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CASE REPORT
Year : 2021  |  Volume : 14  |  Issue : 1  |  Page : 57-59  

Bilateral nevoid hyperkeratosis of nipple: A report of two cases


Department of Dermatology, SKNMC, Pune, Maharashtra, India

Date of Submission07-Aug-2019
Date of Decision29-Sep-2019
Date of Acceptance15-Oct-2019
Date of Web Publication22-Jan-2021

Correspondence Address:
Garima Balpande
Department of Dermatology, SKNMC, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_228_19

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  Abstract 


Nevoid hyperkeratosis of nipple and areola was first described by Tauber in 1923. It is a rare idiopathic disorder characterized by papular warty excrescences on the nipple and areola or both. Histopathology shows hyperkeratosis, acanthosis, papillomatosis, elongation of rete ridges, filiform interconnecting pattern, increased pigmentation in the basal layer, and sparse perivascular lymphocytic infiltrate. We report two cases of nevoid hyperkeratosis of nipple because of rarity of this condition.

Keywords: Benign, calcipotriol ointment, nevoid hyperkeratosis of nipple, warty


How to cite this article:
Balpande G, Khatu S, Chavan D, Palaskar N. Bilateral nevoid hyperkeratosis of nipple: A report of two cases. Med J DY Patil Vidyapeeth 2021;14:57-9

How to cite this URL:
Balpande G, Khatu S, Chavan D, Palaskar N. Bilateral nevoid hyperkeratosis of nipple: A report of two cases. Med J DY Patil Vidyapeeth [serial online] 2021 [cited 2021 Mar 7];14:57-9. Available from: https://www.mjdrdypv.org/text.asp?2021/14/1/57/307666




  Introduction Top


Nevoid hyperkeratosis of nipple and areola (NHNA), first described by Tauber in 1923.[1] It is a rare skin disorder with unknown etiology.[2] Nevoid hyperkeratosis presents as asymptomatic and hyperpigmented filiform or papular warty excrescences on the nipple, areola or both.[3] The eruption of such a malignant looking lesion over areola and/or nipple causes excessive concern in patients, hence effective management with a decent cosmetic outcome is necessary.


  Case Reports Top


Case 1

A 23-year-old unmarried female presented with a rough asymptomatic growth on her both nipples for 12 months [Figure 1]. There was no history of pruritus/pain/discharge.
Figure 1: Brown-to-black verrucous irregular papules and plaque on both the nipples

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Dermatological examination revealed brown-to-black verrucous irregular papules and plaque on both the nipples. On palpation, it was nonindurated, nontender, and nonadherent to the underlying structure. There was no discharge and regional lymphadenopathy. Routine laboratory tests were within normal limits. Clinical differential diagnosis of verruca vulgaris, verrucous epidermal nevus, nevoid hyperkeratosis of nipple, and nipple eczema were thought of.

Skin biopsy specimen showed hyperkeratosis and mild papillomatous hyperplasia, elongation of rete ridges, and filiform interconnecting pattern; there was increased pigmentation in the basal layer. The dermis has dilated capillaries and only sparse perivascular lymphocytic infiltrate [Figure 2]. Clinicohistopathological findings confirmed the diagnosis of nevoid hyperkeratosis of nipple. She was started on topical calcipotriol ointment 0.005% and emollients with partial clearance of the lesion [Figure 3].
Figure 2: Histopathology showing hyperkeratosis and mild papillomatous hyperplasia, elongation of rete ridges and filiform interconnecting pattern (H and E, ×40)

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Figure 3: Posttreatment showing partial clearance of the lesion

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Case 2

A 54-year-old man presented with a rough asymptomatic growth on his both nipples for 15 months [Figure 4]. There was no history of pruritus/pain/discharge/drug intake.
Figure 4: Brown-to-black verrucous papular warty excrescence present over both the nipples

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Dermatological examination revealed brown-to-black verrucous papular warty excrescence of approximate size 4 mm × 3 mm present at 5'o clock position on the left nipple and approximate size 1 mm × 2 mm present at 7'o clock position on the right nipple. On palpation, there was no tenderness and discharge. Clinical differential diagnoses considered were fibroepithelial polyp, nevoid hyperkeratosis of nipple, and verruca vulgaris

Histopathology showed hyperkeratosis, acanthosis, papillomatosis, elongation of rete ridges, filiform interconnecting pattern, and increased pigmentation in the basal layer [Figure 5]. The dermis has dilated capillaries and only sparse perivascular lymphocytic infiltrate. Routine laboratory test were within normal limits. Clinicohistopathological findings confirmed the diagnosis of nevoid hyperkeratosis of nipple. Radiofrequency ablation was done with no recurrence of lesion after 1-year follow-up [Figure 6].
Figure 5: Histopathology showing hyperkeratosis, acanthosis, papillomatosis, elongation of rete ridges, filiform interconnecting pattern. Dermis has dilated capillaries and only sparse perivascular lymphocytic infiltrate (H and E, ×10)

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Figure 6: Posttreatment showing complete clearance

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  Discussion Top


NHNA is a rare benign skin disease. It affects both men and women. Approximately 80% cases occur in women. In females, it occurs during the second and third decades of life. Due to uncertain etiology of the disease, its treatment is challenging with a variable outcome. It is characterized by hyperpigmented verrucous thickening of the nipple and/areola present unilaterally or bilaterally.[2]

Hyperkeratosis of Nipple and Areola (HNA) was classified into three types by Levy–Frenckel in 1938 as follows:[4]

  • Type 1: Associated with epidermal nevus
  • Type 2: Associated with various dermatoses, for example, acanthosis nigricans, chronic eczema, Dariers diesease, and cutaneous T-cell lymphoma
  • Type 3: Idiopathic form and also referred to a nevoid hyperkeratosis.[5]


Perez–Izquierdo suggested alternative classification of two types:

  • Idiopathic or nevoid
  • Secondary.


Secondary type is associated with underlying disease and divided into local associated with acanthosis nigricans, verrucous nevus, and seborrheic keratosis; systemic associated with icthyosis, malignant lymphoma, and Darrier's disease; and drug related diethylstilbestrol and spironolactone.[6],[7]

In the present scenario, both the cases were classified as idiopathic, and there was no association of the cases with the underlying disease. NHNA presents as hyperkeratotic, hyperpigmented plaques of the nipple and areola. It may also present as a verrucous lesion. They may involve the nipple, areola, or both. Out of 45 cases of nevoid hyperkeratosis reviewed by Kubota et al., 17% involved the nipple, 25% the areola, and 58% both the nipple and the areola. The lesions are generally asymptomatic, but occasionally they may be itchy.[8] Histopathology findings of nevoid hyperkeratosis are suggestive of hyperkeratosis, acanthosis, papillomatosis, elongation of rete ridges, filiform interconnecting pattern, and increased pigmentation of the basal layer, and the dermis has dilated capillaries with sparse perivascular lymphocytic infiltrate. NHNA is a diagnosis of exclusion. It needs to be differentiated from epidermal nevus, acanthosis nigricans, chronic eczema, atopic eczema, seborrheic keratosis, Dariers disease, Paget's disease, basal cell carcinoma, dermatophytosis, mycosis fungoide, and Bowen's disease.[9] Mehanna et al. included a suggestion that the term “naevoid” should be replaced by idiopathic.[10] There is no definite treatment for this condition. Management options available are topical retinoids, topical calcipotriol, topical steroids, cryotherapy, carbondioxide laser, shave excision, tangential excision using radiofrequency, and areola reconstruction with a skin graft.[11] We counseled our both patients about benign nature of the disease and explained that disorder is not scary as it looks.

We report these two cases of nevoid hyperkeratosis of nipple because of rarity of this condition and malignancy mimicking lesion with patient's concern regarding cosmetic appearance of the nipple and areola.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Baykal C, Büyükbabani N, Kavak A, Alper M. Nevoid hyperkeratosis of the nipple and areola: A distinct entity. J Am Acad Dermatol 2002;46:414-8.  Back to cited text no. 1
    
2.
Ghanadan A, Balighi K, Khezri S, Kamyabhesari K. Nevoid hyperkeratosis of the nipple and/or areola: Treatment with topical steroid. Indian J Dermatol 2013;58:408.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Elloudi S, Lahlou A, Salim G, Baybay H, Rimani M, et al. Unilateral nevoid hyperkeratosis of the nipple and areola treated successfully with carbon dioxide laser. Int J Clin Dermatol Res 2016;S1:001,1-4.  Back to cited text no. 3
    
4.
Krishnan RS, Angel TA, Roark TR, Hsu S. Nevoid hyperkeratosis of the nipple and/or areola: A report of two cases and a review of the literature. Int J Dermatol 2002;41:775-7.  Back to cited text no. 4
    
5.
Shastry V, Betkerur J, Kushalappa PA. Unilateral nevoid hyperkeratosis of the nipple: A report of two cases. Indian J Dermatol Venereol Leprol 2006;72:303-5.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Burns DA. The breast. In: Burns T, Breathnach S, Cox N, Griffith C, editors. Rook's Textbook of Dermatology. 7th ed. New York: Blackwell Science; 2004. p. 67.8-7.9.  Back to cited text no. 6
    
7.
English JC 3rd, Coots NV. A man with nevoid hyperkeratosis of the areola. Cutis 1996;57:354-6.  Back to cited text no. 7
    
8.
Vestey JP, Bunney MH. Unilateral hyperkeratosis of the nipple: The response to cryotherapy. Arch Dermatol 1986;122:1360-1.  Back to cited text no. 8
    
9.
Rosman IS, Hepper DM, Lind AC, Anadkat MJ. Nevoid hyperkeratosis of the areola misinterpreted as mycosis fungoides. J Cutan Pathol 2012;39:545-8.  Back to cited text no. 9
    
10.
Mehanna A, Malak JA, Kibbi AG. Hyperkeratosis of the nipple and areola: Report of 3 cases. Arch Dermatol 2001;137:1327-8.  Back to cited text no. 10
    
11.
Vats G, Sonare D, Nevoid hyperkeratosis of the nipple and/or areola – A report of 2 cases. Index J Sci Res 2017;78.96:1451-53.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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