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

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