2
Journal of the American Academy of Dermatology Volume 30, Number 4 Brief communications 667 2. RodmanO. Dermatophytosis-tinea pedis and onychomy- cosis. In: Greer K. Common problems in dermatology. Chicago: Year Book, 1988:87-91. 3. Smith E, GrahamJ, UlrichJ. Topica Iclotrimazole in linea pedis. South Med J 1977;70:47-8. 4. Zaias N, Battistini F, Gornez-Urcuyo F, et al. Treatment of tinea pedis with griseofulvin and topical antifungal cream. Cutis 1978;22:196-9. 5. Greer D. Topical treatment for moccasin-type tinea pedis. JAM ACAD DERMATOL 1987;16:554-8. 6. Berman 13, Ellis C, Leyden J, et al. Efficacy of a l-week, twice-daily regimen of terbinafine 1 % cream in the treat- ment of interdigital tinea pedis. J AM ACAo DERMATOL 1992;26:956-60. 7. Bergstresser PR, Elewski B, Hanifin J, et aJ. Topical terbi- nafineand clotrimazole in interdigital tinea pedis: a multi- center comparison of cure and relapse rates with 1- and 4-weck treatment regimens. J AM ACAD DERMATOL 1993;28:648-51. 8. White lE, Perkins PJ, Evans EGV. Successful 2-week treatment with terbinafine (Lamisil'e') for moccasin tinea pedis and tinea manuum. Br J DermatoI1991;125:260-2. 9. Jones H. Therapyof superficial fungal infection. Med Clin North Am 1982;66:873-93. 10. JonesHE. Immune response and host resistance of humans to dermatophyte infection. J AM ACAD DERMATOL 1993;28:S 12-S18. II. Odom R: Pathophysiology of dermatophyte infections. J AM ACAD DERMATOL 1993;28:S2-87. 12. Leyden J. Progression of interdigital infections from sim- plexto complex. J AM ACAD DERMATOL 1993;28:87-8 II. Familial polythelia Fig. 1. Compound nevus (located inferior and to right) that had darkened during pregnancy and supernumerary nipple (located superior and to left) are present below the left breast. CASE REPORT A 19-year-old white woman had a pigmented lesion that had darkened during her pregnancy. Routine ultra- sonography done at 16 weeks of gestation had demon- strated an asymptomatic ectopic kidney on her right side. The lesion with the change in pigmentation was a benign-appearing compound nevus. A 0.5 X 0.5 em su- pernumerary nipple was also noted below the left breast Eugenia Toumbis-Ioannou, MD,a,d and Philip R. Cohen, MDa,b,c Houston, Texas Polythelia refers to supernumerary breast tissue in which only the nipple is present. 1 Sporadic and familial cases of supernumerary nipples have been documented. Familial polythelia has been observed in sequential generations and in siblings. We de- scribe three siblings with supernumerary nipples: a woman with left-sided polythelia and an associated ectopic right kidney, her older sister who also has left-sided polythelia, and her brother who has a complete supernumerary breast on his left side. We also summarize the cases of familial polythelia pub- lished in the English language literature. From the Department ofDermatology' and Patho1ogy,bThe University ofTexas at Houston; Department ofMedical Specialties, Division of Dermatology, theUniversity ofTexas M. D. Andersnn Cancer Cen- ter, Houston"; and the Department ofDermatology, Baylor College of Medicine, Houston." Reprint requests: Philip R. Cohen. MD, Department of Dermatology, The University ofTexas Medical School at Houston, 6431 Fannin, Suite 1.186 Houston, Texas 77030 J AM ACAD DERMATOL 1994;30:667-8. Copyright @ 1994 by the American Academy of Dermatology, Inc. 0190-9622/94 $3.00 + 0 16/54/51522 along the embryonic milk line (Fig. 1). The patient's 30- year-old sister also has a supernumerary nipple in the same location and her 28-year-old brother has a complete supernumerary breast on his left side. Neither the pa- tient's parents nor niece and nephew (brother's and sister's children, respectively) have polythelia. DISCUSSION Supernumerary nipples are uncommon, but not rare. They typically appear as a sporadic finding in

Familial polythelia

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Page 1: Familial polythelia

Journal of the American Academy of DermatologyVolume 30, Number4 Briefcommunications 667

2. RodmanO. Dermatophytosis-tineapedis andonychomy­cosis. In: Greer K. Common problems in dermatology.Chicago: Year Book, 1988:87-91.

3. Smith E,GrahamJ, UlrichJ.TopicaIclotrimazole inlineapedis. South Med J 1977;70:47-8.

4. Zaias N, Battistini F, Gornez-Urcuyo F, et al.Treatmentof tinea pedis with griseofulvin and topical antifungalcream. Cutis 1978;22:196-9.

5. Greer D. Topical treatmentfor moccasin-type tineapedis.JAM ACAD DERMATOL 1987;16:554-8.

6. Berman 13, Ellis C, Leyden J, et al. Efficacy of a l-week,twice-daily regimen of terbinafine 1% cream in the treat­ment of interdigital tinea pedis. J AM ACAo DERMATOL1992;26:956-60.

7. Bergstresser PR, Elewski B, Hanifin J, et aJ. Topical terbi­nafineand clotrimazole in interdigital tinea pedis: a multi-

center comparison of cure and relapse rates with 1- and4-weck treatment regimens. J AM ACAD DERMATOL1993;28:648-51.

8. White lE, Perkins PJ, Evans EGV. Successful 2-weektreatment with terbinafine (Lamisil'e') for moccasin tineapedisand tinea manuum. Br J DermatoI1991;125:260-2.

9. JonesH.Therapyof superficial fungal infection. Med ClinNorth Am 1982;66:873-93.

10. JonesHE. Immuneresponse andhost resistance of humansto dermatophyte infection. J AM ACAD DERMATOL1993;28:S12-S18.

II. Odom R: Pathophysiology of dermatophyte infections. JAM ACAD DERMATOL 1993;28:S2-87.

12. Leyden J. Progression of interdigital infections from sim­plexto complex. J AM ACAD DERMATOL 1993;28:87-8II.

Familial polythelia

Fig. 1. Compound nevus (located inferior and to right)that had darkened during pregnancy and supernumerarynipple (located superior and to left) are present below theleft breast.

CASE REPORT

A 19-year-old white woman had a pigmented lesionthat had darkened during her pregnancy. Routine ultra­sonography done at 16 weeks of gestation had demon­strated an asymptomatic ectopic kidney on her right side.The lesion with the change in pigmentation was abenign-appearing compound nevus. A 0.5 X 0.5 em su­pernumerary nipple was also noted below the left breast

Eugenia Toumbis-Ioannou, MD,a,d and Philip R. Cohen, MDa,b,c Houston, Texas

Polythelia refers to supernumerary breast tissuein which only the nipple is present. 1 Sporadic andfamilial cases of supernumerary nipples have beendocumented. Familial polythelia has been observedin sequential generations and in siblings. We de-scribe three siblings with supernumerary nipples: awoman with left-sided polythelia and an associatedectopic right kidney, her older sister who also hasleft-sided polythelia, and her brother who has acomplete supernumerary breast on his left side. Wealso summarize the cases of familial polythelia pub­lished in the English language literature.

From theDepartment ofDermatology' and Patho1ogy,bThe UniversityofTexas atHouston; Department ofMedical Specialties, Division ofDermatology, theUniversity ofTexas M. D. Andersnn Cancer Cen­ter, Houston"; and theDepartment ofDermatology, Baylor Collegeof Medicine, Houston."

Reprint requests: Philip R. Cohen. MD, Department of Dermatology,TheUniversity ofTexas Medical School at Houston, 6431 Fannin,Suite 1.186 Houston, Texas 77030

J AM ACAD DERMATOL 1994;30:667-8.

Copyright @ 1994 by the American Academy of Dermatology, Inc.0190-9622/94 $3.00 + 0 16/54/51522

along the embryonic milk line (Fig. 1). The patient's 30­year-old sister also has a supernumerary nipple in thesame location and her 28-year-old brother has a completesupernumerary breast on his left side. Neither the pa­tient's parents nor niece and nephew (brother's andsister's children, respectively) have polythelia.

DISCUSSION

Supernumerary nipples are uncommon, but notrare. They typically appear as a sporadic finding in

Page 2: Familial polythelia

668 Brief communications

Table I. Persons with familial polythelia reportedin the English language literature*

No. ofAuthor (year) subjects

Iwai (1907) 20 M, 68 wr 1-3t 2Klinkerfuss (1924) 5W 4 3Graham-Campbell 2W 2 4Weinberg & 6W 2 5

Motulsky (1976)Rintala & Norio 1 M,3 W 2 6

(1982)Hersh et al (1987) 4+ 2 7Leung (1988) 1 M,2W 2 8Bortz et al (1989) 3 M, 1 W 2 9Present case 1 M,2 W 1 CR

CR, Case report; M, man/men; W, woman/women.*The non-English language literature also contains reports of familialpolythelia: 20 persons from four generations of a single family'? andtWins. 11

tThe 88 persons are from 36 families. Supernumerary breasts werepresent in the grandchildren and/or the children and mother (27 fam­ilies); the daughter, mother, and mother's brother (three families); thedaughter and father (one family); two sisters (four families); and twobrothers (l family).tTwo parent-child pairs were described in which the sex of neither theparent nor the child was reported.

an otherwise healthy person. Patients with supernu­merary nipples may also have associated anomalies(most commonly of their urinary tract).' For exam­ple, our patient's ectopic kidney was only discoveredincidentally during a screening ultrasonogram per­formed at 16 weeks of pregnancy. Therefore anevaluation of persons with supernumerary nipplesfor polythelia-associated conditions should be con­sidered.

Although most cases of polythelia are consideredto be sporadic, familial supernumerary nipples havealso been recognized (Table I).2-11 The first series offamilial polythelia in the English language literaturewas reported in 1907 by Iwai/; he observed 31 Jap­anesefamilies (78 persons) in whom there was a he­reditary polymastia and five Japanese families (10persons) in whom there was polymastia in siblings.?In 1924, Klinkerfuss' reported polymastia in fourgenerations. Subsequently, additional patients with

Journal of the American Academy of DermatologyApril 1994

familial polythelia were observed: a woman with 10supernumerary nipples who gave birth to a childwith one supernumerary nipple," a family withbilateral accessory axillary breasts without nipplesor areolae in six women in two gcnerations.f and in­traareolar polythelia bilaterally in a mother, her son,and her two daughters." Hersh et al.7 suggested anautosomal dominant mode of inheritance for super­numerary nipples when they described two parent­child pairs with polythelia. Supernumerary nippleswere also reported by Leung'' and Bortz et a1.9 in aChinese family with polythelia in three membersfrom two generations and an American family inwhich the father, mother, and both sons had a left­sided supernumerary nipple. Finally, in our patient'sfamily, both sisters had polythelia on the left side andtheir brother had a complete left-sided supernumer­ary breast.

REFERENCES

I. Leung AKC, Robson WLM. Polythelia. Int J Dermatol1989;28:429-33.

2. Iwai T. A statistical study on the polymastia of the Japa­nese. Lancet 1907;2:753-9.

3. Klinkerfuss GH. Four generations of polymastia. JAMA1924;82:1247-8.

4. Graham-Campbell R. Polythelia. Br Med J 1936;I:471.5. Weinberg SK, Motulsky AG. Aberrant axillary breast tis­

sue: a report of a family with six affected women in twogenerations. Clin Genet 1976;10:325-8.

6. Rintala A, Norio R. Familial intra-areola polythelia withmammary hypoplasia. Scand J Plast Reconstr Surg 1982;16:287-91.

7. Hersh JH, Bloom AS, Cromer AO, et a1. Does a supernu­merary nipple/renal field defect exist? Am J Dis Child1987;141:989-91.

8. Leung AKC. Familial supranumerary nipples. Am J MedGenet 1988;31 :631-5.

9. Bortz J, Parker S, Ray TL. Lack of associated anomaliesin familial polythelia [Letter]. Am ] Dis Child 1989;143:883.

10. Marie P. Mammelon surnurneraire transmis hereditaire­ment dans une farnille; coincidence avec plusieurs gros­seuses gemellaires. Bull Mem Soc Med Hop Paris1893;18:457-9. Cited in Leung AKC. Am J Med Genet1988;31 :631-5.

11. Birkenfeld W. Beitrag zur Zwillingspathologie derMamma. Arch Klin Chir 1932;J68:568-76.Cited in LeungAKC. Am J Med Genet 1988;31:631-5.