Benign adnexal tumors of late occurrence in verrucoid-sebaceous nevus (Jadassohn). Apropos of 7 cases

Occurrence of basal cell epithelioma and syringocystadenoma papilliferum on sebaceous nevi is well known. But many other adnexal tumors, such as pilar or sweat gland tumours may also be associated with this dysembryoplasia. Out of 99 cases of verruco-sebaceous nevi we find 12 associated basal cell epitheliomas, 7 syringocystadenomas papilliferum and 6 benign adnexal tumors. Our report is about these 7 cases. All of these tumors appeared in adult-hood and were clinically suggesting the diagnostic of basal cell epithelioma. But, after histopathological examination was performed, they revealed to be 2 nodular hidradenomas, 1 chondroid syringoma, 1 trichilemmoma, 1 apocrine cystadenoma, 1 follicular poroma. Similar data are given by Mehregan and Pinkus in 1965 and Wilson Jones and Heyl in 1970, respectively out of 150 and 140 cases of verruco-sebaceous nevi.     

Differential expression of mucin core proteins and keratins in apocrine carcinoma, extramammary Paget's disease and apocrine nevus

Background:  Apocrine carcinomas are rare, the immunohistochemical characterizations that are incomplete. The purpose of this study was to determine the immunohistochemical characteristics of mucin core proteins and keratins in apocrine carcinoma, extramammary Paget's disease (EMPD) and apocrine nevus.
Methods:  We report four cases of apocrine carcinomas along with immunohistochemical analyses: (i) an axillary apocrine carcinoma with an apocrine nevus, (ii) an inguinal apocrine carcinoma, (iii) a vulvar apocrine carcinoma with EMPD and (iv) an axillary apocrine carcinoma with EMPD and an apocrine nevus.
Results:  The tumor cells of apocrine carcinomas, EMPD and apocrine nevi displayed a positive reaction to MUC-1 and CK7 and a negative reaction to CK20. Apocrine carcinomas had high molecular weight (HMW) cytokeratin(+)/CK5(+)/CK14(−)/MUC5AC(−), EMPD with underlying apocrine carcinoma had HMW cytokeratin(−)/CK5(−)/CK14(−)/MUCA5AC(−) and the apocrine nevi had HMW cytokeratin(+)/CK5(+)/CK14(+)/MUCA5AC(+).
Conclusion:  The immunohistochemical findings suggest that apocrine carcinomas, apocrine nevi and EMPD with underlying apocrine carcinomas are quite different, even though they are all derived from apocrine glands.     

Reticulated acanthoma with apocrine differentiation

Reticulated acanthoma with apocrine differentiation (RAAD) represents a rare variant of adnexal neoplasm first described by Ackerman et al. in 1998. It consists of cords and columns of adnexal keratinocytes that form a reticulated pattern. Variable signs of apocrine, sebaceous and follicular differentiation may be present. Since 1998, no further cases of this condition have been published. We report on a lesion excised from the leg of a 46-year-old man, which displayed histopathological features diagnostic of RAAD. Criteria for diagnosis and differential diagnoses are discussed.     

Sebocrine adenoma. An adnexal adenoma with sebaceous and apocrine poroma-like differentiation

Sebocrine adenoma is a benign cutaneous adnexal neoplasm differentiating in the direction of sebaceous and apocrine glands. The sebaceous differentiation is characterized by solitary instances or clusters of sebocytes and sebaceous ducts. The apocrine differentiation is characterized by eccrine poroma-like histology. Reported herein are three such cases.     

Nevus epidérmicos y síndromes relacionados. Parte 2: Nevus derivados de estructuras anexiales

Epidermal nevi are hamartomatous lesions derived from the epidermis and/or adnexal structures of the skin; they have traditionally been classified according to their morphology. New variants have been described in recent years and advances in genetics have contributed to better characterization of these lesions and an improved understanding of their relationship with certain extracutaneous manifestations. In the second part of this review article, we will look at nevi derived from the adnexal structures of the skin and associated syndromes.     

Trichoepithelioma and Pigmented Nevus. A Combined Malformation

The occasional occurrences of combined hamartomas composed of nevus cell nevi and adnexal tumors are not chance happenings. Pigmented nevi are malformations consisting of nevus cells together with various connective tissue and epithelial components. The proliferation of these components results in the combined malformations, whose occurrence, we believe, is more supporting evidence for the idea that pigmented nevi may have other components besides nevus cells.     

Apocrine gland organic hamartoma (apocrine nevus).

A patient had two fleshy, nontender masses, each partially filling an axillary vault. These tumors arose at puberty in normal-appearing axillae. The masses were accompanied by localized hyperhidrosis. Histopathological examination of the lesions showed that the reticular layer of the dermis had been replaced solely by lobular masses of unencapsulated, mature apocrine elements. These very rare tumors are best classified as apocrine gland organic hamartomas, or apocrine nevi. To our knowledge, such a case has never been reported or photographed.     

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.     

Bilateral epidermal nevi in the external auditory canals treated with CO2 laser

Epidermal nevi are benign hamartomas of the epidermis and adnexal structures of the skin. We present the case of epidermal nevi in the bilateral external auditory ear canals of an otherwise healthy 23-year-old woman treated with CO₂ laser ablation.     

Fokale dermale hypoplasie mit apokrinen naevi und streifenförmiger anomalie der knochen

Zusammenfassung Bei einer 22jährigen Patientin mit fokaler dermaler Hypoplasie wurden apokrine Naevi im Sternalbereich nachgewiesen; ein Zusammenhang mit dem Syndrom ist wahrscheinlich. An den langen Röhrenknochen wurde eine streifenförmige Strukturanomalie beobachtet; dieser Befund stellt ein charakteristisches und bisher zu wenig beachtetes Symptom der fokalen dermalen Hypoplasie dar.

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Focal dermal hypoplasia with apocrine nevi and striation of bones

Summary In a 22 year-old female patient affected with focal dermal hypoplasia, multiple apocrine nevi occurred in the sternal region; these nevi may be part of the syndrome. Roentgenograms of the long bones revealed marked longitudinal striations. This anomaly represents a characteristic and hitherto underestimated feature of the syndrome.

     

A complex poroma-like adnexal adenoma

Hidroacanthoma simplex, eccrine poroma, and dermal duct tumor are benign adenomas that develop from excretory ducts of eccrine glands and all three are variants of eccrine acrospiroma. To date, counterparts in apocrine or sebaceous glands have not been reported, but in this study we describe an adnexal, poroma-like adenoma that showed apocrine and sebaceous differentiations. Apocrine structures have the same embryonic origin as does the pilosebaceous system; both are derived from the primary epithelial germ. We suggest that the lesion we describe is truly a sebaceous and apocrine poroma. It must be distinguished from an infundibular adenoma whose pattern reproduces that of follicular poroma.     

Acne arising in an epidermal nevus

Abstract:  Epidermal nevi are benign congenital lesions that follow Blaschko lines. Epidermal nevi can demonstrate a variety of histologic patterns and contain hamartomatous hyperplasia of any epidermal or adnexal structure. We present the first reported instance of acne arising strictly within a preexisting keratinocytic epidermal nevus during puberty, which is a demonstration of genetic mosaicism in pilosebaceous units.     

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.     

Polarizable crystals in apocrine sweat gland tumors: A series of 3 cases

Polarizable calcium oxalate (CaOx) crystals have been well documented in breast biopsies, generally associated with benign apocrine metaplasia. In contrast, polarizable crystals are only rarely reported in skin adnexal neoplasms. We report 3 different cases of sweat gland tumors with polarizable crystals morphologically suggestive of CaOx: 1 apocrine hidrocystoma and 2 tubular apocrine adenomas. The histologic features were examined in 3 cases. Clinical presentation summary included 2 males and 1 female, ages 53 to 74 years, with lesions located on the left cheek, inferior vertex scalp and the left eyebrow. All 3 cases showed polarizable, geometric, plate‐like and fractured, colorless crystals within the lumens of the neoplasm. Of note, these crystals were seen only on the toluidine blue‐stained section of Case #1, but were not present on the corresponding permanent section. We hypothesize that polarizable crystals may be present in sweat gland neoplasms more often than previously documented, but that they may often dissolve with routine processing, accounting for their rare visibility. We highlight this rare finding, and suggest that it may be underreported. We only noted this finding in benign apocrine tumors; further investigation would be necessary to determine whether these crystals are also seen in other cutaneous adnexal neoplasms.     

Pure apocrine nevus. A study of light-microscopic and immunohistochemical features of a rare tumor

The pure apocrine nevus is a rare tumor. A new case of pure apocrine nevi of the bilateral axillary regions in a 48-year-old man is herein reported. The patient had two soft masses of both axillae. The time of onset of the masses was unknown. They were not accompanied by problems of sweating. Histologically, these lesions comprised numerous apocrine glands extending from the reticular layer of the dermis to the subcutaneous tissue. In addition, sections from both of the lesions were stained by immunoperoxidase techniques for the presence of epithelial membrane antigen, carcinoembryonic antigen, and the alpha and beta subunits of S100 protein. Immunohistochemical features of the tumor stained by these antibodies are consistent with a pure proliferation of mature, well-differentiated apocrine glands. The tumor of the present case seems to have developed spontaneously. It is believed, therefore, that the tumor seen in this patient is best categorized as a pure apocrine nevus.     

Expression of Bcl-x in normal skin and benign cutaneous adnexal tumors

BACKGROUND: Bcl-x appears to have an antiapoptotic role in the epidermis. Little is known about the expression of Bcl-x in cutaneous adnexal structures and benign cutaneous adnexal tumors. METHODS: Tissues from 31 cases of benign cutaneous adnexal tumors (five trichofolliculomas, five trichoepitheliomas, two sebaceous adenomas, five apocrine hidradenomas, five eccrine poromas, five eccrine spiradenomas, and four syringomas) were immunostained for Bcl-x. RESULTS: Strong staining for Bcl-x was seen in cells of the epidermal granular layer and inner root sheath of hair follicles. Sebaceous gland cells showed strong staining. Apocrine gland cells showed weak to moderate staining. No staining was seen in eccrine gland cells. The basaloid cells of trichofolliculomas and trichoepitheliomas showed no staining. In sebaceous adenomas, the sebaceous cells showed strong staining while the basaloid cells were negative. The cells of apocrine hidradenomas showed patchy weak staining. No staining was seen in eccrine poromas, eccrine spiradenomas, and syringomas. CONCLUSIONS: The degree of Bcl-x expression in cutaneous adnexal glandular structures appears to be related to their mode of secretion, being strongest in cells with apoptotic degradation of the entire cell (sebocytes). This pattern is recapitulated in the corresponding benign cutaneous adnexal tumors. Bcl-x may be useful in identifying cells with sebaceous differentiation in poorly differentiated adnexal tumors.     

Basiswissen Hautadnextumoren

Adnexal tumors of the skin are epithelial skin tumors with differentiation towards the adnexal epithelial structures of the skin, namely, hair follicle, sebaceous gland, apocrine gland, and eccrine gland. Adnexal tumors include hamartomas, benign and malignant neoplasms, and hyperplasias. The specific diagnosis of adnexal tumors is important because some lesions such as sebaceous neoplasms, cylindromas, or fibrofolliculomas are herald lesions of hereditary tumor syndromes (e.g., Muir-Torre syndrome, familial cylindromatosis, Birt-Hogg-Dubé syndrome). In this article, the classification of adnexal tumors of the skin is explained on the basis of embryology and histology and the main features of tumor-associated syndromes are summarized. Moreover, some conceptual controversies and problems in differential diagnosis of cutaneous adnexal tumors are discussed.     

Low-grade adnexal carcinoma of the skin with multidirectional (glandular, trichoblastomatous, spiradenocylindromatous) differentiation

The conjoint occurrence of follicular, sebaceous, or apocrine differentiations in a cutaneous adnexal neoplasm is a known event, more often encountered in benign neoplasms, whereas reports of cutaneous malignant adnexal tumors with bilineage or trilineage differentiation are few. A new case of a cutaneous malignant adnexal neoplasm with multidirectional differentiation is reported here. A 57-year-old woman presented with a long-standing, slowly growing, asymptomatic solitary tumor the size of a large nut in the coccygeal area, which was surgically excised. Ten years after the surgery, there was no evidence of recurrence or metastasis. Microscopically, the neoplasm was located in the dermis with focal extension into the subcutis. It was asymmetric, horizontally oriented, and mostly composed of small nodules that varied in shape from round and oval aggregations to elongated strands and irregular islands; the nodules were either clustered, formed a jigsaw puzzle-like pattern or were dispersed. The nodules were composed of small basaloid cells sometimes intermixed with larger cells with ample cytoplasm forming glandular structures. Rare nodules resembled elements seen in a spiradenoma by containing scattered lymphocytes and globules of hyalinized eosinophilic basal membrane material. The stroma was paucicellular, but focally it resembled that seen in perifollicular mesenchyme. Mitotic figures, including abnormal ones, were infrequent, but mild nuclear pleomorphism, nuclear crowding, and individual cell necrosis were easily appreciable in both small basaloid cells and cells with clear cytoplasm. Perineural invasion was apparent. We classified this tumor as a well-differentiated adnexal carcinoma demonstrating combined follicular and apocrine differentiation. It differs from previously published cases of malignant adnexal tumors with multidirectional differentiation and further exemplifies the spectrum of diversity encountered in malignant proliferations with differentiation toward the folliculosebaceous-apocrine unit.     

Linear syringocystadenoma papilliferum: an unusual location

Syringocystadenoma papilliferum is a rare benign adnexal skin tumor of apocrine or eccrine glands. We report here an 18-year-old girl who had linear syringocystadenoma papilliferum in the pubic region, which is very rare.     

Multiple apocrine poromas: a new case report

Apocrine poromas are rare and distinctive benign adnexal neoplasms featuring tumor cells differentiating toward folliculosebaceous‐apocrine units. We report an extremely rare case with multiple apocrine poromas in a single patient. Fifteen tumors were distributed on the head, neck, forearm and axilla of a 74‐year‐old man. All tumors were mostly composed of poroid cells that surrounded variably sized duct spaces, some of which exhibited decapitation secretion. The poroid cells were continuous with infundibulum‐like structures that contained aggregates of mature sebocytes. The patient had no family history of similar tumors and no history of immunosuppressive therapy. This is the first report of multiple apocrine poromas, suggesting that predisposing genetic factors might play a part in the development of the tumors.