Skip to main content
Open Access Publications from the University of California

Dermatology Online Journal

Dermatology Online Journal bannerUC Davis

Congenital subungual melanocytic nevus with a pseudo-Hutchinson sign

Main Content

Congenital subungual melanocytic nevus with a pseudo-Hutchinson sign
Ari M Goldminz1 BA, Deon Wolpowitz2 MD PhD, Alice B Gottlieb1 MD PhD, Michael S Krathen3 MD
Dermatology Online Journal 19 (4): 8

1. Department of Dermatology, Tufts Medical Center, Boston, Massachusetts
2. Department of Dermatology, Boston University Medical Center, Boston, Massachusetts
3. Department of Dermatology, Cutaneous Oncology, Stanford University School of Medicine, Stanford, California


We describe a 29-year-old woman with congenital melanonychia striata and compound nevus of the right first digit. There was extension of the hyperpigmentation onto the proximal nail fold, even beyond the borders established by the band of melanonychia striata. A dermal plaque with irregular borders and variegated pigmentation was also present over the distal digit extending from the pigmented region of the hyponychium. A limited number of biopsy proven congenital subungual melanocytic nevi have been reported in the literature. Interestingly, we found that the majority of these cases present with longitudinal melanonychia in association with periungual hyperpigmentation, constituting a pseudo-Hutchinson sign. Currently studies evaluating the diagnostic test characteristics of Hutchinson sign are lacking. While Hutchinson sign is traditionally considered a worrisome feature it is certainly not pathognomonic and a malignant cause should not be assumed without thorough assessment.

Case report

A 29-year-old woman presented for management of acne vulgaris. Examination revealed a concerning hyperpigmented 7.1 x 17.5 mm band within the nail bed extending from the proximal nail fold to the hyponychium. Hyperpigmentation extended onto the proximal nail fold, even beyond the borders established by the band of melanonychia striata. Dermoscopy showed variability in width and color of the nail pigmentation (Figure 1). Also present was a dermal plaque with irregular borders and variegated brown to black pigmentation overlying the distal digit and extending from the pigmented region of the hyponychium (Figure 2).

Figure 1Figure 2
Figure 1. Dermoscopy of the right first fingernail shows varied band color and thickness, with hyperpigmentation of the proximal nail fold (pseudo-Hutchinson sign).

Figure 2. A dermal plaque with irregular borders and brown to black color variegation is seen extending from the pigmented region of the hyponychium down the distal digit (scar from punch biopsy is noted within the lesion as well).

Figure 3
Figures 3A and 3B. Biopsy specimen from the volar distal digit reveals pigmented cells infiltrating deep into the dermis between collagen bundles and along adnexal structures, such as eccrine coils shown here, consistent with a congenital nevus pattern (H&E, 4x and x20).

Neither the nail nor digital pigmentation had undergone significant change throughout her life; there was no history of trauma, even at an early age. Personal and family histories were unremarkable for melanoma, Laugier-Hunziker syndrome, Peutz-Jeghers syndrome, or other mucocutaneous hyperpigmentation syndromes. Medications included a daily estrogen-progesterone oral contraceptive pill as well as tretinoin and hydroxychloroquine cream topically applied to acne areas.

Punch biopsy of the volar distal digit showed nests of banal-appearing melanocytes at the dermal-epidermal junction and within the dermis, admixed with pigment-laden macrophages. Dermal melanocytes infiltrated deep into the dermis and along adnexal structures in a pattern characteristic of congenital nevi (Figures 3A and 3B).


In rare instances, congenital nevi present subungually, with fewer than 20 biopsy-proven cases reported [1-12]. Interestingly, we found that nearly all of these congenital subungual melanocytic nevi feature periungual hyperpigmentation in association with longitudinal melanonychia, constituting a pseudo-Hutchinson sign, as was present in the current case. Additionally, at least four other reports describe congenital subungual melanocytic nevi with distal digital involvement [9, 10, 11, 12].

During fetal development, neural crest derived cell migration leads to the localization of active melanocytes within the dermis starting at 10 weeks gestational age. Whereas active melanocytes become more extensively distributed throughout the dermal tissues with the progression of gestation, dermal melanocytes are normally only found within a few anatomical areas, including the dorsal hands and feet, by the end of fetal development [13]. This process is thought to occur both through redistribution to the epidermis as well as cell death. Around the time of the initial dermal localization at 10 weeks gestation, the nail apparatus also begins to form. Given the temporal relationship of these developmental processes and the nevus’s swirled pattern at the distal digit, the extensive nail unit and distal digital involvement in the current case may be explained by aberrant melanocyte migration within the forming nail matrix and acral skin.

Hutchinson sign, first described in 1886, is defined by longitudinal melanonychia extending into the periungual tissues [14]. Whereas classically associated with an underlying melanoma, false positives or pseudo-Hutchinson sign are not infrequent [15]; studies evaluating the diagnostic test characteristics of Hutchinson sign are lacking in both the adult and pediatric populations. Aside from nail unit melanoma and congenital nevi, other entities to consider in the differential diagnosis include the following: ethnic pigmentation, trauma, systemic medical illness such as hyperthyroidism and Cushing syndrome, depositional disorders, medications, and syndromes of mucocutaneous hyperpigmentation [15]. In our case, although only the distal digit was biopsied, the congenital onset, stable clinical history, morphology, and histopathology support the diagnosis of a congenital nevus involving both the distal digit and subungual areas. Nevertheless, in clinically worrisome melanocytic lesions, benign pathologic findings from punch or incisional biopsies cannot exclude sampling error. Therefore, either additional biopsies or complete excision may be warranted to exclude malignant transformation arising in a benign precursor. As previous authors have suggested, Hutchinson sign may not be an infallible sign of subungual melanoma [15] and requires further validation as a clinical tool. Whereas Hutchinson sign is one criterion for the ABC rule of subungual melanoma [16] and is traditionally considered a worrisome feature, it is certainly not pathognomonic and a malignant cause should not be assumed without thorough assessment.


1. Nagumo M, Masaki H, Yamamoto K, Mishima Y. Junctional nevus in the nail matrix area, nail bed and neighboring skin and its dropping phenomena. Hifu 1976; 18:241-2.

2. Wong DE, Brodkin RH, Rickert RR, McFalls SG. Congenital melanonychia. Int J Dermatol 1991; 30(4):278-80. [PubMed]

3. Tosti A, Baran R, Morelli R, Fanti PA, Peserico A. Progressive fading of longitudinal melanonychia due to a nail matrix melanocytic nevus in a child. Arch Dermatol 1994; 130(8):1076-7. [PubMed]

4. Libow LF, Casey TJ, Varela CD. Congenital subungual nevus in a black infant. Cutis 1995; 56(3):154-6. [PubMed]

5. Léauté-Labrèze C, Bioulac-Sage P, Taïeb A. Longitudinal melanonychia in children. A study of eight cases. Arch Dermatol 1996; 132(2):167-9. [PubMed]

6. Tosti A, Baran R, Piraccini BM, Cameli N, Fanti PA. Nail matrix nevi: a clinical and histopathologic study of twenty-two patients. J Am Acad Dermatol 1996; 34(5 Pt 1):765-71. [PubMed]

7. Jeong Y, Seo JK, Sung KH, Choi HU, Lee SK. A case of melanonychia striata caused by congenital melanocytic nevus. Korean J Dermatol 2006; 44(5):589-92.

8. Buka R, Friedman KA, Phelps RG, Silver L, Calero F, Rudikoff D. Childhood longitudinal melanonychia: case reports and review of the literature. Mt Sinai J Med 2001; 68(4-5):331-5. [PubMed]

9. Ohtsuka H, Hori Y, Ando M. Nevus of the little finger with a remarkable nail deformity: case report. Plast Reconstr Surg 1978; 61(1):108-11. [PubMed]

10. Botet MV, Caro FR, Sánchez JL. Congenital acral melanocytic nevi clinically stimulating acral lentiginous melanoma. J Am Acad Dermatol 1981; 5(4):406-10. [PubMed]

11. Coskey RJ, Magnell TD, Bernacki EG Jr. Congenital subungual nevus. J Am Acad Dermatol 1983; 9(5):747-51. [PubMed]

12. Asahina A, Chi HI, Otsuka F. Subungual pigmented nevus: evaluation of DNA ploidy in six cases. J Dermatol 1993; 20(8):466-72. [PubMed]

13. Zimmermann AA, Becker SW Jr: Melanoblasts and melanocytes in fetal negro skin. In: Illinois Monographs in Medical Sciences, Vol. VI, No. 3, Urbana: University of Illinois Press; 1959:1-59.

14. Hutchinson J. Melanosis often not black; melanotic whitlow. Br Med J. 1886; 1:491.

15. Baran R, Kechijian P. Hutchinson’s sign: a reappraisal. J Am Acad Dermatol. 1996 Jan; 34(1):87-90. [PubMed]

16. Levit EK, Kagen MH, Scher RK, Grossman M, Altman E. The ABC rule for clinical detection of subungual melanoma. J Am Acad Dermatol. 2000 Feb; 42(2 pt 1):269-74. [PubMed]

© 2013 Dermatology Online Journal