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International Journal of Dermatology 2002, 41, 926–927 © 2002 The International Society of Dermatology 926 A 24-year-old man was admitted to our outpatient clinic with lesions in a linear configuration. On dermatologic examination, widespread, dark brown, warty papules and plaques over an erythematous base, following Blaschko's lines, extended from the middle of the chest to the right arm. These lesions had been present on the chest since birth and had gradually extended during childhood. Recently, a nodular lesion had appeared in the pre-existing epidermal nevus in the middle part of the chest (Fig. 1). The nodular lesion was totally excised by a plastic surgeon. Pathologic examination of a section stained with hematoxylin and eosin revealed acanthosis and papillomatosis, as well as numerous sebaceous glands connected to the epidermis (Fig. 2). This histologic feature was compatible with nevus sebaceous. In addition, in the middle of the section, there was a tumor connected to the epidermis. Histologically, the tumor islands, composed of basaloid cells with mostly solid growth pattern, were compatible with basal cell carcinoma. There were also some features of nevus sebaceous, characterized by papillomatosis, and sebaceous glands attached to the epidermis were seen at the edge (Fig. 3). On systemic examination, there was no other developmental abnormality, except that the patient was mildly mentally retarded. There was no family history of a neurocutaneous disorder. All laboratory analyses were within normal limits. Blackwell Science, Ltd Oxford, UK IJD International Journal of Dermatology 0011-9059 Blackwell Science, 2002 41 Cameo Basal cell carcinoma and epidermal nevus Ceylanet al. A case of basal cell carcinoma arising in epidermal nevus Can Ceylan, MD, Fezal Özdemir, MD, Günseli Öztürk, MD, and Taner Akalın, MD From the Departments of Dermatology and Pathology, University of Ege, Medical Faculty, Izmir, Turkey Correspondence Can Ceylan, MD Ege University Medical Faculty Department of Dermatology 35100 Bornova / Izmir Turkey E-mail: [email protected] Discussion Epidermal nevi are hamartomatous lesions arising from the embryonic ectoderm. The pluripotent ectodermal cells evolve into a variety of differentiated cell types, including keratinocytes and the cells forming the various epidermal appendages. Linear epidermal nevi may be either localized or systema- tized. In the localized type, which is present usually but not invariably at birth, only one linear lesion is present. It consists of closely set papillomatous hyperkeratotic papules. It may be located anywhere on the head, trunk, or extremities. The localized type of linear epidermal nevus resembles the inflam- matory linear verrucous epidermal nevus (ILVEN) in con- figuration; however, the latter differs clinically by the presence of erythema, pruritus, and crusting, and histologically by the presence of inflammation and parakeratosis. Figure 1 Basal cell carcinoma (white arrow) appearing on the epidermal nevus in the middle part of the chest Figure 2 Acanthosis and papillomatosis, as well as numerous sebaceous glands connected to the epidermis (hematoxylin and eosin stain; original magnification, × 40)

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  • International Journal of Dermatology

    2002,

    41

    , 926927 2002

    The International Society of Dermatology

    926

    A 24-year-old man was admitted to our outpatient clinic with lesions in a linear configuration.

    On dermatologic examination, widespread, dark brown, warty papules and plaques over an

    erythematous base, following Blaschko's lines, extended from the middle of the chest to the right

    arm. These lesions had been present on the chest since birth and had gradually extended

    during childhood. Recently, a nodular lesion had appeared in the pre-existing epidermal nevus

    in the middle part of the chest (Fig. 1). The nodular lesion was totally excised by a plastic

    surgeon.

    Pathologic examination of a section stained with hematoxylin and eosin revealed acanthosis

    and papillomatosis, as well as numerous sebaceous glands connected to the epidermis (Fig. 2).

    This histologic feature was compatible with nevus sebaceous. In addition, in the middle of the

    section, there was a tumor connected to the epidermis. Histologically, the tumor islands,

    composed of basaloid cells with mostly solid growth pattern, were compatible with basal

    cell carcinoma. There were also some features of nevus sebaceous, characterized by

    papillomatosis, and sebaceous glands attached to the epidermis were seen at the edge (Fig. 3).

    On systemic examination, there was no other developmental abnormality, except that the

    patient was mildly mentally retarded. There was no family history of a neurocutaneous disorder.

    All laboratory analyses were within normal limits.

    Blackwell Science, LtdOxford, UKIJDInternational Journal of Dermatology0011-9059Blackwell Science, 200241

    Cameo

    Basal cell carcinoma and epidermal nevus

    Ceylan

    et al.

    A case of basal cell carcinoma arising in epidermal nevus

    Can Ceylan,

    MD

    , Fezal zdemir,

    MD

    , Gnseli ztrk,

    MD

    , and Taner Akaln,

    MD

    From the Departments of Dermatology and Pathology, University of Ege, Medical Faculty, Izmir, Turkey

    Correspondence

    Can Ceylan,

    MD

    Ege University Medical FacultyDepartment of Dermatology35100 Bornova/IzmirTurkeyE-mail: [email protected]

    Discussion

    Epidermal nevi are hamartomatous lesions arising from theembryonic ectoderm. The pluripotent ectodermal cells evolveinto a variety of differentiated cell types, including keratinocytesand the cells forming the various epidermal appendages.

    Linear epidermal nevi may be either localized or systema-tized. In the localized type, which is present usually but notinvariably at birth, only one linear lesion is present. It consists

    of closely set papillomatous hyperkeratotic papules. It maybe located anywhere on the head, trunk, or extremities. Thelocalized type of linear epidermal nevus resembles the inflam-matory linear verrucous epidermal nevus (ILVEN) in con-figuration; however, the latter differs clinically by the presenceof erythema, pruritus, and crusting, and histologically by thepresence of inflammation and parakeratosis.

    Figure 1 Basal cell carcinoma (white arrow) appearing on the epidermal nevus in the middle part of the chest

    Figure 2 Acanthosis and papillomatosis, as well as numerous sebaceous glands connected to the epidermis (hematoxylin and eosin stain; original magnification, 40)

  • 2002

    The International Society of Dermatology International Journal of Dermatology

    2002,

    41

    , 926927

    927

    Ceylan

    et al. Basal cell carcinoma and epidermal nevus

    Cameo

    Squamous cell carcinomas,

    1

    verrucous

    2

    and adnexal

    3

    carci-nomas, and Bowens disease,

    4

    as well as basal cell carcinoma,

    5

    have been reported within epidermal nevi. The diagnosisof basal cell carcinoma is seldom made in patients youngerthan 40 years of age; however, these malignant tumors arisingwithin epidermal nevi have been described in patients asyoung as 17 years,

    6

    similar to our patient.The dermatologic conditions that predispose a patient to

    the development of basal cell carcinoma include Jadassohnssebaceous nevus, albinism, xeroderma pigmentosum, Ras-mussens syndrome, Rombo syndrome, Bazexs syndrome,and basal cell nevus syndrome, as well as linear epidermalnevus.

    79

    A number of different opinions have been suggestedregarding the histogenesis of basal cell carcinomas. Adam-son

    10

    stated that basal cell carcinomas are nevoid tumorsoriginating from latent embryonic foci aroused from theirdormant state at a later period in life. He believed that thelatent embryonic foci usually are embryonic pilosebaceousfollicles, but occasionally are embryonic sweat ducts.

    On the other hand, Pinkus

    11

    suggested that basal cell carci-nomas occurring later in life do not arise from dormantembryonic primary epithelial germ cells, but from pluripotentcells that form continuously during life and, like embryonicprimary epithelial germ cells, have the potential to form hair,sebaceous glands, and apocrine glands. Because epidermalnevi also arise from the pluripotent cells in the basal layer ofthe embryonic epidermis, the theories mentioned above mayexplain the coexistence of epidermal nevus and basal cellcarcinoma.

    In the biopsy specimen of our patient, in addition tothe histologic features of epidermal nevus, numerous maturesebaceous glands in the upper dermis were seen, similar to

    nevus sebaceous. This coexistence indicates that, during thenormal development of skin, pluripotent cells give rise tokeratinocytes, sebaceous glands, hair follicles, apocrine glands,and eccrine glands. In epidermal nevi, these componentsemerge in an abnormal mixture within a circumscribed site.

    12

    In conclusion, whether the basal cell carcinoma has devel-oped in epidermal nevus or in nevus sebaceous is contro-versial. Nevertheless, the coexistence of basal cell carcinoma,epidermal nevus, and nevus sebaceous is significant, becauseall may arise from the same pluripotent cells in the embryonicectoderm.

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    Figure 3 Basal cell carcinoma characterized by tumor islands composed of basaloid cells, and a component of nevus sebaceous, the sebaceous glands being attached to the epidermis (hematoxylin and eosin stain; original magnification, 40)