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A Pigmented Verrucous Plaque on the Cheek—Diagnosis

A Pigmented Verrucous Plaque on the Cheek—Diagnosis Diagnosis: Nevus sebaceus with syringocystadenoma papilliferum. Microscopic findings Hematoxylin-eosin staining of the biopsy specimen revealed a papillated, thickened, and markedly pigmented epidermis with an increased number of mature sebaceous lobules of various sizes and shapes in the reticular dermis. The side of the biopsy specimen corresponding to the area of clinical change contained a cystlike space marked by papillations lined by 2 layers of cells, the luminal side of which revealed decapitation secretion consistent with apocrine differentiation. Within the fronds of stroma created by the papillations is an infiltrate of plasma cells. Discussion The nevus sebaceus is a congenital hamartoma that occurs primarily on the face and scalp.1 It is a common lesion that affects males and females equally. First described by Jadassohn in 1895, nevus sebaceus is usually depicted as an alopecic, verrucous, yellowish to yellow-orange plaque. The present case is unusual in that the lesion was darkly pigmented. Nevus sebaceus can rarely be associated with the neurocutaneous syndrome known as nevus sebaceus syndrome, which is marked by mental retardation, seizures, and ocular abnormalities in association with a linear nevus sebaceus.2 Nevus sebaceus appears to grow through 3 stages.3-5 At birth, the lesion appears flat and it remains quiescent or regresses during childhood (stage 1). During puberty, it grows larger, darker, and more verrucous (stage 2). These changes are presumably the result of the presence and then the loss of maternal androgens in the neonatal period and of the subsequent flooding of androgens during puberty. Later in adulthood, neoplasms, primarily benign, tend to grow within the markedly verrucous nevus (stage 3). Histopathologically, the lesion presents as a flat or gently papillated epidermis and demonstrates clusters of small, irregularly shaped sebaceous glands in the upper dermis during infancy. The sebaceous glands shrink in childhood (stage 1) until puberty, at which point they burgeon forth again in clusters, the epidermis becomes hyperplastic and papillated, and normal terminal hair follicles are absent (stage 2). In adulthood, the lobules enlarge, the epidermis becomes even more verrucous, apocrine glands can sometimes be found, and cysts and proliferations of epithelial cells with adnexal differentiation are common (stage 3). Neoplasms, most commonly syringocystadenoma papilliferum and trichoblastoma, arise in 5% to 14% of biopsy samples.1,6 While basal cell carcinoma has frequently been cited as the most common neoplasm that arises in nevus sebaceus, reexamination of specimens previously thought to be basal cell carcinomas has led some authors to reclassify them as trichoblastoma.1,3,7,8 Secondary neoplasms arise primarily in adulthood and most commonly in nevus sebaceus of the scalp.1 Other secondary neoplasms that may arise in nevus sebaceus include nodular hidradenoma, apocrine cystadenoma, sebaceoma, tricholemmoma, seborrheic keratosis, and keratoacanthoma. Tricholemmal, squamous cell, and sebaceous carcinomas may arise in lesions of nevus sebaceus but are rarely seen. References 1. Cribier BScrivener YGrosshans E Tumors arising in nevus sebaceus: a study of 596 cases J Am Acad Dermatol 2000;42263- 268PubMedGoogle ScholarCrossref 2. Kucukoduk SOzsan HTuranli AYDinc HSelcuk M A new neurocutaneous syndrome: nevus sebaceus syndrome Cutis 1993;51437- 441PubMedGoogle Scholar 3. Steffen CAckerman AB Neoplasms With Sebaceous Differentiation Philadelphia, Pa Lea & Febiger1994; 4. Mehregan AHPinkus H Life history of organoid nevi: special reference to nevus sebaceus of Jadassohn Arch Dermatol 1965;91574- 588PubMedGoogle ScholarCrossref 5. Ackerman ABRagaz A The Lives of Lesions: Evolution in Dermatopathology New York, NY Masson Publishing1984; 6. Chun KVasquez MSanchez JL Nevus sebaceus: clinical outcome and considerations for prophylactic excision Int J Dermatol 1995;34538- 541PubMedGoogle ScholarCrossref 7. Jaqueti GRaquena LSanchez Yus E Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases Am J Dermatopathol 2000;22108- 118PubMedGoogle ScholarCrossref 8. Kaddu SSchaeppi HKerl HSoyer HP Basaloid neoplasms in nevus sebaceus J Cutan Pathol 2000;27327- 337PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

A Pigmented Verrucous Plaque on the Cheek—Diagnosis

Archives of Dermatology , Volume 140 (11) – Nov 1, 2004

A Pigmented Verrucous Plaque on the Cheek—Diagnosis

Abstract

Diagnosis: Nevus sebaceus with syringocystadenoma papilliferum. Microscopic findings Hematoxylin-eosin staining of the biopsy specimen revealed a papillated, thickened, and markedly pigmented epidermis with an increased number of mature sebaceous lobules of various sizes and shapes in the reticular dermis. The side of the biopsy specimen corresponding to the area of clinical change contained a cystlike space marked by papillations lined by 2 layers of cells, the luminal side of which revealed...
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Publisher
American Medical Association
Copyright
Copyright © 2004 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.140.11.1393-b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Nevus sebaceus with syringocystadenoma papilliferum. Microscopic findings Hematoxylin-eosin staining of the biopsy specimen revealed a papillated, thickened, and markedly pigmented epidermis with an increased number of mature sebaceous lobules of various sizes and shapes in the reticular dermis. The side of the biopsy specimen corresponding to the area of clinical change contained a cystlike space marked by papillations lined by 2 layers of cells, the luminal side of which revealed decapitation secretion consistent with apocrine differentiation. Within the fronds of stroma created by the papillations is an infiltrate of plasma cells. Discussion The nevus sebaceus is a congenital hamartoma that occurs primarily on the face and scalp.1 It is a common lesion that affects males and females equally. First described by Jadassohn in 1895, nevus sebaceus is usually depicted as an alopecic, verrucous, yellowish to yellow-orange plaque. The present case is unusual in that the lesion was darkly pigmented. Nevus sebaceus can rarely be associated with the neurocutaneous syndrome known as nevus sebaceus syndrome, which is marked by mental retardation, seizures, and ocular abnormalities in association with a linear nevus sebaceus.2 Nevus sebaceus appears to grow through 3 stages.3-5 At birth, the lesion appears flat and it remains quiescent or regresses during childhood (stage 1). During puberty, it grows larger, darker, and more verrucous (stage 2). These changes are presumably the result of the presence and then the loss of maternal androgens in the neonatal period and of the subsequent flooding of androgens during puberty. Later in adulthood, neoplasms, primarily benign, tend to grow within the markedly verrucous nevus (stage 3). Histopathologically, the lesion presents as a flat or gently papillated epidermis and demonstrates clusters of small, irregularly shaped sebaceous glands in the upper dermis during infancy. The sebaceous glands shrink in childhood (stage 1) until puberty, at which point they burgeon forth again in clusters, the epidermis becomes hyperplastic and papillated, and normal terminal hair follicles are absent (stage 2). In adulthood, the lobules enlarge, the epidermis becomes even more verrucous, apocrine glands can sometimes be found, and cysts and proliferations of epithelial cells with adnexal differentiation are common (stage 3). Neoplasms, most commonly syringocystadenoma papilliferum and trichoblastoma, arise in 5% to 14% of biopsy samples.1,6 While basal cell carcinoma has frequently been cited as the most common neoplasm that arises in nevus sebaceus, reexamination of specimens previously thought to be basal cell carcinomas has led some authors to reclassify them as trichoblastoma.1,3,7,8 Secondary neoplasms arise primarily in adulthood and most commonly in nevus sebaceus of the scalp.1 Other secondary neoplasms that may arise in nevus sebaceus include nodular hidradenoma, apocrine cystadenoma, sebaceoma, tricholemmoma, seborrheic keratosis, and keratoacanthoma. Tricholemmal, squamous cell, and sebaceous carcinomas may arise in lesions of nevus sebaceus but are rarely seen. References 1. Cribier BScrivener YGrosshans E Tumors arising in nevus sebaceus: a study of 596 cases J Am Acad Dermatol 2000;42263- 268PubMedGoogle ScholarCrossref 2. Kucukoduk SOzsan HTuranli AYDinc HSelcuk M A new neurocutaneous syndrome: nevus sebaceus syndrome Cutis 1993;51437- 441PubMedGoogle Scholar 3. Steffen CAckerman AB Neoplasms With Sebaceous Differentiation Philadelphia, Pa Lea & Febiger1994; 4. Mehregan AHPinkus H Life history of organoid nevi: special reference to nevus sebaceus of Jadassohn Arch Dermatol 1965;91574- 588PubMedGoogle ScholarCrossref 5. Ackerman ABRagaz A The Lives of Lesions: Evolution in Dermatopathology New York, NY Masson Publishing1984; 6. Chun KVasquez MSanchez JL Nevus sebaceus: clinical outcome and considerations for prophylactic excision Int J Dermatol 1995;34538- 541PubMedGoogle ScholarCrossref 7. Jaqueti GRaquena LSanchez Yus E Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases Am J Dermatopathol 2000;22108- 118PubMedGoogle ScholarCrossref 8. Kaddu SSchaeppi HKerl HSoyer HP Basaloid neoplasms in nevus sebaceus J Cutan Pathol 2000;27327- 337PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Nov 1, 2004

Keywords: cheek,linear sebaceous nevus sequence,syringocystadenoma papilliferum

References