Clinical Features of Primary Cicatricial Alopecia in Chinese Patients
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Original Clinical features of primary cicatricial alopecia in Article Chinese patients Shiling Qi, Ying Zhao, Xiaoting Zhang, Shuifeng Li, Hui Cao, Xingqi Zhang Department of Dermatology, ABSTRACT First Affiliated Hospital of Sun Yat‑sen University, Guangzhou, Background: There have been few reports on primary cicatricial alopecias (PCR) especially from Guangdong 510080, People’s Republic of China Asia (PCA). Aims: To study the clinical, pathological and dermoscopic characteristics of PCA among Chinese patients. Methods: A retrospective analysis of the clinical data of 59 patients with Address for corresapondence: PCA was conducted and the dermoscopic, pathological, treatment and prognosis characteristics Dr. Xingqi Zhang, analyzed. Fisher’s Chi‑square exact test, Kruskal‑Wallis and Spearman rank correlation test Department of Dermatology, were performed. Results: The ratio of neutrophilic to lymphocytic cicatricial alopecias was about First Affiliated Hospital of 1.3:1 in this group. The most frequent disorder was folliculitis decalvans. Follicular openings were Sun Yat‑sen University, absent on dermoscopy in all cases except alopecia mucinosa. Patulous follicular openings were Guangzhou, Guangdong 510080, People’s characterisitc of alopecia mucinosa. After treatment, an increase in short vellus hairs was the Republic of China. earliest feature, while telangiectasia, epidermal scale, follicular hyperkeratosis, pustules and hair E‑mail: diameter diversity gradually decreased or even disappeared. Improvement in the areas of hair [email protected] loss after treatment was seen more often in discoid lupus erythematosus, folliculitis decalvans and dissecting cellulitis than in patients with classic pseudopelade of Brocq. Nine patients (13.6%) relapsed after cessation of therapy. Female patients needed longer treatment times. Long duration, large areas of hair loss and shorter treatment courses were the major factors in relapses. Conclusions: Dermatoscopy provides a rapid, practical and useful aid for the diagnosis of PCA and also to assess disease activity. Patulous follicular openings are a specific dermoscopic sign of alopecia mucinosa. Lichen planopilaris is less common in China than in the West. Key words: Cicatricial/scarring alopecia, clinical features, dermoscopy, pathology INTRODUCTION [FD], dissecting cellulitis/folliculitis [DC]) and 3. A mixed group and nonspecific group according Primary cicatricial/scarring alopecias (PCA) are a group to the histopathologic features.[1] of rare disorders with permanent hair loss. These may be divided into: These alopecias are difficult to treat, seriously affecting 1. A lymphocytic group (e.g. chronic cutaneous lupus the patient’s quality of life. In this retrospective study, erythematosus/discoid lupus erythematosus [DLE], we analyzed the clinical, histological, dermoscopic lichen planopilaris [LPP], classic pseudopelade of features, and the treatment and prognosis of primary brocq [PB], alopecia mucinosa [AM]) scarring alopecia in 59 Chinese patients. 2. Neutrophilic group (e.g. folliculitis decalvans METHODS Access this article online Patients Quick Response Code: Website: Between April 2007 and February 2013, 59 cases of www.ijdvl.com PCA were identified among patients attending our Hair DOI: Clinic, accounting for 2.1% of all patients with hair 10.4103/0378-6323.136833 loss. The diagnosis was established using clincal and PMID: histopathologic criteria; direct immunofluorescence ***** was performed in some cases. How to cite this article: Qi S, Zhao Y, Zhang X, Li S, Cao H, Zhang X. Clinical features of primary cicatricial alopecia in Chinese patients. Indian J Dermatol Venereol Leprol 2014;80:306‑12. Received: November, 2013. Accepted: February, 2014. Source of Support: Nil. Conflict of Interest: None declared. 306 Indian Journal of Dermatology, Venereology, and Leprology | July-August 2014 | Vol 80 | Issue 4 Qi, et al. Primary cicatricial alopecia Methods using Spearman rank correlation test. A P value less The age, sex, age of onset, clinical presentation, than 0.05 was considered statistically significant. duration of disease, concomitant diseases, and response to treatment were recorded. Laboratory RESULTS tests included routine blood tests, double‑stranded deoxyribonucleic acid (dsDNA) and antinuclear Clinical findings antibody (ANA) (ELISA, Aesku Diagnostics, The study comprised of 36 male and 23 female Wendelsheim, Germany). Dermatoscopic examination patients (≈1.6:1), aged 5 to 74 years with mean age of was performed by a hand‑held non‑contact polarized 31.7 years. Duration of disease ranged from 1 month to dermoscope (Dermlite, DL3 model, 3Gen, San Juan 10 years with an average of 20.1 months. The patients Capistrano, CA, USA). The area of hair loss was included 13 cases of discoid lupus erythematosus, 2 cases calculated using the Olsen method.[2] Pus swabs each of lichen planopilaris and alopecia mucinosa, 9 were taken for culture and antibiotic sensitivity test. with pseudopelade of brocq, 24 of folliculitis decalvans, The scalp biopsy specimens were obtained from the and 9 cases of dissecting cellulitis. The ratio of biopsies active border of areas of hair loss after obtaining with lymphocytic versus neutrophilic infitrates was signed consent. Biopsy samples were divided into about 0.79 (26 vs. 33). The clinical features of these two parts and horizontal and vertical sections patients are summarized in Table 1, Figure 1 and 2. were stained with haematoxylin and eosin. Direct immunofluorescence (DIF) was performed in 16 cases. About 58% of PCA patients had earlier been Alcian blue staining for mucin was done in 3 cases. misdiagnosed as having conditions with non‑cicatricial hair loss. The rate of misdiagnosis was highest in Treatment regimens patients with lichen planopilaris (100%), followed by The treatment period varied from 3 to 48 months with pseudopelade of brocq and dissecting cellulitis (78%), an average of 10.3 months. Patients with lymphocytic and least in folliculitis decalvans (about 45%). The PCA were given oral methylprednisolone tablets most common misdiagnosis was alopecia areata 0.5 mg/kg for 4 weeks along with hydroxychloroquine followed by androgenic alopecia in patients with in some patients. The dose was then gradually reduced. lymphocytic cicatricial alopecia, whereas the most For patients with neutrophilic PCA, oral antimicrobial common misdiagnosis in patients with neutrophilic therapy was given based on antimicrobial sensitivity cicatricial alopecia was folliculitis. results and isotretinoin was administered to patients with dissecting cellulitis. Discoid lupus erythematosus The hair loss was most often on the parietal Statistical analysis scalp (92.3%). Most alopecia patches were round and The collected data were analyzed using SPSS atrophic, with clear boundary, scales, and hyper‑ or version 16.0. Fisher’s Chi‑square exact test and hypopigmentation. Symptoms were mild. Some patients Kruskal‑Wallis test were performed for inter‑group complained of tickling in the lesion (5 cases, 38.5%), and comparisons. Associations between sex, disease course, 3 patients (23%) had occasional knee pain. In addition onset age, the improvement in hair loss area (the to scalp lesions, four (30.8%) patients had lesions on percentage of reduced area in the original hair loss area), other parts of the body including face, trunk, and hands. treatment course and area of hair loss were analyzed ANA was positive in 4 (30.8%) cases [Figure 1a]. Table 1: Summary of clinical features among the PCA cases Disease Cases, Biopsy cases, Sex (%) Age (year) Course of disease n=59 (%) n=38 (%) (month) Male, n=31 Female, n=23 Mean Range Mean Range DLE 13 (22.0) 12 (31.6) 2 (5.6) 11 (47.8) 35.2 19-74 37.6 3-120 LPP 2 (3.4) 2 (5.3) 1 (2.8) 1 (4.3) 58 57-59 20 4-36 PB 9 (15.3) 9 (23.7) 5 (13.9) 4 (17.4) 32.2 25-46 17.3 2-84 AM 2 (3.4) 2 (5.3) 2 (5.6) 0 (0.0) 38.5 35-42 42 24-60 FD 24 (40.7) 10 (26.3) 18 (50.0) 6 (26.1) 24 5-66 16.4 1-60 DC 9 (15.3) 3 (7.9) 8 (22.2) 1 (4.3) 27.4 12-42 17.6 3-36 DLE: Discoid lupus erythematosus, LPP: Lichen planopilaris, PB: Pseudopelade of brocq, AM: Alopecia mucinosa, FD: Folliculitis decalvans, DC: Dissecting cellulitis Indian Journal of Dermatology, Venereology, and Leprology | July-August 2014 | Vol 80 | Issue 4 307 Qi, et al. Primary cicatricial alopecia a b c d e f g h i Figure 1: Global view (a-c), dermoscopy (d-f) and pathological features (g-i) of discoid lupus erythematosus, lichen planopilaris, classic pseudopelade of brocq Lichen planopilaris inflammatory papules, abscesses, and cysts; pruritus All involved sites of hair loss were located on the or pain were the main symptoms. Incision was vertex. Erythema with pigmentation, erythema, necessary to drain pus in all patients. Four patients follicular hyperkeratosis and scattered hair were found had concomitant acne. [Figures 2c]. in the lesion. No lesions were found in the mouth or on other body sites [Figure 1b]. Bacterial culture results Bacterial culture yielded positive results in Pseudopelade of Brocq 6 cases (25%) of folliculitis decalvans and 1 case (11.1%) Areas of alopecia were multifocal, asymptomatic, with of dissecting cellulitis. Staphylococcus aureus, depigmentation or hypopigmentation and no obvious Staphylococcus epidermidis and other bacteria were erythema or papules. One case also suffered from isolated. All bacteria tested were sensitive to multiple androgenetic alopecia (AGA) [Figure 1c]. antibiotics. Microorganisms were not detected in the other cases after