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Biomedical subjects

W F Bergfeld

Publications and source records attributed to W F Bergfeld.

At least 19 recordsLinked to original sources

The utility of cytokeratin 5/6 in the recognition of cutaneous spindle cell squamous cell carcinoma.

BACKGROUND: Cutaneous spindle cell squamous cell carcinoma (SSCC) is a challenging diagnosis since it may be difficult to distinguish from spindle cell melanoma, leiomyosarcoma and atypical fibroxanthoma. Furthermore, it may be difficult to demonstrate epithelial differentiation by a traditional immunohistochemical panel. We performed an expanded immunohistochemical evaluation of ultrastructurally documented SSCC to assess its utility in diagnosing this entity. METHODS: We identified 16 cases of SSCC that were composed predominantly of spindle-shaped cells and with ultrastructural evidence of epithelial differentiation (i.e. at least rudimentary cell junctions). Immunohistochemical analysis using antibodies to a variety of cytokeratins (AE1/3, K903, CK5/6) and S-100 protein was performed. The extent of immunostaining was graded on a scale of 0 to 4+ (0: no staining; 1+: < or =25%; 2+: 26-50%; 3+: 51-75%; 4+: >75%). RESULTS: Of the 16 cases, 6 expressed AE1/3 (38%), 8 expressed K903 (50%) and 11 (69%) expressed CK5/6. Six cases were positive for all three CK markers and two cases were positive for both K903 and CK5/6 but negative for AE1/3. Three cases (19%) stained for CK5/6 without any staining for AE1/3 or K903. Five cases (31%) were negative for all epithelial markers. The extent of CK5/6 staining was either similar to or greater than K903 staining in 7 of 8 cases that stained with both markers. All 16 cases were negative for S-100 protein. CONCLUSIONS: Including CK5/6 in the initial battery of immunostains performed on a cutaneous spindle cell neoplasm can help demonstrate epithelial differentiation in SSCC, even in the absence of AE1/3 or K903 staining. However, some cases of cutaneous SSCC can only be confirmed ultrastructurally, as up to one-third may not show evidence of epithelial differentiation using an expanded immunohistochemical panel.

Carcinoma, Squamous Cell↗

Evaluation of a new paraffin-reactive CD7 T-cell deletion marker and a polymerase chain reaction-based T-cell receptor gene rearrangement assay: implications for diagnosis of mycosis fungoides in community clinical practice.

BACKGROUND: T-cell deletion and T-cell receptor (TCR) gene rearrangement studies are helpful in the early diagnosis and subsequent management of mycosis fungoides (MF). However, this often requires fresh-frozen tissue that can be difficult to obtain and evaluate in community clinical practice. A new CD7 antibody, the most sensitive and specific T-cell deletion marker, and a new TCR-gamma gene rearrangement polymerase chain reaction (PCR) assay (TCR-gamma PCR) are now available on routine paraffin-embedded biopsy specimens. OBJECTIVE: Our purpose was to assess the utility of CD7 deletion and TCR-gamma PCR in the diagnosis of MF using routine paraffin-embedded biopsy material. METHODS: Cases of MF (n = 17) with matching frozen tissue immunohistochemistry and benign reactive dermatoses (lichen planus; n = 27) were assessed for CD7 (Clone: CD7-272) deletion and TCR-gamma PCR using paraffin-embedded biopsy specimens. RESULTS: Excellent concordance comparing frozen and paraffin embedded CD7 immunostaining (88%) was observed. CD7 deletion and TCR-gamma PCR was sensitive (94%) and specific (96%) for a diagnosis of MF using paraffin-embedded biopsy specimens. CONCLUSION: In the diagnosis of MF, detection of CD7 deletion and monoclonal TCR rearrangements can be successfully performed in a cost-effective, timely fashion using routine formalin-fixed paraffin-embedded biopsy specimens.

Adult↗

Shedding: how to manage a common cause of hair loss.

Although telogen effluvium, or shedding-the most common type of diffuse hair loss in both women and men-is usually self-limiting, the condition may become chronic if the trigger is not identified and corrected. The authors discuss the physiologic and emotional triggers, clinical presentation, diagnosis, and management strategies, including the importance of patient education and reassurance.

Adrenal Cortex Hormones↗

Lipodystrophy.

Congenital lipodystrophy is an uncommon autosomal recessive disorder that occurs mainly in females and is characterized by loss of subcutaneous fat, insulin-dependent diabetes mellitus, and masculinization due to defective metabolism of fat. Acquired lipodystrophy is now most commonly encountered in patients infected with HIV who take protease inhibitors. We present an illustrative case of lipodystrophy and review the presenting signs allowing for an accurate clinical diagnosis.

Female↗

Hair loss: an overview.

Hair loss is a common problem in men and women. Correct diagnosis of hair disorders is complex and requires evaluation of clinical presentation, history, physical examination, and laboratory tests. Hair loss may be categorized as hair shaft abnormalities, permanent alopecia, or nonpermanent alopecia. Nonpermanent alopecia, the most common type, includes androgenetic alopecia, telogen effluvium, alopecia areata, and traction alopecia. The hallmark of this group is the possibility of complete regrowth with adequate treatment.

Alopecia↗

Cutaneous presentation of posttransplant lymphoproliferative disorder.

Posttransplant lymphoproliferative disorder (PTLD) is a known complication of solid organ and bone marrow transplantation. However, cutaneous manifestation is an extremely rare phenomenon of this disorder. We describe a lung transplantation patient with cutaneous PTLD who presented with bilateral extremity nodules and fever.

Female↗

Elastic tissue in scars and alopecia.

A recent report suggests that elastic fibers appear in scars in a time-dependent fashion. This observation prompted our investigation, because we have found elastic tissue stains helpful in determining the pattern of scarring in cases of permanent alopecia. We carried out this investigation to determine if the Verhoeff-Van Gieson (VVG) elastic stain can reliably differentiate scarred from non-scarred dermis and to test our hypothesis that elastic stained sections are helpful in distinguishing lichen planopilaris (LPP) from lupus erythematosus (LE), central progressive alopecia in black females ("follicular degeneration syndrome" and "hot comb alopecia" are other terms used to describe this condition) and classic ivory white idiopathic pseudopelade. We studied histological sections from surgical scars of known duration, stained with the VVG elastic stain and VVG-stained sections of scalp biopsies from patients with established lesions of permanent alopecia. In most cases, both vertical and transverse sections were examined. In every case, the VVG stain clearly differentiated scar from the normal surrounding dermis. Distinct patterns of elastic tissue allowed for correct classification in most of the well-established cases of permanent alopecia studied. We determined that the Verhoeff-Van Gieson stain is an excellent stain to evaluate the pattern of scarring in cases of permanent alopecia and elastic tissue stains may be helpful in the histological evaluation of alopecia.

Alopecia↗

A morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile.

UNLABELLED: Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy characterized by a distinctive storiform growth pattern and frequent local recurrences. In this study, we retrospectively reviewed 48 cases of DFSP diagnosed at the Cleveland Clinic Foundation between 1970 and 1999 to determine the prevalence of morphologic variations including the presence of giant cell fibroblastoma (GCF)-like areas, multinucleated giant cells, hypercellular zones and fibrosarcomatous change. RESULTS: The cohort consisted of 42 patients (20 males, 22 females) with a median age at diagnosis of 40 years (range: 10-73 years). Forty-one primary tumors and seven recurrences were evaluated from these 42 patients. Tumor sites included the trunk (22 cases), head and neck (8 cases), upper extremities (7 cases) and lower extremities (6 cases). GCF-like areas were identified in seven (14.6%), multinucleated giant cells in ten (20.8%), hypercellular zones in 12 (25%) and fibrosarcomatous change in six (12.5%) cases, respectively. Combinations included giant cells and GCF-like areas (two cases), giant cells and hypercellular zone (two cases), and GCF-like areas and hypercellular zones (one case). Our findings suggest that DFSP has a wider range of morphologic features, including GCF-like areas, multinucleated giant cells, hypercellular zones and fibrosarcomatous change, than has been previously recognized in the literature.

Adolescent↗

Hirsutism in women. Effective therapy that is safe for long-term use.

Hirsutism should be considered part of the androgen-excess syndrome unless another cause (e.g., masculinizing tumor, androgenic-drug use) can be established. Medical evaluation for transient or late-onset androgen excess, polycystic ovary syndrome, and insulin resistance is important because of the risks associated with chronic androgen excess. Treatment of insulin resistance with antiandrogen and/or insulin-lowering therapy can reduce ovarian testosterone levels and hirsutism. Simple laboratory evaluation (i.e., measuring total and free testosterone, DHEAS, and androstenedione) identifies about half of patients with hyperandrogenism. More extensive evaluation and testing are required in the remaining half. Combination therapies, specifically oral contraceptives along with antiandrogen agents, are the most effective. Studies suggest that addition of low-dose GnRH agonist therapy prolongs remission of hirsutism. Most methods produce improvement within 6 months, with continued improvement at 12 months. Successful treatment results in finer hair, decreased rate of growth, decreased need for cosmetic camouflage or removal, and improved appearance. All methods, whether used continuously or intermittently, should be considered long term.

Androgen Antagonists↗

Pigmented purpuric dermatosis in a young male.

The pigmented purpuric dermatoses (PPD) are a group of disorders that most often occur on the lower extremities of middle-aged adults as asymptomatic, yellow-orange patches with petechiae. The PPD represent a benign, often chronic, capillaritis of unknown cause. We present a case of PPD on the abdomen of a young male and emphasize careful observation in such a patient to reveal possible progression to purpuric mycosis fungoides.

Adult↗

Once-weekly fluconazole (450 mg) for 4, 6, or 9 months of treatment for distal subungual onychomycosis of the toenail.

BACKGROUND: Fluconazole is a bis-triazole antifungal agent approved for the treatment of oropharyngeal, esophageal, and vaginal candidiasis, serious systemic candidal infections, and cryptococcal meningitis. OBJECTIVE: The purpose of this study was to evaluate three different durations of once-weekly fluconazole for the treatment of onychomycosis of the toenail caused by dermatophytes. METHODS: In a multicenter, randomized, double-blind, parallel, placebo-controlled trial, 384 patients with distal subungual onychomycosis of the toenail received fluconazole, 450 mg once weekly, or placebo for 4, 6, or 9 months. For inclusion, patients were required to have mycologically confirmed distal subungual onychomycosis of the toenail with a large toenail at least 25% clinically affected but having at least 2 mm of healthy nail between the nail fold and the proximal onychomycotic border. Efficacy was assessed by clinical and mycologic (microscopic and microbiologic) measures at screening, at every treatment visit starting at month 3, and at months 2, 4, and 6 after therapy. Observed or volunteered adverse events were recorded and classified at all visits. RESULTS: At the end of treatment, very significantly superior clinical and mycologic results were achieved in all fluconazole groups compared with placebo (p=0.0001). This superiority was largely maintained over 6 months of follow-up. The clinical and mycologic responses of the 9-month treatment duration were significantly superior to the 4- and 6-month durations. Similar percentages of patients in the fluconazole and placebo groups reported adverse experiences for all three durations of the study. CONCLUSION: Results of this study support the efficacy and safety of fluconazole in the treatment of distal subungual onychomycosis of the toenail.

Adolescent↗

Striate palmoplantar keratoderma: a clinical and ultrastructural study.

Palmoplantar keratodermas are poorly understood heterogeneous disorders of cornification. Autosomal dominant and autosomal recessive keratodermas with a varied clinical presentation are recognized. Striate keratoderma is uncommon. We report the case of a 15-year-old girl with keratoderma that began at 5 months of age. Her clinical and ultrastructural findings are reviewed. Routine microscopic examination revealed eosinophilic inclusions in the granular cell layer as well as prominence of the granular cell layer. Electron microscopic examination revealed corneocytes with an imbricated pattern. Ultrastructural findings in this unique case are contrasted with those of other palmoplantar keratodermas.

Adolescent↗

Cicatricial pemphigoid (benign mucous membrane pemphigoid).

Cicatricial pemphigoid is a chronic mucocutaneous bullous condition. It is a heterogenous autoimmune disease, characterized by the production of autoantibodies against basement membrane zone antigens. The target antigens in cicatricial pemphigoid appear to be lamina lucida proteins involved in human keratinocyte adhesion to extracellular matrix. Cicatricial pemphigoid primarily affects persons older than 40 years and appears to have a 2:1 predilection for women, without racial or geographic bias.

Adult↗

Eosinophils in fibrous tracts and near hair bulbs: a helpful diagnostic feature of alopecia areata.

BACKGROUND: When biopsy specimens lack a "swarm of bees" peribulbar lymphoid infiltrate, the diagnosis of alopecia areata depends on the recognition of other histologic features of the disease. OBJECTIVE: Our objective was to determine the frequency of the presence of eosinophils in biopsy specimens of alopecia area'a in relation to the other major histologic features of the disease. METHODS: Biopsy specimens from 71 patients with alopecia areata were studied. RESULTS: Eosinophils were present in 38 of the 71 cases. A peribulbar lymphoid infiltrate was absent in 27 of the 71 cases. Eosinophils were present in 13 of these cases. Multiple catagen hairs and pigment casts (features which may lead to confusion with trichotillomania) were present in 39 cases. The presence of eosinophils was found to be a helpful diagnostic feature in cases with potential for misdiagnoses as trichotillomania. CONCLUSION: Eosinophils are common in all stages of alopecia areata, both within the peribulbar infiltrate and within fibrous tracts. A "swarm of bees" peribulbar infiltrate may be absent. The presence of eosinophils is a helpful diagnostic feature of alopecia areata.

Alopecia Areata↗