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Biomedical subjects

Clay J Cockerell

Publications and source records attributed to Clay J Cockerell.

At least 19 recordsLinked to original sources

The head-tilt maneuver: a clinical aid in recognizing head and neck angiosarcomas.

BACKGROUND: Cutaneous angiosarcoma is a rare, life-threatening tumor that is often initially misdiagnosed. This delay in diagnosis can affect tumor growth, metastatic potential, and prognosis. OBSERVATIONS: We describe the "head-tilt maneuver," which highlights the vascular nature of these lesions and can be of potential benefit in early recognition and better appreciation of the clinical extent of this tumor. CONCLUSION: Early recognition and aggressive management of these tumors can afford the best opportunity for cure.

Diagnosis, Differential↗

Follicular hybrid cysts with infundibular, isthmic-catagen, and pilomatrical differentiation: a report of 2 patients.

The term hybrid cyst was originally coined to describe combination follicular cysts with both epidermoid and trichilemmal (pilar) epithelial zones, separated by an abrupt transition. The concept has since expanded to include many different combinations of pilosebaceous unit differentiation. Entities previously reported also include infundibular and pilomatricomal cyst, trichilemmal and pilomatricomal cyst, eruptive vellus hair cyst combined with steatocystoma or trichilemmal cyst, and trichilemmal, sebaceous, and pilomatricomal cyst. We report 2 cases of follicular hybrid cysts, all mixtures of epidermoid, trichilemmal, and matrical differentiation. The cysts, involving the ear and abdomen, occurred in one woman and one man.

Abdominal Neoplasms↗

Photodermatitis with minimal inflammatory infiltrate: clinical inflammatory conditions with discordant histologic findings.

Dermatoses associated with cutaneous photosensitivity are a group of photodistributed skin eruptions caused or exacerbated by light. Multiple clinical variants of photosensitive dermatoses have been characterized including polymorphous light eruption, chronic actinic dermatitis, solar urticaria, phototoxic and photoallergic dermatitis, reticular erythematous mucinosis, acute cutaneous lupus erythematosus, and dermatomyositis. As there may be significant overlap among the clinical presentation of these conditions, the specific diagnosis of individual photodermatosis relies heavily on characteristic histopathologic features. We present here 5 cases of photodistributed eruptions with virtual absence of histologic epidermal changes and dermal inflammation, yet all were described clinically as being "inflammatory" and erythematous. All cases of this "pauci-inflammatory photodermatitis" presented with photodistributed bright red macular erythema or slightly indurated plaques that developed over a period of weeks to months and clinically resembled photoallergic or phototoxic drug reactions or polymorphous light eruption. Microscopically, however, only very sparse dermal lymphocytic infiltrate was noted with no or minimal epidermal changes. To our knowledge, the observation of clinically evident photodistributed dermatoses that demonstrate such minimal histopathologic findings has not been reported. Clinicians and histologists should be aware of the disparity that may be encountered in this setting, as the clinical features are usually far more impressive than those seen histologically.

Aged↗

Completely regressed primary cutaneous malignant melanoma with nodal and/or visceral metastases: a report of 5 cases and assessment of the literature and diagnostic criteria.

BACKGROUND: Partial regression of primary cutaneous malignant melanoma is not uncommon and may predict a higher likelihood of metastasis and decreased survival. Complete histologic regression of a primary cutaneous melanoma is a rarer occurrence, with only 34 cases reported in the English-language or English language-summarized literature. OBSERVATION: We detail 4 cases of complete histologic regression of primary cutaneous melanoma, discovered at presentation with metastatic disease. A pigmented lesion or its remnant, coupled with historical information, was strongly suggestive of cutaneous melanoma. Histologic examination of the lesions, using multiple levels and immunohistochemical stains, failed to reveal residual melanoma. Our cases are typified by the presence of metastasis of melanoma to regional lymph nodes, with the absence of other suspect skin lesions or malignancies. In addition, we present a fifth case involving a completely regressed lesion on the scalp in a patient with cerebral melanoma metastasis and comment on the implications of this case to accepted diagnostic criteria, proposing that consideration of modification to the criteria be entertained. CONCLUSION: The concept of completely regressed primary cutaneous melanoma is reviewed and the literature critically appraised. When one considers a diagnosis of completely regressed primary cutaneous melanoma, cases must be well documented and biopsy proven. Patients with metastatic melanoma and an occult primary lesion require a thorough skin examination, with serious consideration given to the possibility of completely regressed cutaneous melanoma.

Adult↗

Histologic variants of fibrous papule.

Fibrous papules (FPs) are common benign lesions usually of the face. Most are readily recognizable histologically, although several variants exist that may not be as easily diagnosed. These include hypercellular, clear cell, pigmented, pleomorphic, and inflammatory variants. A granular cell variant has also been described. We microscopically evaluated 212 FPs, and of those, 184 demonstrated features of one of the variants. We conclude that variants of FP may be encountered not uncommonly. Dermatopathologists should be aware of these to avoid misdiagnosis.

Angiofibroma↗

Acrokeratoelastoidosis.

A 45-year-old white woman presented with several years' history of firm, shiny papules on the lateral hands with slight extension to the dorsal fingers. The lesions first appeared between the index fingers and thumbs on both hands. They gradually increased in number, coalescing into plaques and affecting the junction between the palmar and dorsal skin. The patient did not have involvement of her feet. She had been diagnosed previously with chronic eczema that had failed to respond to multiple topical medications. In addition, the patient's sister had similar lesions on both hands. The patient denied any symptoms of hyperhidrosis, excessive sun exposure, or trauma. The plaques were asymptomatic, but were cosmetically unappealing to the patient. On physical examination, small, firm, skin-colored, hyperkeratotic papules, coalescing into plaques, were located on the junction between the palmar and dorsal skin on both lateral margins of the thumb and on the radial side of the index finger (Fig. 1). There were no lesions on the feet. A biopsy taken from a papule on the patient's left hand was consistent histologically with acrokeratoelastoidosis. The biopsy showed marked degeneration of collagen in the dermis with solar elastosis and some smudging of the papillary dermal collagen (Fig. 2). She was treated with clobetasone cream to the affected areas on the hands. After 6 weeks of treatment, she reported no significant improvement.

Clobetasol↗

Decubitus ulcers: a review of the literature.

Decubitus ulcers are a worldwide health care concern affecting tens of thousands of patients and costing over a billion dollars a year. Susceptibility to pressure ulcers comes from a combination of external factors (pressure, friction, shear force, and moisture), and internal factors (e.g. fever, malnutrition, anaemia, and endothelial dysfunction). Often, enough damage is done to create the basis for a decubitus ulcer after as little as 2 h of immobility, a situation which may be difficult to avoid if the patient must undergo prolonged surgery or remain bedridden. Damage owing to pressure may also occur hours before the patient receives medical attention, especially if the patient falls or becomes immobilized owing to a vascular event. Several classification systems for decubitus ulcers have been described, based on where injury first occurs. The histologic progression of decubitus ulcers is a dynamic process involving several stages, each having characteristic histologic features. A team-focused approach integrating all aspects of care, including pressure relief, infection control, nutrition, and surgery, may improve healing rates. With accurate risk assessment and preventative care, we can hope to minimize complications and mortality owing to decubitus ulcers.

Humans↗

Forensic dermatopathology and internal disease.

The gross and microscopic analysis of skin lesions at autopsy can help the pathologist understand diseases and injuries inflicted premortem, perimortem, or postmortem. From January 2003 to January 2004, skin findings at autopsy were closely examined by a dermatologist and sampled for microscopic analysis at the Southwestern Institute of Forensic Sciences. Dermatologic abnormalities in some of these cases led to the discovery of internal disease and allowed for a more complete understanding of the pathologic disease processes affecting the individual. We present four autopsy cases with skin manifestations of internal disease, including pseudoxanthoma elasticum, calciphylaxis, the sign of Leser Trelat, and papular mucinosis, and demonstrate the usefulness of the dermatological assessment at autopsy. In all cases, discovery of these skin lesions and internal disease manifestations allowed contributing factors to the death of the individual to be uncovered.

Adult↗

Is Degos' disease a clinical and histological end point rather than a specific disease?

Degos' disease is described as a rare disorder, with approximately 100 cases detailed in the literature. Nearly all are characterized by the near "pathognomonic" appearance of porcelain-white, atrophic papules with peripheral erythema and telangiectases. Many Degos' disease variants have been described including benign cutaneous Degos' disease, familial Degos' disease, atrophie blanche with Degos'-like features, and connective tissue diseases with similar findings. The course, prognosis, and treatment have substantially varied. We present four patients: the first carries a diagnosis compatible with classic Degos' disease, the second and third demonstrate cutaneous and histological findings of Degos' disease but laboratory evidence suggestive of lupus erythematosus, while the fourth has dermatomyositis with Degos'-like lesions. Because of broad overlap in clinical and histological findings, we contend that Degos' disease may not be a specific entity, but rather, may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated.

Adult↗

Interstitial heparan sulfate in granulomatous inflammatory skin diseases.

BACKGROUND: Heparan sulfate (HS) is a glycosaminoglycan that is anchored to the outside of cell membranes. Under ordinary circumstances, it is not present in the interstitium, but under certain circumstances, mainly in the setting of inflammation and tissue repair, HS can be shed from the cell surface into the interstitium in a regulated fashion. Under these circumstances, interstitial HS seems to have an immunomodulatory function because of its binding of many cytokines. However, it is not known which cell types present at an inflammatory site are responsible for this shedding. OBJECTIVE: We have investigated the presence of interstitial HS by immunohistochemistry in various inflammatory skin diseases characterized by different compositions of the inflammatory infiltrate. RESULTS: Strong interstitial HS immunoreactivity was present only in diseases with a predominantly histiocytic infiltrate but not in diseases with a predominantly lymphocytic or neutrophilic infiltrate. CONCLUSIONS: This indicates that histiocytes have a direct or indirect role in the HS shedding process. In the well-formed granulomas of sarcoidosis, interstitial HS immunoreactivity was spatially associated with the fibrotic ring at the periphery of the granulomas, but not with the center harboring the histiocytes. This suggests that histiocytes can stimulate fibroblasts to shed HS into the interstitium.

Cell Communication↗

Histological features of kaposi sarcoma in a patient receiving highly active antiviral therapy.

The introduction of highly active anti-retroviral therapy (HAART) has changed the clinical presentation of skin diseases in patients with the Acquired Immune Deficiency Syndrome (AIDS). This occurs as a consequence of a newly recognized effect of this therapy known as the "Immune Reconstitution Syndrome," which develops with improvement of immunity. One manifestation of this syndrome is alteration of the natural history of Kaposi sarcoma (KS). While there are multiple reports of KS regression during HAART, there is little documentation of the histologic changes that occur. We present the case of a 55-year-old homosexual male with KS for over 4 years who underwent 6 biopsies over a 3-year period before, during, and after HAART. Kaposi sarcoma lesions prior to HAART were multinodular with ill-defined borders clinically and demonstrated typical features of nodular stage KS histologically. After initiation of HAART in this patient, lesions became uninodular, well circumscribed, and histologically were noted to be less cellular and surrounded by a dense fibrotic stroma. Although the mechanisms for these histologic changes are not clear, it may be due to activity of HAART against human herpesvirus type 8 (HHV-8), the causative agent of KS, to reconstitution of immunity due to diminution in HIV viral load, or both.

AIDS-Related Opportunistic Infections↗

Muir-Torre syndrome: role of the dermatopathologist in diagnosis.

Muir-Torre syndrome (MTS) is an autosomal dominantly inherited disorder characterized by sebaceous lesions and visceral malignancies. The defect is thought to be the result of a mutation in mismatch repair genes and associated with microsatellite instability. Two cases whose diagnoses were suggested first by the dermatopathologist are discussed. The first is a 47-year-old white man who over the past 6 years developed multiple sebaceous lesions. Due to the number of sebaceous lesions and their morphology, the possible diagnosis of MTS was suggested by the dermatopathologist. Subsequently, a lesion in the right colon was found during colonoscopy that proved to be a poorly differentiated cecal adenocarcinoma. A pedigree analysis revealed other family members afflicted with multiple malignancies. Genetic testing of the colonic adenocarcinoma showed microsatellite instability. The second patient is a 50-year-old white man who underwent biopsy of a skin lesion that showed features of both a sebaceous hyperplasia and sebaceous adenoma. Because of the mixed, unusual features of the lesion, the dermatopathologist suggested the diagnosis of MTS. It was later confirmed that the patient had a history of malignancies of the colon and kidney as well as a family history significant for multiple malignant neoplasms. These cases demonstrate the important role of the dermatopathologist in alerting the clinician to the possibility of Muir-Torre syndrome when the diagnosis of a sebaceous neoplasm is made, especially when unusual histologic features are observed.

Adenocarcinoma, Sebaceous↗

Sclerotic fibroma-like change in various neoplastic and inflammatory skin lesions: is sclerotic fibroma a distinct entity?

Sclerotic fibroma was first described in association with Cowden's disease by Weary et al. in 1972. In 1989, Rapini and Golitz detailed 11 cases of solitary sclerotic fibroma (SFS) in the absence of Cowden's disease, suggesting the term SFS of the skin. Classic histological features include hypocellular, hyalinized bands of collagen sharply demarcated from the surrounding skin. Numerous authors have described sclerotic fibroma-like changes in other entities including melanocytic nevi, dermatofibromas, lipomas, tendon sheath fibromas, giant cell collagenomas, neurofibromas, angiofibromas, erythema elevatum diutinum, and folliculitis. Dissension has arisen, with some dermatopathologists asserting that sclerotic fibroma is just an evolutionary end-point of a previous lesion. Others contend that SFS is a distinct lesion and cite recurrent cases and/or proliferation marker studies to corroborate this view. We detail the histopathological findings of lesions consistent with the classic description of SFS and compare these to sclerotic changes observed in an intradermal nevus, blue nevus, erythema elevatum diutinum, neurofollicular hamartoma, angiofibroma, neurofibroma, accessory nipple, and dermatofibromas. Sclerotic fibroma-like change may be seen in a variety of lesions and may represent a common reaction pattern in the skin.

Adolescent↗

Neurofibromatous changes in dermatofibrosarcoma protuberans: a potential pitfall in the diagnosis of a serious cutaneous soft tissue neoplasm.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant neoplasm that has the potential for aggressive local growth and destruction if not treated appropriately. Although the storiform arrangement of spindle cells in DFSP is relatively characteristic, histologic patterns simulating other benign as well as malignant neoplasms such as dermatofibroma, neurofibroma, malignant fibrous histiocytoma, and atypical fibroxanthoma have been described. METHODS: We collected and analyzed six cases of probable DFSP in which a specific diagnosis could not be rendered due to the predominant neurofibromatous changes in the histologic sections. In an attempt to reach a definitive diagnosis, the clinical history and physical characteristics of the lesions were taken into account, and all cases were further evaluated using immunostaining for CD34 and S-100 protein. RESULTS: The average age of the patient was 56 years (range 21-80), and the male to female ratio was 1 : 1. The location of lesions included the scalp, neck, back, and abdomen. All cases displayed two distinct histological patterns: (i) a proliferation of spindle cells with wavy nuclei in a loose mucinous stroma suggesting neural differentiation and (ii) a proliferation of spindle cells which interweaved and filled the reticular dermis extending into the subcutis. The wide variety of clinical impressions and descriptions indicated that the diagnoses were not always straightforward, and clinical information did not always assist in the clinicopathologic correlation. All lesions stained positively for CD34; however, three of six cases also stained positively for S-100. The three cases which were CD34 positive and S-100 negative were likely DFSP, and this was the final diagnosis given. The three cases that were CD34 and S-100 positive did not allow for a straightforward diagnosis. CONCLUSIONS: DFSP may demonstrate areas with features more characteristic of a benign neural lesion, such as a neurofibroma, which can lead to underdiagnosis and subsequent failure to treat. Clinicians and pathologists should recognize this potential diagnostic pitfall and understand that equivocal clinical information, combined with non-specific immunohistochemical staining patterns, can further complicate the dilemma. In these situations, where DFSP is the likely diagnosis but definitive evidence cannot be obtained, full excision of the lesion should be recommended to avoid mistreatment of a potentially malignant lesion.

Adult↗