Organ transplant recipients (OTRs) are at an increased risk of developing a de novo malignant neo... more Organ transplant recipients (OTRs) are at an increased risk of developing a de novo malignant neoplasm compared to the general population. The primary contributor to skin cancer in all patients is sun exposure. In this study, we aim to ascertain both OTR skin cancer awareness and photoprotection practices. A questionnaire-based study of Saskatchewan transplant recipients. Nearly all respondents were aware that sun exposure is the best-known cause of skin cancer and that as an OTR, they are at increased risk of skin cancer (99.3% and 90.5%, respectively). Approximately half of respondents reported wearing a hat regularly, sun avoidance between 10 am and 3 pm, or wearing sunscreen regularly (53.7%, 33.1%, and 47.9%, respectively). Many OTRs are not engaging in photoprotection. Further intervention, which may include access to a dermatologist, is necessary to ensure ORTs do not experience undue morbidity and mortality secondary to skin cancer.
The field of science and medicine has experienced a flood of data and technology associated with ... more The field of science and medicine has experienced a flood of data and technology associated with the human genome project. Over 10,000 human diseases have been genetically defined, but little progress has been made with respect to the clinical application of this ...
Keratolytic winter erythema (KWE) is a rare autosomal-dominant skin disorder characterized by rec... more Keratolytic winter erythema (KWE) is a rare autosomal-dominant skin disorder characterized by recurrent episodes of palmoplantar erythema and epidermal peeling. KWE was previously mapped to 8p23.1-p22 (KWE critical region) in South African families. Using targeted resequencing of the KWE critical region in five South African families and SNP array and whole-genome sequencing in two Norwegian families, we identified two overlapping tandem duplications of 7.67 kb (South Africans) and 15.93 kb (Norwegians). The duplications segregated with the disease and were located upstream of CTSB, a gene encoding cathepsin B, a cysteine protease involved in keratinocyte homeostasis. Included in the 2.62 kb overlapping region of these duplications is an enhancer element that is active in epidermal keratinocytes. The activity of this enhancer correlated with CTSB expression in normal differentiating keratinocytes and other cell lines, but not with FDFT1 or NEIL2 expression. Gene expression (qPCR) an...
American Journal of Medical Genetics, May 29, 2000
Actinic prurigo is an idiopathic, familial photodermatosis seen especially in American Indians. S... more Actinic prurigo is an idiopathic, familial photodermatosis seen especially in American Indians. Segregation analysis was performed on 12 Saskatchewan pedigrees with American Indian ancestry, comprising a total of 1,148 individuals, ascertained via probands diagnosed with actinic prurigo. Although a high degree of familial aggregation has been noted in the past and dominant inheritance has been suggested, no formal segregation analysis has been attempted. Actinic prurigo has a variable age of onset and, therefore, age at the time of censoring must be taken into account in the analysis. However, as these ages of 57% of the unaffected individuals were missing, an algorithm was devised to impute the missing ages from known birth years in the family based on the age differences among relatives and spouses. Using these imputed ages, simple dominant inheritance with incomplete penetrance and a single age of onset distribution was found. The method for imputing the ages at examination was evaluated, as was the correction for ascertainment, by using alternative methods and comparing the results. Regardless of the method used, a dominant mode of inheritance without any multifactorial component remained the best hypothesis.
Background: Neomycin contact sensitization rates in North America range from 7% to 13%, whereas i... more Background: Neomycin contact sensitization rates in North America range from 7% to 13%, whereas in Europe they average approximately 1.9%. Objectives: Given that topical neomycin products are no longer readily available in Canada, the aim of this study was to examine what influence this may have had on neomycin sensitization rates in the 3 western provinces. Methods: On the basis of an observation originally communicated by L. M. Parsons and C. Zhang of the University of Calgary, which suggested significantly reduced rates of neomycin sensitization in Calgary, Alberta, Canada, a multicenter study of patch test results from 5690 patient charts was undertaken. Data from 3 other western Canadian Universities (the University of Saskatchewan, the University of Alberta, and the University of British Colombia) were analyzed. Data were available from 2001 to 2013 for the University of Saskatchewan (except 2006), whereas the University of Alberta and the University of British Columbia had da...
Regular skin self-examination is suggested as a means to detect melanomas at an early stage and t... more Regular skin self-examination is suggested as a means to detect melanomas at an early stage and thus improve prognosis. Compliance, however, has seldom been assessed in a routine clinical setting and anonymously. To assess compliance with self-examination in patients with either a previous melanoma or atypical moles (dysplastic nevi) and to examine the perceived utility of supplied photographs. An anonymous questionnaire was sent to all patients seen with either a melanoma or atypical moles between the years 1995 and 2005. The melanoma cohort consisted of 143 patients. There were 440 patients with atypical moles. Replies to the questionnaire were received from 94 of the melanoma patients and from 185 patients in the atypical mole cohort. Only 22% (12) in the melanoma group performed a total skin examination monthly. Fewer than 10% of those with atypical moles did a monthly skin examination, but about half of the patients examined their entire skin more than once a year. Self-examination is practiced in the majority of patients with either a previous melanoma or atypical moles. Those doing this on a regular monthly basis are a small minority.
Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, painful palmoplantar... more Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, painful palmoplantar keratoderma and blistering, oral leukokeratosis, pilosebaceous cysts (including steatocystoma and vellus hair cysts), palmoplantar hyperhydrosis, and follicular keratoses on the trunk and extremities. PC is diagnosed by clinical findings and by molecular genetic testing. Molecular genetic testing of the four keratin genes (KRT6A, KRT6B, KRT16, and KRT17) in which mutations are known to cause PC is clinically available. Treatment of manifestations: Pain from the palmoplantar keratoderma can be reduced by limiting friction and trauma to the feet by minimizing walking or standing, reducing hydration of the stratum corneum by using wicking socks and ventilated footwear, selecting comfortable shoes, and maintaining ideal body weight. Foot care includes paring down hyperkeratotic areas and topical therapies for hyperkeratosis (emollients and lotions containing keratolyics). Care of thickened ...
Sporotrichosis is a subcutaneous fungal infection caused by the traumatic implantation of the dim... more Sporotrichosis is a subcutaneous fungal infection caused by the traumatic implantation of the dimorphic, pathogenic fungus, Sporothrix schenkii. It constitutes the most common subcutaneous fungal infection in the general population in South Africa. Sporotrichosis in South Africa dates back to 1914, when the disease was first diagnosed in the gold mines. Occupational and recreational circumstances of infection are well established, and the environmental requirements for contracting the disease are better understood. Sporotrichosis cases were recorded from 42 suburbs in the greater Pretoria area as well as from 23 towns outside the Pretoria municipal boundary. It occurred in 154 patients with ages ranging from less than 1 year to 90 years old, with males predominating. Females in the area seemed to be at lesser risk, mainly becoming infected through gardening injuries, insect bites or other minor injuries due to outdoor activities. Exposure to possible sources of the fungus, either fr...
Pachyonychia congenita (PC) is a rare but often debilitating, dominantly inherited disorder. New ... more Pachyonychia congenita (PC) is a rare but often debilitating, dominantly inherited disorder. New treatments require more accurate instruments for evaluating changes in the quality of life in these patients. This study was undertaken to develop and validate a quality of life questionnaire for PC patients (PCQoL). Relevant factors influencing quality of life in PC patients were identified and incorporated into the well-recognized, general questionnaire, the Dermatology Life Quality Index (DLQI), to establish a disease-specific measure, the PCQoL. Classical test theory (CTT) and Rasch analysis (RA) were used to analyze and validate the PCQoL. CTT analysis established test-retest reliability and internal consistency for the PCQoL. Concurrent and construct validity for the DLQI and the PCQoL were also validated. Chi-square-based infit and outfit statistics indicated that the Rasch model fits the observed responses very well. RA reconfirmed reliability, internal consistency, reasonable ho...
Regular skin self-examination is suggested as a means to detect melanomas at an early stage and t... more Regular skin self-examination is suggested as a means to detect melanomas at an early stage and thus improve prognosis. Compliance, however, has seldom been assessed in a routine clinical setting and anonymously. To assess compliance with self-examination in patients with either a previous melanoma or atypical moles (dysplastic nevi) and to examine the perceived utility of supplied photographs. An anonymous questionnaire was sent to all patients seen with either a melanoma or atypical moles between the years 1995 and 2005. The melanoma cohort consisted of 143 patients. There were 440 patients with atypical moles. Replies to the questionnaire were received from 94 of the melanoma patients and from 185 patients in the atypical mole cohort. Only 22% (12) in the melanoma group performed a total skin examination monthly. Fewer than 10% of those with atypical moles did a monthly skin examination, but about half of the patients examined their entire skin more than once a year. Self-examina...
In the multiparametric evaluation of cutaneous B-cell infiltrates (CBIs), immunoarchitectural fea... more In the multiparametric evaluation of cutaneous B-cell infiltrates (CBIs), immunoarchitectural features have been underused, and B-cell clonality alone has limited clinical usefulness. Our aim was to assess the usefulness of immunoarchitectural abnormalities (IAs) and clonality in the diagnosis of CBIs. On 39 CBIs, immunohistochemistry was performed using anti-CD45, anti-CD45RO, anti-CD3, anti-CD20, anti-CD21, and anti-CD35, and polymerase chain reaction was used to detect immunoglobulin heavy chain rearrangement. There were 33 cutaneous lymphoid hyperplasias (CLHs) and 6 cutaneous B-cell lymphomas. IAs were present in 9 lesions and clonal bands in 14 lesions. In 6 cutaneous B-cell lymphomas, IAs were associated with pure and reproducible clonal bands. IAs in 3 CLH lesions with a superficial infiltrate were not associated with clonal bands. Clonal bands in 8 CLH lesions without IAs were not reproducible from deeper sections; furthermore, in 5 of 8 cases, these were present against a ...
South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde, Jan 12, 1983
A case of multiple eruptive skin granulomas caused by Flavobacterium capsulatum is described. The... more A case of multiple eruptive skin granulomas caused by Flavobacterium capsulatum is described. The organism was resistant or poorly sensitive to all antibiotics except carbenicillin. Cure was brought about by using maximal doses of this drug. The source of the infection could not be proved, but it dated from an orthopaedic procedure to the elbow which was followed by a chronic cellulitis at the operation site. Since this is an organism known to occur in stored water, it was presumed that the flavobacterium was introduced into the wound from bottles of boiled and cooled water used in the operating theatre.
Organ transplant recipients (OTRs) are at an increased risk of developing a de novo malignant neo... more Organ transplant recipients (OTRs) are at an increased risk of developing a de novo malignant neoplasm compared to the general population. The primary contributor to skin cancer in all patients is sun exposure. In this study, we aim to ascertain both OTR skin cancer awareness and photoprotection practices. A questionnaire-based study of Saskatchewan transplant recipients. Nearly all respondents were aware that sun exposure is the best-known cause of skin cancer and that as an OTR, they are at increased risk of skin cancer (99.3% and 90.5%, respectively). Approximately half of respondents reported wearing a hat regularly, sun avoidance between 10 am and 3 pm, or wearing sunscreen regularly (53.7%, 33.1%, and 47.9%, respectively). Many OTRs are not engaging in photoprotection. Further intervention, which may include access to a dermatologist, is necessary to ensure ORTs do not experience undue morbidity and mortality secondary to skin cancer.
The field of science and medicine has experienced a flood of data and technology associated with ... more The field of science and medicine has experienced a flood of data and technology associated with the human genome project. Over 10,000 human diseases have been genetically defined, but little progress has been made with respect to the clinical application of this ...
Keratolytic winter erythema (KWE) is a rare autosomal-dominant skin disorder characterized by rec... more Keratolytic winter erythema (KWE) is a rare autosomal-dominant skin disorder characterized by recurrent episodes of palmoplantar erythema and epidermal peeling. KWE was previously mapped to 8p23.1-p22 (KWE critical region) in South African families. Using targeted resequencing of the KWE critical region in five South African families and SNP array and whole-genome sequencing in two Norwegian families, we identified two overlapping tandem duplications of 7.67 kb (South Africans) and 15.93 kb (Norwegians). The duplications segregated with the disease and were located upstream of CTSB, a gene encoding cathepsin B, a cysteine protease involved in keratinocyte homeostasis. Included in the 2.62 kb overlapping region of these duplications is an enhancer element that is active in epidermal keratinocytes. The activity of this enhancer correlated with CTSB expression in normal differentiating keratinocytes and other cell lines, but not with FDFT1 or NEIL2 expression. Gene expression (qPCR) an...
American Journal of Medical Genetics, May 29, 2000
Actinic prurigo is an idiopathic, familial photodermatosis seen especially in American Indians. S... more Actinic prurigo is an idiopathic, familial photodermatosis seen especially in American Indians. Segregation analysis was performed on 12 Saskatchewan pedigrees with American Indian ancestry, comprising a total of 1,148 individuals, ascertained via probands diagnosed with actinic prurigo. Although a high degree of familial aggregation has been noted in the past and dominant inheritance has been suggested, no formal segregation analysis has been attempted. Actinic prurigo has a variable age of onset and, therefore, age at the time of censoring must be taken into account in the analysis. However, as these ages of 57% of the unaffected individuals were missing, an algorithm was devised to impute the missing ages from known birth years in the family based on the age differences among relatives and spouses. Using these imputed ages, simple dominant inheritance with incomplete penetrance and a single age of onset distribution was found. The method for imputing the ages at examination was evaluated, as was the correction for ascertainment, by using alternative methods and comparing the results. Regardless of the method used, a dominant mode of inheritance without any multifactorial component remained the best hypothesis.
Background: Neomycin contact sensitization rates in North America range from 7% to 13%, whereas i... more Background: Neomycin contact sensitization rates in North America range from 7% to 13%, whereas in Europe they average approximately 1.9%. Objectives: Given that topical neomycin products are no longer readily available in Canada, the aim of this study was to examine what influence this may have had on neomycin sensitization rates in the 3 western provinces. Methods: On the basis of an observation originally communicated by L. M. Parsons and C. Zhang of the University of Calgary, which suggested significantly reduced rates of neomycin sensitization in Calgary, Alberta, Canada, a multicenter study of patch test results from 5690 patient charts was undertaken. Data from 3 other western Canadian Universities (the University of Saskatchewan, the University of Alberta, and the University of British Colombia) were analyzed. Data were available from 2001 to 2013 for the University of Saskatchewan (except 2006), whereas the University of Alberta and the University of British Columbia had da...
Regular skin self-examination is suggested as a means to detect melanomas at an early stage and t... more Regular skin self-examination is suggested as a means to detect melanomas at an early stage and thus improve prognosis. Compliance, however, has seldom been assessed in a routine clinical setting and anonymously. To assess compliance with self-examination in patients with either a previous melanoma or atypical moles (dysplastic nevi) and to examine the perceived utility of supplied photographs. An anonymous questionnaire was sent to all patients seen with either a melanoma or atypical moles between the years 1995 and 2005. The melanoma cohort consisted of 143 patients. There were 440 patients with atypical moles. Replies to the questionnaire were received from 94 of the melanoma patients and from 185 patients in the atypical mole cohort. Only 22% (12) in the melanoma group performed a total skin examination monthly. Fewer than 10% of those with atypical moles did a monthly skin examination, but about half of the patients examined their entire skin more than once a year. Self-examination is practiced in the majority of patients with either a previous melanoma or atypical moles. Those doing this on a regular monthly basis are a small minority.
Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, painful palmoplantar... more Pachyonychia congenita (PC) is characterized by hypertrophic nail dystrophy, painful palmoplantar keratoderma and blistering, oral leukokeratosis, pilosebaceous cysts (including steatocystoma and vellus hair cysts), palmoplantar hyperhydrosis, and follicular keratoses on the trunk and extremities. PC is diagnosed by clinical findings and by molecular genetic testing. Molecular genetic testing of the four keratin genes (KRT6A, KRT6B, KRT16, and KRT17) in which mutations are known to cause PC is clinically available. Treatment of manifestations: Pain from the palmoplantar keratoderma can be reduced by limiting friction and trauma to the feet by minimizing walking or standing, reducing hydration of the stratum corneum by using wicking socks and ventilated footwear, selecting comfortable shoes, and maintaining ideal body weight. Foot care includes paring down hyperkeratotic areas and topical therapies for hyperkeratosis (emollients and lotions containing keratolyics). Care of thickened ...
Sporotrichosis is a subcutaneous fungal infection caused by the traumatic implantation of the dim... more Sporotrichosis is a subcutaneous fungal infection caused by the traumatic implantation of the dimorphic, pathogenic fungus, Sporothrix schenkii. It constitutes the most common subcutaneous fungal infection in the general population in South Africa. Sporotrichosis in South Africa dates back to 1914, when the disease was first diagnosed in the gold mines. Occupational and recreational circumstances of infection are well established, and the environmental requirements for contracting the disease are better understood. Sporotrichosis cases were recorded from 42 suburbs in the greater Pretoria area as well as from 23 towns outside the Pretoria municipal boundary. It occurred in 154 patients with ages ranging from less than 1 year to 90 years old, with males predominating. Females in the area seemed to be at lesser risk, mainly becoming infected through gardening injuries, insect bites or other minor injuries due to outdoor activities. Exposure to possible sources of the fungus, either fr...
Pachyonychia congenita (PC) is a rare but often debilitating, dominantly inherited disorder. New ... more Pachyonychia congenita (PC) is a rare but often debilitating, dominantly inherited disorder. New treatments require more accurate instruments for evaluating changes in the quality of life in these patients. This study was undertaken to develop and validate a quality of life questionnaire for PC patients (PCQoL). Relevant factors influencing quality of life in PC patients were identified and incorporated into the well-recognized, general questionnaire, the Dermatology Life Quality Index (DLQI), to establish a disease-specific measure, the PCQoL. Classical test theory (CTT) and Rasch analysis (RA) were used to analyze and validate the PCQoL. CTT analysis established test-retest reliability and internal consistency for the PCQoL. Concurrent and construct validity for the DLQI and the PCQoL were also validated. Chi-square-based infit and outfit statistics indicated that the Rasch model fits the observed responses very well. RA reconfirmed reliability, internal consistency, reasonable ho...
Regular skin self-examination is suggested as a means to detect melanomas at an early stage and t... more Regular skin self-examination is suggested as a means to detect melanomas at an early stage and thus improve prognosis. Compliance, however, has seldom been assessed in a routine clinical setting and anonymously. To assess compliance with self-examination in patients with either a previous melanoma or atypical moles (dysplastic nevi) and to examine the perceived utility of supplied photographs. An anonymous questionnaire was sent to all patients seen with either a melanoma or atypical moles between the years 1995 and 2005. The melanoma cohort consisted of 143 patients. There were 440 patients with atypical moles. Replies to the questionnaire were received from 94 of the melanoma patients and from 185 patients in the atypical mole cohort. Only 22% (12) in the melanoma group performed a total skin examination monthly. Fewer than 10% of those with atypical moles did a monthly skin examination, but about half of the patients examined their entire skin more than once a year. Self-examina...
In the multiparametric evaluation of cutaneous B-cell infiltrates (CBIs), immunoarchitectural fea... more In the multiparametric evaluation of cutaneous B-cell infiltrates (CBIs), immunoarchitectural features have been underused, and B-cell clonality alone has limited clinical usefulness. Our aim was to assess the usefulness of immunoarchitectural abnormalities (IAs) and clonality in the diagnosis of CBIs. On 39 CBIs, immunohistochemistry was performed using anti-CD45, anti-CD45RO, anti-CD3, anti-CD20, anti-CD21, and anti-CD35, and polymerase chain reaction was used to detect immunoglobulin heavy chain rearrangement. There were 33 cutaneous lymphoid hyperplasias (CLHs) and 6 cutaneous B-cell lymphomas. IAs were present in 9 lesions and clonal bands in 14 lesions. In 6 cutaneous B-cell lymphomas, IAs were associated with pure and reproducible clonal bands. IAs in 3 CLH lesions with a superficial infiltrate were not associated with clonal bands. Clonal bands in 8 CLH lesions without IAs were not reproducible from deeper sections; furthermore, in 5 of 8 cases, these were present against a ...
South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde, Jan 12, 1983
A case of multiple eruptive skin granulomas caused by Flavobacterium capsulatum is described. The... more A case of multiple eruptive skin granulomas caused by Flavobacterium capsulatum is described. The organism was resistant or poorly sensitive to all antibiotics except carbenicillin. Cure was brought about by using maximal doses of this drug. The source of the infection could not be proved, but it dated from an orthopaedic procedure to the elbow which was followed by a chronic cellulitis at the operation site. Since this is an organism known to occur in stored water, it was presumed that the flavobacterium was introduced into the wound from bottles of boiled and cooled water used in the operating theatre.
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Papers by Peter Hull