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Clinical and histologic findings in temporal triangular alopecia.

BACKGROUND: Temporal triangular alopecia (TTA; also called "congenital triangular alopecia") is a common disorder that is assumed to be congenital. Little is known about its histologic features. OBJECTIVE: Our purpose was to describe four new cases, review the literature, and present histologic features based on vertical and transverse sectioning. METHODS: The history, clinical features, and histologic findings of four patients with TTA are described and the relevant literature reviewed. RESULTS: Lesions of TTA are seldom congenital, and most are best described as lancet-shaped. The "bald spot" contains normal numbers of hairs, although virtually all are vellus or indeterminate follicles. CONCLUSION: Most cases of TTA appear to develop during the first few years of life, and the designation "congenital" is a misnomer. The appearance of alopecia can be best explained as a focal zone of hair miniaturization leading to vellus hair formation.

Adolescent↗

Immunohistochemical and electron microscopic characterization of the cellular infiltrate in alopecia (areata, totalis, and universalis).

The inflammatory cell infiltrates in scalp skin of 35 patients, 20 with alopecia areata (AA), 7 with totalis, and 8 with universalis were characterized with the ANAE (alpha-naphthylacetate esterase) marker, monoclonal antibodies, and electron microscopy. As demonstrated by the ANAE staining, no clear difference in the main lymphocyte subclasses (T and B cells) or macrophages was seen between the different types of alopecia or as compared to control patients' scalp skin. However, T lymphocytes and macrophages were seen most frequently and in greater numbers perivascularly and infiltrating the hair bulb in those cases of AA where active hair loss took place. Using monoclonal OKT (OKT-3, -4, and -8) antibodies and the avidin-biotin immunoperoxidase method on frozen sections, a concentration of OKT-8 reactive cells (suppressor/cytotoxic T cells) was seen peribulbarly and invading the hair infundibulum. The cells affecting the hair infundibulum were further studied by electron microscopy. They could be classified into three main types: small lymphocytes (60%), macrophages (30%) and cells closely resembling large granular lymphocytes (LGL) (10%). LGL have previously been considered to be human natural killer (HNK) cells. Thus the hair follicle seems to be the target for the cellular immune response in alopecia. Whether HNK cells participate in the destruction of hair bulbs remains to be investigated.

Adolescent↗

Induction of hair growth in alopecia totalis with diphencyprone sensitization.

The therapeutic use of the induction of an allergic contact sensitivity to diphencyprone in alopecia areata has been studied. Fourteen subjects had alopecia totalis and four had extensive and long-standing alopecia areata. Topical diphencyprone was capable of inducing an allergic contact sensitivity in 16 subjects (89%), and 13 of these agreed to proceed to repeated intermittent applications to the left side of the scalp, the right side acting as an untreated control; six subjects achieved cosmetically useful regrowth of normally pigmented terminal hair. No clinical features seemed to distinguish those who showed a favourable response from those who did not.

Adult↗

Differential patterns of elastic fibers in scarring and non-scarring alopecias.

Elastic fiber stain (acid alcoholic orcein) reveals diagnostically significant differences between several types of alopecia of the scalp. A short outline of elastic fiber distribution on the normal hair follicle emphasizes the elastic coat of the follicular isthmus, the sparsity of elastic fibers on the cyclic lower portion of the hair root, and the presence in the neck of the dermal papilla of an elastin-like body which is formed anew with each hair cycle. This body provides a marker of the gradual shortening of successive anagen hairs in male pattern alopecia. Patterns of elastic fibers in the perifollicular and interfollicular dermis are helpful in differentiating idiopathic pseudopelade of Brocq from pseudopeladic states secondary to lupus erythematosus and other disease processes. Within the idiopathic group, the development of elastic fibres on the lower cyclic portion of the hair root identifies a sub-group that may have a different, non-inflammatory pathogenesis and is provisionally designated as fibrosing alopecia.

Alopecia↗

[New therapeutic aspects in non-cicatricial alopecias].

We discuss the promising results of recent pharmacological and clinical studies on non-cicatricial alopecia: (1) The development of effective antiandrogens for topical application will provide some possibilities of 'causal' treatment of androgenetic alopecia in male patients. (2) Further investigations on patients treated with minoxidil may lead to new insight of the factors regulating the follicular functions. (3) Our preliminary experiences with the various therapeutic modes of alopecia areata, based on the autoimmune pathogenesis of this disease, supply the foundation for effective therapeutic regimens for practical use.

Alopecia↗

T lymphocyte subpopulations in alopecia areata and psoriasis: identification with monoclonal antibodies and Fc receptors.

A comparison of T lymphocyte subpopulations as defined by Fc receptors and monoclonal antibodies was investigated in 9 patients with alopecia areata and alopecia universalis (AA and AU) and in 6 patients with psoriasis. It was shown that there was higher proportion of T lymphocytes with Fc receptors for IgG (Tg cells) in patients with alopecia (AA and AU) and psoriasis. The proportions of total T lymphocytes (Tt), T lymphocytes with Fc receptors for IgM, T suppressor/cytotoxic cells (Leu2A), T helper/inducer (Leu3A) as defined by monoclonal antibodies were within normal range as compared to the normal donors. The possible reason of the dissociation between Tg and T suppressor (Leu2A) cells could be that these cells belong to different subpopulations.

Adult↗

Suppressor cell number and function in alopecia areata.

Several values of immunologic function were studied and correlated with disease activity and extent in 14 patients with alopecia areata, alopecia totalis, or alopecia universalis and in a concurrently studied age- and sex-matched control group. As compared with the control group, the patients showed a significantly increased incidence of autoantibody formation, increased concanavalin A-induced suppression of the normal lymphocyte response to mitogens, an increased proportion of suppressor-cytotoxic cells in the peripheral blood, and a decrease in the absolute B-cell count. Absolute total T-cell counts, quantitative serum immunoglobulin determinations, and lymphocyte proliferation after exposure to the mitogens--concanavalin A, phytohemagglutinin, and pokeweed--and to tetanus antigen were comparable for both groups. Neither the percentage of concanavalin A-induced suppression of the normal lymphocyte response to mitogens nor the helper-suppressor ratio correlated significantly with the extent of hair loss. However, patients, particularly those who demonstrated spontaneous regrowth of hair, had increased concanavalin A-induced suppression in conjunction with an increase in the proportion of peripheral suppressor cells.

Adolescent↗

Congenital temporal triangular alopecia.

Congenital temporal triangular alopecia (CTTA) is a condition that occurs very rarely and is often unresponsive to treatment. It is necessary to differentiate it from other conditions such as alopecia areata in order to avoid prolonged treatment which may cause adverse side effects. Two additional cases of CTTA are presented herein. The first case was initially seen eighteen years ago. CTTA was diagnosed after a short period of time, but only after the patient had been mistakenly treated for alopecia areata. In the second case, CTTA was diagnosed during the first examination and no treatment was given. I believe that this condition is not as rare as formerly thought, and I also think that some cases may be misdiagnosed when the physician is not aware that he is presented with a case of CTTA.

Alopecia↗

Familial focal alopecia. A new disorder of hair growth clinically resembling pseudopelade.

A 14-year-old girl was evaluated for patchy hair loss present from early childhood. Her mother was found to have a similar condition. When studied in transverse section, biopsy specimens from both women showed marked anagen-telogen transformation that circumstantially appears to be irreversible. Preservation of telogen epithelium with absence of inflammation and scarring readily separate focal familial alopecia from the pseudopelade state and from localized alopecia areata. To our knowledge, this is the first description of a new familial disorder causing focal alopecia.

Adolescent↗

Alopecia areata. Autoreactive T cells are variably enriched in scalp lesions relative to peripheral blood.

BACKGROUND AND DESIGN: Alopecia areata is a condition characterized by hair loss in association with perifollicular infiltration of T cells and antigen-presenting cells. Autoreactive T cells are postulated to amplify this abnormality by interacting with DR+ follicular epithelium. These cells may recognize either autologous major histocompatibility complex class II antigen or an autoantigen restricted by major histocompatibility complex class II. Limiting dilution analysis was used to determine the frequency of autoreactive lymphocytes in scalp biopsy specimens and peripheral blood from seven adult patients with alopecia areata. Autoreactive T cells are defined for this study as those that proliferate in response to autologous irradiated peripheral blood mononuclear cells. RESULTS: Autoreactive lymphocytes were enriched in scalp biopsy specimens relative to peripheral blood in five of seven patients. This enrichment was statistically significant in four of five patients. Five autoreactive T-cell clones derived from lesional scalp were characterized. Four of these clones were CD3+CD4+CD8- and one clone was CD3+CD4-CD8+. CONCLUSIONS: Enrichment of autoreactive cells in lesions of alopecia areata supports a role for these cells in the pathogenesis of this condition. Enrichment of autoreactive lymphocytes is also found in allergic contact dermatitis. Thus, these autoreactive lymphocytes may have a general role in inflammation.

Adult↗

Acne keloidalis is a form of primary scarring alopecia.

OBJECTIVE: To better define the pathogenesis of acne keloidalis (AK). DESIGN: Prospective, blinded study of histologic material collected from 10 patients with clinically typical AK. SETTING: Outpatient dermatology clinic of a military tertiary care medical center. PATIENTS: Ten male volunteers 18 years or older with early AK lesions (1- to 4-mm firm papules on the lower occipital/nuchal region). DATA SOURCE: Biopsy specimens from small, early lesions and from clinically uninvolved skin, studied histologically with transverse sectioning. INTERVENTION: Three separate 4-mm punch biopsy specimens of the scalp (lesional, perilesional, and "normal" scalp) were obtained from each volunteer. The specimens were processed using transverse sectioning. MAIN OUTCOME MEASURES: The primary variables for data analysis were the presence or absence of the following histologic features: premature loss of the inner root sheath; eccentric placement of shaft, with thinning of the outer root sheath; lamellar fibroplasia surrounding the follicle; loss of sebaceous glands; evidence of follicular destruction or scarring; inflammation; and intrafollicular or perifollicular microorganisms. The number and type of hairs were also recorded. RESULTS: The most common findings in the 19 histologically abnormal specimens were perifollicular, chronic (lymphocytic and plasmacytic) inflammation, most intense at the level of the isthmus and lower infundibulum; lamellar fibroplasia, most marked at the level of the isthmus; complete disappearance of sebaceous glands, associated with inflamed or destroyed follicles; thinning of the follicular epithelium, most marked at the level of the isthmus; and total epithelial destruction (superficial and deep), with residual "naked" hair fragments. Even some "normal" specimens contained true follicular scars, demonstrating that normal-appearing scalp skin had previously been affected by the disease. CONCLUSIONS: Acne keloidalis is a primary form of scarring alopecia, and many of the histologic findings closely resemble those found in certain other forms of cicatricial alopecia. Extensive subclinical disease may be present in patients with AK and can account for some of the permanent hair loss. Overgrowth of microorganisms does not appear to play an important role in the pathogenesis of the disease. There is no etiologic relationship between AK and pseudofolliculitis barbae. Therapies found to be useful in other forms of inflammatory scarring alopecia are useful in the treatment of early AK.

Acne Keloid↗

Evaluation of sexual function with an international index of erectile function in subjects taking finasteride for androgenetic alopecia.

OBJECTIVE: To evaluate variations in sexual and erectile function in subjects taking 1 mg of finasteride for androgenetic alopecia by administering the abridged 5-item version of the International Index of Erectile Function (IIEF-5) questionnaire before and during treatment. DESIGN: In a multicenter study, 186 patients with androgenetic alopecia were asked to complete the IIEF-5 regarding the domain of erectile function before (at baseline) and 4 to 6 months after beginning finasteride treatment. The test was self-administered. SETTING: The study was conducted in 7 institutional dermatology departments in Italy (Bologna, Rome, Genoa, Cagliari, Milan, Florence, and Bari). PATIENTS: A total of 186 patients with androgenetic alopecia were evaluated before and 4 to 6 months after the initiation of finasteride therapy (1 mg). All patients (age range, 19-43 years; mean age, 28.3 years) were followed up as outpatients. RESULTS: The score on each of the 5 domains of the IIEF-5 did not show any significant change after 4 to 6 months of treatment. CONCLUSIONS: Our results support the clinical impression that sexual side effects are actually much less common than reported in clinical trials. The sexual function of all patients remained stable during treatment with 1 mg of finasteride.

5-alpha Reductase Inhibitors↗

Tissue expansion in the treatment of alopecia.

Tissue expanders have been used in multiple body sites. We successfully used the Radovan tissue expander in the scalp of patients with cicatricial alopecia and in hair replacement surgery for male pattern alopecia. The expander is placed in the subgaleal plane beneath a hairbearing area and is inflated over a six- to eight-week period. In a second procedure, the device is removed and the bald area is excised. Closure of the defect is accomplished, with the stretched hairbearing scalp replacing the area of alopecia. Scalp reduction and pedicle scalp flap procedures have benefited by preliminary scalp expansion.

Adolescent↗

Child with manifestations of dermotrichic syndrome and ichthyosis follicularis-alopecia-photophobia (IFAP) syndrome.

We report on a boy with short stature, mental retardation, seizures, follicular ichthyosis, generalized alopecia, hypohydrosis, enamel dysplasia, photophobia, congenital aganglionic megacolon, inguinal hernia, vertebral, renal and other anomalies, and a normal chromosome constitution. The clinical findings include all the features that dermotrichic and ichthyosis follicularis-alopecia-photophobia (IFAP) syndrome have in common and in addition those that characterize IFAP syndrome (photophobia, recurrent respiratory infections, etc.), those that are present only in dermotrichic syndrome (nail anomalies, hypohydrosis, megacolon, vertebral defects, etc.) and additional ones (enamel dysplasia, renal anomalies, inguinal hernia, etc.). Two maternal uncles were referred as being affected by alopecia and ichthyosis suggesting X-linked recessive transmission. Various hypotheses concerning the relationship between the 2 syndromes and the present case are discussed.

Alopecia↗

Simultaneously occurring alopecia areata and Hodgkin's lymphoma: complete remission of both diseases with MOPP/ABV chemotherapy.

A 43-year-old man presented with simultaneously occurring alopecia areata and stage IIIB nodular sclerosing Hodgkin's disease. Systemic symptoms of Hodgkin's disease were present for 6 months, and rapidly developing patchy hair loss of the scalp was present for 2 weeks prior to presentation. The patient was treated with eight cycles of MOPP-ABV chemotherapy that resulted in complete remission of Hodgkin's-disease. Four months after the completion of chemotherapy, the patient had normal hair regrowth with no evidence of alopecia areata. Alopecia areata may be an autoimmune disease and may respond to chemotherapy.

Adult↗

Surgical interventions for the treatment of radiation-induced alopecia in pediatric practice.

Permanent alopecia can occur following treatment for pediatric malignant disease, especially cranial irradiation, resulting in identity and self-image problems. This late effect is usually addressed through external cosmesis and psychological adjustment. Surgical options are less commonly utilized. The experience of reconstructive procedures in patients at RLC NHS Trust, Alder Hey with alopecia is presented. Four patients had scalp tissue expansion and one had hair transplantation. The reconstructive options available are discussed as well as the potential opportunities and difficulties in this population. Post-radiotherapy alopecia can be successfully addressed by reconstructive surgery, and should be considered more often in this population.

Adolescent↗

Immunopathological studies on alopecia areata.

Anti-endothelial cell antibodies could be removed from circulating lymphocytes by means of acid elution techniques in eight patients with different degrees of alopecia areata. These antibodies were specifically directed against the endothelial cells in the capillary network of the hair bulb, indicating the existence of an antigen, which is unique to these particular endothelial cells. These antibodies do not bind complement "in vitro" and are species-specific. Circulating ANA (speckled type) were only noticed in case with alopecia areata in spots. A significant decrease in circulating T cells was noticed in six of eight patients with a certain degree of alopecia.

Adolescent↗

Suppressor cell decrease in alopecia areata.

There is considerable evidence to suggest that autoimmunity plays a role in the pathogenesis of alopecia areata. Since it is known that T cells regulate the immune system, a study was undertaken to measure T helper (OKT-4) and T suppressor (OKT-8) cells in the peripheral blood of patients with alopecia areata (both active and stable) and in controls. Total T cells, B cells, immunoglobulins, and autoantibodies were also measured. There was a highly significant decrease in the T-suppressor cell population of patients with alopecia areata (P less than 0.001). Two of ten patients had microsomal antibodies and three of ten had elevated IgE levels. Other parameters were not significantly different. The decrease in suppressor cells suggest an impairment of the prime negative regulator of the immune system, with loss of tolerance and resultant autoimmunity.

Adolescent↗