SPECIAL SECTION

Pediatric Dennatology Vol. 6 No. 4 336-343

Proceedings of the 14th Annual Meeting of the Society for Pediatric Dermatology Quebec City, Quebec, Canada June 22-24, 1989

Sharon S. Raimer, M.D.,*= NeU S. Prose, M.D.,t Adelaide A. Hebert, M.D.,t and James E. Rasmussen, M.D.§

*University of Texas Medical Branch at Gaiveston, f State University of New York Health Science Center at Buffalo, tUniversity of Texas Health Science Center at Houston, and §University of Michigan, Ann Arbor, Michigan The fourteenth annual meeting of the Society for commonly in children 2 to 6 years of age, is charac- Pediatric Dennatology was held in Quebec City, terized by bony defects, diabetes insipidus, exoph- Quebec, Canada and hosted by Drs. Beraice thalmos, and skin lesions (present in 30% to 50% of Krafchik, Lionel Boxall, and the dennatology staff patients). Skin lesions are frequently similar to of L'Hotel-Dieu de Quebec, particularly Drs. Ray- those seen in Letterer-Siwe disease but occasion- mond Lessard and Richard Cloutier. The fifth an- ally also involve mucous membranes. nual Sidney Hurwitz Lecture was presented by Pro- Skin lesions are rare in eosinophilic granuioma, fessor Ruggero Caputo of the University of Milan. the benign localized form of the disease. When He discussed the histiocytoses, which he classified present, they may involve the periorificial region by their clinical, histopathological, ultrastructural, and mucous membranes. The lesions of congenital and immunohistochemical characteristics. self-healing reticulohistiocytosis appear at birth or Histiocytosis X is a proliferation of unknown shortly thereafter as large reddish brown nodules cause of a distinct histiocytic cell type containing which generally begin to involute after approxi- Langerhans' granules in the cytoplasm and staining mately 1 month. positively for S-100 and OK-T6. The disease can be Approximately 50% of the histiocytes in all four divided into four clinical subtypes: Letterer-Siwe clinicEil forms of histiocytosis X contain Langer- disease, Hand-SchuDer-Christian disease, eosino- hans' granules. These granules differ from those philic granuioma, and congenital self-healing reticu- found in Langerhans' cells in that they may be lohistiocytosis. Letterer-Siwe is the acute dissemi- longer, are frequently attached to plasma mem- nated form of histiocytosis X. Cutaneous lesions branes, may appeetr clumped, and may be present in are present in approximately 80% of cases, particu- nuclei as a result of abnormal mitosis. larly on the trunk and scalp, and these lesions are The non-X histiocj^oses lack Langerhans' gran- frequently the presenting sign of the disease. Le- ules and include juvenile xanthogranuloma, gen- sions may present as papules (often with scaling, eralized eruptive histiocytoma, benign cephalic purpura, or crusts), pustules, petechiae, and purpu- histiocytosis, sinus histiocytosis with massive ric striae of the nail beds. lymphadenopathy, xanthoma disseminatum, papu- Hand-SchuUer-Christian disease, seen most lar xanthoma, and reticulohistiocytosis.

Address correspondence to James E. Rasmussen, M.D., De- partment of Dermatolt^, University of Michigan Medical Cen- ter, 1910 Taubman Health Care Center, Ann Arbor, MI 48109- 0314.

336 Raimer et al: 14th Annual Meeting 337

Juvenile xanthogranuloma in the small nodular polyarthritis have been reported in children. The (papular) form may present with 2 to 5 mm irregu- skin lesions associated with the disease are treuislu- larly scattered papules occasionally associated with cent yellow or yellow-brown papulonodules ranging caf£ au lait spots. In the less frequent large nodular in size from a few millimeters to two centimeters form, which may involve mucous membranes and that occur most commonly on the hands and occa- occasionally internal organs, it may present with sionally on the knees and fcu:e. Nasal and pharyn- one or a few 10 to 20 mm nodules. All lesions re- geal mucosal lesions may occur in approximately solve within 3 to 6 years. The most common extra- 50% of cases. cutaneous manifestation of the small nodular form The next presentation was given by William is ocular involvement, which may precede or follow Macrae, M.D., of the University of Toronto, who cutaneous involvement. Only six cases of general- discussed diseases in which changes occur in both ized eruptive histiocytoma, a condition in which the skin and eye. Fifty percent of childhood blind- hundreds of 3 to 10 mm reddish to bluish papules ness is due to inherited conditions and ten percent appear in successive crops, have been reported in of visually-impaired children have oculocutaneous children. No visceral lesions have been described. albinism as the basis of impairment. Albinism has The condition eventually resolves spontaneously. several different genetic varieties: recessively in- Benign cephalic histiocytosis develops during the herited tyrosinase-positive or tyrosinase-negative first 3 years of life. Papular or maculopapular 2 to 3 ocuiocutaneous albinism affecting hair, skin, and mm yellow-brown lesions develop principally in the eyes; or ocular albinism affecting eyes only, classi- head and neck region, particularly on the posterior cally X-linked in inheritance, but occasionally in- side, and regress spontaneously during childhood herited as an autosomal recessive condition. Albi- without scarring. Mucous membranes and viscera nos demonstrate visual loss, photosensitivity, and are uninvolved. Approximately 20 cases have been nystagmus. The visual loss is due to central retinal described. hypoplasia, resulting in reduced central vision but Sinus histiocytosis is associated with massive normal visual fields. Optic fibers from the temporal lymphadenopathy, fever, anemia, leukocytosis, el- retina to the ipsilateral geniculate nucleus are mis- evated erythrocyte sedimentation rate, and poly- routed so that binocular vision is severely impaired. clonal hypergammaglobuiinemia. Approximately Tyrosinase-negative individuals generally have 10% of patients develop red-brown or yellow-brown greater visual impairment. Dr. Macrae stressed that papulonodular lesions without any particular distri- the hair bulb incubation test for the presence of ty- bution. Spontaneous resolution generally occurs. rosinase is not reliable under 1 to 2 years of age. Xanthoma disseminatum, a benign histiocytosis Lisch nodules may be helpful in the diagnosis of very rarely seen in children, presents with red- neurofibromatosis. They are elevated, smooth, brown papules involving the face, trunk, and prox- translucent lesions that are present bilaterally on imal extremities. The papules tend to become con- the iris in 90% of neurofibromatosis patients over 6 fluent on flexural surfaces and in the perioral years of age. region. The eyes and mucous membranes are gen- Infants with Sturge-Weber syndrome should be erally involved and the disease is associated with examined for the presence of congenita] glaucoma diabetes insipidus. Both the skin iesions and the di- due to choroideil hemangioma, and patients with a abetes insipidus resolve after several years. nevus of Ota should be followed closely because of Dr. Caputo has recently described papular xan- an approximate 4% incidence of melanoma in these thoma (previously reported only in adults) in two nevi. children. Yellowish papulonodular lesions without Patients with atopic dermatitis frequently have visceral involvement are clinically very similar to an associated keratoconjunctivitis, which can be juvenile xanthogranuloma; histology is necessary treated with cold compresses, antihistamines and for differentiation. Spontaneous resolution has been the judicious use of topical steroids. Eight percent observed only in children. of atopies develop cataracts. Reticulohistiocytosis is a form of histiocytosis Thirty-five percent of patients with Stevens- very rarely seen in children. A purely cutaneous Johnson syndrome have permanent sequelae be- solitary papulonodular form involving mainly the cause of scarring. Treatments during the etcute head and neck, and a diffuse widespread form not phase of the disease include topical steroids, lubri- associated with arthritis may occur. Only two cases cants, and bandage contact lenses to maintain the of multicentric reticulohistiocytosis associated with integrity of the cornea. 338 Pediatric Dennatology Vol. 6 No. 4 December 1989

Herpes simplex of the cornea is the most com- mon porphyria of childhood and features symptom- mon infectious cause of blindness. Keratitis is the atic mild photosensitization. When present, skin most important manifestation; ulceration caused by changes consist of thickening on the nose and dor- the virus can be seen on fluorescein staining. Dr. sum of the hands with mild scarring of the cheeks. Macrae prefers topical tdfluridine (Viroptic) and Red blood cells (RBC), which contain high levels of oral acyclovir as treatment. Chronic conjunctivitis protoporphyrin, fluoresce briUiantly for 20 to 30 may be due to herpes simplex; steroid-containing seconds under a fluorescent microscope. Plasma eyedrops should be avoided in patients with chronic protoporphyrin levels are elevated due to loose conjunctivitis unless herpes simplex infection has binding of protoporphyrins to RBCs. Urine and stool been ruled out. porphyrins are normal. Iron deficiency anemia and Eye involvement may occur with Lyme disease. lead intoxication also result in elevated RBC proto- In stage I of the disease, conjunctivitis may occur porphyrin levels, although such patients are not and, rarely, a panophthalmitis resulting in blind- photosensitive because the protoporphyrins are ness. In stage III of the disease, keratitis and uveitis tightly bound to RBCs. Iron deficiency anemia in have been described, as well as cranial nerve palsy. association with a polymorphic Ught eruption could Large strawberry hemangiomas of the eyelid be misdiagnosed as EPP due to an increase in red may cause loss of vision due to occlusive ambly- cell porphyrins associated with photosensitivity. opia, strabismic amblyopia, or compression of the Cirrhosis of the liver occurs in a small number of globe. Intervention with such modalities as argon patients with EPP. laser, excision, or intralesional steroids must be at- The hepatic porphyrias are acute intermittent tempted without delay to prevent permanent visual porphyria, variegate porphyria, hereditary copro- loss. piorphyria, and porphyria cutanea tarda. Variegate Hans-Michael Dosch, M.D., from the University porphyria (VP) and hereditary coproporphyria of Toronto presented his recent studies on the for- (HCP) may present with acute systemic symptoms mation of the IgE molecule. The genome for immu- identical to those of acute intermittent porphyria, noglobulins consists of approximately 200 kilobases with skin findings which are identical to those of beginning with those coding for IgM then, sequen- porphyria cutanea tarda (PCT), or a combination of tially, IgD, IgG, IgA, and lastly, IgE. It is presently both. Investigation of porphyrin levels in VP dem- accepted that individual B cells begin as IgM secre- onstrates coproporphyrin levels greater than uro- tors then subsequently become IgD, IgG, and IgA porphyrin in urine and protoporphyrin greater than secretors. Finally, a few cells reach the "end of the coproporphyrin in stool. Porphyrin levels are nor- line" and become IgE secretors. mal in red blood cells. Hereditary coproporphyria Dr. Dosch's laboratory noted that lymphocytes aiso shows levels of coproporphyrin higher than with IgE on their plasma membranes also had the uroporphyrin in urine, but stool shows much higher IgM molecule. When IgM-secreting cells were iso- levels of coproporphyrin than protoporphyrin. Dur- lated from atopies, all cells that made IgE were also ing acute episodes of either disease, the urine may found in this group of cells. He believes that ap- also contain aminolevulinic acid and porphobilino- proximately 90% of B lymphocytes progress from gen. Porphyria cutanea tarda, which is quite rare in making IgM to making IgA or IgG, while approxi- childhood, should only be diagnosed when VP and mately 10% continue to make IgM and also make HCP have been ruled out thoroughly. All available IgD and IgE. Further studies on the regulation of family members of patients with VP or HCP should this system are needed. be screened. Diagnosing asymptomatic patients al- Colin Ramsay, M.D., from the University of To- lows education about inciting drugs, thus prevent- ronto discussed porphyria in children. The porphy- ing serious acute episodes of these diseases. rias can be divided into erythropoietic and hepatic Lynn From, M.D., University of Toronto, pre- types. The former group includes erythropoietic sented a discussion on nevi in children. She began porphyria (Gunter's disease), erythropoietic proto- by discussing her view that large congenital nevi porphyria (EPP), and erythropoietic copropor- should not be removed as a cancer-preventing mea- phyria. Gunter's disease presents in early childhood sure. She noted that death from melanoma in chil- with marked hypertrichosis, mutilating skin dren under 10 years of age is extremely rare in the changes, splenomegaly, and a marked hemolytic United States and no such death has been reported anemia that is frequently the cause of death. All tis- in Ontario in the last 30 years. It is well recognized, sues will fluoresce under a Wood's light. however, that deaths do occur from melanoma in Erythropoietic protoporphyria is the most com- association with large congenital nevi. Some of Raimer et al: 14th Annual Meeting 339 these melatiomas actually arise in the central ner- edge of the lesion. Recent studies suggest that the vous system, which frequently contains increased more pigmented nevi one has, dysplastic or not, the numbers of melanocytes in association with large greater the chance of developing melanoma. The congenital nevi. Because large congenital nevi usu- number of pigmented nevi may be related to the ally extend deeply into subcutaneous tissue, it is number of sunburns an individual has had. frequently impossible to remove all the nevus cells. The "Cases of the Year" session was moderated Deaths have occurred from melanomas arising un- by Sam Weinberg, M.D. Susan Mallory, M.D., pre- der skin grafts after extensive surgery to remove the sented the first case, a 4-year-old white girl with lesions. dermatomyositis in whom marked hypertrichosis It has been noted in recent years that patients was the presenting clinical sign of the disease. with a diagnosis of melanoma in a large congenital Amy Paller, M.D., discussed a 5-year-old girl nevus frequently survive long-term. Dr. From who developed left leg pain and edema followed by noted that these nevi may develop nodules that con- vasculitic changes with palpable purpura and necro- tain areas of necrosis and hemorrhage and numer- sis. Computerized tomographic scans demonstrated ous mitotic cells that may be very difficult to distin- thrombosis in the iliac and femoral veins. Direct guish from melanoma. She feels that many of these immunofluorescent studies of a skin biopsy showed are due to trauma and have been misdiagnosed in granular deposits of IgG, IgM, and C3 around der- the past as melanoma. Approximately 60% of large mal blood vessels. An extensive immunologic congenital nevi have many mitotic figures in the workup was essentially normal except for the pres- deep dermis. She noted that worrisome nodules that ence of anticardiolipin antibodies. are present at birth frequently regress. Spitz nevi Antiphospholipid antibodies, which include anti- also can occur within congenital nevi and may be cardiolipin antibodies, and lupus anticoagulant are misdiagnosed as melanoma. Parents or patients frequently present in association with collagen vas- may desire removal of congenital nevi for cosmetic cular diseases, particularly systemic lupus erythe- reasons, but there appears to be no urgency in the matosus and juvenile rheumatoid arthritis, but occa- removal and one may not be preventing the occur- sionally are seen in normal individuals. In adults rence of melanoma by such removal. these antibodies may be associated with recurrent In discussing small congenital nevi. Dr. From thrombotic episodes, fetal loss from placental in- noted that in a 2-year study in Ontario approxi- farcts, thrombocytic anemia. Coombs' and hemo- mately 1.9% of melanomas had evidence of the clas- lytic anemia, livedo reticularis, vasculitis, and sic histology of a congenital nevus associated with rjirely migraine headaches and seizures. This syn- the melanoma. She manages congenital nevi by giv- drome has not been reported in children. ing parents a low-power photograph of the nevus Maureen Rogers, M.D., described the case of a for positional orientation and a close-up so that par- 4-month-old boy with a viral pneumonitis managed ents can look for changes in shape or color. She re- under a croup tent who developed vesicles over the moves any nevus that has changed. sites of venipuncture. The vesicles rapidly enlarged The speaker mentioned that she had recently into buUae. Histology was consistent with miliaria, seen two cases in which malignant melanoma devel- showing hyperkeratosis, very marked dilated sweat oped in nevus spilus-like lesions. The dark areas in ducts with extravasation of sweat into the dermis, this type of lesion frequently have the histologic and a mild inflammatory infiltrate. No typical nul- changes of dysplastic nevi. Spindle cell nevi typi- iaria rubra was present. Although the lesions re- cally arise rather quickly during childhood. Histo- solved spontaneously, two small lesions recurred logic changes occur mostly in the epidermis. Nu- with a subsequent febrile illness. Ron Hansen, merous mitotic figures are frequently present, and M.D., reported having seen a similar eruption in a the lesion may be misdiagnosed as a malignant mel- terminally ill young infant. anoma. Spitz nevi, which typically have more der- Patricia Treadwell, M.D., presented an 8-year- mal involvement than spindle cell nevi, occasion- old girl with a lesion on the helix of the ear. The ally may be very difficult to distinguish from patient's mother had a similar sore on the lower leg. melanoma histologically, and clinical judgment may Sporothrix schenkii was isolated from both patients. be of greater value than histopathology in children. Sporotrichosis in more than one family member has In dysplastic nevi, the atypical cells are present been reported, but is a rare occurrence. principally at the edges of the lesion. Generally the Daniel Hogan, M.D., discussed the case of a entire nevus should be removed for biopsy, but if a healthy 7-year-old girl who presented with a two smaller biopsy is done, it should be taken from the week history of an enlarging nodule of the wrist that 340 Pediatric Dermatology Vol. 6 No. 4 December 1989 progressed to ulceration. Routine histology showed tion following their second exposure to the antibi- a granulomatous infUtrate with giant cells. Numer- otic cefaclor (Ceclor). ous acid-fast bacilli were demonstrated with special On Friday morning, 23 June 1989, a series of clin- stains. A mycobacterial infection was diagnosed ical cases was presented to the meeting participants and the child was treated for 2 months with INH at L'H6tel-Dieu de Quebec, the oldest hospital in and Rifampin. Cultures subsequently grew Rhodo- North America. This remarkable program, orga- coccus terrae, a partially acid-fast, fairly ubiquitous nized by Drs. Raymond Lessard and Richard sofl organism. Treatment was discontinued and the Cloutier, included 20 patients from throughout Que- lesion healed spontaneously. bec and Ontario, and eight poster presentations. Andrea Dominey, M.D., presented an infant with The discussion that followed the patient viewing a targe congenital tumor of the heeul and neck, was led by Dr. Cloutier. which proved to be a rhabdoid sarcoma on histo- Dr. Danielle Marcoux of Hopital Ste-Justine in logic examination. Cutaneous metastases to the Montreal presented a 6-year-old boy who devel- thigh and back were present at birth. This rare tu- oped a large area of atrophy on the right buttock mor has a rapidly fatal course. The mean age of on- and three smaller areas on the posterior aspect of set in children is 18 months and the usual primary the leg. A biopsy of the lesion showed atrophy of location in this age group is the kidney. In adults, the subcutis with a perivascular iymphohistiocytic this sarcoma may present as a subcutaneous tumor infiltrate. Conference participants agreed with the of the extremities or the oral cavity. The histologic suggested diagnosis of lipodystrophy centrifugalis, hallmark of a rhabdoid sarcoma is the presence of and most felt that the prognosis for spontaneous eosinophilic cytoplasmic inclusions that are PAS resolution was quite good. positive, diastase resistant, and stain immunohisto- Dr. Marcoux also presented an 8-year-old girl chemicatly for vimentin. with erythrokeratoderma variabilis. The patient had Linda Rabinowitz, M.D., described two infants widespread erythematous, hyperkeratotic plaques with capillary hemangiomas treated initially at 4 involving the trunk and extremities. Cutaneous in- days and 8 days of age with the 585 nM pulse dye volvement ended sharply at the base of the neck, laser. The lesions initially became gray in appear- and there was a bizarre, spiralled gyrate erythema ance, then purpuric, and faded over 1 to 2 weeks. superimposed upon the areas of hyperkeratosis. The 585 nM pulse is within the absorption spectrum Treatment with intermittent courses of oral 13-cis of oxyhemoglobin. The energy absorbed by oxyhe- retinoic acid and topical keratolytics has been well moglobin presumably damages endothelial cells, re- tolerated, but the skin disease remains severe and sulting in vessel closure, generally without scarring. extensive. Steven Resnick, M.D., reported a child who de- A 12-year-old girl with severe lamellar ichthyosis veloped a Bechet's-Uke syndrome with high fevers, who had collodion features at birth was presented uveitis, retinal vasculitis, facial pustules, and an er- by Dr. Bertrand Rodrigue of Hospital St-Sacre- ythema nodosum-Uke eruption on the legs shortly ment, Quebec. He reported significant therapeutic after having a nickel-containing orthodontic appli- success with 5% glycolic acid in water washable ance placed in her mouth. The appliance was re- base. Dr. Bernice Krafchik reported that collodion moved and the child has responded to a slow taper babies respond to therapy with the visible light that of systemic steroids. is used in the treatment of hyperbilinibinemia. Anne Lucky, M.D., presented an infant with Dr. L. J. Margesson of Kingston, Ontario, pre- congenital ichthyosiform erythroderma who was sented a 32-month-old male who was bom as a har- hospitetlized three times before 3 months of age for lequin fetus. Treatment with oral etretinate began at severe failure to thrive. Oxygen consumption stud- 3 days of life. The child has persistent, diffuse ery- ies done at 3 months of age indicated that the infant thema and scaling and is still on etretinate therapy. had an increased energy expenditure and that his Long-term survival of a harlequin fetus remains an caloric requirement was almost twice that of a nor- extremely unusual phenomenon. mal infant. With adequate caloric intake the infant Dr. Bernice Krafchik's case was a 5-year-old boy gained weight and the skin became less erythroder- with recurrent asymptomatic reddish papules witji mic. ulceration and crusting since age 4 months. Re- The last cases of the session were presented by peated biopsies of these skin lesions led to the diag- Carl Frix, M.D. He reported several children who nosis of lymphomatoid papulosis. The occurrence developed urticaria and a serum sickness-4ike erup- of this disorder is extremely rare during childhood, Raimer et al: 14th Annual Meeting 341 and histologic confirmation will often require serial restrictive dermopathy (J Invest Dennatol 1987; biopsies. 88:330-339); 2) Pena-Shokeir phenotype (J Pediatr Dr. Jacques Soucy presented a 6-month-old girl 1974;85:373-375); and 3) sclerema from in-utero with a cavernous hemangioma involving the face hypoxia (Am J Obstet Gynecol 1986;155:38O-381). and neck. Dr. Anne Lucky noted that me^netic res- Dr. Andrew Margileth h£ul seen similar cases that onance imaging may be useful in determining the he felt resulted from congenital cytomegalovirus in- degree of anatomical involvement in the neck re- fection and congenital varicella. No conclusive di- gion, and may indicate when steroid therapy is agnosis was reached, however. needed. The second case was from Dr. Weston's depart- Dr. Julie Powell and Dr. A. Bolduc presented a ment in Denver. A 6V2-year-old boy had been in 7-year-old boy with a progressively enlarging good health until January 1989, when he developed asymptomatic lesion on the left thigh since age 2 streptococcal pharyngitis and 2 weeks of hematuria. years. The lesion was an indurated, violaceous After the resolution of the hematuria, he developed plaque with focal areas of epidermal atrophy. After recurrent episodes of fever up to 103° Fsdu-enheit exhaustive laboratory investigation, the discovery for 3-day intervals. He had an episode of urticadal of budding spores in giant cells of the skin and rash on the distal extremities and was thought to lymph nodes suggested the diagnosis of North have juvenile rheumatoid arthritis. Therapy with as- American blastomycosis. Dr. Ilona Frieden pointed pirin resulted in only slight improvement. out that granulomatous slack skin disease may have Dermatologic evaluation was sought when the a similar clinical appearance and suggested that it child developed new, bullous skin lesions that be- be considered as an alternative diagnosis. gan on the dorsal feet, spreading to other parts of A 15-year-old girl with extensive porokeratosis the body. The bullae were often hemorrhagic. The of Mibelli was described by Dr. Richard Cloutier. child was on enteric coated aspirin when the erup- The patient also had a thick area of hyperkeratosis tion occurred. A skin biopsy showed a subepider- involving the left heel that interfered with walking. ma! split with no vasculitis. Direct and indirect im- The lesion has responded well to laser therapy. munofiuorescence was negative on two occasions. Dr. Danielle Marcoux presented a 16-year-old The sedimentation rate, which had been 70 mm/hr, girl for diagnosis of a progressive dermatosis that was 7 mm/hr at the time the acute bullous eruption had been present since the first year of life. The pa- began. A normochromic anemia and a slightly ele- tient had developed linear atrophic bands of the vated eosinophil count were reported. The antinu- posterior lower extremities, with similar lesions on clear antibody titer was negative, and the antistrep- the breasts and in the axillae and inguinal ar- tolysin titer was not elevated. Dr. Lynn Roberts eas. Conference participants favored the diagnosis recommended that indirect immunofluorescence be of focal dermal hypoplasia. Several discussants done on sodium chloride split skin. The differential pointed out that the biopsy of this disorder may be diagnosis included linear IgA dermatosis, bullous difficult to interpret if obtained from an early lesion. drug eruption, bullous pemphigoid, lupus erythema- Histologic comparison with an area of normal skin tosus, and Henoch-Schonlein purpura. Therapy had may sometimes be helpful. included 500 mg oral sulfapyridine twice daily for 12 The Saturday session was moderated by Walter days, which resulted in nausea, vomiting and diar- Tunnessen Jr., M.D., and opened with Dr. William rhea. Steroids had not been used in therapy. Weston's "Cases in Search of a Diagnosis." The The second speaker w^ Carol Redmond, M.D., first case was that of a 36-week-old female infant from the Department of Obstetrics and Gynecology with a diffuse, "woody" nonpitting induration of at the Hospital for Sick Children in Toronto, who the skin, arthrogryposis, and hypoplastic lungs. discussed lichen sclerosus et atrophicus (LSA) in Thick skin was noted by ultrasound done at 26 children. Dr. Redmond supports the view that LSA weeks for decreased fetal movement. Presenters in girls is similar to the form seen in adult women in Moise Levy, M.D., and Andrea Dominey, M.D., of its symptomatology, appearance and histologic Houston, Texas, described sclerematous indiuation changes. The two forms differ, however, in their of the skin at birth. At autopsy, the infant had "po- natural history. LSA may not be rare in children, liceman's tip" positioning of the arms and fatty hy- and most often Caucasian females are afflicted with poplasia of the muscles. Biochemical studies were this disorder. The earliest recognized case occurred unremarkable and viral cultures were negative. In at 1 month of age with a peak incidence at 3 to 6 his differential diagnosis, Dr. Weston included 1) years. 342 Pediatric Dermatology Vol. 6 No. 4 December 1989

Symptoms may be intennittent, with episodes of childhood with less than 2% of all cases seen in pa- vulvar pruritus lasting I to 2 weeks. Anal fissures tients under 20 years of age. If the LP is familial it may develop and predispose to constipation. Clini- may occur at a younger age. Although oral mucous cally apparent genital lesions tend to be symmetric membrane lesions are rare in childhood lichen pla- and can involve the vulva (primarily the labia ma- nus, the nails may be involved (20 nail dystrophy). jora), the clitoris, or the vestibule. The skin cheinges Characterized by asymptomatic 2 to 3 mm pap- can include leukoderma, erythema, telangiectases, ules, lichen nitidus is easily differentiated from LP hemorrhagic buliae, and fissures. The alterations based on clinical and histologic features. The hair, may wax and wane and, on rare occasions, predis- nails, face and oral mucous membranes are typi- pose to gross contractures of the vaginal introitus. cally spared. Males are affected four times as often Rraurosis vulvae is an exceedingly rare sequelae of as females. LSA in girls. Friedrich {Friedrich G, Jr. Vulvar Dis- Greater than 50% of all cases of lichen striatus ease, 2nd ed. Philadelphia: WB Saunders, 1983) de- occur in children. Onset is usually between the eiges scribes a phenomenon known as agglutination of 2 and 15 years. More than one linear band may be where there is loss of the labia minora due to adhe- seen distributed along the lines of Blaschko. sions from LSA. Papular acrodermatitis of childhood (PAC) was The differential diagnosis of LSA presented by distinguished from papulovesicular acrolocated syn- Redmond included 1) monilia (although the absence drome (PAS) (J Am Acad Dermatoi 1982 ;6:862-866). of glycogen in prepubertal girls makes this diagnosis In Europe, the hepatitis virus subtype ayw is the unlikely), 2) nonspecific vulvovaginitis, 3) herpes form most frequently associated with PAC, and vulvitis, 4) sexual abuse, 5) vitiiigo, and 6) mastur- children with HLA Bw 54 are significantly more bation. likely to develop the disease. The oldest patient to de- Does LSA spontaneously resolve in young girls? velop PAC in Dr. Caputo's series was 12 years of age. Dr. Redmond found that 50% to 60% of 72 girls Pityriasis lichenoides was divided into three studied previously got better symptomatically. The groups (Table 1) based on distribution and duration recovery period averaged 5 years with a range of 1 of the eruption. No relationship existed between the to 10 years. In data collected at the Hospital for age of onset and the ultimate course of the disease. Sick Children LSA Clinic in Toronto, 42 of 55 pa- Similarly, no relationship has been recognized be- tients (76%) got better symptomatically (i.e., still tween topographical distribution and the severity of had itching, but less often) with an average recov- the disease. None of Dr. Caputo's cases went on to ery period of 3 years (range 0.2 to 7 years). Only develop lymphomatoid papulosis. 23% had resolution of their cutaneous changes. Re- Seveso, Italy was the site of a large TCDD spill lapses have occurred even after 4 years of being on 10 July 1976. The chemical spread through the symptom free. Involution of prepubertal anogenital air to the surrounding 16 kilometers. Both acute and LSA occurred in two-thirds of cases before or delayed onset of cutaneous reactions to the chemi- around menarche. In Dr. Redmond's study, 75% cal were illustrated by Dr. Caputo. The lesions felt had symptomatic improvement unrelated to pu- to be due to direct toxic exposure were noted within berty and 60% were still symptomatic at puberty. 24 to 48 hours. Erythema and edema of exposed ar- No known predictive factors exist that determine eas of skin, vesiculobuUous and necrotic papules of which patients will have resolution of their LSA at the palms and soles, and papulonodules on the arms menarche. Therapeutic modalities include topical and chest developed acutely. One to two months af- steroids and topical hormonal agents. Topical ste- ter the chemical spill, cases of chloracne occurred, roids are the mainstay of therapy in girls with LSA believed due to both direct exposure of the skin to and do seem to help control the itching. TCDD and ingestion of contaminated foods. Le- In his second address. Professor Ruggero Caputo sions of chloracne resolved slowly, leaving pitted presented an overview of papular eruptions in chil- scars. dren and briefly described the cutaneous manifesta- tions that followed the 1976 tetrachlorodibenzo- dioxin (TCDD) spill in Seveso, Italy. The lichenoid TABLE 1. Pityriasis Lichenoides in Children eruptions encompass lichen planus, lichen nitidus, Duration lichen striatus, papular acrodermatitis of childhood, Form Location (months) papulovesicular acrolocated syndrome, and pityria- Diffuse Face, trunk, limbs 11.4 sis lichenoides. Central Localized on trunk 17.3 Peripheral Lichen planus (LP) occurs rarely in infancy and Limbs, face 31.3 Raimer et al: 14th Annual Meeting 343

Judah Folkman, M.D., introduced many emerg- The pharmacologic diagnosis of drug-induced ing concepts with regard to angiogenesis (N Engl J skin disease was presented by Stephen Spielberg, Med 1989;320:12! 1-1212). He described the six an- M.D. Drugs may induce multisystem disease with giogenic polypeptides now recognized: fibroblast fever and rash. In any given individual, a drug erup- growth factor, angiogenin, transforming growth fac- tion may develop as an expression of a hypersensi- tors alpha and beta, tumor necrosis factor alpha, tivity reaction. These reactions may be related to and platelet-derived endothelial cell growth factor. the innate ability to acetylate a drug or to biochem- Theoretically, if we could control angiogenesis, we ical abnormalities. Currently, studies are underway could gain control of a number of diseases, such as to define the pharmacogenetic basis of drug reac- hemangiomas and psoriasis. An alternative hypoth- tions. The Pandix plate with fluorescent probes esis is that port wine stains are not the result of ex- serves as a tool in those investigations. cess angiogenesis, but arise due to a defect in Dr. Samuel Weinberg introduced each speaker in smooth muscle. The ratio of endothelial cells to the final session, "Cases of the Year." Some of the smooth muscle cells may serve as a reliable equa- cases are described below. Dr. Moise Levy de- tion to determine the nature of vascular malforma- scribed several cases of Naprosyn photosensitivity tions. In port wine stains, the ratio of these ele- in children. Histologically, a subepidermal buUa ments is 396 endothelial cells to 42 smooth muscle was characteristic, as well as photodistributed in- cells (9:1). One of the most promising drugs that creased skin fragility in some patients. may have clinical implications in managing vascular A child with manifestations of congenital leuke- abnormalities is interferon a. This drug has shown mia within the first 3 days of life was detailed by Dr. promise in the treatment of pulmonary capillary William Bason. Dr. Julie Prendiville's case had hemangiomatosis (N Engl J Med 1989;320:1197- granular cell tumors, a giant lentiginous nevus and a 1200). nevus flammeus. A child with both relapsing con- The Resident Award paper was presented by genital syphilis and severe combined immunodefi- Glenn Kline, M.D., whose colleagues were Oscar ciency was presented by Dr. Karen Sherwood. L. Frick, M.D., Ph.D.; Edward L. Strem, M.D.; Dr. Lynne Roberts emphasized the use of so- and Mary L. Williams, M.D. His research entailed a dium chloride split skin studies to help distinguish study of ten patients with atopic dermatitis who bullous pemphigoid from epidermolysis bullosa ac- were treated with a slow-release form of ascorbic quisita, her patient's disease. An infant with epider- acid (Geriatric Pharmaceutical Corporation). All molysis bullosa dystrophica with harlequin features patients were between the ages of 3 and 21 years was described by Dr. Liz Beightler. (average age, 8 years), six were male, four were fe- Two cases were reported by Dr. Lawrence male, and all had IgE levels in excess of 2000 IU/ml. Eichenfeld. The first involved an acrodermatitis en- No adverse effects of the vitamin C therapy were teropathica-like eruption with cystic fibrosis. The noted. Fewer courses of antibiotics were required second case was a l!^-year-old with an enlarging by patients on the vitamin C during the 3-month tongue mass that was diagnosed as Riga-Fede dis- treatment period as compared to controls. ease. The child was also found to have familial dys- Practical pointers on how to approach drug stud- autonomia. ies were offered by Dr. John Arlette. The use of The statistics of sporadic dysplastic nevi as mel- multiple sites in performing drug studies is helpful anoma risk markers in relatives were reviewed by in offsetting local variations. The disease being Dr. Lee Albert. Dr. Ronald Hansen discussed the studied should be clearly defined in terms of clini- use of cooling suits for hypohidrotic ectodermal cal, biochemical and/or histologic findings. Ensur- dysplasia. A handout listing the address and phone ing compliance is often a major obstacle in clinical number of the Hypohidrotic Ectodermal Dysplasia drug study trials. Compliance may be enhanced by Foundation (1-804-826-0065) was provided. Dr. frequent patient follow-up visits, giving additional Adelaide Hebert described two siblings with the attention to each patient, selecting patients on their DeSanctis-Cacchione syndrome. The fibroblast ability to comply, and counting the pills prescribed analysis on these patients was performed by Dr. at each visit. Errors in drug studies may suise as a James Cleaver, who will address the Society for Pe- function of the impact of environmental conditions diatric Dermatology at the pre-American Academy on the disease, the patient's desire to perform, and of Dermatology meeting in San Francisco, Califor- intolerance to vehicles being studied. nia in December 1989.