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Biomedical subjects

M H Brownstein

Publications and source records attributed to M H Brownstein.

At least 19 recordsLinked to original sources

Basaloid follicular hamartoma: solitary and multiple types.

BACKGROUND: Multiple skin tumors often show autosomal dominant inheritance; solitary neoplasms are typically nonhereditary. OBJECTIVE: The purpose of this study was to investigate a possible hereditary pattern in patients with multiple and solitary basaloid follicular hamartomas. METHODS: Four new familial cases of multiple basaloid follicular hamartomas and 56 solitary nonhereditary examples were identified and their inheritance pattern recorded. RESULTS: Clinically, basaloid follicular hamartomas were typically 1 to 2 mm, smooth facial papules. Histologically, they were well-circumscribed lesions composed of anastomosing strands of squamoid and basaloid cells in a loose stroma. Horn cysts and pigmentation were common. No significant association with other cutaneous or internal disease was found. CONCLUSION: Basaloid follicular hamartoma, a unique benign follicular tumor, was often diagnosed previously as trichoepithelioma or basal cell carcinoma. It is another cutaneous neoplasm in which patients with multiple lesions show autosomal dominant inheritance, whereas solitary growths with identical clinical, microscopic, and biologic features are nonhereditary.

Adult↗

Lichenoid epidermal nevus: "linear lichen planus".

A 3-year-old girl had an eruption that showed clinical and histologic evidence of both a verrucous epidermal nevus and a lichenoid tissue reaction. Linear plaques had been present on her right lower limb for 2 years. Microscopic features included hyperkeratosis, papillomatosis, acanthosis, a bandlike lymphohistiocytic infiltrate that obscured the dermoepidermal junction, Civatte bodies, and melanophages. Lichenoid epidermal nevus is a new variant of linear verrucous epidermal nevus. At least some of the cases previously diagnosed as linear lichen planus probably represent lichenoid epidermal nevus. There is a parallel between lichenoid epidermal nevus and the entity described as inflammatory linear verrucous epidermal nevus, which frequently was misinterpreted as linear psoriasis before it was recognized as a linear verrucous epidermal nevus with psoriasiform features.

Child, Preschool↗

Acantholytic acanthoma.

This article describes 31 examples of acantholytic acanthoma, a newly recognized, solitary, benign cutaneous tumor. Acantholytic acanthoma was typically an asymptomatic, keratotic papule or nodule. Patients ranged in age from 32 to 87 years (median 60 years); the ratio of men to women was 2:1; the most frequent clinical diagnosis was keratosis; and half of the growths were on the trunk of the body. Histologically, the lesions showed hyperkeratosis, papillomatosis, and acanthosis. Acantholysis was an outstanding finding in all cases; the patterns resembled pemphigus vulgaris, pemphigus vegetans, superficial pemphigus, or Hailey-Hailey disease, but no patient had evidence of any of these disorders. The term acantholytic is used because acantholysis is the outstanding histologic feature in these neoplasms; acanthoma was chosen because the growths are benign tumors of epidermal keratinocytes. The relationship of acantholytic acanthoma to acantholytic blistering disease is similar to that of solitary lichen planus-like keratosis to lichen planus and epidermolytic acanthoma to bullous congenital ichthyosiform erythroderma.

Acantholysis↗

Desmoplastic trichoepithelioma and intradermal nevus: a combined malformation.

We studied 13 desmoplastic trichoepitheliomas associated with intradermal nevi. Ten intradermal nevi were found among 76 new cases of desmoplastic trichoepithelioma (13%); three additional examples of the combined malformation were seen in consultation. Clinically, desmoplastic trichoepithelioma associated with an intradermal nevus was typically a small, firm or hard, sometimes annular, nodule on the face, particularly the cheek, of a relatively young woman. Microscopically, the combined malformation contained narrow strands of basaloid cells and keratinous cysts in a desmoplastic stroma, intimately mixed with intradermal nests of nevocytes. Melanocytic nevi have been associated with epidermal hyperplasia resembling seborrheic keratoses, follicular cysts, trichostasis spinulosa, syringomas, basal cell carcinomas, and hair follicle formation on the soles. The frequency of the occurrence of intradermal nevus with desmoplastic trichoepithelioma and the close anatomic association of the two elements may indicate that this combined malformation is another example of epithelial induction by melanocytic nevi.

Biopsy↗

Fibrofolliculoma: solitary and multiple types.

Multiple skin tumors generally show autosomal dominant inheritance and are sometimes associated with other cutaneous and internal lesions. Solitary growths are typically nonhereditary and unassociated with other abnormalities. We found only two previous reports of solitary fibrofolliculoma and only one of multiple fibrofolliculomas without associated lesions; a few publications describe multiple fibrofolliculomas in patients with perifollicular fibromas, trichodiscomas, acrochordons, and connective tissue nevus. We studied five new cases of solitary nonhereditary fibrofolliculomas and one of multiple familial fibrofolliculomas without associated lesions. Clinically, fibrofolliculomas were typically small growths with a central dilated follicle containing horny material. Histologically, there was characteristically a hyperplastic follicular infundibulum with a central keratinous plug, and anastomosing strands of basaloid cells branching into an angiofibromatous stroma. Fibrofolliculoma is thus another skin tumor in which patients with multiple growths showing autosomal dominant inheritance sometimes have associated abnormalities, while solitary lesions with identical clinical and microscopic features are relatively trivial and nonhereditary.

Adult↗

Papillary adenoma of the nipple: analysis of fifteen new cases.

Fifteen cases of papillary adenoma (florid papillomatosis, erosive adenomatosis) of the nipple were compared with forty examples of syringadenoma papilliferum, twenty-four of hidradenoma papilliferum, and one hundred of breast carcinoma metastatic to skin. Clinically, papillary adenoma of the nipple typically occurred in a female patient as an erosion, ulceration, or mass that was sometimes accompanied by serous or bloody discharge from the nipple and was frequently misdiagnosed as Paget's disease. Histologically, there was usually a papillary and adenomatous growth in the corium that connected with the surface and showed a lining of apocrine type secretory epithelium, a sometimes plasmacytic stroma, and horn cysts in the upper corium. Papillary adenoma of the nipple is often histologically misinterpreted as syringadenoma papilliferum, hidradenoma papilliferum, or low-grade adenocarcinoma, but it has received almost no attention in the dermatology literature.

Adenoma, Sweat Gland↗

The cutaneous pathology of Cowden's disease: new findings.

Histological, histochemical and immunohistochemical findings were studied in 40 cutaneous biopsies from 7 patients with Cowden's disease. Most facial biopsies showed a spectrum of trichilemmomas and related follicular malformations, including cylindrical trichilemmomas, lobulate trichilemmomas (14 lesions), and a keratinizing type sharing features with inverted follicular keratosis. One facial growth showed trichilemmomal changes without apparent follicular origin. Studies for common papilloma virus structural antigens were negative, apart from a typical common wart in one patient. Immunohistochemical studies in 6 facial trichilemmomas and acral keratoses, using a panel of anti-keratin antibodies, disclosed only abnormal differentiation with lack of large keratins in the lobulate trichilemmomas. Nine biopsies revealed a distinctive type of fibroma characterized by an organized pattern of interwoven fascicles of collagen bundles with a laminated or tortuous appearance, embedded in abundant mucin. A number of fibromas showed striking hyalinization; these may represent a second microscopic hallmark of Cowden's disease in addition to facial trichilemmomas.

Antigens, Viral↗

The genodermatopathology of adnexal tumors.

Several adnexal tumors (e.g., trichoepitheliomas, trichilemmomas, steatocystomas, sebaceous adenomas, and cylindromas) tend to occur as multiple lesions showing autosomal dominant inheritance, sometimes in association with other cutaneous or internal findings. Solitary tumors with the same clinical and histopathologic features are usually trivial non-hereditary lesions. Multiple trichilemmomas are part of Cowden's syndrome, which includes a high incidence of breast cancer in women; multiple sebaceous adenomas and other sebaceous tumors associated with carcinomas of the gastrointestinal tract are part of a syndrome that was first delineated by Bakker and Tjon A Joe.

Adenoma↗

Hybrid cyst: a combined epidermoid and trichilemmal cyst.

Seven cases of hybrid cyst, a combined epidermoid and trichilemmal cyst, are reported. Hybrid cysts occurred in five men and two women, and involved the scalp, cheek, back, arm, elbow, and forearm. The clinical diagnosis was usually cyst or sebaceous cyst. Microscopically, the upper portion of the cyst showed epidermoid keratinization in continuity with the surface epidermis, and was indistinguishable from an epidermoid cyst. Showing sharp transition from it, the inferior portion of the lesion displayed trichilemmal keratinization and was indistinguishable from a trichilemmal cyst. Hybrid cyst is not merely a newly described pathologic curiosity; rather, it forms a meaningful link between the two most common types of follicular cysts and adds weight to the body of evidence that most epidermoid cysts are related to the follicular infundibulum, while trichilemmal cysts are related to the follicular isthmus.

Adult↗

The invisible dermatoses.

It is understandable that clinically normal skin may show abnormalities when examined with the light microscope, but paradoxical that biopsy of a clinically significant skin disorder may show a histologic picture that looks like normal skin. From the perspective of the dermatopathologist, the invisible dermatoses are clinically evident skin diseases that show a histologic picture resembling normal skin. A strategy for approaching the problem of the invisible dermatoses is to first examine the epidermis for fungi, cornoid lamellae (disseminated superficial actinic porokeratosis), and absence of the granular layer (dominant ichthyosis vulgaris). The cutis is then studied for hyalin deposition (macular amyloidosis), mast cells, microfilaria, dermal melanocytosis, silver granules, and absence of sweat glands (anhidrotic ectodermal dysplasia). Special stains may be required to uncover conditions like anetoderma and nevus elasticus. Comparison of the specimen with normal skin may disclose atrophoderma, lipoatrophy, vitiligo, or café au lait spot. Finally, technical problems should be considered, including sampling errors and mixup of specimens, either by the clinician or the laboratory.

Biopsy↗

Steatocystoma simplex. A solitary steatocystoma.

Steatocystoma simplex, an entity not previously described, to my knowledge, occurred in 16 women and 14 men. Eleven cysts were situated on the face, six on the limbs, and five on the chest. The clinical diagnosis was usually some type of cyst. The lesions contained yellow fluid. Microscopic findings showed a partially collapsed, thin-walled cyst lined by stratified squamous epithelium with a thick, undulating hyaline cuticle and sebaceous glands in or near the wall. When compared with steatocystoma multiplex and dermoid cysts, the differential features of steatocystoma simplex included its solitary nature, occurrence in adulthood, lack of family history, and intracutaneous location. Just as other dominantly inherited syndromes of multiple benign adnexal tumors, such as cylindromas, trichoepitheliomas, and trichilemmomas, have their counterparts in nonheritable solitary tumors, so steatocystoma multiplex has its parallel in steatocystoma simplex.

Adolescent↗

Proliferating trichilemmal cyst: a simulant of squamous cell carcinoma.

Comparison of 50 proliferating trichilemmal cysts with 50 "ordinary" trichilemmal cysts indicated that both types almost invariably occurred on the scalps of women, were diagnosed clinically as cysts, followed a benign course, and featured trichilemmal keratinization. A spectrum was observed from trichilemmal cyst with minimal hyperplasia, to full-blown proliferating trichilemmal cyst. Occasionally, patients had ordinary trichilemmal cysts on their scalps associated with a proliferating trichilemmal cyst. In addition to trichilemmal keratinization, which is characteristic of the follicular isthmus, proliferating trichilemmal cysts showed a wider range of differentiation, including features of the follicular infundibulum, the lower nonkeratinizing portion of the follicular outer root sheath, and sebaceous cells. Intense inflammatory infiltrate and cellular atypia, sometimes of significant proportions, were also seen in proliferating trichilemmal cysts. It is concluded that trauma and inflammation may induce a trichilemmal cyst to proliferate and show a broader spectrum of pilosebaceous differentiation and cellular atypia of pseudocarcinomatous proportions, while maintaining its benign biologic behavior.

Adenoma, Sweat Gland↗

Trichilemmoma. Benign follicular tumor or viral wart?

Trichilemmoma is a follicular tumor because of its resemblance to follicular outer root sheath, overlapping features with other tumors in the spectrum of benign follicular neoplasms, occurrence as multiple dominantly inherited and solitary noninheritable tumors, and occasional complication of organoid nevus. The dangerous misinterpretation that trichilemmomas are viral warts, unsupported by scientific evidence, has harmed patients. All electron-microscopic, immunologic, and clinical transmission attempts to demonstrate viral etiology of trichilemmoma have failed. If trichilemmomas were simply old warts, we might expect to see them frequently in children, extrafacially, of long duration, associated with multiple authentic ("young") warts, as multiple lesions, and associated with authentic warts in patients with Cowden's syndrome (multiple trichilemmomas). Actually, trichilemmomas are rarely seen in children, extrafacially, of long duration, associated with multiple authentic warts, as multiple lesions in patients without Cowden's syndrome, and associated with authentic warts in Cowden's syndrome. Unfortunately, patients with multiple trichilemmomas, Cowden's syndrome, and associated internal disease have been passed off as "simply old viral warts." The missed opportunity to identify women with a 50% risk of breast cancer is a disservice to the patient, to say nothing about malpractice implications for the physician when generally accepted medical standards are not applied.

Breast Neoplasms↗

Trichilemmal horn: cutaneous horn showing trichilemmal keratinization.

A unique and distinctive clinicopathological entity occurred in nineteen patients who ranged in age from 16 to 72 (median 50) years. Clinically, these were solitary cutaneous horns. Nine were on the limbs, four on the back, two on the face, three on the scalp, and in one the site was not known; the median duration was 2 years. Histologically, there was a benign picture with a protrusion of massive horn and trichilemmal keratinization at the base. This tumour, which I have named trichilemmal horn, must be differentiated from other lesions that show trichilemmal keratinization (trichilemmal cyst, proliferating trichilemmal cyst, keratoacanthoma) and from other cutaneous horns, including trichilemmomal horn (cutaneous horn overlying trichilemmoma).

Adolescent↗

The dermatopathology of Cowden's syndrome.

During the past 2 years we have studied eighty-nine biopsy specimens from nineteen patients with Cowden's syndrome. Among fifty-three facial lesions, twenty-nine were trichilemmomas. Twenty-three of the others were consistent with trichilemmoma, showed a non-specific benign verrucous acanthoma, or were not diagnostic; one was a blue naevus. All fourteen oral mucosal biopsy specimens were benign fibromas. Nineteen of twenty-two biopsy specimens from the hands and feet showed the pattern of benign keratosis. Multiple trichilemmomas were found in all patients with Cowden's syndrome, but at times several biopsy specimens were required before a diagnostic picture was uncovered. All patients with multiple facial trichilemmomas were found to have Cowden's syndrome. The combination of multiple facial trichilemmomas, oral fibromas, and benign acral keratoses enables one to diagnose Cowden's syndrome at a stage before serious internal complications develop.

Abnormalities, Multiple↗

Cowden's disease: a cutaneous marker of breast cancer.

Cowden's diseases features facial trichilemmomas (a benign tumor of follicular epithelium), acral keratoses on the limbs, and oral mucosal papillomas and fibromas; it may also involve thyroid, gastrointestinal tract, ovaries, uterus, and breasts. Among 32 known cases of Cowden's disease, 21 are women, in 10 of whom breast cancer has already developed (bilateral in 4). The 11 women in whom breast cancer has not yet developed have fibroadenomas, fibrocystic disease, virginal hypertrophy of the breast, and malformations of nipples and areolae. Their median age is only 36 years. Two have mothers with breast cancer and in one both mother and maternal grandmother had breast cancer. Dermatologic lesions, including pathognomonic multiple facial trichilemmomas, precede the development of malignancy and can identify women with ahigh risk of developing breast cancer.

Adult↗

Pilar sheath acanthoma.

A newly recognized, benign follicular tumor occurred in nine patients who ranged in age from 46 to 75 (median, 55) years. The neoplasms were solitary, asymptomatic, skin-colored nodules with a central pore-like opening plugged with keratin. All lesions had been present for years. Eight were on the skin of the upper lip and one was on the forehead. Histologically, a central cystic cavity showing epidermoid keratinization extended from the surface into the deep cutis. Multiple tumor lobules that were composed of hair sheath epithelium extended from the cyst wall into the surrounding corium. This neoplasm, which we have named "pilar sheath acanthoma," must be differentiated from trichofolliculoma and dilated pore.

Aged↗