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Competing Interests: Conflicts of interest None disclosed.

There are a multitude of diseases that commonly affect both the skin and the eye. Part II of this 2-part series reviews the oculocutaneous manifestations of neoplasms, both benign and malignant, and adverse drug reactions affecting the skin and the eye. Though rare, a number of neoplasms that primarily involve the skin, such as melanoma and basal cell carcinoma, can metastasize to the eye, leading to permanent damage if not properly treated. In addition, periocular neoplasms can irritate the conjunctiva and lid, reducing a patient's ability to see clearly. Neoplastic diseases, such as xeroderma pigmentosum, Sturge-Weber syndrome, and multiple myeloma, can also lead to permanent changes in the eye if not discovered and managed promptly. Furthermore, there are a multitude of drugs, including those commonly used by dermatologists, which can result in permanent damage to the eye. With proper knowledge of the ocular manifestations and treatment recommendations described in this 2-part series, dermatologists with the assistance of their ophthalmology colleagues can help avoid the complications, including permanent blindness, associated with infectious, inflammatory, genetic, neoplastic, and drug-related conditions., (Copyright © 2014 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Sebaceous neoplasms have long been a source of confusion to dermatologists and pathologists alike. Disagreements regarding nomenclature, classification, and management have been longstanding. Sebaceous lesions represent a broad spectrum of interesting entities that range from hamartomas, hyperplasias, and benign tumors to highly malignant neoplasms. This article discusses the clinical and pathologic features of sebaceous hyperplasia, nevus sebaceous of Jadassohn, sebaceous adenoma, seboacanthoma, sebaceous epithelioma, sebaceoma, mantleoma, basal cell carcinoma with sebaceous differentiation, sebomatricoma (sebomatrixoma), and sebaceous carcinoma. Controversies regarding these lesions will be explored, and any relationship with Muir-Torre syndrome will be discussed.

Nevus sebaceous is a tumor of primary epithelial germ cells that is generally first noticed at birth. It is relatively quiescent until puberty when a variety of benign and potentially malignant tumors may arise within it. We describe a nevus sebaceous with immature fat cells, vascular ectasia, and extramedullary hematopoiesis that was present at birth. At 4 months, the immature adipose and hematopoietic elements, as well as the vascular changes, had resolved.

Background: Carcinoembryonic antigen (CEA) is a well-known marker for sweat gland differentiation in adnexal neoplasms., Objective: The aim of this study was to examine the expression of glycoproteins of the CEA family, that is, CEA-180, nonspecific cross-reacting antigens (NCAs), and biliary glycoprotein (BGP), in sebaceous glands and in neoplasms with sebaceous differentiation., Methods: Normal adult and fetal skin, hyperplasias, hamartomas, and neoplasms with sebaceous or follicular differentiation were stained immunohistochemically with a panel of polyclonal and monoclonal antibodies highly specific for CEA-180, NCAs, and BGP. Double immunostaining was performed to correlate the CEA expression with that of epithelial membrane antigen (EMA), a glycoprotein consistently found in differentiating sebocytes., Results: Whereas sweat glands coexpressed CEA, NCAs, BGP, and EMA, sebaceous glands were exclusively labeled with the antibodies recognizing BGP or EMA. Staining of the sebaceous glands was restricted to mature sebocytes, sparing immature cells. At the ultrastructural level immunoreactivity for BGP and EMA was demonstrable in the golgi area, in small vesicles, and along the cell membranes. During fetal development BGP was not found until the sebaceous glands matured. The expression of BGP and EMA was highly conserved in reactive, hamartomatous, and neoplastic proliferations of adnexal structures with sebaceous differentiation., Conclusion: The expression of BGP, a CEA glycoprotein, in differentiating sebocytes accounts for the reactivity of many anti-CEA antibodies with sebaceous glands and thus disqualifies the CEA family as a monospecific marker for sweat gland differentiation.

Background: Sebaceous epithelioma is a benign tumor that must be differentiated from basal cell carcinoma and other appendageal tumors., Objective: Histologic and clinical findings were reviewed in 21 patients with sebaceous epitheliomas., Methods: Twenty-one cases of sebaceous epithelioma collected during a 5-year period were reviewed., Results: The tumors appeared as yellow to flesh-colored papules, most often on the face and neck. They consisted of basaloid cells with foci of sebaceous differentiation. Cystic spaces formed by holocrine degeneration or simulating sebaceous duct lining were common findings. Patients with sebaceous epitheliomas were more likely than the general population to have an internal malignancy., Conclusion: Patients with sebaceous epithelioma should be examined for an underlying malignancy.

It is important that clinicians suspect malignancy in patients who have persistent or nonhealing periorbital lesions. Basal cell carcinoma, squamous cell carcinoma, and sebaceous gland carcinoma of the eyelids are frequently misdiagnosed clinically and, in the case of squamous cell and sebaceous gland carcinomas, histologically as well. Prompt and adequate biopsy can facilitate early diagnosis and thus avoid unnecessary metastatic spread in the case of squamous cell carcinoma and sebaceous gland carcinoma, as well as the extensive local destruction that basal cell carcinoma may produce.

We describe a patient with both Muir-Torre syndrome and alpha 1-antitrypsin deficiency. A keratoacanthoma developed after immunosuppressive therapy for necrotizing vasculitis. To our knowledge, this is the first reported case of Muir-Torre syndrome associated with alpha 1-antitrypsin deficiency.

Two patients with multiple sebaceous adenomas associated with visceral carcinoma (Torre's syndrome) are described, and reported cases in the literature are summarized. Certain aspects of Torre's syndrome are emphasized: that the presence of multiple sebaceous adenomas is a cutaneous marker for visceral carcinomas, that the associated carcinomas are of comparatively low-grade malignancy, and that it is a genetic condition probably related to the Cancer Family syndrome. In addition, three morphologic patterns of the sebaceous adenoma are described: solid, cystic, and "keratoacanthoma-like"; then, the potential pitfalls in the histopathologic interpretation of this tumor are discussed.

Meibomian carcinoma is rare but carries a grave prognosis. The lesion may be easily mistaken for inflammatory condition. Therefore, any nodular or recurrent lesion on the eyelid of an elderly person should be examined histologically. A case is presented in which the tumor was successfully treated by the Mohs microscopically controlled excision.

Tumors of the ceruminous glands may involve the auricular and preauricular skin and thus should be included in the differential diagnosis of neoplasms involving these areas. A rational decision as to the management of ceruminous gland tumors depends on the precise histologic classification of these tumors and the extent of involvement. We present seven patients with tumors of the ceruminous glands: ceruminous adenoma in one, ceruminous adenocarcinoma in three, and adenoid cystic carcinoma in three. The literature is reviewed with respect to signs and symptoms, pathology, and therapy.

One of the two types of complications that may occur with organoid nevus (nevus sebaceus) is the category of brain malformations and seizure disorders. The second type is the late occurrence of various epithelial growths that are usually observed to develop in long-standing organoid nevi. Among these later-occurring lesions, we are describing the occurrence of proliferating trichilemmal cysts in association with organoid nevi.

Torre's syndrome is characterized by the association of sebaceous neoplasms and keratoacanthomas with visceral malignancies. We report on a patient in whom the cutaneous manifestations of Torre's syndrome dramatically increased following immunosuppression.

Two representative cases of familial Muir-Torre syndrome are presented. Multiple benign sebaceous neoplasms in both cases and a solitary keratoacanthoma in one were successfully treated with oral isotretinoin. Low-dose maintenance therapy has stabilized the cutaneous manifestations in the two patients, and no new epithelial neoplasms have appeared. This report emphasizes (1) the rationale for the use of isotretinoin in the Muir-Torre syndrome and (2) the potential for a familial pattern of inheritance and a possible association with the cancer family syndrome. It speculates on the prevention of future internal malignancies in Muir-Torre syndrome patients by maintenance oral isotretinoin treatment.