Dermal mucin in alopecia areata – tell tale sign or incidental finding?

Alopecia areata is an autoimmune disease causing patchy hair loss, which occurs with an increased incidence in patients with lupus erythematosus. We report a 27-year-old African-American female with systemic lupus erythematosus and alopecia areata, whose biopsy showed a marked increase in mucin in the deep dermis and subcutis. Archival biopsies of alopecia areata were then reviewed to see if this finding occurs in patients without systemic lupus. Of 13 recent biopsies diagnostic of alopecia areata, we detected deposition of mucin in 3 (23%), but all mild in degree and in a superficial location. We speculate that the marked deposition of mucin in this patient's biopsy of alopecia areata may be related to her underlying systemic lupus, and that the presence of marked, deep dermal deposition of mucin might serve as a diagnostic clue for the presence of underlying systemic lupus in patients with alopecia areata.     

Lupus panniculitis clinically simulating alopecia areata

A 27-year-old woman with a known history of lupus erythematosus presented with two circumscribed patches of non-scarring alopecia closely resembling alopecia areata. Scalp biopsy showed a predominantly subcutaneous and deep dermal lymphocytic infiltrate that surrounded the deep follicular segments and hair bulbs, as well as the eccrine glands. There was associated hyaline fat sclerosis. The epidermis, infundibular and isthmus segments of follicles were relatively spared and lacked the lichenoid inflammation and fibrosis seen with lupus erythematosus. The biopsy findings illustrate that the deep variant of lupus panniculitis may be concentrated around the hair bulbs and deep temporary segments of hair follicles and spare the permanent stem cell-rich follicular segments. This pattern is capable of producing a temporary hair-loss, clinically simulating alopecia areata. The clinical history, presence of subtle erythema and scalp tenderness on physical examination, as well as the biopsy findings, were important clues in distinguishing our case from a true combination of alopecia areata and lupus erythematosus.     

ANTISMOOTH MUSCLE AND ANTIPARIETAL CELL ANTIBODIES IN INDIANS WITH ALOPECIA AREATA

Background. Alopecia areata is suspected to be an autoimmune disease. We studied 104 consecutive patients with alopecia areata for the presence of autoantibodies and associated autoimmune diseases. Methods. A detailed history and examination was carried out in all patients to look for associated atopy, diabetes mellitus, hypertension, rheumatoid arthritis, vitiligo, lupus erythematosus, and thyroid disorders, etc. in the patients or their family members. Venous blood for estimation of fasting and postprandial blood glucose was collected in 30 patients, especially in those with family history of diabetes mellitus. Antimitochondrial (AMA), antismooth muscle (SMA), antinuclear antibodies (ANA), antiparietal cell antibody (PCA), and antibody against thyroid microsome (TMA) were detected employing indirect immunofluorescence on a composite section of rat liver, stomach, kidney, and human thyroid. Skin biopsy was processed for direct immunofluorescence by a conventional technique. Results. Disseminated discoid lupus erythematosus, lichen planus, urticaria, psoriasis, and seronegative spondylarthritis were associated with alopecia areata in one case each. Anti-smooth-muscle-antibodies and PCA were found in 36 (34.6%) and 44 (42.3%) patients respectively, followed by TMA in 8 (7.7%), AMA in 6 (5.7%), antithyroglobulin antibodies in 3 (2.8%), and ANA in 2 (1.9%) patients. The incidence of SMA was higher in men with alopecia areata (P< 0.001). Direct immunofluorescence carried out in 24 patients did not reveal significant findings, except for occasional immunoglobulin deposits around hair follicles and blood vessels. Conclusion. Alopecia areata in India is associated more often with antismooth muscle and antiparietal cell antibodies.     

Nuchal nevus flammeus as a skin marker of prognosis in alopecia areata

In this work, the incidence of nuchal nevus flammeus was studied in 205 patients suffering from various forms of alopecia areata, as well as in a group of 555 volunteers without alopecia areata examined in our outpatient clinic. The incidence of nuchal nevus flammeus in the totalis-universalis form of alopecia areata was 58.2% (examined patients, n = 79), in ophiasis-extensive forms 22.8% (examined patients, n = 70) and in simple forms of alopecia areata 3.6% (examined patients, n = 56). In the group of 555 volunteers without alopecia areata the incidence of nuchal nevus flammeus was 4.5%. Our results show that nuchal nevus flammeus could be a valuable skin marker indicating a more severe course of alopecia areata.     

Severe alopecia areata treated with systemic corticosteroids

Background Treatment of severe alopecia areata is difficult, and most efforts to successfully treat this condition have been disappointing. Systemic corticosteroids have been demonstrated as an effective treatment of severe alopecia areata. Methods Eighteen patients with alopecia areata (extensive patchy and totalis universalis types) were treated with systemic corticosteroids. Results Satisfactory hair regrowth was achieved in seven patients (38.9%). Hair fall subsequently occurred in all of these patients on discontinuation or tapering of corticosteroid therapy. Conclusions Systemic corticosteroid therapy does not prevent the spread or relapse of severe alopecia areata and, when complete regrowth is obtained, it is rarely maintained off therapy.     

Frontal fibrosing alopecia: a survey in 16 patients

BACKGROUND: Postmenopausal frontal fibrosing alopecia (PFFA) was described by Kossard et al. as a progressive recession of the frontal hairline affecting particularly postmenopausal women. Further cases of PFFA have been reported to date, all of them considering it as a variant of lichen planopilaris on the basis of its clinical, histological and immunohistochemical features. OBJECTIVE: To describe clinical features, and response to treatment of 16 cases of frontal fibrosing alopecia diagnosed at our department in the last 6 years. METHODS: In addition to clinical data, biopsies and laboratory tests (antinuclear antibodies, sex hormones, thyroid hormones) were performed in order to rule out other causes of scarring alopecia. Patients were treated with intralesional corticosteroids, finasteride, and minoxidil, depending on the stage of the disease and association to androgenetic alopecia. RESULTS: All patients presented progressive alopecia localized to the frontal and temporal hairlines. Eight patients (50%) had loss of eyebrows, and six patients (37.5%) had axillar alopecia. Ages ranged from 45 to 79. Three of these women were premenopausal. Androgenetic alopecia was evident in seven patients (43.8%). All patients biopsied showed perifollicular lymphocitic infiltrate with lamelar fibrosis limited to the upper portions of the follicle. The progression of the condition stopped in most patients after a variable period on treatment. When treatment was abandoned the alopecia progressed to 'clown alopecia' appearance. DISCUSSION: Cases of Kossard's type scarring alopecia affecting premenopausal women made us consider that this condition is not exclusive of postmenopausal women. Differential diagnosis should take into account conditions like female androgenetic alopecia, fibrosing alopecia in a pattern distribution, alopecia areata, and chronic lupus erythematosus. Except for the pattern of alopecia, lichen planopilaris and frontal fibrosing alopecia are indistinguishable, thus the latter is included as a variant of lichen planopilaris. Although the disease tends to spontaneous stabilization, intralesional and topical corticosteroids, and anti-androgens may stop the progression of the disease and improve the female androgenetic alopecia that usually is associated to FFA.     

Coexistence of chronic cutaneous lupus erythematosus and frontal fibrosing alopecia

Lupus erythemathosus is a chronic, relapsing disease with acute, subacute, and chronic lesions. Effluvium telogen occurs in the setting of systemic activity of the disease, and cicatricial alopecia results from discoid lesionsin on the scalp. Other types of alopecia, like alopecia areata, may rarely be found in lupus. Frontal fibrosing alopecia is characterized by frontotemporal hairline recession and eybrow loss. Histophatologically, it cannot be differentiated from lichen planopilaris.It is controversial whether frontal fibrosing alopecia is a subtype of lichen planopilaris.. A pacient with chronic lichenoid lupus erythematosus is described with clinical, histophatological and dermoscopic features of frontal fibrosing alopecia.We have not been able to find in the literature cases of frontal fibrosing alopecia as a clinical manifestation of lupus.     

Discoid lupus erythematosus exacerbated by contact dermatitis caused by use of squaric acid dibutylester for topical immunotherapy in a patient with alopecia areata

A 57-year-old Japanese male patient with an 18-year history of discoid lupus erythematosus (DLE) presented with alopecia on his scalp, and was clinically diagnosed to have alopecia areata. He was started on topical immunotherapy with squaric acid dibutylester (SADBE) for the treatment of alopecia areata. The patient was first sensitized with the application of 2% SADBE on the right upper arm, followed subsequently by re-exposure to a low concentration of SADBE to provoke contact dermatitis on the scalp as treatment. Approximately 2 months later, he developed multiple red scaly lesions on his scalp and face, which were diagnosed histopathologically as DLE. DLE is known to be exacerbated by a variety of factors, including sunlight, X-rays, tattoos, burns, and some forms of cutaneous trauma, including dermatitis. However, to the best of our knowledge, there have only been two reported cases of DLE exacerbated by contact dermatitis.     

Efficacy and safety of methotrexate in alopecia areata

BACKGROUND

Alopecia areata is a chronic disorder of the hair follicles and nails, of unknown etiology, with clear autoimmune components and genetic factors. Several therapeutic options have been suggested; however, no treatment is able to modify the disease course. Methotrexate is an immunosuppressant used in various dermatoses and recently introduced as a therapeutic option for alopecia areata.

OBJECTIVES

To evaluate the efficacy and safety of methotrexate in alopecia areata.

METHODS

In a retrospective, non-controlled study, we evaluated 31 patients with alopecia areata in current or prior treatment with methotrexate to assess the therapeutic response according to sex, age, pattern of alopecia areata, disease duration, cumulative dose of methotrexate, use of systemic corticosteroids or other treatments, and drug safety.

RESULTS

Regrowth greater than 50% was observed in 67.7% of patients, with the best responses observed in those with <5 years of disease progression (79%), age over 40 years (73.3%), male patients (72.8%), cumulative dose of methotrexate 1000-1500 mg, and multifocal alopecia areata (93%). Among patients receiving systemic corticosteroids in combination with methotrexate, 77.3% had greater than 50% regrowth, compared with 44.4% in those who used methotrexate alone. The therapeutic dose ranged from 10-25 mg/week. No patient had serious adverse effects. Relapse was observed in 33.3% of patients with more than 50% regrowth.

CONCLUSION

Methotrexate appears to be a promising and safe medication for the treatment of severe alopecia areata when used alone or in combination with corticosteroids.     

Treatment of persistent alopecia areata with sulfasalazine

Background  Alopecia areata is an autoimmune disease with no definitive treatment, and some cases persist despite standard therapies. Sulfasalazine has been reported to show success in the treatment of persistent cases of alopecia areata.
Objective  To assess the efficacy of sulfasalazine in cases of recalcitrant alopecia areata that do not respond to topical and intralesional corticosteroids, 5% minoxidil, or psoralen plus ultraviolet-A (PUVA) therapy.
Methods  Thirty-nine patients with persistent alopecia areata received 3 g of oral sulfasalazine for 6 months, and terminal hair regrowth was quantified as no response, moderate response, or good response.
Results  A good response occurred in 10 of the 39 patients (25.6%), a moderate response in 12 (30.7%), and a poor or no response in 17 (43.5%).
Conclusion  Sulfasalazine can be used as an alternative drug in patients with persistent alopecia areata.     

Ant-induced alopecia: report of 2 cases and review of the literature

Localized scalp hair loss is associated with many processes, including alopecia areata, trichotillomania, tinea capitis, and early lupus erythematosus. There are several reports of localized alopecia after tick- and flea-bites and bee stings, but there are only two reports of ant-induced alopecia in the literature. We present two cases of alopecia induced by ants of genus Pheidole (species pallidula) and review the literature for insect-induced alopecia. Ant-induced alopecia should be considered in the differential diagnosis of localized sudden-onset alopecia, at least in some geographic areas of the world.     

Dermoscopic Approach to a Small Round to Oval Hairless Patch on the Scalp

Background

Various kinds of alopecia can show small round or oval hairless patch. Dermoscopy could be a simple, useful tool for making a correct diagnosis.

Objective

The aim of this study is to investigate clinical usefulness of dermoscopy for diseases with small round or oval hairless patch on the scalp.

Methods

Dermoscopic examination was performed for 148 patients with small round or oval hairless patch using DermLite® II pro. The type and its patient number of alopecia investigated in the study were as below: alopecia areata (n=81), trichotillomania (n=24), tinea captis (n=13), traction alopecia (n=12), lichen planopilaris (n=8), discoid lupus erythematosus (n=7), congenital triangular alopecia (n=2) and pseudopelade of Brocq (n=1). The significance of dermoscopic findings for each disease were evaluated.

Results

Characteristic dermoscopic findings of alopecia areata were tapering hairs and yellow dots. Those of trichotillomania and traction alopecia were broken hairs. Dermoscopic findings of tinea capitis included bent hairs, perifollicular white macules and greasy scales. Discoid lupus erythematosus and lichen planopilaris were characterized by dermoscopic findings of lack of follicular ostia. Furthermore, keratin plugs were frequently seen in discoid lupus erythematosus whereas perifollicular hyperkeratosis and erythema were frequently seen in lichen planopilaris.

Conclusion

Dermoscopic examination for small round or oval hairless patch showed characteristic findings for each disease. Based on these results, we propose dermoscopic algorithm for small round or oval hairless patch on the scalp.     

ALOPECIA AREATA THYROID DISEASE AND AUTOIMMUNITY

SUMMARY.— The incidence of thyroid disease in patients with alopecia areata was greater than in patients with psoriasis and also greater than in a control series of the general population. There was also an increased incidence of alopecia areata and diabetes mellitus in the relatives of patients with alopecia areata. No significant difference was found in the incidence of thyroglobulin and thyroid complement-fixing antibodies in patients with alopecia areata as compared with a general practice population. A negative association between psoriasis and thyroid antibodies was demonstrated.     

斑秃的治疗现状

斑秃是一种累及生长期毛囊的免疫相关性疾病.斑秃的治疗主要是依据患者的年龄、疾病的严重程度及持续时间来选择合适的治疗方法,包括糖皮质激素、米诺地尔、免疫疗法、生物制剂及试验性治疗和疾病管理措施等,但至今尚无确切有效的治疗和预防的方法,有些疗法也没有系统的随机、对照试验证据,其治疗方面仍是一大挑战.目前认为,斑秃是一种毛囊免疫赦免破坏的自身免疫性疾病,免疫抑制可控制病情,进一步重建免疫状态成为斑秃治疗的理想观念.     

Functional polymorphisms of the FAS/FASLG genes are associated with risk of alopecia areata in a Chinese population: a case–control analysis

Background The Fas/Fas ligand system plays a key role in regulating cell growth and apoptosis. Previous findings have suggested that FAS and FASLG polymorphisms are associated with systemic lupus erythematosus, autoimmune hepatitis, vitiligo and other autoimmune‐related disorders. However, to the best of our knowledge, there is no reported study on the associations between FAS and FASLG polymorphisms and the risk of alopecia areata. Objectives To investigate the associations between FAS and FASLG polymorphisms and the risk of alopecia areata in a Chinese Han population. Methods In a hospital‐based case–control study of 84 patients with alopecia areata and 84 controls, we genotyped FAS 1377G>A, FAS 670A>G and FASLG 844T>C polymorphisms and assessed their association with alopecia areata risk. Results We found that a reduced risk of alopecia areata appeared to be associated with the FAS 670AG genotype [adjusted odds ratio (OR) 0·43; 95% confidence interval (CI) 0·22–0·86] when compared with the FAS 670AA genotype, but no risk was associated with any of the FAS 1377G>A and FASLG 844T>C genotypes. In the combined analysis, we found that the presence in individuals of two at‐risk alleles of the three FAS/FASLG polymorphisms was associated with a lower risk of alopecia areata (adjusted OR 0·21; 95% CI 0·05–0·89) when compared with the presence of six at‐risk alleles. Conclusions These results suggest that genetic variants in the FAS and FASLG genes may contribute to the aetiology of alopecia areata.     

Dramatic Response of Scarring Scalp Discoid Lupus Erythematosus (DLE) to Intravenous Methylprednisolone, Oral Corticosteroids, and Hydroxychloroquine in a 5-Year-Old Child

Abstract:  Discoid lupus erythematosus (DLE) is rare in childhood. We report the case of a 5-year-old girl who presented with erythematous scaly plaques, with scarring alopecia, involving approximately 40% of her scalp. Histopathology confirmed the diagnosis of DLE. Treatment with intravenous methylprednisolone, hydroxychloroquine, oral prednisone, topical corticosteroids, and sunscreen lead to reversal of scarring alopecia and re-growth of hair.     

Investigation of the hair follicle inner root sheath in scarring and non-scarring alopecia

BACKGROUND: Premature desquamation of the inner root sheath (IRS) is described as a defining histologic feature of follicular degeneration syndrome/central centrifugal cicatricial alopecia (CCCA). However, IRS abnormalities have been noted in other types of alopecia. DESIGN: We evaluated the IRS in terminal hair follicles with transverse sections stained with hematoxylin-eosin and Ziehl-Neelsen stains in 22 non-scarring (7 areata and 15 androgenetic) and 21 scarring (13 CCCA, 2 lichen planopilaris, 2 lupus erythematosus, 1 folliculitis decalvans and 3 end stage) alopecia cases. In addition, we evaluated 15 normal controls with longitudinal sections to establish the level of IRS desquamation. RESULTS: The IRS was present in 99.5 +/- 0.01% (mean +/- standard error) of normal follicles at the level of the arrector pili muscle/sebaceous gland/sweat gland coil (L2) and variably present at higher levels. The IRS was present at L2 in 97.9 +/- 1.5% of alopecia areata, 87.4 +/- 5.3% of androgenetic alopecia, 59.3 +/- 7.0% of CCCA and 49.4 +/- 11.3% of other scarring alopecia follicles. CONCLUSIONS: Premature desquamation of the IRS was identified in CCCA; however, it was also noted in other scarring alopecia cases. IRS premature desquamation is a non-specific histologic feature in scarring alopecia and cannot be used alone as a defining feature of CCCA.     

The pattern and profile of alopecia areata in Singapore--a study of 219 Asians

BACKGROUND: Alopecia areata is believed to be an autoimmune condition with a worldwide occurrence. It usually presents as patchy, nonscarring hair loss. There is a paucity of clinical data in Asians. OBJECTIVE: To study the epidemiology, clinical aspects, associations, and treatment of alopecia areata in an Asian population over a 1-year period. METHODS: Records of all newly diagnosed alopecia areata cases seen from May 1998 to April 1999 at the National Skin Center were collated with regard to the epidemiology, pattern of alopecia, and associations according to the investigational guidelines published by Oslen et al. The treatment and psychologic impact of alopecia areata were also assessed. RESULTS: Two hundred and nineteen new case referrals of alopecia areata were seen from May 1998 to April 1999. The incidence of alopecia areata was 3.8%. There were 173 Chinese (79%), 35 Indians (16%), and 11 Malays (5.0%). The male to female ratio was 1 : 1.3. The median age at presentation was 25.2 years. The majority of patients (85.5%) had their first episode of alopecia areata before the age of 40 years. Of the patients with onset of alopecia areata before the age of 40 years, 36.5% presented with extensive alopecia, compared with 5.5% above the age of 40 years (P < 0.05). Nail changes, consisting of pitting, trachyonychia, and longitudinal ridging, were reported in 23 patients (10.5%). A significant percentage of patients had an associated personal and family history of atopy (60.7%). There was no significant association between a personal history of atopy and the extent of alopecia areata. The frequencies reported for the following associated diseases were: thyroid disease, 2.3%; vitiligo, 4.1%; diabetes mellitus, 3.2%; Down's syndrome, 1.4%; and rheumatic arthritis, 0.9%. A family history of alopecia areata was reported in 4.6%. Intralesional triamcinolone acetonide was the first-line treatment for limited alopecia areata, while squaric acid dibutyl ester was used for extensive involvement. The majority of patients with limited alopecia areata (82.1%) had more than 50% improvement with intralesional triamcinolone acetonide after 3 months. The majority of patients who received squaric acid dibutyl ester (87.5%) achieved more than 50% regrowth at the end of 6 months. Poor prognostic factors for alopecia areata were extensive involvement, early age of onset, and Down's syndrome. Thirteen out of 132 respondents (9.8%) recalled stressful events preceding hair loss. Patients with extensive alopecia areata experienced more psychologic adverse effects than those with limited alopecia areata (P < 0.05). Males with extensive alopecia areata experienced more severe psychologic ill-effects, such as depression and feelings of inability to improve hair loss. CONCLUSIONS: Our findings are similar to those reported in the Western literature where alopecia areata is predominantly a disease of the young. A holistic approach is important in the management of alopecia areata as the disease can have a severe psychologic impact on an individual's well-being.     

Trichomegaly in a 3-Year-Old Girl with Alopecia Areata

Abstract:   We report here a case of bilateral trichomegaly associated with alopecia areata in a 3-year-old girl, healthy except for mild atopic dermatitis. Trichomegaly is a rare condition and, in many cases, is a side effect of medication such as ophthalmic solution prostaglandin analogs and epidermal growth factor receptor inhibitors. Trichomegaly has also been associated with acquired medical conditions such as HIV, systemic lupus erythematosus, anorexia nervosa, porphyria cutanea tarda, hypothyroidism, and dermatomyositis. In very rare circumstances, trichomegaly has been described as part of congenital conditions such as Oliver-McFarlane syndrome. We believe that the development of bilateral trichomegaly in conjunction with alopecia areata in this patient represents a novel finding as it occurred in the absence of any significant health problems, congenital abnormalities, or medications.