAXOL 10:187-192, 1984.

Zaias N: The nail of health and disease. Jamaica, NY, 1980, SP Medical & Scientific Books, pp. 219-222.