The Spectrum of Epidermal Nevi: A Case of Verrucous Epidermal Nevus Contiguous with Nevus Sebaceus

During the normal development of skin, pluripotential cells give rise to keratinocytes, sebaceous glands, hair follicles, apocrine glands, and eccrine glands. In epidermal nevi, these components emerge in an abnormal mixture within a circumscribed site. Many authors have categorized epidermal nevi based on their predominant component; however, there is often notable overlap that occurs within a single area or within contiguous areas. We report a verrucous epidermal nevus contiguous to a nevus sebaceus of Jadassohn. The categories of epidermal nevi are somewhat artificial. Our case supports the view that epidermal nevi have a spectrum of manifestations, including verrucous epidermal nevi and nevus sebaceus of Jadassohn.     

Sebaceous gland neoplasia

Neoplasms with sebaceous differentiation cover a broad spectrum. They range from benign sebaceous hyperplasia and adenomas to locally infiltrative sebaceous epitheliomas which have biologic behaviors similar to basal cell carcinomas, to sebaceous carcinomas which can have very malignant biologic behaviors. In this paper, we have purposely excluded the nevus sebaceus of Jadassohn, a nevoid tumor composed of several cutaneous elements. As sebaceous glands are only one part of this tumor, it is better classified as an organoid nevus.     

Syringocystadenoma papilliferum with sebaceous differentiation in an intradermal tubular apocrine component. Report of a case

Syringocystadenoma papilliferum is a benign tumor most commonly located on the scalp or face, and it often arises within a nevus sebaceus of Jadassohn. We describe a 61-year-old man with syringocystadenoma papilliferum developing within a nevus sebaceus with sebaceous differentiation in an intradermal tubular apocrine component of the syringocystadenoma papilliferum. Although some adnexal neoplasms that develop in association with a nevus sebaceus may exhibit conjoint sebaceous, follicular, or apocrine differentiation, reflecting close embryological relations of the folliculosebaceous-apocrine unit, the feature we report on has not previously been described to the best of our knowledge.     

Basal Cell Carcinoma and Syringocystadenoma Papilliferum Arising in Nevus Sebaceous on Face—A Rare Entity

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprising of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual to develop two different types of tumor, benign and malignant, to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 27-year-old male with two neoplastic proliferations including a syringocystadenoma papilliferum a benign tumor and basal cell carcinoma a malignant tumor arising in a long-standing nevus sebaceus on the face. Neoplastic changes are common in nevus sebaceous present on scalp but our case is unique due to the presence of two different types of neoplasm in a nevus sebaceous which was present on face.     

Cutaneous spindle cell squamous cell carcinoma in nevus sebaceous

A case of a 28 year-old Chinese male who presented with a rapidly growing tumor within a nevus sebaceous on his right cheek. The tumor was excised and immunohistochemical analysis and histology were consistent with spindle-cell squamous cell carcinoma. To the best of our knowledge, it is the first report of cutaneous spindle-cell squamous cell carcinoma developing in nevus sebaceous in the English literature. This report highlights the importance of early excision of any nevus sebaceous with a history of change.     

Basal cell carcinoma appearing in a facial nevus sebaceous of Jadassohn: dermoscopic features

The nevus sebaceous of Jadassohn usually affects the face or scalp. It tends to evolve in three stages, and the final stage is characterized by the appearance of tumours. We present the case of a facial nevus sebaceous of Jadasshon in which a basal cell carcinoma developed. We also explore the diagnosis of this disease, which was established through dermoscopy, and propose using this technique in the clinical follow-up of this type of hamartoma, thereby allowing the early detection of cancer development.     

Nevus sebaceous revisited

Nevus sebaceous of Jadassohn is a hamartoma with a combination of abnormalities of the epidermis, hair follicles, and sebaceous and apocrine glands. Herein, we discuss the results of an extensive literature review on the topic of nevus sebaceous with a particular focus on the debate about the necessity for prophylactic excision. We also focus on the documentation of associated malignant tumors that were reported to develop within NS. In addition to reporting the number and types of neoplasms, we documented the recommendations of all authors for therapeutic handling of these nevi.     

Trichoblastoma in a Child

Abstract:   A trichoblastoma is a benign adnexal neoplasm composed of follicular germinative cells. It most commonly presents as a solitary nodule on the scalp, face, or perineum and occurs equally in males and females. It is diagnosed most commonly between the fourth and the sixth decades, and it is extremely rare in childhood. To our knowledge there is no prior case report of a primary trichoblastoma occurring outside the setting of a nevus sebaceous of Jadassohn in a child before adolescence. Herein we report a case of trichoblastoma of the right cheek in an 11-year-old Korean girl.     

Squamous cell carcinoma and basal cell carcinoma arising in a naevus sebaceous of Jadassohn: case report and literature review

The development of a basal cell carcinoma within a naevus sebaceous of Jadassohn (NSJ) has commonly been reported. However, the development of a squamous cell carcinoma (SCC) is rare. There have been several reports of SCC arising within a NSJ but many contain little or ambiguous clinical information. A thorough review of the current English language literature has identified only three other well documented cases of SCC arising in a NSJ. Of these only one was a case of simultaneous occurrence of squamous and basal cell carcinoma. We report the second case of both malignancies arising within the same NSJ.     

Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases

Nevus sebaceus of Jadassohn is a hamartoma that combines epidermal, follicular, sebaceous, and apocrine gland abnormalities. Classically, several types of cutaneous neoplasms have been associated with this hamartoma, with basal cell carcinoma being the most frequently described malignancy. We studied a series of 155 examples of nevus sebaceus of Jadassohn with clinicopathologic correlation. Several histopathologic changes related to the age of the patients were found. In our series, we could not identify any cases of authentic basal cell carcinoma. In contrast, several examples of primitive follicular induction and of trichoblastomas were seen. Other cutaneous hamartomas, hyperplasias, and neoplasms found in our series of nevus sebaceus of Jadassohn included syringocystadenoma papilliferum, sebomatricoma, apocrine gland cyst, poroma, different histopathologic variants of warts (classic warts, tricholemmoma, and desmoplastic tricholemmoma), primitive follicular induction, and ductal induction. In our series, no examples of malignant neoplasms were identified. On the basis of these findings, the classically recommended treatment for this hamartoma, which consists of early excision to prevent the development of malignancy, seems to be inappropriate.     

Sebaceous nevus of Jadassohn: review and clinical-surgical approach

BackgroundNevus sebaceous of Jadassohn is defined as a rare congenital malformation characterized as a non-hereditary hamartoma of the adnexal structures of the skin. Its etiology is not yet well understood, but it is believed to be related to post-zygotic mutations in the HRAS, NRAS and KRAS genes.ObjectiveTo describe the clinical manifestation of nevus sebaceous, as well as the main management techniques addressed in the medical literature. Moreover, the present study discusses a case report of a congenital linear nevus in the left retroauricular region found in a male patient, without extracutaneous manifestations.MethodA narrative review of the literature was carried out.DiscussionNevus sebaceous occurs as lesions with a linear or oval appearance, with a smooth or verrucous texture, generally alopecic and with very variable color. Moreover, nevus sebaceous is one of the components of the so-called linear nevus syndrome or Schimmelpenning-Feuerstein-Mims syndrome, which is associated with multisystemic complications. The treatment of the lesions is still controversial; however, most experts indicate surgical excision as the most frequently adopted treatment method, in addition to multidisciplinary follow-up when the diagnosis of Schimmelpenning-Feuerstein-Mims syndrome is established.ConclusionThe linear nevus syndrome constitutes a rare manifestation; however, its diagnosis should be considered in children born with nevus sebaceous. There is no consensus yet on the best therapy, but surgical removal has shown to be a viable option.     

Apocrine cystadenoma arising in a nevus sebaceus of Jadassohn

An increased incidence of benign and malignant adnexal tumors has been noted to occur within the nevus sebaceus of Jadassohn. We report a case of a 41-year-old white woman with a blue cyst on her scalp which had recently developed within a congenital "birthmark." Excision of the lesion revealed that the nodule was an apocrine cystadenoma within a nevus sebaceus of Jadassohn. The clinical features of nevus sebaceus with apocrine cystadenoma are discussed and the various tumors which may arise within the nevus sebaceus are reviewed.     

Sebaceous carcinoma arising in nevus sebaceus of Jadassohn: a clinicopathological study of five cases

The authors report a small series of sebaceous carcinoma developing in nevus sebaceus of Jadassohn (also known as organoid nevus) and analyze similar cases reported in the literature. All of our patients were females (age range 57-71 years; median 60 years) who had the organoid nevus on the scalp, face, or nuchal area, the rest of which was recognized clinically and/or histologically. All sebaceous carcinomas manifested unquestionable architectural (asymmetry, invasive growth) and cytological features of a carcinoma (cellular and nuclear pleomorphism, plentiful atypical mitoses, necroses en masse), demonstrated unambiguous sebaceous differentiation in the form of mature sebocytes, and lacked other differentiations. The sebaceous carcinoma was always accompanied by other benign or malignant adnexal lesions such as sebaceoma, syringocystadenoma papilliferum, syringocystadenocarcinoma papilliferum, trichoblastoma, tricholemmoma, desmoplastic tricholemmoma, or syringoma. In three cases, prominent mucinous metaplasia of sweat ducts and glands was seen. In two of these cases, sweat ducts exhibited hyperplastic changes. The analysis of the previously published material and our cases indicates that sebaceous carcinoma arising in organoid nevus has a female predilection and tends to occur in elderly patients. It may involve any site where nevus sebaceus typically occurs. Clinically, the tumor presents as a solitary nodule, ulcerated tumor, or mass, often with a recent history of rapid growth. It may arise alone, but it occurs more frequently as part of multiple benign and malignant adnexal tumors. The lesion does not seem to be associated with Muir-Torre syndrome. The rest of organoid nevus is usually recognized both clinically and microscopically, although large tumors may overgrow and mask the nevus. The tumor seems to be a low-grade carcinoma in terms of clinical behavior.     

Immunohistochemistry in ocular carcinomas

Background:  The distinction between ocular sebaceous carcinoma, poorly differentiated ocular squamous cell carcinoma and ocular basal cell carcinoma can be challenging. An appropriate immunohistochemical panel may help to differentiate these lesions.
Methods:  To determine the distribution and use of several immunostains in these specimens, formalin-fixed, paraffin-embedded tissue from several of each was studied using an immunohistochemical technique.
Results:  Positive staining for cytokeratin (CK)7 was seen in 100% of sebaceous carcinomas, 77.8% of basal cell carcinomas and 67.7% of squamous cell carcinomas. One hundred percent of sebaceous and basal cell carcinomas were positive for cytokeratin CAM 5.2, while only 83.3% of squamous cell carcinomas were positive. Using epithelial membrane antigen (EMA), 100% of squamous cell carcinomas and 80% of sebaceous carcinomas were positive, while basal cell carcinomas were uniformly negative. One hundred percent of basal cell carcinomas and 80% of sebaceous carcinomas were positive for Ber-EP4, while all squamous cell carcinomas were negative. Finally, 77.8%, 20% and 16.7% of basal cell carcinomas, sebaceous carcinomas and squamous cell carcinomas showed immunoreactivity for the androgen receptor.
Conclusion:  An EMA positive, Ber-EP4 positive immunophenotype supports sebaceous carcinoma, EMA positive, Ber-EP4 negative result supports squamous cell carcinoma and an EMA negative, Ber-EP4 positive result supports basal cell carcinoma.     

Apocrine carcinoma developed in nevus sebaceus of Jadassohn

Apocrine skin carcinoma (ASC) is a rare aggressive cutaneous tumor. We describe a case of ASC occurring on a nevus sebaceus of Jadassohn (NSJ) in a 66-year-old man. Malignant degeneration of NSJ is a rare complication usually found in elderly patients. Most of these tumors are basal cell carcinomas or squamous cell carcinomas. Only five cases of ASC on NSJ have been previously reported. This report recalls the potential of NSJ for degeneration.     

Congenital panfollicular nevus: report of a new entity

The various forms of non-melanocytic nevi (hamartomas) are usually encountered in pediatric patients, and nevus sebaceous of Jadassohn is the most common to have undifferentiated pilosebaceous units. We report a unique congenital follicular nevus that fails to meet the criteria of any previously described follicular neoplasm, despite the plethora of alternatives. Clinically considered a syringocystadenoma papilliferum, the excised lesion contained multiple dermal nodules that exhibited nearly all stages of follicular differentiation. The periodicity of the follicular proliferations was akin to normal terminal hair, and a prominent perifollicular sheath surrounded each. This benign lesion of abortive hair follicles was unassociated with any established genodermatous syndrome or other adnexal neoplasm.     

Nevus sebaceous syndrome with facial hemihypertrophy

Abstract:  Nevus sebaceous syndrome is a member of the epidermal nevus syndromes group, and is characterized by extensive nevus sebaceous, seizures, and mental retardation. We present an affected 5-month-old boy who had facial hemi-hypertrophy and recurrent seizures.     

Multiple tumors arising in nevus sebaceus.

A forty-three year old woman with nevus sebaceus of Jadassohn involving the scalp is described. The unusual features of this case include the presence of adenoidal basal cell epithelioma, pigmented basal cell epithelioma, syringocystadenoma papilliferum, and nevocytic nevus. This case suggests the importance of prophylactic removal and close follow-up of nevus sebaceus.     

Epidermal nevus syndrome and didymosis aplasticosebacea

Abstract:  The epidermal nevus syndrome is a disease complex consisting of the association of an epidermal nevus with various developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular, and urogenital systems. The epidermal nevi are classified according to their predominant component; nevus sebaceus (sebaceous glands), nevus comedonicus (hair follicles), and nevus verrucosus (keratinocytes). We report a neonate who presented with a nevus sebaceus on the scalp and face as well as a coloboma and dermoid on his left eye. Within the sebaceous nevus on the scalp, circumscribed lesions of aplasia cutis congenita were detected, which is consistent with the recently proposed term in the literature didymosis aplasticosebacea.