Syringocystadenoma papilliferum developing over hyperkeratosis of the nipple in a pregnant woman
Authors: Ghina Ghazeeri, Ossama Abbas
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: Syringocystadenoma papilliferum (SCAP) is usually a solitary lesion that most commonly occurs on the scalp.1Shindo M. Yamada N. Yoshida Y. Yamamoto O. Morino S. Syringocystadenoma papilliferum on the male nipple.J Dermatol. 2011; 38: 593-596Crossref PubMed Scopus (9) Google Scholar Rarely, lesions have been described on the breast.1Shindo M. Yamada N. Yoshida Y. Yamamoto O. Morino S. Syringocystadenoma papilliferum on the male nipple.J Dermatol. 2011; 38: 593-596Crossref PubMed Scopus (9) Google Scholar It usually presents at birth or develops in childhood as a warty crusted papule. Although there is increasing evidence for apocrine histogenesis, eccrine origin could also be a possibility as well as derivation from pluripotent cells.1Shindo M. Yamada N. Yoshida Y. Yamamoto O. Morino S. Syringocystadenoma papilliferum on the male nipple.J Dermatol. 2011; 38: 593-596Crossref PubMed Scopus (9) Google Scholar In approximately one third of cases, it is associated with organoid nevus2Cribier B. Scrivener Y. Grosshans E. Tumors arising in nevus sebaceus: a study of 596 cases.J Am Acad Dermatol. 2000; 42: 263-268Abstract Full Text Full Text PDF PubMed Scopus (363) Google Scholar; it has also been described in association with nevus comedonicus.3Lee H.J. Chun E.Y. Kim Y.C. Lee M.G. Nevus comedonicus with hidradenoma papilliferum and syringocystadenoma papilliferum in the female genital area.Int J Dermatol. 2002; 41: 933-936Crossref PubMed Scopus (25) Google Scholar Here we report, to the best of our knowledge, the first case of SCAP developing over hyperkeratosis of the nipple. A 27-year-old woman in her thirty-fifth week of first pregnancy presented with a 5-week history of enlarging asymptomatic skin lesion on the nipple of her right breast (Fig 1, A). She had had an asymptomatic verrucous hyperpigmented plaque diffusely involving the right nipple for 5 years that was diagnosed as hyperkeratosis of the nipple (HNA) based on biopsy findings of hyperkeratosis, keratotic plugging, acanthosis, papillomatosis, and a sparse perivascular lymphocytic infiltrate (Fig 1, B). The HNA was stable until a new 0.5-cm skin-colored papule developed over the HNA starting in her thirtieth week of gestation. The patient was otherwise healthy. Shave excision of the newly developed lesion revealed papillary foci in continuity with the surface squamous epithelium. These papilla had a bilayered epithelium of inner columnar cells showing decapitation secretion and outer flattened cells as well as a fibrovascular core containing abundant plasma cells (Fig 1, C and D). These findings were consistent with SCAP. First described in 1923, HNA is a benign condition of unknown origin.4Baykal C. Büyükbabani N. Kavak A. Alper M. Nevoid hyperkeratosis of the nipple and areola: a distinct entity.J Am Acad Dermatol. 2002; 46: 414-418Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar, 5Higgins H.W. Jenkins J. Horn T.D. Kroumpouzos G. Pregnancy-associated hyperkeratosis of the nipple: a report of 25 cases.JAMA Dermatol. 2013; 149: 722-726Crossref PubMed Scopus (18) Google Scholar It is usually bilateral, affecting young adult women and occurring as a hyperpigmented verrucous lesions on the nipple (17%), areola (25%), or both (58%).4Baykal C. Büyükbabani N. Kavak A. Alper M. Nevoid hyperkeratosis of the nipple and areola: a distinct entity.J Am Acad Dermatol. 2002; 46: 414-418Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar, 5Higgins H.W. Jenkins J. Horn T.D. Kroumpouzos G. Pregnancy-associated hyperkeratosis of the nipple: a report of 25 cases.JAMA Dermatol. 2013; 149: 722-726Crossref PubMed Scopus (18) Google Scholar Although HNA shares clinical and histopathologic features of epidermal nevus, it is usually acquired after puberty and is not associated with systemic disease. In 1938, Lévy-Franckel classified HNA into 3 types. Type 1 is an extension of epidermal nevus. Type 2 is usually associated with several other dermatoses including ichthyosis, Darier disease, or lymphoma. Type 3 is the isolated nevoid variant. Hormonal factors may contribute to its etiopathogenesis in that lesions appear or worsen in pregnancy or with hormonal therapy.5Higgins H.W. Jenkins J. Horn T.D. Kroumpouzos G. Pregnancy-associated hyperkeratosis of the nipple: a report of 25 cases.JAMA Dermatol. 2013; 149: 722-726Crossref PubMed Scopus (18) Google Scholar There are yet no reports of an association of HNA with SCAP or other tumors. The fact that HNA shows features of an epidermal nevus and may theoretically be an extension of epidermal nevus makes the development of different neoplasms, including SCAP, within it possible, similar to what has been reported with nevus sebaceous and nevus comedonicus. The basic defect producing the hamartomatous process in epidermal nevi that may result in immature cells capable of differentiating into follicular and glandular cells and tumors could also be playing a role in SCAP development over HNA. This is especially true given that a recent study demonstrated that SCAP could itself be a hamartomatous tumor arising from pluripotent cells.1Shindo M. Yamada N. Yoshida Y. Yamamoto O. Morino S. Syringocystadenoma papilliferum on the male nipple.J Dermatol. 2011; 38: 593-596Crossref PubMed Scopus (9) Google Scholar