300 Fediatric Dennatology VoL 10 No. 3 September 1993

TABLE 2. Type of Nevus Depigmentosus andAssociated Disorders

Type No. (%) Association No. (%)

Eocal 37 (74) Seizures, mentalretardation None

Hemangioma 2 (4)Erictional alopecia 1 (2)Pityriasis alba 2 (4)Melanocytic nevus 3 (6)Papular urticaria 1 (2)Halo nevus 1 (2)Mongolian spot 1 (2)Leukotrichia 2 (4)Atopic dermatitis 5 (10)

Segmental 11 (22)

Generalized 2 (4)

Of 50 patients, in 38 (76%) age at onset was 5years or less. This indicates that the disorder ismostly a concern in chUdren of preschool age. Thisis in contrast to vitiligo, which often first appears atschool age (>5 yrs) (5—7). Two of our patients hadgeneralized NDP. Some authors believe that incon-tinentia pigmenti achromians is a variant of general-ized NDP (8,9). Others, however, believe that theyare two different entities (2).

In contrast to observations by others that NDPmost commonly involves the trunk and proximalextremities and only rarely the face and neck (2), 19(38%) of our patients had lesions on the face andneck. Among the associated diseases, atopic der-matitis in 5 (10%) patients and a family history ofatopy in 14 (28%) are significant observations notmentioned before in the literature.

REFERENCES

1. Lesser E. In; Ziemssen HV, ed HandbuchderHautkrankheiten, 2nd ed. Leipzig: Vogel, 1884:183.

2. Mosher DB, Fitzpatrick TB, Ortonne JP, Hori Y.Disorders of pigmentation. In; Fitzpatrick TB, EisenAZ, Wolff K, et al, eds. Dermatology in general med-icine, 3rd ed. New York; McGraw-Hill, 1987:826-828.

3. Jimbow E, Eitzpatrick TB, Szabo G, et al. Congenitalhypomelanosis: a characterization based on electronmicroscopic study of tuberous sclerosis, naevus de-pigmentosus and piebaldiesm. J Invest Dermatol1975;64;50-62.

4. Bolognia JL, Pawelek JM. Biology of hypopigmenta-tion. J Am Acad Dermatol 19;217-255.

5. Grimes PS, Kelly AP. Management of vitiligo in chil-dren—a symposium, Pediatr Dermatol 1986;3:498-510.

6. Banerjee BN, Pal SK. Leucoderma. Ind J Dermatol1956;2;{-10.

7. Rogers M. Management of vitiligo in children—asymposium. Pediatr Dermatol 1986;3;498-5I0.

8. Maise JC, Headington JH, Lynch PJ. Systematized

hypochromic nevus: incontinentia pigmenti achromi-ans of Ito. Arch Dermatol 1972;106; 184-185.

9. Ito M. A singular case of nevus depigmentosus sys-tematicus bilateralis. Jpn J Dermatol 1951;61:31-32.

SANDIPAN DHAR. M.D.. D.N.B.AMRINDER J. KANWAR. M.D.SURRINDER KAUR. M.D.. FAMSChandigarh, India

ACCESSORY NIPPLES AND URINARYTRACT MALFORMATION

To the Editor:In a recent issue oi Pediatric Dermatology (1992;

9:239-240) Armoni et al reported that none of 102Israeli children who had an accessory nipple (AN)and who were screened with an abdominal ultra-sound had evidence of a urinary tract malformation(UTM). The authors concluded that abdominal ul-trasound was unnecessary in patients who have anAN and who reside in their geographic region.

Sixty-four percent of the children in that studywere of Oriental origin. This patient population isnot truly representative of the general population inIsrael. As such, the conclusion that an abdominalultrasotind is not indicated in patients who have anAN and who reside in Israel may not be valid.

The frequency of UTM in patients with AN is re-ported to vary from 1.2% to 27% (1-5). The fre-quency of UTM detected by abdominal ultrasoundin the general population is reported to be 1.0%.Therefore, even if the frequency in patients whohave an AN is only 2% to 3%, this would be 2 or 3times the expected frequency in the general popula-tion. The study by Armoni et al included only 102patients who had an AN. Failure to identify any pa-tients with UTM in this small population may bedue to chance alone. A larger prospective study istherefore necessary prior to concluding that no as-sociation exists. Until such a study is performed,we believe that an abdominal ultrasound should beconsidered in all patients who have an AN.

REFERENCES

1. Kenny RD, Flippo JK, Black EB. Supernumerarynipples and renal anomalies in neonates. Am J DisChild 1987; 141:987-988.

2. Mehes K. Association of supernumerary nipples withother anomalies. J Pediatr l979;95:274-275.

3. Varsano IB, Jaber L, Garty BZ, Mukamel MM,Grunebaum M. Urinary tract abnormalities in chil-dren with supemumerary nipples. Pediatrics 1984 ;33:103-105.

4. Meggyessy V, Mehes K. Association of supernumer-

Correspotjdence 301

ary nipples with renal anomalies. J Pediatr i987;i i i:412-413.

5. Leung AK, Robson WL. Polytheiia. Int J DermatoiI988;28:429-433.

ALEXANDER K. C. LEUNG, MBBS. FRCP(Edin)WM. LANE M. ROBSON, M.D., FRCPCCalgary, Alberta, Canada

REPLY

To the Editor:We gratefully acknowledge the letter of Leung

and Robson. In reply to their letter, we wish to clar-ify that otir paper "Accessory Nipples; Any Rela-tionship to Urinary Tract Malformation?" reportedthe results of the/iwf part of our study. The numberof chiidren who were subsequently examined by ul-trasound with suspected urinary tract malformationwas larger than we have published, but does notchange the results.

In our cotintry the policy of performing ultra-sound in children with accessory nipples is waning.

It is important to emphasize that in our conclu-sion we stated that "this issue is far from settled,"and will require further studies in different geo-graphic areas to reach clear conclusions concerningdifferent populations and racial stock.

MICHAEL ARMONI. M.D.DANIEL FILK, M.D.MENACHEM SCHLESINGER, M.D.SHLOMO POLLAK. M.D.ARYEH METZKER. M.D,Ashkelon. Israel

DIFFUSE CUTANEOUS MASTOCYTOSIS:A RARE ENTITY

To the Editor:Diffuse cutaneous mastocytosis is an extremely

rare mast cell disorder (1,2) characterized by diffusemast eel! infiltration of the skin. Compared withother types of cutaneous mastocytosis, systemicfeatures, namely, diarrhea, flushing, hypotension,and shock, are fairly common in patients with thisdisorder. The frequency of systemic mast cell dis-ease is said to be greatest in these patients (2). Wedescribe a patient with diffuse cutaneous mastocy-tosis who, to the best of our knowledge, is the firstto be reported from India.

A 6-month-old girl was brought to our dermatol-ogy outpatient department with complaints of in-tense pruritus all over the body, with developmentof linear urticarial wheals of four months' duration.She had history of passing loose stools 8 to 10 times/

day dudng attacks of prtirittis. She was born dudngan uncomplicated vaginal delivery and had a normalantenatal and pednatal history. Up to 2 months ofage she was absolutely healthy.

On examination, there was generalized involve-ment of the skin, which was yellowish, rough,thickened, and infolded over flexural areas (Eig. 1).On palpation, the skin appeared doughy and indu-rated, like elephantine skin (Eig. 2). There were nonodules, blisters, hyperpigmentation, depigmenta-tion, or scarring. Darier sign was positive. Systemicexamination revealed no hepatosplenomegaly,lymphadenopathy, or bony tenderness. Examina-tion of the eyes was within normal limits.

Histopathology of a skin sample showed in-creased number of mast cells distributed uniformlythroughout the dermis with pedvascular localiza-tion at places. Mast cells were recognized by virtueof their metachromatic staining of granules withGiemsa stain. There were occasional eosinophilsbut the number of melanocytes and their state ofmelanization were within normal limits.

Laboratory investigations showed a total eosino-phil count of 400/mm ,̂ and stool cultures were ster-ile during diarrheal episodes. Chest skiagram andradiologic examination of long bones and skull re-vealed no abtiormality.

The condition was explained to the child's par-

s .

Figure 1. Yellowish, rough, thickened skin over thethigh, with some mfoiding.