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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

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

Desmoplastic trichoepithelioma.

Fifty examples of a distinctive clinicopathologic entity occurred in 49 patients who ranged in age from 8 to 70 (median 46) years. Forty-nine tumors were on the face, mainly the cheek, chin and forehead; most were present one to five years, and 85% of the patients were female. The growths varied in size from 3 to 8 mm; many were hard and annular, with a raised border and depressed nonulcerated center. Histologically, narrow strands of basaloid cells and epidermoid cyts infiltrated a fibrotic stroma. This tumor, which we have named desmoplastic trichoepithelioma, must be differentiated histologically from morphealike basal cell carcinoma, desmoplastic cutaneous metastasis, and certain benign adnexal neoplasms.

Adenoma

Halo congenital nevus.

A 24-year-old woman noted a depigmented halo around a pigmented lesion that had been present on her abdomen since birth. The halo and the central pigmented zone showed no change during the following year. Light and electron microscopic studies disclosed an uninflamed nevus extending to the deep reticular dermis; peripherally, the basal layer of the epidermis was depigmented. These findings indicate that a halo may develop about an uninflamed nevus that does not involute, and that the nevus may show the specific histopathology of congenital nevus.

Adult

Verrucous carcinoma of skin: epithelioma cuniculatum plantare.

Eight patients had plantar growths that were usually irregularly shaped, sharply outlined, verrucous, and several centimeters in greatest dimension. Clinical diagnoses included viral wart, deep mycosis, pyogenic granuloma, amelanotic melanoma, basal cell carcinoma, and eccrine poroma. The median age of the patients, and duration of the growth, were 52 and 16 years, respectively. Therapeutic modalities other than total excision were ineffective. The tumor, derived from surface epidermis, was composed of masses of pale-staining benign-appearing prickle cells, deeply invaginated by thick, compact, partially parakeratotic horn. Several lesions have recurred; amputation was required in one patient. This distinctive entity, apparently not described in the American literature, has been reported from Europe under such titles as epithelioma cuniculatum and papillomatosis cutis carcinoides. We believe that it is best interpreted as a form of verrucous carcinoma, the first cutaneous tumor--as opposed to lesions of mucous membranes and mucocutaneous junctions--to be so classified.

Adult

Desmoplastic malignant melanoma.

A 68-year-old man sought dermatologic attention for a tumor of the arm. Biopsy specimen showed abnormal, essentially amelanotic, spindle-shaped cells in the cutis, greatly fibrotic stroma, and focal epidermal invasion. Desmoplastic malignant melanoma was diagnosed. The lesion was widely excised and axillary lymphadenectomy performed; one node showed metastasis. Nine months later, he died with widespread metastatic disease. To our knowledge, this is the first report of this entity since its delineation in 1971 and the only case in which diagnosis was established on initial biopsy and followed by definitive therapy. Desmoplastic melanoma has been confused with benign fibrosis, invasive fibromatosis, and fibrosarcoma, and is another example, with morpheaform basal cell carcinoma and sclerodermoid metastatic lesions from breast carcinoma, in which desmoplastic stroma may obscure the epithelial nature of cutaneous neoplasm.

Aged