ARTICLE
Hyperkeratosis of the nipple and areola (HKNA) is an uncommon dermatosis
first described by Tauber in 1923 [1]. No more than 50 cases have been
reported, although it is possible that some cases may pass unnoticed.
It is characterized by uni or bilateral irregular, verrucous thickening
and brown hyperpigmentation of the nipple or/and areola.
It has been classically classified by Levy-Frankel [2] in three types:
A) extension of an epidermal nevus; B) associated with other skin diseases
(ichthyosis, acanthosis nigricans, Darier's disease, ichthyosiform erythroderma,
T-cell lymphoma, chronic eczema); and C) an isolated nevoid form. Some
authors suggested another fourth type associated with estrogen (diethylstilbestrol)
and spironolactone therapy [3].
We report herein a case of the isolated nevoid form of hyperkeratosis
of the nipple and areola in which histopathological findings resembled
mycosis fungoides.
Case report
A 21-year-old woman presented to the Department of Dermatology with
a two-year history of asymptomatic, irregular thickening of both areolas.
Her medical history was unremarkable (no pregnancies) and she had not
been taken any medication or oral contraceptive.
On physical examination verrucous, ill-defined plaques with brown-gray
pigmentation were observed in the areolas and extending to the nipples
(Figs. 1 and 2). No
lymphadenopathies were encountered.
A skin biopsy specimen showed papillomatosis, elongated rete ridges,
associated with slight acanthosis and focal hyperkeratosis (Fig.
3). There was also a moderate superficial dermal lymphocytic infiltrate.
The presence of cell aggregates in the epidermis was noteworthy, composed
of lymphocytes and Langerhans cells, resembling the so-called Pautrier's
microabscesses (Fig. 4).
These cells did not have an abnormal size or shape, and it was not associated
with spongiosis. Papillary dermal fibrosis was also observed. Clonal analysis
using polymerase chain reaction (PCR) did not give any reliable data.
Laboratory and instrumental examinations (chest X-ray, CT scan) were all
normal. An extensive workup did not show any other involvement.
Topical tretinoin was given with moderate improvement. Two years after
the diagnosis, areolar lesions have remained without any significant changes
and no other lesions have appeared.
Discussion
Nevoid hyperkeratosis of the nipple and areola (NHNA) appears predominantly
in female (80%) patients in the second or third decade, as slowly growing
irregular verrucous plaques, almost always with a bilateral distribution
[4]. It may affect the nipple or areola alone, or both. It is usually
asymptomatic, although moderate pruritus has been reported. It has an
excellent prognosis being only of cosmetic importance. The condition may
worsen with pregnancy and it may make breast feeding difficult [5]. Usually,
after a period of growth, the lesions remain static and asymptomatic.
Histological features of NHNA are not specific [2-5], sharing similarities
with epidermal nevus and acanthosis nigricans. It is characterized by
variable degrees of acanthosis, hyperkeratosis, keratin plugging, papillomatosis,
and hyperpigmentation. Dermal inflammatory infiltrate, if present, is
sparse.
Treatment is usually unsatisfactory. Keratolytic agents (6% salicylic
acid gel, 12% lactic acid lotion), topical tretinoin and cryotherapy,
have been used with good results in some cases [6, 7].
The cause of NHKNA is unknown. Endocrine factors have been advocated,
because it may worsen in pregnancy and it has been associated with estrogen
therapy [3]. Some authors suggested it may be a localized form of acanthosis
nigricans [8]. An association with human papillomavirus was encountered
in one case [9].
This case is remarkable because although the clinical presentation was
characteristic, histopathological findings resembled mycosis fungoides.
Lymphocytes and Langerhans cells were disposed within the epidermis mimicking
the so-called Pautrier's microabscesses, associated with dermal
fibrosis. However, the shape and size of lymphocytes were normal, and
the rest of the clinical and laboratory studies and clonal analysis did
not show any data suggestive of cutaneous lymphoma. Finally, lesions have
remained unchanged for two years and no other lesions have appeared. Only
in one other case of NHNA [10] were intraepidermal lymphocytic microabscesses
observed, but associated with spongiosis and itching.
There are two reports in the literature in which cutaneous T-cell lymphoma
was associated with hyperkeratosis of the nipple and areola [11, 12],
but both patients had generalized skin erythematous plaques and lymph
node involvement.
On the other hand, verrucous lesions are among the rarest morphological
appearance of cutaneous T-cell lymphoma. They occur specially in the palms
and soles. Verrucous changes of the nipple and areola have been described
in one woman with mycosis fungoides and follicular mucinosis [13], and
associated with erythrodermic MF [14], but it has never been described
located exclusively in the nipple and/or areolas.
In conclusion, we report a case of nevoid hyperkeratosis of the areola
with histopathological features that resembled mycosis fungoides.
We emphasise that these patients should be studied carefully to discard
any associated skin disorder, specially a malignant disease as cutaneous
T-cell lymphoma.
Article accepted on 3/7/01
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