Puzzling out Difficult Pediatric Skin Diagnoses

32 Skin Disorders FAMILY P RACTICE N EWS • September 15, 2005 Puzzling Out Difficult Pediatric Skin Diagnoses

BY HEIDI SPLETE celes or hetero- Senior Writer topic brain tissue.

O RLANDO — Pediatric skin conditions Pilomatricoma often pose diagnostic challenges because A 15-month-old many cutaneous disorders have similar girl presented clinical features. with a bump on Annular lesions of granuloma annulare her cheek—a may be mistaken for tinea corporis; fol- firm, bluish, cys- licular papules of keratosis pilaris may be tic papule that confused with follicular eczema; and nail moved back and psoriasis may be misdiagnosed as ony- forth under pres- chomycosis. sure. When one At a meeting sponsored by the Ameri- end of the lesion can Academy of Pediatrics, Albert C. Yan, was palpated, the A collar of hair around membranous aplasia cutis Pilomatricomas may be associated with systematic M.D., director of pediatric dermatology at other end would congenita can indicate cranial dysraphism. disorders such as Gardner’s syndrome or sarcoidosis. the Children’s Hospital of Philadelphia, pop up, a phe- provided some helpful diagnostic tips for nomenon also known as a “teeter-totter had swollen hands and feet. She had been distinguishing some of these potentially sign.” otherwise healthy and was taking no med- puzzling skin problems. Although these lesions may resemble ications. The condition arose suddenly; the dermoid cysts or epidermal inclusion parents noticed the rings and swelling one The Hair Collar Sign cysts, the diagnosis in this case was pilo- morning when picking up the child from HILADELPHIA A boy is born with an area of localized, cir- matricoma, distinguished by its bluish col- her bed. On closer inspection, the rings P cular alopecia covered by a glossy mem- or and the presence of the teeter-totter were red and blanchable, with clear white brane. The area is surrounded by a col- sign. The lesions most often occur on the centers. Some were imperfect circles. OSPITAL OF lection of dark, terminal hairs. Palpation head or neck, although other areas occa- The diagnosis is annular urticaria. H S reveals that a lump is present. sionally are affected. Pilomatricomas gen- “These types of cases are frequently re- ' Occasionally mistaken for fetal scalp erally are solitary, benign, frequently cal- ferred for suspected erythema multi- HILDREN

monitor trauma, neonatal herpes simplex cified, and arise from hair follicles. In forme,” Dr. Yan noted. “Lesions of annu- C infection, or a nevus sebaceus of Jadas- some cases, the lesions resolve sponta- lar urticaria are evanescent; the lesions sohn, this characteristic pattern—a collar neously, but more often, they persist and fade and move, and the lesions can form of coarse hair surrounding an area of grow, and surgical intervention is recom- imperfect circles with clear centers. The le- membranous aplasia cutis congenita—can mended. Pilomatricomas may rupture, sions may disappear within 24 hours, only HOTOS COURTESY be a marker for cranial dysraphism, a de- which can cause inflammation and scar- to show up elsewhere,” he said. By con- P velopmental defect of the skull potential- ring. Although pilomatricomas generally trast, erythema multiforme appears as Urticaria multiforme, often mistaken for ly associated with structural neurologic are isolated findings, they may be associ- fixed target, or “bull’s-eye,” lesions with annular urticaria, is linked to allergy. defects. The scalp defect may represent ated with systemic disorders such as Gard- dusky centers and is associated with mu- only the tip of the iceberg, Dr. Yan noted, ner’s syndrome, myotonic dystrophy, and cous membrane ulcers. history to determine underlying causes, since underlying bony defects or ectopic sarcoidosis. The two conditions are treated quite dif- removal or treatment of those causes, and brain tissue may be present. ferently, Dr. Yan emphasized. Annular ur- consideration of steroid therapy if indi- In such cases, magnetic resonance imag- Annular Urticaria/Urticaria Multiforme ticaria responds to combinations of anti- cated. Dr. Yan often refers to annular ur- ing is essential to rule out underlying ab- A 3-year-old girl presented with red, histamines or occasionally steroids; ticaria as “urticaria multiforme” because normalities, including atretic encephalo- swollen, annular plaques on her skin, and erythema multiforme requires a detailed these cases are so regularly mistaken. ■ Early, Aggressive Surgery Is Best Distinguish Diaper Psoriasis Course for Neurofibroma in Children From Seborrheic Dermatitis BY SHARON quence of exposure to stool BY PATRICE WENDLING Tumors may grow back and in neurofibromatosis 2 (NF2), WORCESTER and urine. Chicago Bureau greater numbers, and they could which is rarer, occurring in 1 in Southeast Bureau It can be difficult to distin- be fibrosarcoma, said Dr. Mc- 40,000 births. guish napkin psoriasis from se- PARIS — Resection can’t be too Kinnon, a plastic and reconstruc- NF2 is characterized by multi- B AL H ARBOUR, FLA. — borrheic dermatitis, but there early or too aggressive when tive surgeon with the University ple tumors on the cranial and Diaper-area psoriasis, also are certain characteristics that treating neurofibroma in chil- of Chicago. Early surgery, even in spinal nerves and by other le- known as napkin psoriasis, is a can help, said Dr. Paller, pro- dren, reported McKay McKin- infancy, may be indicated. sions of the brain and spinal cord. fairly common finding in chil- fessor and chair of dermatol- non, M.D., at the Fourth Inter- “In my experience, a very ag- Its primary characteristic is bilat- dren, Amy Paller, M.D., said at ogy at Northwestern Universi- national Academy of Cosmetic gressive surgery can often pro- eral tumors of the eighth cranial the annual Masters of Pediatrics ty, Chicago. Dermatology World Congress. duce nonrecurrence for many nerve, resulting in hearing loss conference sponsored by the Plaques associated with nap- Neurofibroma is a usually be- years or forever,” he said. “That’s beginning in the patient’s teens University of Miami. kin psoriasis are sharply de- nign tumor of the peripheral hard to prove, but I think that or early 20s. Although inclusion of this fined, brightly erythematous, nerves caused by abnormal pro- should be the principle.” Because patients with neurofi- “rash” in the realm of psoriasis and larger than those in sebor- liferation of Schwann cells. Viable structure may remain broma may have recurrence has been controversial, Dr. rheic dermatitis. They also tend The development of neurofi- after excision of large tumors throughout life and/or develop Paller said she “truly believes” it to have drier scale, which in the bromas, multiple café au lait from the ear. But ocular tumors neurosarcoma, they need con- should be considered a psoriat- diaper area often can be seen spots, and freckling in the pose a wide range of problems, tinued surgical surveillance, Dr. ic condition. only when the skin is scratched. armpits or groin area are com- particularly if the tumor extends McKinnon said. About 13% of children pre- This is caused by the constant mon symptoms of neurofibro- into the extraocular muscles. The National Institutes of sent with napkin psoriasis with moisture in the area, which also matosis 1 (NF1), which occurs in If at all possible, surgeons Health is launching a study to in- dissemination; about 4% pre- can obscure diagnosis in some 1 in 4,000 births. should not sacrifice the impor- vestigate whether genetic differ- sent with localized disease. cases, Dr. Paller noted. There is no way to tell which tant structures of the face or the ences explain differences in disease The frequency of this condi- For treatment, she uses a patients will develop a mild case facial nerve, he said. severity among patients with NF1. tion in infants reflects the short course (about 3 weeks) of and which patients will have se- A careful and early diagnosis of Participants must be at least 16 Koebner phenomenon—a re- nonhalogenated topical steroid rious complications such as neurofibroma is important and years old. For more information, sponse to the constant trauma or topical calcineurin inhibitor painful or disfiguring tumors. can help distinguish NF1 from write to [email protected]. ■ to the skin that is a conse- and a lot of protective paste. ■