Biomedical subjects
Publications and source records attributed to Klaus F Helm.
We describe a patient with localized chronic fibrosing vasculitis who presented with a rhinophymalike eruption of the nose. To our knowledge, the manifestation of this condition on the nose has not been previously reported. The differential diagnosis also is examined.
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BACKGROUND: The CD30-positive lymphoproliferative disorders lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (C-ALCL), and systemic anaplastic large cell lymphoma (S-ALCL) are lesions that overlap clinically, histopathologically, and immunophenotypically. Their biologic behaviors, however, vary considerably. In particular, lesions of LyP regress spontaneously while those of S-ALCL persist and often progress. Apoptosis has been suggested as the mechanism by which the lesions of LyP regress, but the underlying signaling pathways remain unclear. In this study, we used newly developed activation state-specific antibodies to demonstrate apoptosis signaling through the death receptor-mediated pathway regulated by FADD and caspase 3. METHODS: Dual immunohistochemistry for CD30 and activated forms of FADD and caspase 3 was performed on cutaneous biopsy specimens from 27 patients with CD30-positive lymphoproliferative disorders involving the skin. The patients included 18 with primary cutaneous CD30-positive LPDs (15 with LyP and 3 with C-ALCL) and 9 with S-ALCL. RESULTS: The proportion of CD30-positive cells expressing activated FADD was significantly different between primary cutaneous CD30-positive lymphoproliferative disorders and S-ALCL (36.4% vs. 14.5%, P = 0.0083). Expression of cleaved caspase 3 was also significantly different between primary cutaneous lesions and S-ALCL (9.2% vs. 1.9%, P = 0.048). CONCLUSIONS: Although a larger number of cases should be studied to validate these results, these data provide evidence that differences in signaling through the death-receptor apoptosis pathway mediated by FADD may be responsible for the varying biologic behaviors of CD30-positive lymphoproliferative disorders involving the skin.
Dermatopathologists have noted an increased number of smaller biopsy specimens. Our objective was to determine whether dermatologists are changing their choice of biopsy technique and the size of biopsy specimens. We conducted a retrospective study of 500 biopsies performed by dermatologists for the purpose of diagnosis in 1988, 1993, 1998, and 2003. Our study results indicate that dermatologists are performing fewer punch biopsies and more shave biopsies. What's more, there has been a decrease in the size of shave biopsy specimens. We believe that the decreased number of punch biopsies being performed combined with the decreased size of shave biopsy specimens may affect patient care.
BACKGROUND AND OBJECTIVE: Technological improvements have helped digital cameras become widely available and inexpensive. Dermatopathologic diagnosis is often aided by clinical information. The purpose of this study was to determine whether submitting a digital image along with a skin biopsy provides additional helpful information to the dermatopathologist. METHODS: Digital pictures were taken of the lesion being biopsied and were submitted along with routine paper work to the dermatopathologists. The dermatopathologists interpreted the skin biopsy as usual without viewing the digital image, and then later viewed the submitted picture. The dermatopathologists evaluated the help provided by the image. RESULTS: A clinical digital image was helpful in confirming the initial diagnosis and narrowing down the differential diagnosis (p < 0.001). Including pictures did not change the diagnosis, result in an increase in the differential diagnosis, or result in fewer or additional step sections being performed. Digital imaging of inflammatory disorders was felt to be good help in 43% of the biopsies and narrowed down the differential diagnosis in 31%. CONCLUSION: In certain cases the inclusion of a digital image may help a dermatopathologist. Digital imaging was found to be most helpful in evaluation of inflammatory disorders.
Basal cell carcinoma (BCC) has many histologic variants. These variants may show differentiation toward benign and malignant tumors of cutaneous appendages, especially hair follicles. Herein we describe a lesion that was clinically thought to be a BCC, but a superficial biopsy showed histologic features suggestive of an apocrine hidrocystoma. Because of some cytologic atypia, complete excision was recommended. The excision specimen revealed a cystic BCC. The importance of examining the entire neoplasm before reaching a final pathologic diagnosis is emphasized.
BACKGROUND: The mnemonic ABCD, which stands for asymmetry, border irregularity, color variation, and diameter greater than 6 mm alerts physicians to features that suggest melanoma. Although the ABCD criteria are guidelines for diagnosis, clinicians may overlook many melanomas that do not follow the ABCD rule. OBJECTIVE: We examined the sensitivity of the diameter portion of the ABCD rule. METHODS: Retrospective study examining the pathology reports of 383 melanomas. Data were compiled for each melanoma regarding its diameter, depth, body location, patient age, and sex. A 95% confidence interval (p=0.05) was used to identify the proportion of melanomas less than or equal to 6 mm in diameter. A two-tailed p value approach (p value =0.05, degrees of freedom=28) was used when evaluating two independent populations, lesions less than or equal to 6 mm and lesions greater than 6 mm in diameter. RESULTS: A total of 38.21% of melanomas were less than or equal to 6 mm in diameter after processing. Melanomas greater than 6 mm in diameter occurred in significantly older patients and at a greater Breslow's thickness than smaller melanomas. CONCLUSIONS: We demonstrated that a significant proportion of melanomas may be smaller than 6 mm. The ABCD criteria are not absolute; melanomas have many different appearances and start as small lesions.
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Primary mucinous carcinoma is a rare malignant tumor that most frequently occurs in the periorbital area. This tumor originates from the deepest portion of the eccrine sweat duct. This normally asymptomatic and slow-growing tumor has demonstrated a local recurrence rate of 30% after excision with narrow surgical margins and can have local metastases. It is difficult to differentiate this tumor histologically from metastatic lesions. Immunohistochemical staining and cytokeratin profiles have been studied to aid in the differentiation between primary lesions and metastatic mucinous carcinomas. We present a case of a 54-year-old man with recurrent primary eccrine mucinous carcinoma and review the clinical, histologic, and immunohistochemical features of this tumor.
BACKGROUND: Antibodies to the B-cell-specific antigen CD20 are widely used for immunohistochemical identification of B-cell lymphomas, approximately 95% of which are strongly CD20-positive. METHODS: We report a 51-year-old male with a CD20-positive systemic B-cell lymphoma who developed a CD20-negative relapse with secondary cutaneous involvement after therapy with the anti-CD20 monoclonal antibody rituximab (Rituxan). RESULTS: Biopsy of a skin nodule demonstrated an atypical lymphocytic infiltrate that was negative for CD20, CD3, lysozyme, and myeloperoxidase, but strongly positive for CD45rb (LCA) and the B-cell marker CD79a. CONCLUSIONS: We conclude that loss of CD20 expression in cutaneous B-cell lymphoma (both primary and secondary) is important to recognize, that immunohistochemistry for CD79a, another widely expressed B-cell marker, is useful in the identification of CD20-negative B cells in such cases, and that loss of CD20 expression may become more common, as use of rituximab is expected to increase.
Pyoderma gangrenosum (PG) is a rare, ulcerative skin disease. Diseases with cutaneous manifestations resembling PG may lead to the misdiagnosis of PG. We discuss two patients presenting with cutaneous ulcers originally thought to be PG, and review the literature on the clinical and pathologic characteristics of PG ulcers compared to PG-like ulcers in order to determine possible distinguishing features.
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BACKGROUND: Alopecia mucinosa has been shown to be associated with many benign and malignant conditions. It can be seen in childhood but is seen more commonly in adulthood. Alopecia mucinosa is generally felt to occur in three settings: a primary idiopathic form, a form associated with malignancy, and a form secondary to inflammatory conditions. The histologic hallmark is the accumulation of mucin in the follicular epithelium, called follicular mucinosis. Therapy for alopecia mucinosa remains problematic. OBJECTIVE: We present a representative case of alopecia mucinosa and discuss the etiology, histology, epidemiology, treatment, and prognosis. CONCLUSION: We report a case of idiopathic alopecia mucinosa that responded to minocycline with a complete remission and we review the literature on alopecia mucinosa.
BACKGROUND: Erdheim-Chester disease is a rare non-Langerhans' cell histiocytosis. OBJECTIVE: This case report is presented to familiarize clinicians with Erdheim-Chester disease and its differential diagnosis. RESULTS AND CONCLUSION: Erdheim-Chester disease presents with unique clinical and pathologic findings. Its xanthoma-like lesions can cause significant morbidity and mortality.
BACKGROUND: Physicians inevitably receive a pathology report after excision of a basal cell carcinoma that indicates that it is incompletely excised. The physician and patient are then left with the dilemma of whether immediate re-excision or close clinical follow-up is indicated. OBJECTIVE: Our purpose was to identify characteristics of incompletely excised basal cell carcinomas that are at low risk for recurrence. METHODS: We retrospectively reviewed the charts and pathology slides of all incompletely excised basal cell carcinomas from 1991 to 1994 in a university hospital tumor registry. RESULTS: Incompletely excised basal cell carcinomas of superficial or nodular subtype, less than 1 cm in diameter, located anywhere except the nose or ears, with less than 4% marginal involvement on the initial inadequate excision had no evidence of tumor persistence. CONCLUSION: When physicians receive a pathology report indicating the incomplete excision of a basal cell carcinoma, they face the dilemma of further management. The majority of patients should undergo immediate re-excision or Mohs micrographic surgery because tumor persistence was found in 28% of cases. Occasionally, for a small group of select patients, close clinical follow-up may be indicated if the risk of recurrence is very low.
Malignant myxoid melanoma (MMM) is a rarely reported variant of malignant melanoma, which can often be confused with other mucin-containing neoplasms. A retrospective study of 3 cases of MMM and a review of the English-language literature was performed. MMM affects an older population and is frequently misdiagnosed. The major pathologic features are atypical spindle cells embedded in a myxoid stroma. Immunohistochemistry analysis of the tumor shows uniform staining of the spindle cells with S-100. In our 3 cases, there were noticeably more mast cells that could be detected with Giemsa stain and with antibody against transforming growth factor. The prognosis appears to be equivalent to other primary melanomas. Diagnosing MMM requires a high index of suspicion. We hypothesize that mast cells and secretion of transforming growth factor beta stimulates fibroblast secretion of mucin, which contributes to the tumor's invasive potential.
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We reviewed the pathologic findings on a family with multiple hereditary trichoepitheliomas. Although the majority of the lesions were trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma) were present, representing a spectrum of lesions exhibiting folliculosebaceous (trichoepithelioma, basal cell carcinoma) and apocrine (spiradenoma, spiradenocylindroma) differentiation. Multiple familial trichoepitheliomas may be a syndrome whereby tumors develop from undifferentiated germinative cells of the folliculosebaceous-apocrine unit. Published findings regarding the genetics of this syndrome and solitary trichoepitheliomas are reviewed; although the molecular basis for the tumors has yet to be determined, current data suggest that a tumor suppressor gene may be involved.