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Biomedical subjects

S J Hunt

Publications and source records attributed to S J Hunt.

At least 19 recordsLinked to original sources

Cutaneous T-cell lymphoma: utility of antibodies to the variable regions of the human T-cell antigen receptor.

BACKGROUND: The clonotypic 90 kd Ti heterodimer of the human T-cell antigen receptor is composed of two distinct chains (alpha beta or rarely tau delta) that result from the recombination of variable (V), constant, joining, and, in the case of beta chains, additional diversity regions. OBJECTIVE: The variable region expression of human cutaneous T-cell lymphoma (CTCL) was studied. METHODS: Biopsy specimens from 13 patients with CTCL (7 plaque, 3 tumor stage, 3 Sézary syndrome) were examined immunohistochemically by a panel of seven commercially available monoclonal V-region antibodies. RESULTS: Two patients had significant anti-V-region staining. One patient with Sézary syndrome had two lesions, subjected to biopsy 4 months apart, that reacted with beta V5(a), a specificity previously documented by flow cytometry of leukemic cells. A patient with plaque-stage CTCL, negative for T-cell gene rearrangement by Southern blot, demonstrated reactivity with beta V5(c) largely limited to epidermotropic lymphocytes. CONCLUSION: Panels of V-region antibodies should be useful reagents for diagnosis and follow-up of CTCL.

Adult

Primary cutaneous fungal infection after solid-organ transplantation: report of five cases and review.

Solid-organ transplant recipients who are receiving immunosuppressive therapy are at increased risk of acquiring opportunistic infections, particularly fungal infections. We present the cases of five liver transplant recipients who developed primary cutaneous opportunistic fungal infections that remained localized to the skin. These cases are compared with 27 previously reported cases of primary cutaneous fungal infections. In these previously reported cases, administration of systemic antifungal medications, including amphotericin B, ketoconazole, griseofulvin, and miconazole, resulted in a 71% survival rate. Medical and surgical therapy together resulted in an 86% survival rate, and surgical excision resulted in a 100% survival rate. Thus, regardless of the age of the patient, type of immunosuppressive therapy, clinical presentation, or organisms involved, surgical excision yielded the highest cure rate. When possible, surgical excision should be performed on solid-organ transplant recipients who acquire opportunistic fungal infections.

Adult

Primary cutaneous aspergillosis near central venous catheters in patients with the acquired immunodeficiency syndrome.

BACKGROUND: Aspergillosis in patients with the acquired immunodeficiency syndrome is unusual, and the clinicopathologic features of primary cutaneous aspergillosis in this setting are undefined. Our findings show that the manifestations can differ from those of primary cutaneous aspergillosis in other immunocompromised patients. OBSERVATIONS: Two men with the acquired immunodeficiency syndrome developed foci of primary cutaneous aspergillosis beneath adhesive tape near central venous catheter sites. Typical lesions were flesh-colored to pink, umbilicated papules that clinically resembled molluscum contagiosum. Biopsy specimens showed variably ruptured follicles that contained collections of fungal hyphae typical of Aspergillus species. Cultures in one case identified Aspergillus fumigatus. The use of nonocclusive dressings and local wound care resulted in involution of several lesions. CONCLUSIONS: Primary cutaneous aspergillosis begins as saprophytic involvement of hair follicles secondary to the altered microenvironment beneath adhesive tape. Systemic antifungal therapy is prudent, but in the absence of neutropenia or other traditional risk factors for dissemination, it appears that Aspergillus in patients with the acquired immunodeficiency syndrome can produce relatively indolent cutaneous lesions with a tendency to resolve once precipitating factors are removed.

Acquired Immunodeficiency Syndrome

Acquired benign and "borderline" vascular lesions.

In recent years, the classification of vascular lesions has been expanded and modified with the addition of several newly described entities, the redefinition of others, the recognition of lesions of borderline biologic behavior, and the need to avoid misdiagnosis with early Kaposi's sarcoma. This review clarifies the nomenclature, updates information on previously known lesions, and summarizes data on several recently discovered, lesser-known entities such as glomeruloid hemangioma, microvenular hemangioma, and multinucleate cell angiohistiocytoma. Clinicopathologic features and differential diagnosis are emphasized.

Angiokeratoma

Necrolytic migratory erythema: dyskeratotic dermatitis, a clue to early diagnosis.

A 57-year-old woman with a 6-year history of a dermatitis that evolved into typical necrolytic migratory erythema is reported. Four biopsy specimens were obtained in 5 years. The early lesions revealed superficial perivascular inflammation in the dermis, minor epidermal spongiosis, and scattered dyskeratotic cells in the upper epidermis. The differential diagnosis of this pattern of dyskeratotic dermatitis, particularly in a chronic eruption, should include consideration of hyperglucagonemia and the possibility of an associated pancreatic islet cell tumor.

Dermatitis

Linear and punctate porokeratosis associated with end-stage liver disease.

A periodic eruption of porokeratosis developed in a 31-year-old black woman with chronic idiopathic hepatitis requiring liver transplantation. The clinicopathologic features were chiefly those of linear and punctate porokeratosis but overlapped those of porokeratosis plantaris, palmaris et disseminata and hyperkeratotic or verrucous porokeratosis. Typical cornoid lamellae were visible on histologic examination. Outbreaks of the lesions occurred during exacerbations of the liver disease. The skin condition rapidly improved after operation, with concomitant improvement in liver function.

Adult

Malignant hair matrix tumor ("malignant trichoepithelioma") arising in the setting of multiple hereditary trichoepithelioma.

An unusual adnexal tumor on the scalp of a 59-year-old woman with hereditary trichoepithelioma is reported. After the initial removal, the tumor recurred twice during a 4-year period. Mohs micrographic surgery resulted in full excision of the lesion. Two years later there was no evidence of recurrence. Histologic examination of the tumor showed it to merge with adjacent areas of classic trichoepithelioma. The tumor showed cytologic atypia, mitotic figures, necrosis, deep infiltration, and features of pilar differentiation including clear cells, reminiscent of cells of the follicular outer root sheath, and focal shadow-cell formation. The differential diagnosis of this malignant follicular neoplasm included pilomatrix carcinoma and basal-cell carcinoma with matrical differentiation. The unique clinicopathologic features, however, raised consideration of a "malignant trichoepithelioma."

Adenoma

Microvenular hemangioma.

Ten examples of a distinctive vascular tumor are reported. These benign acquired lesions typically occur as small, enlarging lesions that favor the extremities, particularly the forearms, of young to middle-aged adults. Clinically, they are purple to red lesions generally thought to be hemangiomas. Histologically, there is a pattern of irregular, branching venules with inconspicuous lumina and lack of cellular atypia. Because the lesions do not conform to existing classifications of vascular tumors, they have been designated with the histologically descriptive name of microvenular hemangioma. Although speculative, they are felt to represent a form of acquired venous hemangioma.

Adolescent

Mucinous syringometaplasia mimicked by a clear cell hidradenoma with mucinous change.

An adnexal tumor with features of both clear cell hidradenoma and mucinous syringometaplasia is reported. The lesion occurred as a solitary, asymptomatic, slow growing nodule of 6-months duration on the anterior neck of a 55-year-old woman. Serous fluid could be expressed from a central skin opening. Histologically, there was a squamous-lined invagination with foci of mucin-laden cells. A ductal component with a variable admixture of mucinous cells merged with a nearby clear cell hidradenoma. Because clinicopathologic similarities exist between mucinous syringometaplasia and clear cell hidradenoma with mucinous change, thorough sectioning of mucinous syringometaplasia-like lesions is necessary to exclude an associated sweat gland neoplasm.

Adenoma, Sweat Gland

Eosinophilic cellulitis: histologic features in a cutaneous mastocytoma.

A cutaneous mastocytoma with associated histologic features of eosinophilic cellulitis is reported. The tumor occurred as a small, asymptomatic lesion on the left thigh of a 4-year-old boy. Microscopically, an accumulation of mast cells, microgranulomas, eosinophils and 'flame figures' was present. A pathogenesis involving mast cell degranulation, eosinophil chemotactic factors and eosinophil major basic protein is discussed.

Cellulitis

Neonatal intracranial teratomas.

Two neonates with intracranial teratomas presented with cranial enlargements a few weeks after birth. Both cases underwent surgery: one died intraoperatively; the other is the longest known survivor, alive 7 years and 9 months after subtotal excision of a mature teratoma of the left sylvian fissure. Previous operations have been relatively few and nearly all have been unsuccessful. Size and favorable location may be the most important prognostic features regardless of the histologic classification as mature or immature. One of our cases demonstrates that even subtotal excision of a mature teratoma can result in long-term survival.

Brain Neoplasms

Giant eccrine acrospiroma.

Four cases of large eccrine acrospiroma (three benign, one malignant) are reported. The benign tumors involved the lower extremities of two women and one man (73 to 89 years of age). The duration of the lesions ranged from 10 to 20 years. The malignant tumor involved the left side of the chest of a 60-year-old man. Its occurrence in a lesion that had been present for 40 years suggested malignant transformation of a pre-existing benign eccrine acrospiroma. Each tumor showed little to no cellular atypia. Mitotic rates (mitotic figures per 10 high-power fields) varied both between and within lesions. Average mitotic rates did not differentiate the benign from the malignant tumors. The most important distinguishing features of large benign eccrine acrospiromas are the relative circumscription, the lack of cellular atypia, and the absence of stromal, perineurial, and angiolymphatic invasion.

Adenoma, Sweat Gland

Malignant rhabdoid tumor of the liver. A distinct clinicopathologic entity.

A malignant rhabdoid tumor occurring as a primary hepatic neoplasm in a six-month-old white infant is reported. It was treated by an attempt at total resection involving right hepatic lobectomy and by chemotherapy with cis-platinum, VP-16, and Adriamycin. Despite this, recurrence of the tumor resulted in the girl's death within three months. The neoplasm showed typical light microscopic features of malignant rhabdoid tumor as well as filamentous cytoplasmic inclusions by electron microscopic examination and staining for both cytokeratin and vimentin by immunohistochemistry. The classic clinicopathologic features of this tumor support the concept that malignant rhabdoid tumors similar to those of the kidney may occur in extrarenal sites.

Female

Cutaneous epithelioid angiosarcoma.

Three cases of cutaneous epithelioid angiosarcoma with solid pattern were studied by immunohistochemistry and electron microscopy. The neoplasms followed a slow, protracted course with local recurrences and regional lymph node metastases. The correct histological diagnosis was delayed by the close histological simulation of carcinomas, misleading ultrastructural findings, and largely negative immunohistochemical markers. Two of the patients have been followed for at least 48 months and are still alive. Some seemingly undifferentiated epithelioid angiosarcomas may entail a better prognosis than originally suspected.

Adult

Cellular angiolipoma.

Three examples of cellular angiolipoma are presented. They occurred as part of multiple, occasionally painful, subcutaneous nodules on the extremities and trunk of healthy men. There was no familial tendency. Grossly, the cellular angiolipomas were small lesions, approximately 1 cm. Histologically, dense cellular angiomatous tissue comprised more than 95% of the lesions. Much of this component contained prominent spindle cells. Lesser degrees of involvement in the other subcutaneous nodules illustrated the continuum of histology between cellular angiolipomas and more typical angiolipomas. The differential diagnosis of cellular angiolipomas includes spindle cell lipoma, Kaposi's sarcoma, and other vascular tumors. The most important distinguishing features are encapsulation, intravascular fibrin thrombi, septation, association with other more typical angiolipomas, and occurrence in healthy individuals.

Adult

Desmoplastic trichilemmoma: histologic variant resembling invasive carcinoma.

A clinical and histologic review of 22 patients (13 males, 9 females) with cutaneous lesions classified as desmoplastic trichilemmoma is reported. Typically, the lesions occur as solitary dome-shaped papules on the face during (at least) the fifth decade of life. The average age was 64 years (range 19-89) with a median age of 66.5 years. The most frequent clinical diagnosis was basal cell carcinoma, and the most common sites were the lip, eyebrow and nose. Two lesions occurred in examples of nevus sebaceus of Jaddasohn. The proportion of desmoplasia varies, but is generally between 20% to 60% of the lesion. Ulceration is seen in a minority of lesions but when present, is closely associated with underlying desmoplasia. Desmoplasia may be seen in small, as well as large, trichilemmomas; it generally occurs centrally but at times may be seen peripherally. The appearance of strands of epithelial cells entrapped in dense fibrosis and hyalinization may mimic desmoplastic variants of squamous cell carcinoma and basal cell carcinoma. This appearance may be particularly troublesome when the desmoplasia occurs at the base of the biopsy specimen. Knowledge of this phenomenon and the search for more typical features of trichilemmoma such as clear cells and peripherally palisaded columnar cells upon a thickened, eosinophilic basement membrane will allow the correct diagnosis to be made. We believe that the desmoplasia occurs as a secondary change in pre-existing trichilemmomas.

Adult

Cholesterotic fibrous histiocytoma. Its association with hyperlipoproteinemia.

A 63-year-old woman with fibrous histiocytomas showed cholesterol deposition in the setting of type IIB hyperlipidemia. The two lesions involved the left leg and right thigh. One had typical features of a fibrous histiocytoma including changes of the overlying epidermis. The other was essentially replaced by cholesterol deposits and could not be differentiated from a tuberous xanthoma. This case illustrates the histiocytic response of fibrous histiocytomas to a hyperlipoproteinemic microenvironment.

Cholesterol