Correspondence

Letters to the Editor are welcomed for publication (subject to ed-iting). Letters must be signed by all autisors, typewritten doublespaced, and must not exceed two pages of text inctoding references.Two copies of all letters shotiM be submitted. Letters should notduplicate material submitted or published in otber joumals. Pre-publicatlon proofs will not be provided.

EARLY-ONSET NEVI

To the Editor:The recent study by MacKie et al (1) demonstrat-

ing a high frequency of small, early-onset nevi (de-fined as rtevi present before 2 yrs of age) in contigu-ity with melanomas in young adults (<30 yrs) isprovocative to all of us who wrestle with the mela-noma risks associated with small congenital nevi. Iam concemed, however, that readers unfamiliarwith the original report (1) may be misled by Dr.MacKie's brief summary of this work (2) to equateearly-onset nevi with congenital nevi. Although Dr.MacKie did not so recommend (2), some readersmight even conclude that removal of all congenitalnevi, occurring in about 1% of newborns (3-6)would be warranted if this would prevent at leastabout 3% of all melanomas. In their study (I), 6% ofmelanomas occurred in patients less than 30 yrs old,and approximately half of these arose within early-onset aevi. Thus, it is important to bear in mind thatthe prevalence of early-onset nevi in their controlpopulation was 20% (1). This is much higher thanthe frequency of congenital nevi in any populationthat has been studied by nursery examination (3-6).Hence, clearly most (=95%) early-onset nevi arenot cotigenital.

In support of this notion, Clemmetisen andKrooo (7) reexamined 2- to 3-year-old children thaton nursery examination had no congenital nevi. Ap-proximately 25% of these children had developedone or more melanocytic nevi, half of whichshowed the histopathoiogy of congenital nevi. To-gether, these studies suggest that the at risk pig-mented lesion, linked in this (1) and other (8,9) stud-ies to melanoma, actually may be of later onset andmuch more common in the population thati congen-ital nevi. If so, removing all small congenital tieviwill do little to diminish this risk.

REFERENCES

1. MacKie RM, Watt D, Doherty V, Aitchison T. Ma-lignant melanoma occurring in those under 30 in westof Scotland, 1979-1986: a study of incidence, ciinicaifeatures, pathological features and survival. Br J Der-matol 1991;124:560-564.

2. MacKie RM. Nevi as dsk factors for melanoma. Pe-diatr Dermatol 1992 ;9:340-341.

3. Walton RG, Jacobs AH, Cox HJ. Pigmeoted lesionsin newborn infants. BrJ Dermatol }976;95:389-396.

4. Alper J, Holmes LB, Mihm MC. Birthmarks with se-dous medical significance: nevocellular nevi, seba-ceous nevi. and multiple cafe-au-lait spots. J Pediatr1979;95:696-700.

5. Hidano A, Purwoko R, Jilsuhawg K. Statistical sur-vey of skin changes in Japanese neonates. PediatrDermatol 1986;3:140-144.

6. Kroon S, Clemmensen O, Mastniip N. The incidenceof congenital melanocytic nevi in newborn babies inDenmark. J Am Acad Dermatol 1987;17:422-426.

7. Clemmensen OJ, Kroon S. The histology of "congen-ital features" in early acquired melanocytic nevi. JAm Acad Dermatol 1988;15:742-746.

8. Rhodes AR, Sober AJ, Day CL, et al. The malignantpotential of small congenital nevocellular nevi. Anestimate of association based on a histologic study of234 pdmary cutaneous melanomas. JAAD 1982;6:230-241.

9. lilig L, Weidner F, Hundeiker M, et a!. Congenitalnevi =s 10 cm as precursors to melanoma. Arch Der-mato! 1985;121:1274-1281.

MARY L. WILLIAMSSan Francisco. Califomia

LEUCOTRICHIA IN NEVUS DEPIGMENTOSUS

Jo the Editor:The clinical features of nevus depigmentosus are

quite characteristic. The lesions are of varioussizes, pale white to tan-colored macules, with ser-rated margins (I). At times they may be clinicallyconfused with vitiliginous lesions, but a stable (incolor and size) hypopigmented patch with serratedfeathery margins present from birth is quite charac-teristic and allows differentiation from vitiligo. Di-agnosis is further confirmed by dopa reaction show-ing the usual number of melanocytes in nevusdepigmentosus as against absent melanocytes in vi-tiligo. Electron microscopy shows a transfer defectof melanosomes from melanocytes to basal kerati-nocytes in the former as against absent melatiocytesin the latter. Although leukotrichia is a common fea-ture of vitiligo, it has not been mentioned in relationto nevus depigmentosus to the best of our knowl-edge.

We recently saw two patients with nevus depig-mentosus, a 9-year-old boy and a 10-year-old girl,who had characteristic hypopigmented patchesfrom birth. Of interest was the presence of scatteredhypopigmented hairs over the patches. The hairsover the tinaffected skin and scalp hair were of nor-mal color (dark). There was no personal or familyhistory of vitiligo in either patietit. It is possible thata defect in the transfer of melanosomes also exists

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in the hair follicles as in the epidermis and this re-sults in leukotrichia.

REFERENCE

1. Jimbow K, Fitzpatrick TB, Szabo G, et al. Congeni-tal hypomelanosis: a charactedzation based on elec-tron microscopic study of tuberous sclerosis, naevusdepigmentosus and piebaldism. J Invest Dermatol1975;64:50-62.

SANDIPAN DHAR, M.D., D.N.B.,AMRINDER J. KANWAR, M.D.. andSRABANl GHOSH, M.D,Chandigarh, India

ACUTE LYMPHOBLASTIC LEUKEMIAASSOCIATED WITH DERMATOMYOSITIS IN

A CHILD

To the Editor:A 14-year-old girl was diagnosed as having acute

lymphoblastic leukemia. Complete remission wasachieved with polychemotherapy. Maintenancechemotherapy (6-mercaptopurine 50 mg/m"/day andmethotrexate 20 mg/m' once/wk) was started sixmonths later. After eight months of complete remis-sion of hemopathy, she developed facial erythemawith butterfly lupus (Fig. 1) and edema predominanton the upper eyelids (Fig. 2), but without rash onthe dorsal surfaces of the hands. Two weeks latershe began to complain of fatigue in the legs. Exam-ination revealed muscular weakness predominant inproximal muscles of the limbs resulting in func-tional impotence (difficulty standing and climbingstairs). Laboratory data showed rises in serum cre-atinine phosphokinase (CPK) and aldolase levels.Lymphopenia and decreased serum globulin levelswere noted. Histologic specimens obtained fromthe biceps muscles showed degenerative changes(vacuolization and necrosis) of muscle fibers. Peri-fascicular atrophy, interstitial infiltrate, and occlu-sive vasculitis of small vessels were also observed.There was no evidence of ultrastructurally pseudo-viral inclusion in muscle specitnens. A skin biopsyspecimeti showed perivascular infiltratioti by mono-nuclear cells. These findings were consistent with adiagnosis of dermatomyositis (DM). Concomi-tantly, no progressive viral infection was found, andmyelogram indicated continuance of complete re-mission. Treatment consisted of intensification ofmaintenance chemotherapy (methotrexate 13 mg/m' twice/wk) and plasma exchanges (3 times/wk).Clinical improvemetJt was noted. The patient wasable to stand up without support, facial edema and

• ;

\Figure 1. Facial erythema.

Figure 2. Eyelid edema.

erythema diminished, and CPK and aldolase con-centrations returned to normal range over a fewweeks.

Facial erythema reappeared, and weakness wasobserved 10 months later. More frequent plasmaexchanges failed to bring about clinical improve-ment, and the patient experienced an isolated