Naevus sebaceous papilliferum
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh
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Rai R, Saraswat A, Kumar B. Naevus sebaceous papilliferum. Indian J Dermatol Venereol Leprol 2002;68:148-149
AbstractAn unusual form - large linear lobulated-of naevus sebaceous on the ear is reported in a 40-year-old man. It simulated syringocystadenoma papillitferum clinically.
Sebaceous naevi are circumscribed hamartomatous lesions comprising of sebaceous glands and are found in 10% of all neonates. Traditionally they are described as circumscribed, only slightly raised, pinkish, yellow, orange, or tan hairless plaques with a smooth or somewhat velvety surface. The great majority are said to occur on the head and neck favouring the scalp, areas around the ears, the ears, the temples, forehead and the central part of face. Towards puberty these lesions become more thickened and elevated. A case of cerebriform sebaceous naevus has been described in literature in which the surface of the lesion showed pronounced furrows. We present a patient with a markedly lobulated linear naevus sebaceous provisionally diagnosed as syringocystadenoma papilliferum due to its clinical appearance of exophytic linear grouped nodules.
A 40 - year -old man presented with a linear lobulated growth over the pinna and retroauricular region of his right ear. The lesion was present since birth. Initially it was a 2.5 x 2.5 cm verrucous growth in the retroauricular region, which over the years by gradual extension attained a size of 8x4.5 cm [Figure - 1]. The lesion continued to extend further in the post pubertal phase and the patient gave a history of a characteristic smell emanating from the lesion accompanied by an occasional whitish discharge. There was no family history of a similar lesion.
Examination revealed a large skincoloured linear lobulated lesion measuring 8 x 4.5 cm, situated in the right retroauricular area and extending on to the helix of the right ear. It was made up of multiple smooth grouped exophytic nodules which showed no overlying skin changes.
The lesion was excised taking care to preserve the normal contour of the ear. During surgery it was found to be closely adherent to the pinna, although we were able to excise the growth without breaching the perichondrium. The base was allowed to heal by granulation. Histopathology of the excised tissue revealed marked papillomatosis with hyperkeratosis and acanthosis of the epidermis.The dermis showed extensive hyperplasia of sebaceous glands. Features were consistent with those of naevus sebaceous. The whole lesion was examined for the histological presence of cystic invaginations with dense plasma cell infiltrate suggestive of syringocystadenoma papilliferum, but no such areas were found.
On follow-up after two weeks, the base of the lesion had healed well. Electrodesiccation of a few areas of remnant tissue helped us to achieve a cosmetically acceptable result. The patient is on regular follow - up. Two months post -surgery,there has been no recurrence of the lesion.
Our case was unusual due to the large size and linear lobulated appearance of the lesion of naevus sebaceous which has not been described earlier. Continued growth of the lesion after puberty and extension and fixation to the cartilage of the ear was unusual too. Morphologically, naevus sebaceous is said to have a yellow or velvety surface as compared to the pink and nodular syringocystadenoma papilliferum. Both can have a linear configuration although it is more common in syringocystadenoma papilliferum. Development of syringocystadenoma papilliferum in lesions of naevus sebaceous is seen in 8-19% cases. This is clinically suggetsed by rapid growth of the lesion with increased nodularity of its surface. A diligent search of the excised lesion from our patient failed to reveal any areas histopathologically resembling syringocystadenoma paplliferum. To the best of our knowledge, a large linear lobulated sebaceous naevus of this nature clinically resembling syringocystadenoma papilliferum has not been described earlier. We would like to christen the lesion naevus papilliferum.
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