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Alopecia and periorificial dermatitis in biotin-responsive multiple carboxylase deficiency.

Three siblings with infantile-onset biotin-responsive multiple carboxylase deficiency are described. Recognition of the characteristic dermatologic manifestations, alopecia and periorificial dermatitis, should result in early diagnosis and institution of potentially lifesaving therapy with biotin. Other metabolic disorders may present a similar clinical picture. Immunologic dysfunction and/or aberration in lipid or branched chain amino acid metabolism may be the common pathophysiologic link in some or all of these disorders.

Alopecia↗

Bullous pemphigoid and multiple autoimmune diseases. Alopecia universalis, bullous pemphigoid, hypothyroidism, rheumatoid arthritis, and neutropenia in one patient.

A 57-year-old white man developed Hashimoto's thyroiditis, bullous pemphigoid, and autoimmune neutropenia during the last year of his life. In addition, he had alopecia universalis since the age of 21 and rheumatoid arthritis since the age of 38. This unique constellation of autoimmune diseases has not been reported previously. Its significance is discussed in relation to the polyglandular failure syndrome and thymoma-related autoimmune disease.

Alopecia↗

T cell subpopulations in alopecia areata.

Alopecia areata remains a disease of unknown cause and unsatisfactory treatment. Histologically it is characterized by a lymphocytic infiltrate that surrounds the lower half of the hair follicle. Our findings show that 64% to 92% of this infiltrate is composed of T lymphocytes bearing the Leu 3a (helper-inducer) phenotype. The remaining lymphocytes demonstrate the Leu 2a (cytotoxic-suppressor) phenotype. This helper T cell predominance is present in all patients, irrespective of clinical activity or duration of disease. The Leu 3a/Leu 2a ratio ranged from 2.6 to 19.8, the higher ratios being seen in patients with clinically active disease. The cutaneous infiltrate also is Ia-positive, indicating the activated nature of these lymphocytes.

Adult↗

Potential mechanisms of minoxidil-induced hair growth in alopecia areata.

In vivo, topical minoxidil therapy is associated with changes in the follicular epithelium, tissue and blood lymphocyte populations, lymphocyte blastogenic response to mitogens, and perifollicular vasculature. Biopsy specimens taken from areas of terminal hair regrowth show a dose-dependent increase in hair follicle length, a decrease in tissue lymphocyte populations associated with a simultaneous increase in peripheral blood lymphocyte counts, and reopening of previously closed lumina of perifollicular vessels. Responder lymphocytes show pretreatment-increased concanavalin A and phytohemagglutinin-induced blastogenesis, which decrease toward control values after treatment. In vitro, at concentrations approximating the range of tissue levels in patients treated topically with the 5% solution, minoxidil affects both epithelial cells and lymphocytes in tissue culture. Cultured murine epithelial cells show increased cell proliferation and delayed senescence. Cultured human lymphocytes show suppression of mitogen-induced blast transformation. Differential effects on responder, nonresponder, and control lymphocytes are seen. Minoxidil may induce hair regrowth in alopecia areata by a synergistic stimulatory effect on follicular epithelium and a suppressive effect on lymphocyte-mediated immunologic phenomena. A contributing role for its vasodilatory properties must also be considered.

Alopecia Areata↗

Safety and efficacy of topical minoxidil in the management of androgenetic alopecia.

Of 149 subjects with androgenetic alopecia, 102 completed 1 year of a double-blind, randomized study comparing 2% minoxidil and 3% minoxidil solutions for safety and efficacy. One third of the subjects used a vehicle placebo for the first 4 months and then switched to 3% minoxidil. At 12 months the 2% minoxidil group switched to a 3% solution. During months 5 to 12 a steady increase in terminal hair counts occurred to an equal degree within the 2% and 3% minoxidil groups and the 3% treatment group switched from placebo. Total hair counts at 12 months increased from a baseline mean of 63.5 to 180.6 in the 2% treatment group, from 61.0 to 179.9 in the 3% group, and from 65.0 to 191.1 in the placebo to 3% crossover group. Although all 102 subjects completing 12 months of the study thought that visible hair growth had resulted, 89 were considered by the investigators to have visible growth. Dense hair growth, defined as hair long enough to cut or comb, was present in 48 subjects by their own evaluation and in 33 subjects by investigator evaluation. There were no serious side effects. Two instances of allergic contact dermatitis and four of pruritus were attributed to use of the drug. Two individuals complained of impotence, which disappeared within a few days of discontinuation of topical minoxidil. This effect has not been reported during the use of minoxidil in its oral form (Loniten) for the treatment of hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Topical↗

Androgenetic alopecia: treatment results with topical minoxidil.

A double-blind 12-month trial was conducted to evaluate the safety and efficacy of topical minoxidil in patients with androgenetic alopecia. During the first 4 months of the study, patients applied a topical solution containing either 2% minoxidil, 3% minoxidil, or placebo to their scalps twice daily. At the end of the fourth month, patients taking placebo were crossed over to treatment with 3% minoxidil solution. Of 60 patients enrolled in the study, 43 were evaluable at month 12. Hairs were counted in a 1-inch target area and classified as vellus, indeterminate, and terminal; the latter two classifications were combined as nonvellus hairs for further statistical analysis. All three groups had significant increases in total, nonvellus, and terminal hair counts between baseline and month 4 and between month 4 and month 12. At month 4 the average total hair counts increased from a baseline mean of 158.2 to 270.2 in the 2% minoxidil group, from 156.6 to 287.0 in the 3% minoxidil group, and from 162.6 to 246.9 in the placebo group. At month 12 the means were 415.6, 448.5, and 471.1 in the 2% minoxidil, 3% minoxidil, and placebo-3% minoxidil crossover subjects, respectively. The increases from month 4 to month 12 were highly significant for each group (p = 0.0001). Average increases in nonvellus hair counts between months 4 and 12 were 216, 181, and 264 in the 2% minoxidil, 3% minoxidil, and placebo-to-3% minoxidil crossover groups, respectively, all highly significant differences from zero (p = 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Topical↗

Congenital triangular alopecia. Report of fourteen cases.

Fourteen patients affected by congenital triangular alopecia are presented. The clinical and histologic features of this condition are discussed. I suggest that the condition is considerably more common than hitherto has been thought.

Adolescent↗

Follicular mucinosis associated with scarring alopecia, oligoclonal T-cell receptor V beta expansion, and Staphylococcus aureus: when does follicular mucinosis become mycosis fungoides?

A diagnosis of alopecia mucinosa, occurring as a single scalp lesion, was made in a 40-year-old white woman who had a history of trauma. Follicular mucinosis, Staphylococcus aureus, and oligoclonal expansion of the T-cell receptor V beta chain genes 6 and 7 were present in the skin. Epidermotropic T-cell skin diseases with oligoclonal T-cell proliferations may be the result of HLA- and cytokine-determined reaction patterns to persistent antigens.

Adult↗

Finasteride in the treatment of men with androgenetic alopecia. Finasteride Male Pattern Hair Loss Study Group.

BACKGROUND: Androgenetic alopecia (male pattern hair loss) is caused by androgen-dependent miniaturization of scalp hair follicles, with scalp dihydrotestosterone (DHT) implicated as a contributing cause. Finasteride, an inhibitor of type II 5alpha-reductase, decreases serum and scalp DHT by inhibiting conversion of testosterone to DHT. OBJECTIVE: Our purpose was to determine whether finasteride treatment leads to clinical improvement in men with male pattern hair loss. METHODS: In two 1-year trials, 1553 men (18 to 41 years of age) with male pattern hair loss received oral finasteride 1 mg/d or placebo, and 1215 men continued in blinded extension studies for a second year. Efficacy was evaluated by scalp hair counts, patient and investigator assessments, and review of photographs by an expert panel. RESULTS: Finasteride treatment improved scalp hair by all evaluation techniques at 1 and 2 years (P < .001 vs placebo, all comparisons). Clinically significant increases in hair count (baseline = 876 hairs), measured in a 1-inch diameter circular area (5.1 cm2) of balding vertex scalp, were observed with finasteride treatment (107 and 138 hairs vs placebo at 1 and 2 years, respectively; P < .001). Treatment with placebo resulted in progressive hair loss. Patients' self-assessment demonstrated that finasteride treatment slowed hair loss, increased hair growth, and improved appearance of hair. These improvements were corroborated by investigator assessments and assessments of photographs. Adverse effects were minimal. CONCLUSION: In men with male pattern hair loss, finasteride 1 mg/d slowed the progression of hair loss and increased hair growth in clinical trials over 2 years.

Adult↗

Pulse methylprednisolone therapy for severe alopecia areata: an open prospective study of 45 patients.

BACKGROUND: Oral corticosteroids may be effective in the treatment of severe alopecia areata (AA), but the side effects of prolonged therapy limit their use. The benefit of a single intravenous pulse of methylprednisolone has not been evaluated in patients with ongoing hair loss of less than 12 months' duration. OBJECTIVE: Our purpose was to determine the effectiveness of an intravenous pulse of methylprednisolone at 1, 3, 6, and 12 months in patients with active severe AA of less than 12 months' duration. METHODS: Forty-five patients were included in this open study. All had rapid and extensive hair loss for less than 1 year (first occurrence or relapse), with the bald area exceeding 30% of the scalp. There were 20 multifocal, 10 ophiasic, 9 universalis, and 6 totalis cases. Intravenous methylprednisolone, 250 mg, was administered twice a day on 3 successive days. Follow-up for at least 12 months (up to 29 months) was performed. The percentage of pretreatment bald area covered by hair regrowth at 1,3,6, and 12 months was measured. RESULTS: No major side effects were observed. Patients with multifocal AA (n = 20) showed the best response rate, with 9, 12, 13, and 12 showing 100% or 50% to 100% regrowth at 1, 3, 6, and 12 months, respectively. Relapse occurred at 3 months in 1 patient, at 6 months in 2, and at 12 months in 4. A second pulse was tried in 2 patients with relapse with 100% regrowth that was stable at 12 and 28 months. In patients with ophiasic AA (n = 10), no total regrowth was observed; 6 had no response, 4 showed 20% to 70% regrowth at 1 month with relapse at 3 and 6 months. A second series of pulses was given to the 4 initial responders 3 to 13 months after the first series; the response rate to this second treatment was better than the first. In patients with universalis and totalis AA (n = 15), no total regrowth was observed initially; 8 patients had no response, and 3 showed 50% to 90% regrowth at I month, with subsequent improvement at 3 and 6 months. In 4 patients who did not show an initial response, a significant (90% to 100%) delayed regrowth was observed between 9 and 16 months after the pulse therapy. CONCLUSION: A single series of intravenous pulse of methylprednisolone appears to be well tolerated and effective in patients with rapidly progressing extensive multifocal AA, but not those with ophiasic and universalis AA.

Adolescent↗

Alopecia areata and cytomegalovirus infection in twins: genes versus environment?

BACKGROUND: Alopecia areata (AA) is hypothesized to be an organ-specific autoimmune disease mediated by T cells directed to the hair follicle. Genetic susceptibility may be conferred by HLA, and an environmental trigger, such as a viral infection, is suspected. The incidence of AA in the population is estimated to be 1.7%, with an average of one in four patients having a positive family history. OBJECTIVE: Our purpose was to examine the concordance rate of AA among identical versus fraternal twins and the correlation between stress, cytomegalovirus (CMV) infection, and disease. METHODS: Families with AA were solicited from dermatologists in the United States and through a Website on the Internet. HLA class 2 typing and identification of CMV early and late genes were performed by polymerase chain reaction (PCR) on genomic peripheral blood DNA. Serum antibodies for CMV were determined by enzyme-linked immunosorbent assay. RESULTS: From 114 families, we identified 11 sets of monozygotic twins and 3 sets of dizygotic twins. The concordance rate was 55% for monozygotic twins and 0% for fraternal twins. Most identical twins were male. The severity of the AA phenotype varied and appeared most severe in the first affected twin. Five of 24 twins were CMV seropositive but CMV DNA was not detected in blood lymphocytes of any of the subjects when studied after the onset of AA. The presence of AA in twins was not correlated with evidence of CMV. CONCLUSION: A 55% concordance rate in identical twins and AA occurring in families support a genetic component as well as possible environmental triggers that remain unknown.

Adolescent↗

Changes in hair weight and hair count in men with androgenetic alopecia, after application of 5% and 2% topical minoxidil, placebo, or no treatment.

Quantitative estimation of hair growth using hair weight and number was recorded for 120 weeks in 4 groups of 9 men with androgenetic alopecia. Three double-blind groups applied either 2% or 5% minoxidil solution, or vehicle. The fourth group, unblinded, received no treatment. Measurements of hair weight and number were continued for 96 weeks, when treatment (if any) was stopped, though measurements were continued for another 24 weeks. Although not compared statistically, the placebo and untreated groups behaved in a similar fashion. In contrast, the 5% and 2% minoxidil treatment groups showed a statistically significant increase in mean percentage change in interval weight from baseline compared with placebo; results for number counts were usually less significant. Over 96 weeks, topical minoxidil induced and maintained an increase in interval weight over baseline of about 30%. After treatment was stopped, hair weight and number counts for the minoxidil groups returned to about the same levels as placebo in 24 weeks.

Administration, Topical↗

Tufted hair folliculitis: a pattern of scarring alopecia?

We present a patient with pemphigus vulgaris who, over the years, experienced the development of tufted hair folliculitis as a result of scalp involvement. Multiple hairs emerged from widely dilated follicular ostia surrounded by indurated, scarred skin. Histopathologic findings were typical for tufted hair folliculitis. We believe that because a specific host response to scalp injury might be crucial to the development of this rare disorder, it should be regarded as a type of scarring alopecia.

Adult↗

Sex hormone-related and growth hormone-related alopecias.

Canine endocrine dermatoses are characterized by bilateral symmetrical alopecia. Although growth hormone-related and sex hormone-related dermatoses are less common than hypothyroidism and hyperadrenocorticism, they are important causes of hormonal skin disease. Several new syndromes associated with growth and sex hormones recently have been described.

Alopecia↗

Focal and generalized alopecia.

Focal or generalized alopecia is defined as hair loss affecting the ventral, lateral, perineal, and dorsal aspects of the trunk of the cat, usually in a symmetric pattern. This may be attributable to failure of hair coat production, excess loss of hair due to self trauma, or excess shedding of whole hairs. Self trauma is the most common cause of hair loss and is associated particularly with flea allergy dermatitis. Other causes of hair loss are reviewed.

Alopecia↗

Alopecia in three women of Southeast Asian descent with chronic renal failure: possible association with erythropoietin use.

Recombinant human erythropoietin (Epo) has been used successfully to correct the anemia caused by chronic renal failure in patients undergoing dialysis, as well as the anemia associated with other conditions, including cancer therapy. Despite its benefits, it can be associated with adverse side effects. These include hypertension, headaches, increased seizure activity, clotted vascular access, and occasional thromboembolic events, such as myocardial infarction or stroke. We report a potentially new side effect associated with Epo of a cosmetic nature. Three Southeast Asian women with chronic renal failure developed diffuse, nearly total, hair loss during erythropoietin use. Two cases were strongly associated with Epo use, and a third had other intercurrent illnesses as well. Alopecia may be associated with Epo use in certain ethnic populations.

Adult↗

Ketocazole as an adjunct to finasteride in the treatment of androgenetic alopecia in men.

Dihydrotestosterone (DHT) binding to androgen receptors (AR) in hair follicles is commonly accepted as the first step leading to the miniaturizing of follicles associated with androgenetic alopecia (AGA). Testosterone is converted to DHT by the enzyme 5alpha-reductase. Finasateride a 5alpha-reducase inhibitor blocks the production of DHT and is currently used to treat AGA. The inhibition is not complete but a reduction of DHT systemically and in the scalp is accomplished. Ketoconazole has been clinically shown to be effective in the treatment of AGA. In this paper, evidence is presented to support the hypothesis that ketoconazole 2% shampoo has a local disruption of the DHT pathway. It is proposed that using ketoconazole 2% shampoo as an adjunct to finasteride treatment could lead to a more complete inhibition of DHT and thus better treat AGA.

Administration, Oral↗