Clinical presentations of alopecia areata

Alopecia areata (AA) may can occur on any hair-bearing region. Patients can develop patchy nonscarring hair loss or extensive loss of all body hair. Hair loss may fluctuate. Some patients experience recurrent hair loss followed by hair regrowth, whereas others may only develop a single patch of hair loss, never to see the disease again. Still others experience extensive loss of body hair. The heterogeneity of clinical presentations has led investigators conducting clinical therapeutic trials to typically group patients into three major groups, those with extensive scalp hair loss [alopecia totalis (AT)], extensive body hair loss [alopecia universalis (AU)], or patchy disease (AA). Treatment outcomes have been correlated with disease duration and extent. Recently, guidelines were established for selecting and assessing subjects for both clinical and laboratory studies of AA, thereby facilitating collaboration, comparison of data, and the sharing of patient-derived tissue. For reporting purposes the terms AT and AU, though still used are defined very narrowly. AT is 100% terminal scalp hair loss without any body hair loss and AU is 100% terminal scalp hair and body loss. AT/AU is the term now recommended to define the presence of AT with variable amounts of body hair loss. In this report the term AA will be used broadly to encompass the many presentations of this disease. Development of AA may occur with changes in other ectodermal-derived structures such as fingernails and toenails. Some investigators have also suggested that other ectodermal-derived appendages as sebaceous glands and sweat glands may be affected in patients experiencing AA. Whether or not function of these glands is truly impaired remains to be confirmed. Many patients who develop patchy or extensive AA complain of changes in cutaneous sensation, that is, burning, itching, tingling, with the development of their disease. Similar symptoms may occur with hair regrowth. The potential involvement of the nervous system in AA has led to morphologic investigations of the peripheral nervous system as well as analysis of circulating neuropeptide levels. In this article the clinical presentations of AA are reviewed. The guidelines for conducting treatment studies of AA are presented and observations on changes in cutaneous innervation are introduced. Throughout the text, unless otherwise noted, AA will be used in a general way to denote the spectrum of this disease.     

Dry dermoscopy in clinical treatment of alopecia areata

Although dermoscopy is conventionally utilized with immersion gel for diagnosis of pigmented tumor, we utilized dry dermoscopy, which is dermoscopy without immersion gel, for clinical treatment of alopecia areata (AA). The scalp skin and hair of a 38-year-old Japanese male, and 23-, 22- and 47-year-old Japanese females with AA, whose normal hair color was black, were examined by dry dermoscopy. Exclamation mark hairs, short hairs, fractured hairs and black dots, all characteristic of AA, were detected by dry dermoscopy of the scalp of the 23-year-old female with ophiasis type AA. In the case of the 47-year-old female with round hair loss on the occipital scalp and diffuse hair loss over the fronto-vertical region, dry dermoscopy was useful for diagnosis of AA based on hair characteristic of AA. After she received corticosteroid pulse therapy with 500 mg of i.v. methylprednisolone on 3 successive days, her hair showed apparent regrowth and disappearance of the abnormal hairs characteristic of AA, evidenced by dry dermoscopy 1 month later. In a case of long-lasting AA in the 23-year-old female, we found a follicular plaque-like appearance at the opened hair follicle pores by dry dermoscopy. Histopathologically, the incompletely differentiated remnant hair shaft was packed in the follicular infundibulum. In addition, regrowing vellus hairs, which were difficult to clinically recognize, were detected by dry dermoscopy. Dry dermoscopy is therefore useful for both diagnosis and follow up of AA.     

Prominent follicular mucinosis with diffuse scalp alopecia resembling alopecia areata

A 56‐year‐old Caucasian female presented with a 2‐month history of alopecia. On examination, she had diffuse hair loss of her scalp with some discrete patches of nonscarring alopecia. Histopathology revealed an inflammatory nonscarring alopecia with prominent follicular mucinosis and findings suggestive of alopecia areata. The patient's alopecia completely resolved with oral prednisone. The histopathologic findings and clinical presentation are most consistent with a diagnosis of alopecia areata with follicular mucinosis, although the differential diagnosis is broad. As follicular mucinosis may be associated with both benign and malignant conditions, it is important to be cautious regarding the clinical diagnosis when this reaction pattern is observed histopathologically.     

Use of trichoscopy for the diagnosis of alopecia areata coexisting with primary scarring alopecia in a female hair loss patient

Despite patchy hair loss being typically observed in alopecia areata (AA), similar lesions can be seen in other forms of alopecia and the diagnosis is sometimes challenging. Of note, patchy primary scarring alopecia (SA) needs to be clearly distinguished from AA as SA can leave permanent hair loss. Herein, we report a previously unreported case of AA coexisting with SA successfully diagnosed by detailed trichoscopic investigation. A 42‐year‐old woman visited us with patchy hair loss lesions on the scalp. On physical examination, alopecic lesions sized up to 2 cm in diameter were observed in the right temporal and parietal regions. A gentle hair pull test collected dystrophic anagen hairs from some patches. Trichoscopy detected tapering hairs and black dots. The diagnosis of AA was made. However, some reddish patches were totally hair pull test negative, urging us to further evaluate the remaining lesions. Additional trichoscopic investigation revealed the disappearance of follicular ostia and the presence of a white and milky‐red area and peripilar scales, suggestive of SA. In histology, the clinically AA lesion showed peribulbar cell infiltration, while the potentially SA lesion demonstrated inflammatory cell infiltration around the isthmus and the decrease in hair follicles, some of which were replaced by fibrotic tissue. The final diagnosis of AA coexisting with SA was made. Intralesional corticosteroid injection improved AA but not SA. These findings emphasize the need for thorough trichoscopic examination for accurate diagnoses of rare hair loss conditions.     

Topical and intralesional therapies for alopecia areata

Alopecia areata is a common form of nonscarring alopecia. It affects males and females equally and has no racial predilection. It usually affects the scalp, but any hair-bearing area can be involved. It presents as patchy hair loss, loss of hair on the entire scalp (alopecia totalis), or the whole body (alopecia universalis). The histopathology varies according to the disease stage, but usually a perifollicular lymphocytic infiltrate is seen. The course of the disease and response to treatment are unpredictable. Various therapeutic modalities are used including topical, intralesional, and systemic agents, although none are curative or preventive. This article will review the available topical and intralesional agents that are used in the treatment of alopecia areata and suggest a management approach based on the age of the patient and extent of the disease.     

Histopathology of alopecia areata,acute and chronic: Why is it important to the clinician?

Alopecia areata (AA) is often easy to diagnose but a scalp biopsy for horizontal sectioning is routine in this research clinic. The characteristic histological feature of AA is the peribulbar and intrabulbar mononuclear cell infiltrate, which occurs in the acute stage of the disease but may be absent in biopsies taken at a later stage. AA evolves through acute, subacute, chronic, and recovery phases. Increased numbers of terminal catagen and telogen hairs are found in the acute and perhaps subacute stages with increased numbers of miniaturized, vellus-like hairs in the subacute and chronic stages. Thus, it is important for clinicians and pathologists to recognize the different phases of AA, so that in the absence of the classic findings of a peribulbar lymphocytic infiltrate, a diagnosis of AA can still confidently be made.     

Trichostasis spinulosa of the scalp mimicking Alopecia Areata black dots

Alopecia areata is a common autoimmune disorder that leads to nonscarring hair loss. Black dots, also called comedo-like cadaver hairs, can be found in almost 50% of alopecia areata patients and indicate disease activity. Trichostasis spinulosa is a follicular disorder resulting from the retention of numerous hairs surrounded by a keratinous sheath in dilated follicles. Trichostasis spinulosa is a relatively common but underdiagnosed disorder of hair follicles. Here, we describe a man with alopecia areata of the eyebrows, androgenetic alopecia and trichostasis spinulosa at the vertex and show how dermoscopy can be useful in distinguishing black dots from Trichostasis spinulosa lesions.     

Hair shaft abnormalities in alopecia areata evaluated by optical coherence tomography

Background/purpose: Optical coherence tomography (OCT) is able to provide highly reproducible measurements of hair shaft thickness, including hair shaft diameter, cross‐sectional surface area and hair shape, similar to histology but in vivo. Variations in the caliber of hair shafts have been described in patchy hair loss like alopecia areata (AA) using electron microscopy. The aim of this study was to evaluate whether OCT is useful for the evaluation of hair shaft abnormalities in AA. Methods: The measurements were performed on patients with AA (n=9), aged 2–66 years. Fifty hairs from the border of an alopecic area and 50 hairs from an unaffected area without hair loss were examined using the OCT technique. The hair parameters were characterized by the cross‐section (CS) and the form factor. The ratio of the maximal and minimal diameters of the hair at a fixed measuring distance from the scalp surface determined the form factor (d_max/d_min). Results: In all cases, the CS of hairs from an AA patch was significantly lower compared with hairs of an unaffected area. However, the form factor did not indicate any disturbances in hair growth. Conclusions: The results demonstrate that structural abnormalities of hair shafts are found in active lesions of AA, but not in clinically unaffected hairs. The OCT technique is a promising method to gain more insight into the pathogenesis of AA in a non‐invasive way.     

Etiopathogenesis of alopecia areata: Why do our patients get it?

Alopecia areata (AA) is a nonscarring, inflammatory skin disease that results in patchy hair loss. AA is unpredictable in its onset, severity, and duration making it potentially very stressful for affected individuals. Currently, the treatment options for AA are limited and the efficacy of these treatments varies from patient to patient. The exact etiology of AA is unknown. This article provides some insights into the etiopathogenesis of AA and why some people develop it. The current knowledge on the pathogenesis of AA is summarized and some of the recent hypotheses and studies on AA are presented to allow for a fuller understanding of the possible biological mechanisms of AA.     

Alopecia areata: autoimmune basis of hair loss

Alopecia areata (AA) is a heterogeneous disease characterized by nonscarring hair loss on the scalp or any hair-bearing surface. A wide range of clinical presentations can occur -- from a single patch of hair loss to complete loss of hair on the scalp (alopecia totalis) or the entire body (alopecia universalis). Particularly in severe or chronic cases, AA may cause considerable psychological and emotional distress for affected individuals. The estimated lifetime risk of developing AA is 1.7%. While the precise etiology of this common disorder has not been elucidated, a substantial body of evidence suggests that AA is an organ-specific, autoimmune disease, targeted to hair follicles. However, the antigenic target(s), mechanisms, and consequences of autoimmune attack in AA have yet to be determined. Here, we critically explore the evidence supporting the hypothesis that AA is an autoimmune disease and propose specific pathways by which self-directed immune responses are generated.