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PubMed2006

[Hair darkening close to a patch of frontal fibrosing alopecia].

BACKGROUND: Fibrosing frontal alopecia is scarring form alopecia and is most often seen in menopausal women. It is currently considered as an anatomoclinical form of lichen planopilaris of selective topography. We report a case of hair repigmentation during the course of post-menopausal frontal fibrosing alopecia. CASE REPORT: A 78-year-old woman developed alopecia of the scalp and eyebrows in 1997. Her eyebrows had been white for some ten years. In 1999, she noted repigmentation of a strip of hair at the edge of her scalp, but the rest of her hair remained white. The patient had not been taking any long-term drugs or any hair treatment. Histological examination of the regions of alopecia revealed lesions of lichenoid appearance at the junction with erosion by lymphocytes and keratinocytic necroses of the lower layers of the epidermis. Direct cutaneous immunofluorescence testing was negative. A diagnosis of post-menopausal fibrosing frontal alopecia was made on the basis of the clinical and laboratory evidence. DISCUSSION: 96 cases of post-menopausal fibrosing frontal alopecia have so far been reported in the literature, but to our knowledge, this is the first case combined with or inducing hair repigmentation. The other cases of repigmentation were eliminated by history-taking, and clinical or laboratory examinations. The mechanism of hair repigmentation in our patient could have been the result of a post-inflammatory process.

Aged↗
PubMed2004

Anxiety-related behaviour in C57BL/6 <--> BALB/c chimeric mice.

In a previous study on anxiety-related behaviours of the genetically and behaviourally distant inbred mouse strains C57BL/6 and BALB/c using the Elevated plus-maze (EPM) and Open-field (OF) apparatuses, we identified a number of variables, the factorial scores of which were grouped by principal component analysis (PCA) into factors specifically describing each inbred strain. We have now studied the effect of C57BL/6 and BALB/c haploid sets of genes on this behaviour by comparing EPM and OF variables of C57BL/6 and BALB/c versus C57BL/6 x BALB/c F1 hybrids (B6CBF1) and chimeric C57BL/6 x BALB/c (CHIM) mice. CHIM mice were made by embryo aggregation and the chimerism degree of their brain was inferred from coat black/white distribution. Discriminant analysis of EPM and OF factorial scores of C57BL/6, BALB/c and CHIM mice showed that CHIM mice with an exceeding (> or =80%) C57BL/6 or BALB/c coat component had behaviours similar to those of the predominant strain, whereas CHIM mice with intermediate chimerism differed from both inbred strains. Additional MANOVA analysis showed that the anxiety behaviour of CHIM mice with intermediate chimerism was similar to that of B6CBF1 mice as for factors not describing the inbred strains, including a motor activity mostly limited to protected areas, with attempts to approach the anxiogenic areas while processing/storing the external information. We conclude that the balanced presence of both C57BL/6 and BALB/c genetic backgrounds, either when carried by the same cell or by different cells, gives rise to a novel stress coping strategy described by factors different from those of the inbred strains.

Animals↗
PubMed1998

Dermatological indicators of coronary risk: a case-control study.

OBJECTIVE: We examined the association of dermatological signs such as baldness, thoracic hairiness, hair greying and diagonal earlobe crease with the risk of myocardial infarction in men under the age of 60 years. METHODS: A hospital-based, case-control study included 842 men admitted for the first non-fatal myocardial infarction, the controls were 712 men admitted with noncardiac diagnoses, without clinical signs of coronary disease. The relative risks were estimated as odds ratios. Logistic regression was used to control for the confounding variables. RESULTS: Baldness, thoracic hairiness and earlobe crease were approximately 40% more prevalent in cases (P<10(-6) in each case). In both cases and controls, baldness and thoracic hairiness were frequently coexistent, as well as hair greying and earlobe crease (P<10(-4) in each case). After allowing for age and other established coronary risk factors, the relative risk of myocardial infarction for fronto-parietal baldness compared with no hair loss was 1.77 (95% CI 1.27-2.45) and it was 1.83 (95 CI 1.4-2.3) for men with thick, extended thoracic hairiness. The presence of a diagonal earlobe crease yielded a relative risk of 1.37 (95% CI 1.25-1.5), while hair greying was associated with myocardial infarction only in men under the age of 50 years. CONCLUSION: It appears that baldness, thoracic hairiness and diagonal earlobe crease indicate an additional risk of myocardial infarction in men under the age of 60 years, independently of age and other established coronary risk factors.

Adult↗
PubMed1995

Insight into the microphthalmia gene.

The murine microphthalmia gene (mi) is one of the last multi-allelic, classic coat-colour genes to be cloned in the mouse and, similar to many of these genes, encodes an exciting molecule that is is involved in multiple developmental processes. The existence of the numerous alleles has allowed the molecular dissection of the function of the MI bHLH-Zip transcription factor in vivo and offers a unique opportunity to understand the function of a multimeric transcription factor throughout development and in many tissues. It is also the gene mutated in some patients with the human deafness syndrome, Waardenburg's syndrome type II, and hence helps to understand this syndrome.

Alleles↗
PubMed1999

The mahogany mouse mutation: further links between pigmentation, obesity and the immune system.

Completely different lines of experimentation have identified attractin, a protein that seems to have multiple roles in regulating physiological processes. It affects the balance between agonist and antagonist at receptors on melanocytes, modifies behaviour and basal metabolic rate, and mediates an interaction between activated T cells and macrophages. It may well be a target for development of drugs to treat obesity.

Agouti Signaling Protein↗
PubMed2000

Host-related and environmental risk factors for cutaneous basal cell carcinoma: evidence from an Italian case-control study.

BACKGROUND: Despite its frequency, there is a paucity of data on risk factors for basal cell carcinoma. OBJECTIVE: We assessed potential risk factors for basal cell carcinoma in a population from southern Europe. METHODS: This multicenter case-control study involved 528 newly diagnosed cases and 512 controls. RESULTS: In the multivariate analysis, red hair, lighter colored eyes, high nevus counts on the upper limbs, and the presence of solar lentigines and actinic keratoses were all associated with basal cell carcinoma. The risk of the tumor increased in subjects who reported burning easily and experiencing sunburn episodes before 15 years of age. An association was documented with indices of recreational sun exposure but no clear evidence of exposure-effect relationship was found. No relation was found with occupational sun exposure. Finally, basal cell carcinoma appeared to be significantly associated with a family history of skin tumors, a personal history of tumors other than those on skin, and radiotherapy. CONCLUSION: Genetic and environmental factors appear to be involved in the onset of basal cell carcinoma.

Adult↗
PubMed1984

Common and uncommon cutaneous findings in patients with ataxia-telangiectasia.

This series describes some common and uncommon cutaneous findings in twelve patients with ataxia-telangiectasia (A-T). All patients had the characteristic telangiectasia as described previously. However, the telangiectases did not conform to a classic photodistribution . Our series did include one patient with a pathologic reaction to light-simulating hydroa aestivale or vacciniforme . In addition, there were three patients with acanthosis nigricans in two unrelated families. Vitiligo, impetigo, recurrent herpetic gingivostomatitis, hirsutism, lipoatrophy, gray hair, progeroid changes and hyper- and hypopigmented macules were noted in our patients. Many of our patients had one or more caf'e au lait like-lesions in a dermatomal distribution. In addition, several of the family members showed one or more café au lait-like lesions, suggesting the possibility that this finding may represent a phenotypic expression in the skin of carriers of this cancer-prone syndrome.

Acanthosis Nigricans↗
PubMed1984

Vitiligo and melanoma: can genetically abnormal melanocytes result in both vitiligo and melanoma within a single family?

We found twelve families with melanoma who had close family members with halo nevi, early graying of hair, a halo primary melanoma, or ordinary vitiligo. On the basis of these findings and the observation of others in fish, horses, and pigs with melanomas, we suggest that the melanocytes of people with vitiligo or with a genetic background for vitiligo are predisposed to undergo a malignant transformation. The presence of vitiligo appears as a manifestation of host suppression of malignant melanocytes.

Adolescent↗
PubMed1987

Childhood vitiligo.

Eighty-two children (ages 6 months to 12 years) with clinical and/or histopathologic diagnoses of vitiligo were evaluated; 35 were male and 47, female. Fifty-six were black, 25 white, and 3 classified as "other." Children were compared with control groups of children with other skin diseases and with adults with vitiligo. Children had an increased incidence of segmental vitiligo (p less than 0.01). Children had an increased incidence of autoimmune and/or endocrine disease and also of premature graying in their immediate and extended family members (p less than 0.001). Six of 33 children with vitiligo tested had positive organ-specific serum autoantibodies, which was a higher incidence than in the control group of children (p less than 0.05). Eighteen percent of children treated with topical psoralens and long-wave ultraviolet light (PUVA) therapy had an acceptable response, which was less than an adult group similarly treated. We have found childhood vitiligo to be a distinct subset of vitiligo, showing increased segmental presentation; strong autoimmune and/or endocrine disease background and high incidence of premature graying in the families of affected children; the presence of organ-specific serum autoantibodies and a poor response to topical PUVA therapy.

Adult↗
PubMed1987

Number of melanocytic nevi as a major risk factor for malignant melanoma.

A study of 121 melanoma patients and 139 control subjects from the University of California, San Francisco clinics was conducted among whites to examine the relationship between number of melanocytic nevi and cutaneous melanoma. Nevi that measured 2 mm or more in diameter were counted over the body by a dermatologist and a dermatology fellow. The average number of nondysplastic melanocytic nevi that were 2 mm or greater in diameter was 97 for melanoma patients and 36 for control subjects (p less than 0.001). Relative risks were 1.6 (p = 0.43) for 11 to 25 nevi, 4.4 (p = 0.01) for 26 to 50 nevi, 5.4 (p = 0.008) for 51 to 100 nevi, and 9.8 (p = 0.001) for more than 100 nondysplastic melanocytic nevi. Relative risks were 3.8 (p = 0.001) for 1 to 5 dysplastic nevi and 6.3 (p = 0.003) for 6 or more of these lesions. Report of blistering sunburns or of a previous skin cancer and having red or blond hair at the age of 20 were also independently associated with an increased risk of cutaneous melanoma. If confirmed in larger studies, the results presented on number of nevi and melanoma risk suggest a readily identifiable melanoma-prone group that could be followed to detect early malignant melanoma.

Dysplastic Nevus Syndrome↗
PubMed1998

Partial albinism with immunodeficiency: Griscelli syndrome: report of a case and review of the literature.

Partial albinism with immunodeficiency (Griscelli syndrome) is an uncommon disorder characterized by pigmentary dilution and variable immunodeficiency. Features include a silvery-gray sheen to the hair, large clumped melanosomes in hair shafts, and prominent mature melanosomes in cutaneous melanocytes with sparse pigmentation of adjacent keratinocytes. Immunologic abnormalities most often include impaired natural killer cell activity, absent delayed-type hypersensitivity, and impaired responses to mitogens. Impaired helper T cell function and hypogammaglobulinemia have also been described. The syndrome can be differentiated from Chediak-Higashi syndrome by pathognomonic light and electron microscopic features in skin and hair, and absence of consistent granulocyte abnormalities, but similarly carries a poor prognosis without bone marrow transplantation. We describe a patient with Griscelli syndrome who presented with hepatosplenomegaly, hepatitis, pancytopenia, and silvery hair in the newborn period.

Albinism↗