SPECIAL ARTICLE Pediatric Dermatology Vol. 9 No. 1 66-76 Proceedings of the 16th Annual Meeting of the Society for Pediatric Dermatoiogy WiUiamsburg, Virginia June 3a-July 3, 1991 Eleanor £. Sahn, M.D. Medical University of South Carolina Charleston, South Carolina A. Howiand Hartley, M.D. Children's Hospital National Medical Center Washington, D.C. Stephen Gellis, M.D. Children's Hospital Medical Center Boston, Massachusetts James E. Rasmussen, M.D. University of Michigan Medical Center Ann Arbor, Michigan Monday, July 1, 1991 ture by the newspaper account he received, dated December 17, 1799, telling of General George Dr. Alfred T. Lane (Stanford University) orga- Washington's death. We learn the story of General nized the sixteenth annual meeting of the Society Washington's rapid demise, probably from bacterial for Pediatric Dermatology, held in Wiliiamsburg, infection, hastened by the medical treatments of the Virgitiia. The seventh annual Sidney Hurwitz Lec- day, including frequent and copious blood letting. ture was delivered by Dr. Rona M. MacKie (Uni- There was a current saying, "more people died an- versity of Glasgow) on "Melanoma: Risk Factors in nually from lancets than from swords." Dysplastic Nevus Syndrome." President Anne Lucky (Cincinnati, Ohio) welcomed the society MELANOMA: RISK FACTORS AND members to Wiliiamsburg and introduced the first DYSPLASTIC NEVUS SYNDROME speaker. Dr. Rona MacKie first discussed risk factors in mel- anoma, citing several large case control studies car- COLONIAL MEDICINE ried out in western Canada, Scotland, Scandinavia, Dr. Tor A. Shwayder (Henry Ford Hospital) pre- and Germany. The frequency of melanoma has dou- sented a delightful and professional "Character In- bled each decade in Scandinavia, the United King- terpreter Portrayal of Iseiac Shwayder, Medical dom, and Germany. The frequency increases with Doctor (circa 1799)." Complete in eighteenth- high altitudes, low latitudes, pale skin, outdoor rec- century costume, including three-cornered hat and reation, and total number of banal, nondysplastic silk hose. Dr. Shwayder discussed the art and sci- nevi. Other risk factors are the tendency to freckle, ence of medicine as it evolved in colonial Willisuns- a history of severe sunburn, three or more clinically burg. However, he became distracted from his lec- atypical nevi, and fair hair. Factors that are associ- No reprints available. 66 Proceedings 67 ated with acquisition of large numbers of banal nevi nevus at the site. The melanomas that developed on include one or more years of childhood spent in the these nevi tended to be thick. Dr. MacKie recom- tropics, Mediterranean holidays when less than 5 mended a photographic record of dysptastic nevi in years old, light skin that bums rather than tatis, se- children, and removing those that developed char- vere sunburn in childhood, and freckling. The acteristics suggestive of melanoma. working hypothesis is that these early childhood events act as initiating factors that may induce be- HERPES SIMPLEX INFECTIONS IN CHILDREN nign meianocytic lesions. Later, further sun expo- Dr. J. Clark Huff (University of Colorado) dis- sure may lead to both promotion and progression, cussed the four types of herpes simplex virus (HSV) with the development of melanoma. infection in children: initial (primary) infection; re- An additional risk factor for the development of activation (recurrent) infection; infection in the im- melanoma is the presence of clinically atypical and munocompromised host; and herpes-associated er- pathologically dysplastic nevi (DN). There is much ythema multiforme (EM). confusion regarding the use of the term "dysplastic Initial or primary HSV infection is most com- nevus" because experts do not agree on pathologic motily subclinical or unrecognized. Older literature criteria. Elder's clinical criteria are size greater than stated that 95% of the United States population was 5 mm, irregular outline, "fried egg" profile, varie- seropositive, but current studies revised this figure gated pigmentation, and erythema. His pathologic to 52% to 65%. criteria are basilar meianocytic hyperplasia, cyto- Neonatal HSV infection occurs in about 1 in 14(X) logic meianocytic atypia, fusion of meianocytic live births—that is, 1500 to 2200 affected infants per nests, lymphocytic infiltrate, lamellar or concentric year in the United States. Seventy percent of the fibroplasia, and rete ridge elongation. cases are due to HSV-2. Risk factors for neonatal The four categories of DN according to the NIH HSV infection are thought to include asymptomatic Consensus Conference are (A) sporadic; (B) famil- shedding from the maternal genital tract, especially ial nevi with no melanoma; (C) sporadic personal the cervix, at the time of delivery; recently acquired melanoma (not in the fEunily, patient only); (D,) fa- subclinical first episode infection; reactivation milial nevi with personal melanoma and one family herpes with shedding (although risk is only about member with melanoma, and (D2) familial nevi with 3% in these infants); and the absence of maternal personal melanoma and two family members with antibodies to HSV-2. Neonatal HSV localized to melanoma. The increased risk of melanoma de- the skin, eye, or mucous membrane is associated pending on the type of dysplastic nevus is as fol- with no mortality. When encephalitis is present, lows: type A, 4-fold increase; type B, 7-fold in- mortality is 15%, and of those who survive, 64% crease; type C, 80-fold increase; type D, 108-fold have severe sequelae, usually mental retardation. If increase. It was noted that melanoma was ex- the infection is disseminated but no encephalitis is tremely rare prepubertally, but in the few patients present, mortality is 57% and morbidity at one year in whom it has occurred, 50% developed in giant is 41%. Treatment is acyclovir 30 mg/kg/day intra- meianocytic nevi. venously for 10 days. In Dr. MacKie's current study of melanomas in An HSV infection in the immunocompromised individtials under age 30 years, a significant associ- host is often extremely severe. Treatment is intra- ation exists between the presence of a nevus since venous acyclovir 30 mg/kg/day for 10 days. early life and the development of melanoma at that The HSV-associated EM is usually a minor form site. This suggests that a portion of congenital or of EM, and follows recurrent HSV by 3 to 21 days. early-onset small nevi have the potential for malig- The polymerase chain reaction (PCR) has been used nant change. Continuing studies wiU attempt to es- to identify herpes virus DNA in skin lesions of EM. tabhsh the rate at which this occurs and guidehnes In HSV-associated EM, 100% of lesions had HSV for the management of small congenital nevi. Be- DNA. Fifty percent of patients with idiopathic EM cause these melanomas do not seem to develop had evidence of HSV DNA in lesions. Prophylaxis prior to puberty—approximately 1 chance in 100 ex- includes oral acyciovir 400 to 800 mg/day. ists that a smdl congenital nevus will become ma- The diagnosis of HSV can be made on culture, lignant by 13 years of age—it is reasonable to offer which is usually positive in one to two days. A new excision of small congenital nevi at puberty. Of 95 technique involves culturing for 24 to 48 hours and patients who developed melanoma at age 30 or less, then fluorescent antibody staining. Tzanck prepara- 44% had evidence of a preexisting, small congenital tion is reliable in new vesicles. Immunocytology, 68 Pediatric Dermatology Vol. 9 No. 1 March 1992 enzyme-linked immunosorbent assay of blister nychia, angular stomatitis, glossitis, and alopecia fluid, and spin amplification techniques are avail- often are present. Impaired wound healing, growth able at some institutions. retardation, marked irritability, diarrhea, and im- mune abnormalities may be seen. NUTRITIONAL DEFICIENCY COAGULATION DISORDERS Dr. Moise L. Levy (Baylor College) discussed the cutaneous signs of four types of nutritional defi- Dr. Hazel Vemon (Medical College of Virginia) dis- ciency: protein-energy malnutrition, vitamin defi- cussed three coagulation disorders with manifesta- ciencies, essential fatty acid deficiency, and trace tions in the skin: protein C deficiency, purpura ful- element deficiency. Protein-energy malnutrition is minans, and the Kasabach-Merritt syndrome. the most common worldwide and is recognized clin- Homozygous protein C deficiency is extremely ically as kwashiorkor or marasmus. Either can fol- rare, with only 17 confirmed cases in the United low poor dietary intake, maiabsorption, or catabolic States and Europe. Protein C activity is usually less state. than 1%, with protein C antigen ranging from less The skin in kwashiorkor shows hypopigmenta- than 1% to 30%. The three major clinical findings in tion and diffuse pitting edema. Waxy elevated these newboms are the onset of ecchymoses and plaques ("enamel paint") or erosions ("flaky necrotic lesions within 2 to 12 hours of birth, central paint") are often seen. In marasmus the skin is dry, nervous system thromboses, and blindness. Hema- wrinkled, and loose, often producing "monkey tologic studies show a picture of disseminated intra- facies." The hair is reddish and thin in kwashior- vascular coagulation with decreased platelets and kor, and alternating periods of adequate and inade- fibrinogens, elevated fibrin split products, and pro- quate nutrition are reflected in alternate bands of longed activated partial thromboplastin and pro- color in the hair, the so-called flag sign. In maras- thrombin times. PVotein C levels are low or absent. mus the hair is also thin and slow growing, and Histologic examination of a skin biopsy specimen lanugo hair may be seen. reveals extensive thrombosis with fibrinoid necro- Deficiencies of vitamins A, B, and C also pro- sis of vessel walls and hemorrhage into subcutane- duce skin signs. Vitamin A deficiency produces foi- ous fat, but without vasculitis. Treatment includes hcular papules, so-called phrynoderma. Xerosis is fresh-frozen plasma given daily, or protein C and S prominent, as are ophthalmologic abnormalities in- concentrates.