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PubMed2005

[Alopecia totalis, hypotension and erectile dysfunction in a 34 year old patient. Difficult clarification of a common cause].

The occurrence of both autoimmune endocrinopathies and endocrinopathies caused by other reasons is called polyglandular autoimmune syndrome (PAS-syndrome). In a 34 years old man with weakness, weight loss and erectile dysfunction we found low cortisol caused by an autoimmune adrenalitis and low testosterone caused by a hypophysitis with impaired gonadotropin secretion. Thyroid autoantibodies and islet cell autoantibodies without any hormone deficiencies were further signs of a broad endocrine autoimmune syndrome. In the following 11 years the patient developed three autoimmune disorders: paradrenal glandular insufficiency, hypogonadism caused by hypophysitis, Diabetes mellitus type 1. In the same time several non endocrine autoimmune diseases became manifest: alopecia totalis, vitiligo, retrobulbar neuritis and keratoconjunctivitis.

Adult↗
PubMed2002

Strong expression of CD40, CD54 and HLA-DR antigen and lack of evidence for direct cellular cytotoxicity are unique immunohistopathological features in alopecia areata.

The pathological role played by T cells infiltrating hair follicles in lesions of alopecia areata (AA) is unknown. We examined the expression in cryostat sections of scalp skin obtained from a total of 28 patients with AA and from five normal control subjects of (1) molecules related to the induction of cell death including Fas, Fas ligand (FasL), perforin, granzyme B (GB), and TIA-1, (2) molecules related to antigen presentation including CD1a, CD40, CD54, CD80, and CD86, and (3) molecules induced by interferon gamma (IFN-gamma) including CD40, CD54, Fas, and HLA-DR. CD3(+) T cells infiltrated perifollicularly, perivascularly and in the hair structure and there was a predominance of CD4(+) over CD8(+) cells. Antigen-presenting cells expressing CD1a, CD40, CD54, or HLA-DR were also seen. Expression of CD40, CD54, HLA-DR and CD95 was also seen in the hair structure including the dermal papilla. Consistent with these observations, IFN-gamma-producing cells were also detected in the perifollicular infiltrate. In contrast, few Fas-L(+), perforin(+), GB(+) or TIA-1(+) cells were found adjacent to the follicles. Apoptotic cells were recognized only in the outer root sheath of catagen hairs. These findings suggest that infiltrating T cells interact with perifollicular or follicular antigen-presenting cells to produce IFN-gamma, which deprives dermal papilla cells of their ability to maintain anagen hair growth.

Adolescent↗
PubMed2004

Patients with a large prostate show a higher prevalence of androgenetic alopecia.

Androgenetic alopecia (AGA) and benign prostatic hyperplasia (BPH) are both androgen-dependent disorders, displaying in situ high levels of dihydrotestosterone with a good therapeutic response to finasteride. Embryological development of both the hair follicle and the prostate depends on mesenchymal-epithelial interaction, which is influenced by the expression of type 2 5alpha-reductase. The aim of this study was to elucidate the association between the size of the prostate gland and the prevalence and severity of AGA. A total of 46 patients age between 56 and 87 years were retrospectively recruited. They fulfilling the clinical diagnosis of BPH defined as (1) prostate volume > 30 cm3, measured by transrectal ultrasound, (2) maximal urine flow rate < 15 ml/s and mean urine flow rate < 10 ml/s, and (3) prostate serum albumin < 10 ng/ml. The control group comprised 34 patients aged between 49 and 81 years with urogenital infection, cystitis or urolithiasis. The expression and severity of AGA were evaluated by dermatologists using modified Norwood/Hamilton classification. Patients with a prostate volume > 30 cm3 had a higher prevalence of AGA than patients with a smaller prostate (< 30 cm3) (83.3% vs 61.3%; P < 0.05). The prostate size, however, did not correlate with the severity of AGA in either group or in the whole patient group. The prevalence of AGA was not significantly different in patients with or without BPH (85.7% vs 70.6%). The prostate was slightly larger among patients with AGA than among those without AGA (mean+/-SD 42.7+/-17.4 vs 35.4+/-14.9 cm3), but this was not statistically significant. There was no significant correlation between the age of onset of AGA and the development of BPH. Our results suggest that a larger prostate is associated with a higher prevalence of AGA. It remains to be seen if long-term use of finasteride in AGA patients will prophylactically lower the incidence of BPH.

Aged↗
PubMed2005

Expression of beta-catenin, p63 and CD34 in hair follicles during the course of androgenetic alopecia.

During the hair growth cycle, the hair follicle appears to recapitulate part of its embryogenesis where both beta-catenin and p63 participate. The aim of the present study was to investigate the hypothesis that beta-catenin and p63 protein may be involved in the pathogenesis of androgenetic alopecia. Second, expression of CD34 protein was used to assess the capillary density of the affected skin. Cadavers were used as samples and the results showed that analysis of beta-catenin, p63 and CD34 expressions in human cadaverous scalp skin by immunohistochemical techniques were possible. We detected a higher expression of p63 in occipital skin in comparison to the affected frontal areas. However, we found only minimal changes in beta-catenin expression comparing frontal and occipital areas. A completely new finding was the expression of CD34 positive cells in the outer root sheath of hair follicles.

Adult↗
PubMed2007

Serum anti-Fcgamma receptor autoantibodies in patients with alopecia areata.

Although anti-Fcgamma receptor antibodies (Abs) are detected in various autoimmune diseases, there have been no studies about the anti-Fcgamma receptor Abs in alopecia areata (AA). To detect the anti-Fcgamma receptor Abs in patients with AA and their clinical correlations, Serum samples from 72 patients with AA and 23 normal controls were examined by enzyme-linked immunosorbent assay assessing anti-Fcgamma receptor Ab levels. Anti-Fcgamma receptor I Abs were significantly frequently detected in patients with AA compared with normal controls. Furthermore, the detection of anti-Fcgamma receptor I Abs significantly inversely correlated with the disease duration. These results suggest that anti-Fcgamma receptor I Ab and Fcgamma receptor I play an important role in the regulation of AA, are useful for a marker of the disease prognosis and are worth intense research for the reasonable and specific therapy of AA.

Adolescent↗
PubMed2000

Conjunctival edema and alopecia of the external third of the eyebrows in a patient with Meige syndrome.

PURPOSE: To describe a patient with Meige syndrome in whom we observed the coexistence of hereditary lymphedema of the lower legs, conjunctival edema and alopecia of the lateral third of the eyebrows. METHODS: Case report. RESULTS: Histological examination of the conjunctival and skin specimens showed dermal edema and a slight reduction in the number of severely ectatic lymphatics in the reticular dermis. The vessel were identified as lymphatics on the basis of immunohistochemical evidence of discontinuity and/or absence of basement membrane. CONCLUSIONS: Clinical and histological findings suggest that the etiopathogenesis of the edema in Meige syndrome is related to a structural ectatic defect of lymphatics. This anomaly seems to involve both skin and other sites, such as conjunctival mucosa.

Adult↗
PubMed2002

Cerebellar hypoplasia, hypergonadotrophic hypogonadism, retinitis pigmentosa, alopecia, microcephaly, psychomotor retardation, and short stature: "D-CHRAMPS syndrome".

We here report a rather novel syndrome of dysmorphic features, short stature, microcephaly, alopecia, psychomotor retardation, retinitis pigmentosa and secondary amenorrhoea. This may present a new complex multisystem disorder distinct from those reported in the literature and we propose the acronym D-CHRAMPS for this novel disorder.

Adolescent↗
PubMed2002

Alopecia universalis congenita, XY gonadal dysgenesis and laryngomalacia: a novel malformation syndrome.

We report on five individuals with the following consistent findings: alopecia universalis congenita, XY gonadal dysgenesis and laryngomalacia persisting beyond infancy. The clinical presentation of the XY gonadal dysgenesis was ambiguous genitalia, appearing as male or, more commonly, female. In one affected individual müllerian structures were present. The affected individuals come from two unrelated families. While in the first family the two affected individuals come from two related sibships, three affected individuals come from one sibship in the second family. Parents of affected individuals in the three sibships are first cousins. To our knowledge, this association has not been reported before. We speculate that the mode of inheritance of this disorder is autosomal recessive with probable sex limitation.

Alopecia↗
PubMed2000

Structure and polymorphism of the human gene for the interferon-induced p78 protein (MX1): evidence of association with alopecia areata in the Down syndrome region.

Alopecia areata (AA) is a chronic inflammatory disease characterised by patchy hair loss with T cell infiltration of hair follicles. AA occurs in approximately 0.1% of the general population, but this is increased to 9% in Down syndrome (DS). DS is associated with an additional copy (full or partial) of chromosome 21, and the DS region may potentially include genes involved in the pathogenesis of AA. MX1 is the gene encoding the interferon-induced p78 protein (MxA). MxA protein confers resistance to influenza viruses, and we have previously shown that MxA protein is strongly expressed in lesional anagen hair bulbs from patients with AA but not in normal follicles. We therefore studied the possible involvement of MX1 in the pathogenesis of AA. To establish markers in the MX1 region which could be screened by PCR-based methods, we defined the human MX1 exon/intron organisation and screened the exons and the introns by conformation-sensitive gel electrophoresis. We found that the MX1 gene contains 17 exons extending over 33 kb. The size and sequence of the region from exon 6 to exon 16 are highly conserved between human and mouse. Screening of 4747 bp within the MX1 gene revealed four single nucleotide polymorphisms in intron 6. These polymorphisms are concentrated within 147 bp and show strong linkage disequilibrium. In a case-control association study for the MX1 (+9959) polymorphism in 165 AA patients and 510 controls we found a significant association of this marker with AA (odds ratio 1.79, 95% CI 1.21-2.66, chi2 = 8.464, P = 0.0036). The risk of disease was greater for patchy AA (mild disease) and with early age at onset (odds ratio 2.34, 95% CI 1.24-4.43, P = 0.0072), providing new evidence of genetic heterogeneity in AA. Our demonstration of genetic association between the MX1 gene and disease supports the hypothesis that this is a new candidate gene in AA.

Alopecia Areata↗
PubMed2003

Scalp hypothermia to prevent chemotherapy-induced alopecia is effective and safe: a pilot study of a new digitized scalp-cooling system used in 74 patients.

GOALS: The aim of this study was to examine the efficacy and safety of a new digitized, controlled, scalp-cooling system to prevent chemotherapy-induced alopecia. METHOD: Seventy-four female cancer patients who received 13 varying chemotherapy regimens were included in a nonrandomized pilot study. The Digni 2-3 with Dignicap system consists of a refrigerator unit and a control unit integrated into a mobile cabinet and connected to a tight-fitting cooling cap. This system maintains a constant scalp temperature of +5 degrees C for many hours. In this study, 60 patients were treated for ovarian cancer with either taxane or epirubicin combination chemotherapy. Eight patients with Hodgkin's lymphoma, three with breast cancer, two with endometrial cancer, and one with sarcoma were also included. Photo documentation and patient assessment of hair loss and discomfort were performed. RESULTS: In anthracycline-treated patients, total prevention of hair loss was observed, whereas hair loss in paclitaxel/docetaxel-treated patients was minimal to none. The combination of anthracycline and taxane resulted in more hair loss, but only three of six patients used a wig. Scalp cooling was generally very well tolerated; only two of 74 patients discontinued use of the cold cap due to discomfort. No scalp metastases occurred over a median follow-up period of 15 months. CONCLUSIONS: The digitized, controlled, scalp-cooling system represents an effective and safe device that should be clinically evaluated in a randomized trial and in studies using other chemotherapy regimens to determine optimal temperatures and durations of cooling for maximal efficacy.

Adult↗
PubMed2006

Protection against chemotherapy-induced alopecia.

PURPOSE: The goal is to provide an overview on the advances in protection against chemotherapy-induced alopecia (CIA). MATERIALS AND METHODS: The four major parts of this review are (a) overview of the hair follicle biology, (b) characteristics of CIA, (c) state-of-the-art animal models of CIA, and (d) experimental approaches on protection against CIA. RESULTS: The hair follicle represents an unintended target of cancer chemotherapy. CIA is a significant side effect that compromises the quality of life of patients. Overcoming CIA represents an area of unmet needs, especially for females and children. Significant progresses have been made in the last decade on the pathobiology of CIA. The pharmacological agents under evaluation include drug-specific antibodies, hair growth cycle modifiers, cytokines and growth factors, antioxidants, cell cycle or proliferation modifiers, and inhibitors of apoptosis. Their potential applications and limitations are discussed. CONCLUSION: Multiple classes of agents with different action mechanisms have been evaluated in animal CIA models. Most of these protective agents have activity limited to a single chemotherapeutic agent. In comparison, calcitriol and cyclosporine A have broader spectrum of activity and can prevent against CIA by multiple chemotherapeutic agents. Among the three agents that have been evaluated in humans, AS101 and Minoxidil were able to reduce the severity or shorten the duration of CIA but could not prevent CIA.

Alopecia↗
PubMed1975

Alopecia, ascites, and incomplete regeneration after 85 to 90 per cent liver resection.

A nineteen year old female underwent 85 to 90 per cent partial hepatectomy to treat a minimal deviation hepatoma. Observations afterwards suggested that the limit of resection compatible with survival had been reached. She recovered perfect health after many months, although liver regeneration was not complete. Severe but eventually reversible alopecia and ascites developed postoperatively, undoubtedly as a complication of the massive hepatic resection.

Adult↗
PubMed1986

A rare case of numerous oral hairs in a 13-year-old boy with alopecia areata.

An extremely rare case of numerous oral hairs in a 13-year-old boy with alopecia areata is presented. The hairs growing from gingival pockets were black in color, similar to the hair on the patient's scalp. Microscopic examination of hairs from the oral cavity and those from the scalp showed differences in their structure.

Adolescent↗
PubMed1991

Epidermal dexamethasone receptors in dogs with confirmed hyperadrenocorticalism, hypothyroidism or undiagnosed hormonal alopecia.

Low capacity, high affinity [3H] dexamethasone binding receptors were identified in cytosolic preparations of the skin (mean number 42.0 +/- 25.2 fmol mg-1 protein, apparent dissociation constant (1 nM +/- 0.23) of clinically normal dogs. No [3H] dexamethasone binding was observed in the skin of nine out of 10 dogs with confirmed spontaneous hyperadrenocorticism or in the skin of three out of six dogs with undiagnosed hormonal alopecia. A reduction was detected in the number of [3H] dexamethasone binding receptors in the skin of one dog with confirmed hypothyroidism. This study provides evidence for the susceptibility of canine glucocorticoid receptors to down regulation by imbalances of endogenous hormones, particularly increased glucocorticoid concentrations.

Adrenocortical Hyperfunction↗
PubMed1988

Alopecia areata during lithium therapy. A case report.

A case of total Alopecia areata is discussed in a patient who has received lithium for 2 months. Recent literature is reviewed and several critical observations are made on the possibility of attributing this pathology to the use of lithium.

Alopecia Areata↗
PubMed1990

Topical cyclosporine in male pattern alopecia.

We previously demonstrated a systemic and topical effect of cyclosporine on hair growth in an experimental model composed of human scalp skin transplanted onto nude mice. The aim of this study was to determine whether topical cyclosporine affects male pattern alopecia. For 4 months in a double-blind study, 10 subjects were treated with cyclosporine and three were treated with olive oil. Hair growth was evaluated by photographs and hair counts. Significant hair growth was observed in two of the eight patients who completed the study. In one the hair growth was cosmetically satisfactory. No systemic or cutaneous side effects were noted.

Administration, Topical↗
PubMed1990

Role of topical immunotherapy in the treatment of alopecia areata. Quality analysis of articles published between January 1977 and January 1988 about three treatments. Reading Group.

We conducted a survey of clinical trials to assess the scientific evidences presented for the practical use of dinitrochlorobenzene, squaric acid dibutylester, and diphencyprone in the treatment of alopecia areata. Twenty-six papers published between January 1977 and January 1988, in English, French, and Italian were selected. We used a standardized protocol of evaluation, which focused principally on the reporting of methods. Twelve papers were uncontrolled studies. Of the controlled studies, 11 had a self-controlled design, two studies involved the use of parallel concurrent controls, and seven were randomized trials. With regard to criteria for entry, follow-up schedules, and criteria for evaluation of response to treatment, the studies were scored generally poorly; therapeutic regimen, patients' characteristics, withdrawals, and description of side effects in these studies were rated more highly. In light of our results, further and better-designed studies are needed for acceptance of dinitrochlorobenzene, squaric acid dibutylester, and diphencyprone in current therapy.

Administration, Cutaneous↗
PubMed1991

Treatment of alopecia areata with diphenylcyclopropenone.

One hundred thirty-nine patients with severe alopecia areata (the majority with the subtotal, total, or universalis type) were treated with topical immunotherapy (diphenylcyclopropenone). Patients were initially treated unilaterally; the other side of the scalp served as a control. In 50.4% of the patients the response was either excellent (total regrowth) or satisfactory (subtotal regrowth with only a few remaining bald patches). The most frequent side effects were eczematous reactions with blistering, spreading of the induced contact eczema, and sleep disturbances.

Administration, Topical↗