PubMed Health⌕ Search

Biomedical subjects

Clay J Cockerell

Publications and source records attributed to Clay J Cockerell.

33 records · Page 2Linked to original sources

Buschke-Ollendorff syndrome: report of a case and interpretation of the clinical phenotype as a type 2 segmental manifestation of an autosomal dominant skin disease.

Buschke-Ollendorff syndrome is a rare, autosomal dominant disease consisting of osteopoikilosis and skin manifestations. A case is reported, and the literature is reviewed with special reference to the clinical distribution patterns of skin lesions. The 2 main types of skin manifestations in this entity are widely disseminated, symmetrically distributed papules and localized, asymmetrically distributed plaques. Both types of lesions have been observed within the same family or within the same person. This particular phenotype can be explained by type 2 segmental manifestation of an autosomal dominant cutaneous trait: Symmetrically distributed papules are a manifestation of the heterozygous state acquired by inheritance, and asymmetrically distributed plaques develop in areas that have undergone a somatic mutational event of the wild-type allele at an early developmental stage, the result being loss of heterozygosity.

Female↗

Late-stage nodular erythema elevatum diutinum.

Erythema elevatum diutinum (EED) is a rare chronic vasculitic process of unknown etiology that presents as bilateral, symmetrical, periarticular, red-brown papules and plaques, often over dorsa of joints. Early histologic changes are characterized by leukocytoclastic vasculitis. With chronicity, the lesions develop dense neutrophilic infiltrate and fibrosis. We describe an unusual case of late-stage nodular EED present for 35 years in a seronegative 75-year-old man. Asymptomatic papules and plaques measuring from 0.7 to 9.0 cm were seen over the interphalangeal joints, elbows, knees, and ankles. The histologic findings were characterized by predominant concentric fibrosis that formed well-circumscribed dermal and subcutaneous nodules. Awareness of this unusual presentation of EED helps to avoid misdiagnosis as cutaneous neoplasms.

Aged↗

Reliability and validity of a histologic score as a marker for skin cancer chemoprevention studies.

OBJECTIVE: To develop a reliable and valid scoring system for grading skin biopsies from actinic keratosis (AK) and sun-damaged skin for use in evaluating the efficacy of skin cancer chemopreventive agents. STUDY DESIGN: A panel of dermatopathologists developed histologic criteria and diagnostic definitions for the progression of lesions from early AK to AK. The criteria were then applied to a sample of 335 histologic slides from an ongoing chemoprevention study. A 10% sample of 35 slides was reread in order to assess intrarater reliability. RESULTS: Six of the 7 criteria demonstrated high reliability (> 85%). The total histologic score, calculated using the 6 criteria, was found to significantly differentiate between (blinded) biopsy location (normal, pre-AK, AK and adjacent to squamous cell carcinoma) and histologic diagnosis (normal, pre- or early AK, AK and squamous cell carcinoma). CONCLUSION: The total histologic score, having demonstrated reliability on repeated readings and validity in its association with biopsy location and histologic score, is a reliable and valid end point for judging the efficacy of agents in skin cancer chemoprevention studies. Additional interrater reliability tests utilizing larger test sets and a rigorous statistical design should be undertaken to establish its portability.

Biopsy↗

Abundance of interstitial heparan sulfate in granuloma annulare but not in other mucinous skin diseases.

BACKGROUND: Heparan sulfate (HS), unlike other glycosaminoglycans, is mainly located on cell surfaces but can be shed into the interstitium by a regulated process. It has been found in interstitial fluid drained from cutaneous wounds, but otherwise the conditions under which the release of HS from the cell surface occurs are unknown. To better characterize this process, we have investigated the presence of interstitial HS in various skin diseases with glycosaminoglycan accumulation. METHODS: Histologic routine material was stained immunohistochemically using an antibody recognizing HS. RESULTS: Heparan sulfate immunoreactivity is present in the interstitium of young cutaneous scars and in the interstitium of the inflammatory infiltrate of granuloma annulare. No reactivity was found in a number of non-inflammatory skin diseases with mucin deposition. CONCLUSIONS: The selective presence of interstitial HS in only two of the investigated skin conditions supports the existence of a regulated mechanism to release HS from the surface of cells into the interstitium. It is suggested that HS modulates the biologic actions of growth factors and cytokines not only during wound repair but possibly also in inflammatory skin diseases such as granuloma annulare.

Cicatrix↗

Dermatology and the Americans With Disabilities Act: a review of the case law.

The Americans With Disabilities Act (ADA) defines disability as a physical or mental impairment that substantially limits one or more major life activities. Although dermatology has received relatively little attention in the context of disability law, dermatologic diseases are properly covered by the ADA and are subject to the same criteria as other medical conditions. A Lexis-Nexis search of federal court decisions covering the ADA produced 23 cases dealing with dermatologic impairments as disabilities. In Cehrs v Northeast Ohio Alzheimer Research Center, a federal appeals court held that psoriasis constituted a disability under the Act. Skin diseases not only cause physical and mental impairments, but they are also visible to others. Persons with skin diseases may be "regarded as" disabled, and this can constitute discrimination under the law.

Dermatology↗

Characterization of a subgroup of patients with dermatitis herpetiformis with nonclassical histologic features.

Dermatitis herpetiformis (DH) is an autoimmune disease mediated by IgA antibodies. The diagnosis of DH is based on clinical presentation, biopsy for hematoxylin and eosin, and direct immunofluorescence. The chief hematoxylin and eosin finding is a subepithelial blister with neutrophils in dermal papillae. The direct immunofluorescence findings are deposition of IgA and sometimes C3 at the basement membrane with accentuation in dermal papillae. Immunofluorescence is thought to be a sensitive and specific assay in DH, and there are no other known diseases with this pattern of immunofluorescence. The aim of this project was to determine the prevalence of nonclassical histologic findings in DH. We studied 24 cases of DH received at our institution. All cases had clinical findings of DH as well as positive direct immunofluorescence with IgA. We found that 9 of 24 cases (37.5%) had nonspecific H&E findings of a lymphocytic infiltrate only with fibrosis in the dermal papillae and ectatic capillaries. The remaining 15 cases had classic findings of multilocular neutrophilic microabscesses in the dermal papillae. These findings were reproduced on step sectioning. These findings suggest that routine histology may be quite nonspecific in DH and direct immunofluorescence or other more specific immunologic assay is an essential adjunct to diagnosis.

Basement Membrane↗

Primary cutaneous B-cell lymphoma in a 74-year-old Caucasian male.

Primary cutaneous B-cell lymphoma is an uncommon neoplasm with classification schemes that may be confusing, but with clinical presentations that are relatively consistent. Clinical exam and history can raise the index of suspicion for this condition, although the diagnosis is confirmed only by pathological evaluation. We report such a scenario in the case of a 74-year old male with a slowly expanding oval plaque on his right upper arm.

Aged↗