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[Androgenetic alopecia. Current aspects of a common phenotype].

Androgenetic alopecia is the most common type of hair loss in men and women. The disorder represents a quantitative phenotype with an underlying genetic disposition. So far none of the causative genes have been identified. Under the influence of androgens there is a shortening of the anagen phase as well as a reduction of the cellular hair matrix volume in the involved scalp area. This results in the transformation of thick terminal hair follicles into thin vellus-like hair follicles. Clinically, patients present with an alopecia that follows a defined pattern (pattern baldness) and progresses continuously but in varying degrees. In advanced cases, men may develop baldness with remaining hair exclusively in the temporal and occipital regions. Women are prone to exhibit a more diffuse type of hair loss with pronounced thinning in the parietal region. Whereas the diagnosis of androgenetic alopecia is easy, its treatment is often difficult. The physician is commonly confronted with high patients' expectations regarding hair regrowth. Today, with minoxidil and finasteride, effective therapies are available which can lead to cessation of hair loss. The identification of underlying genes will make a more specific therapy easier to achieve.

5-alpha Reductase Inhibitors↗

[Postmenopausal frontal fibrosing alopecia].

We report on two patients with postmenopausal frontal fibrosing alopecia. This recently described disease affects postmenopausal women and is characterized by progressive recession of the frontotemporal and parietal hair margins. It leads to a symmetrical band-like scarring area of alopecia 3-6 cm wide. Histological examination shows perifollicular fibrosis and lymphocytic inflammation concentrated around the isthmus and infundibular areas of the follicles. One of our two patients also had vulvar lichen sclerosus et atrophicus. An effective treatment for this type of postmenopausal alopecia is yet not known, but the course of the disease seems to be self-limited.

Aged↗

[Kossard frontal fibrosing alopecia in a man].

In 1994 Steven Kossard described a new and peculiar type of hair loss that he named postmenopausal frontal fibrosing alopecia. In 6 elderly women he observed a symmetric regression of the frontal hair line. Often the eyebrows were also affected. Histology showed lichen planopilaris. There were no clinical signs of lichen planus on the rest of the body. Since the original description by Kossard, several cases of frontal fibrosing alopecia have been described--almost all of them in elderly women. We report a man with frontal fibrosing alopecia of the Kossard type.

Aged↗

Lesional alopecia areata T lymphocytes downregulate epithelial cell proliferation.

Alopecia areata is characterized by peribulbar infiltration by activated T cells. The function of these T cells in the pathogenesis is unknown. To elucidate the potential role of lesional T cells in the regulation of hair growth, T-cell clones from the margin of involved alopecia areata lesions from three patients were obtained by cloning, using the limiting dilution method. Of these T-cell clones, 31 were CD4+CD8-, 15 were CD8+CD4- and 2 were CD4-CD8-. The T-cell clones were activated and the supernatant harvested 24 h later and tested for its capacity to regulate proliferation of neonatal keratinocytes. The majority of the T-cell clone supernatants inhibited epithelial cell proliferation in a dose-dependent fashion. When the cytokine profiles of conditioned T-cell medium were compared with the growth-regulatory capacity, it was found that T-cell clones that released high amounts of interferon gamma and/or tumour necrosis factor alpha inhibited keratinocyte growth. In conclusion, T cells derived from the margin of active alopecia areata lesions are able to downregulate epithelial cell proliferation. This points to an important role of the immune system, especially the T cells, in this disease.

Alopecia Areata↗

Localization of a novel locus for alopecia with mental retardation syndrome to chromosome 3q26.33-q27.3.

Alopecia with mental retardation syndrome is a rare autosomal recessive disorder characterized clinically by total or partial alopecia and mental retardation. In an effort to understand the molecular bases of this form of alopecia syndrome, large Pakistani consanguineous kindred with multiple affected individuals has been ascertained from a remote region in Pakistan. Genome wide scan mapped the disease locus on chromosome 3q26.33-q27.3. A maximum two-point LOD score of 3.05 (theta = 0.0) was obtained at marker D3S3583. Maximum multipoint LOD score exceeding 5.0, obtained with several markers, supported the linkage. Recombination events observed in affected individuals localized the disease locus between markers D3S1232 and D3S2436, spanning 11.49-cM region on chromosome 3q26.33-q27.3. Sequence analysis of a candidate gene ETS variant gene 5 from DNA samples of two affected individuals of the family revealed no mutation.

Alopecia↗

Chronic active hepatitis associated with vitiligo, nail dystrophy, alopecia and a new variant of LKM antibodies.

In this report we describe the case of a 7-year-old boy, suffering from autoimmune-type chronic active hepatitis (AI-CAH) associated with vitiligo, nail dystrophy, alopecia areata and a variant of liver kidney microsomal (LKM) autoantibodies. This patient's antibodies are different from LKM-1 which are directed against cytochrome P450 db1. They react predominantly with perivenous hepatocytes in contrast to LKM-1 antibodies which homogeneously stain the whole liver lobule in immunofluorescence. In Western blot analysis this LKM variant reacts with a liver microsomal protein of approx. 50 kDa, but not with recombinant LKM-1 (cytochrome P450 db1) antigen. Immunosuppressive treatment led to a normalization of liver histology after 1 year and a significant improvement of vitiligo and alopecia areata. In summary, a case of autoimmune-type chronic active hepatitis is presented which is associated with a new variant of LKM antibodies reacting with a 50 kDa microsomal protein different from cytochrome P450 db1. Furthermore, this patient suffers from extrahepatic syndromes (alopecia, nail dystrophy) that have not been described previously in LKM antibody-positive chronic active hepatitis.

Alopecia↗

Essential syphilitic alopecia revisited.

There has been a resurgence of syphilis in the past decade. Uncommonly, diffuse hair loss, termed essential alopecia, is the only sign of syphilitic infection. We describe two patients with syphilis in whom the first sign of disease was alopecia and discuss the clinical and histopathologic findings of essential syphilitic alopecia.

Adult↗

Subcutaneous or topical administration of 16,16 dimethyl prostaglandin E2 protects from radiation-induced alopecia in mice.

Alopecia, a common sequel of radiation treatment of brain tumors, increases patient stress to the extent that refusal of treatment may occur. The expectation that loss of hair will be prevented, or that regrowth will occur, is extremely important to patients. To investigate prostaglandin-induced radiation protection against alopecia, the hair of B6D2F1 male mice was plucked from the right thigh and surrounding area to induce anagen. Fourteen days later, mice were injected subcutaneously in the neck with 10 micrograms 16,16 dm PGE2 in 0.2 ml of vehicle, or with the vehicle alone. In another group of previously plucked mice, 16,16 dm PGE2 in the same concentration, or the vehicle was applied topically. One hour later, graded single doses from 6.5 to 12.5 Gy 137Cs gamma irradiation were given to groups of six animals. On day 21 post-plucking, all animals were killed and a portion of the irradiated site was excised. The average hair counts per field in irradiated animals were 85 +/- 4 (6.5 Gy), 25 +/- 5 (8.5 Gy), and 5.5 +/- 0.7 (10 Gy). Animals receiving the prostaglandin systemically had values of 60 +/- 10 (6.5 Gy), 54 +/- 3 (8.5 Gy), 66 +/- 6 (10 Gy), and 30.1 +/- 8 (12.5 Gy). Topical application of the prostaglandin resulted in protection that yielded 52 +/- 3 (8.5 Gy), 34 +/- 4 (10 Gy), and 3.2 +/- 0.9 (12.5 Gy) hairs per field. Both systemic and topical application of 16,16 dm PGE2 protected from some degree of radiation-induced alopecia, which supports the conclusion that prostaglandins may be useful in the protection of hair follicles in patients treated with radiation for brain tumors.

16,16-Dimethylprostaglandin E2↗

Congenital alopecia, seizures, and psychomotor retardation in three siblings.

Three siblings, devoid of hair at birth, had an unusual autosomal recessive disorder characterized by universal congenital alopecia, microcephaly, seizures, psychomotor retardation, and severe growth failure. Metabolic and chromosome studies were normal. Skin biopsies disclosed immature hair follicles, some of which were filled with keratotic material but had no hair shafts. Neuropathologic features included cerebral cortical hypoplasia, neuronal depletion, and microcalcifications. The familial occurrence of universal congenital alopecia conjoined with nonprogressive central nervous system abnormalities in this and other kindreds defines a nosologic group of neurocutaneous disorders in which congenital alopecia is the solitary cutaneous manifestation.

Alopecia↗

Turner syndrome female with a small ring X chromosome lacking the XIST, an unexpectedly mild phenotype and an atypical association with alopecia universalis.

Rearranged X chromosome in Turner syndrome (TS) are generally well tolerated but in cases of ring X chromosomes and of X/autosome translocations the incidence of mental retardation and other congenital abnormalities can be significantly higher. These abnormal phenotypes can be ascribed to failed or partial X inactivation. Here, we report a 10-year-old female who was referred for a cytogenetic analysis because she developed an alopecia universalis. The patient, of normal intelligence, had been found to have traits of TS, especially short stature. A first cytogenetic analysis showed a no mosaic 45,X karyotype. Since, the risk of developing gonadoblastoma in TS patients with mosaicism for a Y derivative chromosome and because association of alopecia universalis and TS is uncommon, fluorescence in situ hybridization (FISH) was performed to search for a second cell population. Our patient was found to have a mosaic 45,X/46,X,+r. FISH analysis using sex chromosome probes permitted us to identify the very small marker as a ring X chromosome, detected in 90% of cells. The ring appeared to be formed almost totally of alphoid sequences with breakpoints in the juxtacentromeric region. The r(X) does not include the XIST locus and may, therefore, not be subject to X-inactivation. Unexpectedly mild phenotype in our patient and its association with alopecia universalis will be discussed.

Abnormalities, Multiple↗

Topical 5-fluorouracil is ineffective in the treatment of extensive alopecia areata.

We report the results of a pilot study of topical 5% 5-fluorouracil (FU) cream for the treatment of alopecia areata, an immunologically modulated disorder of hair growth. Patients with extensive (>50% scalp surface area involvement) alopecia areata that was refractory to previous treatments applied 5-FU to one side of their scalp twice daily for 3 to 6 months. In all, 9 patients enrolled, and 8 completed the study. No patient experienced measurable hair growth on the treated side. Only mild irritation was observed in a subset of patients with application of 5-FU to the nonphotodamaged scalp skin. Based on these results, we cannot recommend the use of topical 5-FU for treatment of alopecia areata without further evidence of therapeutic benefit.

Administration, Topical↗

Lipedematous scalp and lipedematous alopecia: a clinical and histologic analysis of 3 cases.

BACKGROUND: Lipedematous alopecia and lipedematous scalp are two similar unusual conditions mostly affecting healthy black women. OBJECTIVE: The purpose of our study was to report three cases of this condition with emphasis on clinical and histologic findings, and to review the literature on the subject. METHODS: The study includes clinical, echographic, and histologic findings of 3 patients, complemented with a literature review. RESULTS: Two cases of lipedematous alopecia and one of lipedematous scalp in 3 white women had echographic confirmation of an increased subcutaneous layer. The presence of ectatic lymphatic vessels in the two cases with hair loss was particularly emphasized. CONCLUSIONS: Our findings suggest a lessened role of racial factors, but confirms the sex implications in these related conditions, and stress the potential significance of lymphangiectatic vessels in the development of alopecia in these patients.

Adipose Tissue↗

The medical treatment of cicatricial alopecia.

The best outcome of current treatments of cicatricial alopecia is induction of a clinical remission with arrest of symptoms and signs, but the progression of hair loss may continue insidiously. Current treatments do not arrest the underlying disease process. A scalp biopsy is the first step in management. Selection of treatment described herein is guided by the histopathologic findings, including the type, location and extent of the predominant cellular inflammatory infiltrate, and clinical disease activity. Cicatricial alopecias with predominantly lymphocytic infiltrates are treated with immunomodulating agents, and those with predominantly neutrophilic infiltrates are treated with antimicrobial agents. Treatment selection may be challenging and requires flexibility, as histopathologic features frequently overlap, are not clear cut, or change over time. In the future, cellular and molecular biology studies will hopefully identify unique markers for the clinically distinct cicatricial alopecias and lead to better treatments and a cure.

Alopecia↗

Antibody deficiency and alopecia.

Three patients are described with antibody deficiency, recurrent infections, and alopecia. One patient had congenital agammaglobulinemia; the other two patients, a brother and sister, had an incomplete antibody deficiency syndrome. The loss of hair in each patient was total; the history was typical of alopecia areata. The association of alopecia and antibody deficiency has not previously been described to our knowledge in children.

Adolescent↗

Celiac disease-associated alopecia in childhood.

We report the association of celiac disease and alopecia in 3 children. In one, the alopecia developed after 4 years' nonadherence to a gluten-free diet; the other 2 patients presented with alopecia. Administration of a gluten-free diet resulted in partial regrowth of hair in the first child and complete hair growth in the others.

Adolescent↗

Early androgenetic alopecia as a marker of insulin resistance.

The previously proven association between androgenetic, alopecia and serious cardiovascular events raises a question of the common pathogenetic mechanism of these disorders. Our practice-based case-control study in men aged 19-50 years showed a strikingly increased risk of hyperinsulinaemia and insulin-resistance-associated disorders such as obesity, hypertension, and dyslipidemia in men with early onset of alopecia (<35), compared with age-matched controls. This finding supports the hypothesis that early androgenetic alopecia could be a clinical marker of insulin resistance.

Adult↗

Urinary steroids in men with male-pattern alopecia.

Enzyme hydrolysis, solid phase extraction, methoxym-silyl derivatization and capillary gas chromatographic analysis were used to examine the changes in urinary steroid metabolites in men with androgenic alopecia. A total of 23 men with androgenic alopecia and 7 age-matched control healthy men collected 24-h urine. Significantly increased values were found in the metabolites of testosterone (T): androsterone (A) (p<0.02), and etiocholanolone (E) (p<0.05) in patients with androgenic alopecia, compared to the control values. Elevated levels of 16-hydroxy-dehydroepiandrosterone (16-OHD) (p<0.03) and cortisol (F) (P<0.05) were found, but the levels of cortisol metabolites were unchanged. Calculating the ratio of total 5 alpha/5 beta metabolites provided information on the activity of 5 alpha-reductase. The ratio of total 5 alpha/5 beta metabolites was increased in the patients showing the increased 5 alpha-reductase activity. The elevated 16-OHD level could be indicative of patients who had mild hyperadrenal activity.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗

Syringolymphoid hyperplasia with alopecia: two case reports and review of the literature.

Syringolymphoid hyperplasia with alopecia is an uncommon, but histopathologically distinct, skin disorder that has been reported to occur with and possibly represent a syringotropic variant of cutaneous T-cell lymphoma. We report 2 patients with syringolymphoid hyperplasia with alopecia. Both had CD4-positive infiltrates; 1 also demonstrated loss of CD7. One patient had evidence of T-cell clonality by gene rearrangement studies, but neither had histologic evidence of cutaneous T-cell lymphoma. Because the natural progression of syringolymphoid hyperplasia with alopecia remains to be fully explained, close follow-up of patients is advised.

Adult↗