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Background@#Primary cicatricial alopecia (PCA) is a rare disease that causes irreversible destruction of hair follicles and affects the quality of life (QOL). @*Objective@#We aimed to investigate the disease awareness, medical use behavior, QOL, and real-world diagnosis and treatment status of patients with PCA. @*Methods@#A self-administered questionnaire was administered to patients with PCA and their dermatologists. Patients aged between 19 and 75 years who visited one of 27 dermatology departments between September 2021 and September 2022 were included. @*Results@#In total, 274 patients were included. The male-to-female ratio was 1:1.47, with a mean age of 45.7 years. Patients with neutrophilic and mixed PCA were predominantly male and younger than those with lymphocytic PCA. Among patients with lymphocytic PCA, lichen planopilaris was the most common type, and among those with neutrophilic PCA, folliculitis decalvans was the most common type. Among the total patients, 28.8% were previously diagnosed with PCA, 47.0% were diagnosed with PCA at least 6 months after their first hospital visit, 20.0% received early treatment within 3 months of disease onset, and 54.4% received steady treatment. More than half of the patients had a moderate to severe impairment in QOL. Topical/intralesional steroid injections were the most common treatment. Systemic immunosuppressants were frequently prescribed to patients with lymphocytic PCA, and antibiotics were mostly prescribed to patients with neutrophilic PCA. @*Conclusion@#This study provides information on the disease awareness, medical use behavior, QOL, diagnosis, and treatment status of Korean patients with PCA. This can help dermatologists educate patients with PCA to understand the necessity for early diagnosis and steady treatment.
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Background@#Alopecia areata (AA) is a chronic disease with an unpredictable course and can have a severe psychological impact on an individual. @*Objective@#To provide evidence and consensus-based statements regarding the treatment of patients with AA in Korea. @*Methods@#We searched for relevant studies from inception to May 2021 regarding the systemic treatment of AA. Evidence-based recommendations were also prepared. The evidence for each statement was graded and classified according to the strength of the recommendations. Hair experts from the Korean Hair Research Society (KHRS) voted on the statement, and an agreement of 75% or greater was considered as having reached consensus. @*Results@#Current evidence supports the efficacy of systemic corticosteroids, oral cyclosporine monotherapy or combination with systemic corticosteroids, and oral Janus kinase inhibitors in severe AA patients. Systemic steroids may be considered for pediatric patients with severe AA. A consensus was achieved in three out of nine (33.3%), and one out of three (33.3%) statements pertaining to systemic treatment in adult and pediatric AA, respectively. @*Conclusion@#The present study produced up-to-date, evidence-based treatment guidelines for AA associated with the consensus obtained by experts based on the Korean healthcare system.
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Background@#Alopecia areata (AA) is a chronic disease with an unpredictable disease course and severe psychological impact. @*Objective@#To provide evidence- and consensus-based insights regarding the treatment of patients with AA in Korea. @*Methods@#We searched for relevant studies on the topical and device-based treatment of AA in the literature from inception until May 2021. Evidence-based recommendations were also prepared. The evidence for each statement was graded and classified according to the strength of the recommendations. Hair experts from the Korean Hair Research Society (KHRS) voted on the statements, and an agreement of 75% or greater was considered as consensus. @*Results@#Currently, there remains a scarcity of topical treatments, which is supported by robust evidence from a number of high-quality randomized controlled trials. Current evidence supports the efficacy of topical corticosteroids, corticosteroid intralesional injection, and contact immunotherapy in AA patients. Topical corticosteroids and contact immunotherapy are recommended for pediatric AA. A consensus was achieved in 6 out of 14 (42.8%), and 1 out of 5 (20.0%) statements pertaining to topical and device-based treatments in AA, respectively. The expert consensus was from a single country, and the study may not cover all the treatments used. @*Conclusion@#The present study provides up-to-date, evidence-based treatment guidelines for AA based on the consensus reached among experts after considering regional healthcare circumstances, adding diversity to the previous guidelines.
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Background@#Injectable soft tissue fillers are important elements in facial rejuvenation as they provide volume restoration without significant inconvenience to the patient or substantial associated recovery time. Complications can be classified into immediate, delayed, or late adverse reactions. Most complications are temporary and common throughout the filler classes. @*Objective@#To describe the long-term side effects of fillers. @*Methods@#A retrospective study of 10 patients who experienced long-term side effects of soft tissue filler injections between 2007 and 2018 was conducted. A long-term reaction was defined as a complication that occurred 1 year after soft tissue filler injection at any facial site. @*Results@#Ten patients were included in the study. All the patients visited our department because of a palpable subcutaneous nodular lesion on their face. The mean duration from receiving the filler injection to the appearance of side effects was 4.3 years (range, 1∼12 years). Based on their clinicopathological features, complications were roughly classified into granulomatous inflammation (60.0%), non-inflammatory palpable nodule formation (20.0%), abscess (10.0%), and dermal fibrosis with inflammation (10.0%). @*Conclusion@#Regardless of the filler type, side effects can appear up to 12 years after injection. The most common type is a granulomatous lesion; however, it can appear as a non-granulomatous lesion. Therefore, when a patient visits with a nodule or an edematous lesion without any recall reason, careful history taking is needed to find any associated clues. With close follow-up and appropriate treatment, complications associated with injectable soft tissue fillers can be limited and competently managed.
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Bronchogenic cysts develop from tracheal diverticula or abnormal budding of the anterior foregut during embryological development. The most common extrapulmonary site of such cysts is the mediastinum; however, remote locations such as the lingual, intra-abdominal, and cutaneous regions have also been reported. Moreover, the postauricular location is an uncommon site for this entity. An 11-year-old boy visited our hospital with a long-standing mass in the postauricular area. Ultrasonography revealed a well-circumscribed anechoic nodule measuring 1.02×1.03 cm in size with posterior enhancement. The lesion was then completely excised. Pathological examination revealed a cystic lesion lined with ciliated pseudostratified columnar epithelium, consistent with a bronchogenic cyst. The patient had no local recurrence at 6th month follow-up. Herein, we report the first case of a bronchogenic cyst that developed in the postauricular area, and provide a review of the literature on cutaneous bronchogenic cysts.
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Primary cutaneous marginal zone B-cell lymphomas (PCMZLs) are classified as lowgrade as they run an indolent course. They are histologically characterized through nonepidermotropic nodular or diffuse infiltrates consisting of small or medium heterogeneous atypical lymphoid cells. In the past few years, chemotherapy has increased the survival rate of breast cancer patients. However, the adverse effects of treatment, such as leukemia, have also been shown to emerge gradually. Additionally, cases of occurrence of non-Hodgkin lymphoma (NHL) post chemotherapy have also been reported. A 48-year-old female patient was presented with a violaceous nodule on her left thigh. Around 15 months ago, she completed breast cancer chemotherapy. Eight months later, a skin lesion appeared. Histological findings revealed dense and nodular lymphocyte infiltration. Immunohistochemical staining was positive for CD20 and BCL2. Clinical and histological examination of the lesions confirmed PCMZL. After systemic evaluation, lymphoma was found to be limited to the skin, and thus, she underwent complete excision of the lesion. At the first month followup, there was a recurrent lesion on the right wrist, which was excised successfully. However, recurrences occurred again in the calf and forearm in the following five and two months, respectively. These lesions were also confirmed with PCMZL using biopsy. We assume that this case is related to chemotherapy as it was presented and recurred abruptly post chemotherapy. Additionally, there are several reported cases of NHL post breast cancer chemotherapy. However, this is the first case report of PCMZL associated with chemotherapy.
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Background@#Pediatric alopecia areata (AA) can affect the quality of life (QoL) of patients and their family members. Research on the QoL and burden on family members in pediatric AA is limited. @*Objective@#This nationwide multicenter questionnaire study described the QoL and burden of the family members of patients with pediatric AA. @*Methods@#This nationwide multicenter questionnaire study enrolled AA patients between the ages of 5 and 18 years from March 1, 2017 to February 28, 2018. Enrolled patients and their parents completed the modified Children’s Dermatology Life Quality Index (CDLQI) and the modified Dermatitis Family Impact (mDFI). The disease severity was measured using the Severity of Alopecia Tool (SALT) survey scores. @*Results@#A total of 268 patients with AA from 22 hospitals participated in this study. Our study found that the efficacy and satisfaction of previous treatments of AA decreased as the severity of the disease increased. The use of home-based therapies and traditional medicines increased with the increasing severity of the disease, but the efficacy felt by patients was limited. CDLQI and mDFI scores were higher in patients with extensive AA than those with mild to moderate AA. The economic and time burden of the family members also increased as the severity of the disease increased. @*Conclusion@#The severity of the AA is indirectly proportional to the QoL of patients and their family members and directly proportional to the burden. Physicians need to understand these characteristics of pediatric AA and provide appropriate intervention to patients and their family members.
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Background@#Lipomas are the most common benign tumors and surgical removal is the treatment of choice. However, some deep-seated lipomas are technically challenging to surgically excise from a dermatologist’s perspective. This study was planned to help predict the depth of lipomas depending on their anatomical site of occurrence. @*Objective@#To determine whether there is a relationship between the anatomical site and the depth of lipomas. @*Methods@#We retrospectively reviewed the medical records of 459 patients with 459 lesions diagnosed as lipoma, surgically treated between June 2006 and June 2019. Histopathologic evaluation was performed to determine the relationship between the depth and the specific subtype of the lipoma. @*Results@#The most common site of occurrence was the trunk (177; 38.6%). Most of the lipomas (388; 84.5%) were located in the subcutaneous fat layer. Some lipomas (71; 15.5%) were found in deeper tissues, such as the intramuscular, intermuscular, and submuscular layers. The forehead and flank had a higher incidence of deep-seated lipomas than other areas. @*Conclusion@#Our results revealed that lipomas in the forehead and flank are more likely to occur in deeper layers. In clinical practice, physicians often skip radiologic evaluation before surgery. However, we recommend preoperative radiologic evaluation before surgical excision of lipomas of the forehead and flank.
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Background@#In 2015, the Korean Atopic Dermatitis Association (KADA) working group published consensus guidelines for treating atopic dermatitis (AD). @*Objective@#We aimed to provide updated consensus recommendations for systemic treatment of AD in South Korea based on recent evidence and experience. @*Methods@#We compiled a database of references from relevant systematic reviews and guidelines on the systemic management of AD. Evidence for each statement was graded and classified based on thestrength of the recommendation. Forty-two council members from the KADA participated in three rounds of voting to establish a consensus on expert recommendations. @*Results@#We do not recommend long-term treatment with systemic steroids forpatients with moderate-to-severe AD due to the risk of adverse effects. We recommend treatment with cyclosporine or dupilumab and selective treatment with methotrexate or azathioprine for patients with moderate-to-severe AD. We suggest treatment with antihistamines as an option for alleviating clinical symptoms of AD. We recommend selective treatment with narrowband ultraviolet B for patients with chronic moderate-to-severe AD. We do not recommend treatment with oral antibiotics for patients with moderate-to-severe AD but who have no signs of infection. We did not reach a consensus on recommendations for treatment with allergen-specific immunotherapy, probiotics, evening primrose oil, orvitamin D for patients with moderate-to-severe AD. We also recommend educational interventions and counselling for patients with AD and caregivers to improve the treatment success rate. @*Conclusion@#We look forward to implementing a new and updated consensus of systemic therapy in controlling patients with moderate-to-severe AD.
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Background@#Trichotillomania (TTM) is characterized by recurrent hair pulling, resulting in hair loss. TTM is typically encountered in dermatology settings; however, few reports on the clinical characteristics of TTM in a large population are available. @*Objective@#To evaluate the clinical characteristics of TTM. @*Methods@#We performed a retrospective chart review of patients diagnosed with TTM between 2006 and 2019. @*Results@#Clinical records of 198 patients were analyzed. The average age of TTM onset was 10.8 years, and 119 patients experienced childhood-onset (at ＜12 years). Sex prevalence showed a female predominance, and the proportion of females was lower in the childhood-onset TTM than in the late-onset TTM (onset at ≥12 years). Vertex was the most commonly involved site. A history of stressful situations was noted in 48 patients (24.2%). In the group with a poor prognosis, the number of patients with ≥25% scalp invasion was significantly more than the other group. The average duration of hair loss to more than 50% recovery was 4.88 months for childhood-onset TTM cases and 9.83 months for late-onset TTM cases. The prognosis was significantly better in the childhood-onset TTM than in the late-onset TTM. Nail biting and folliculitis were the most common co-occurring repetitive behavior and comorbid disease, respectively. @*Conclusion@#Unlike previous domestic studies, patients with childhood-onset TTM had a female preponderance. Moreover, the proportion of patients who experienced a stressful situation was higher than that reported in a previous study. In addition, patients with severe disease and late-onset TTM had a significantly poorer prognosis.
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Many cutaneous adverse reactions including eczematoid dermatitis and lichenoid eruption have been reported with the increased usage of tumor necrosis factor-α inhibitors. However, there are very few reports of generalized pustular eruptions with adalimumab administration. A 44-year-old woman visited our clinic with multiple skin lesions scattered across her body. The patient developed these lesions three days after the initiation of adalimumab treatment. At the time of the patient’s visit, physical examination revealed multiple erythematous papules and pustules across the body. Histopathological findings revealed subcorneal neutrophilic microabscess formation with dense perivascular and perifollicular infiltration that was mainly composed of lymphocytes and neutrophils. We diagnosed the patient with cutaneous pustular eruptions due to adalimumab exposure and initiated treatment with prednisolone and discontinued adalimumab injections. After 1 month of treatment, the patient’s skin lesions improved. Herein, we report a case of generalized pustular eruptions, a rare cutaneous adverse reaction to adalimumab.
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Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. We report a case of MF patient who showed atypical histological findings. A 76 year-old Asian male patient visited our dermatology clinic with multiple skin lesions on the trunk since 2 years ago. Histopathological findings showed hyperchromatic enlarged atypical lymphocytes perivascular infiltration. Immuno-phenotyping showed numerous dermal lymphocytes with positive for CD3, 4, 8. In T-cell receptor gene rearrangement study, which revealed monoclonality. Patient was finally diagnosed with MF by confirming the monoclonality of the TCR gene rearrangement. The patient was treated with MTX, NBUVB therapy. Skin lesions were mostly improved and patient remained in good condition for 15 months without recurrence. Diagnosis of MF in early stages is important. Clinically, when encountering refractory erythematous plaques with peripheral rims, dermatologist should consider the possibility of MF and histologic remission of epidermis due to previous treatments.
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Background@#Acute diffuse and total alopecia (ADTA) is characterized by rapid hair loss and rapid recovery without treatment. If a non-invasive method such as dermoscopy can identify ADTA, clinicians will be able to diagnose ADTA faster and reassure patients. @*Objective@#The purpose of this study was to evaluate the characteristics of ADTA using dermoscopy. @*Methods@#Patients with ADTA diagnosed by clinical and histological findings from October 2016 to November 2019 were examined by dermoscopy during their first visit to the Department of Dermatology at Kyung Hee University Hospital at Gangdong. @*Results@#Among 300 lesions observed via dermoscopy, yellow dots or black dots were observed in 225 (75.0%) and 240 (80.0%) lesions, respectively. Short vellus hairs or broken hairs were seen in 231 (77.0%) and 190 (63.3%) lesions, respectively. Exclamation mark hairs appeared in only 71 lesions (23.7%). @*Conclusion@#In this study, yellow dots, black dots, broken hairs, short vellus hairs, and exclamation mark hairs were observed in the ADTA cases as well as in previously reported Alopecia areata (AA) studies. These results could be due to the characteristic rapid disease progression of ADTA. Exclamation mark hairs are known to take a few weeks that the transformation of an anagen hair bulb to a club shape of exclamation mark hairs of alopecia areata.Therefore, exclamation mark hairs might be less commonly found at the very early stage of ADTA. There are some limitations in this study. The number of subjects was small, and we did not set up a control group for comparison.
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Nivolumab (anti-PD-1) currently used in many cancers. With the usage of nivolumab increased, many cutaneous side effects were reported including maculopapular rash, lichenoid reactions, vitiligo, bullous disorders, psoriasis exacerbation, and alopecia areata (AA). Here, we report AA after nivolumab for treatment of hepatocellular carcinomas (HCC). A 55-year-old male presented with multiple hairless patch from 1 month ago. He suffered HCC and treated with nivolumab for 6 months after hepatectomy. He treated for hair loss with triamcinolone intra-lesional injection without improvement.We performed skin biopsy on the scalp. Histopathologic findings revealed decreased of hair follicles on the horizontal section with lymphocyte infiltration on the perifollicular area on the vertical section. Clinicopathologic findings were agreed with AA. Considering lack of previous history of AA and hairless patches with 6 months after nivolumab injection, we diagnosed him as nivolumab induced AA. Treatment included topical steroid, and minoxidil. No regrowth of hair was noted after 4 months of follow-up. Nivolimumab induced AA is rare side effect. Pathogenesis of nivolumab induced AA remain unclear. But our case is likely related to nivolumab, known to induce immune related adverse events, and given in the delay of a few months between introduction and the occurrence of the hair loss. Here, we reports nivolmumab induced AA; rare side effect.
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Sweet’s syndrome was first described as a reactive dermatosis characterized by sudden onset of fever, leukocytosis, and erythematous plaques infiltrated with neutrophils.Therefore, Sweet’s syndrome is also known as acute febrile neutrophilic dermatosis. However, subsequently, it became clear that fever and neutrophilia in Sweet’s syndrome vary depending on the case, and several other characteristics have been described. The lesions in Sweet’s syndrome are typically observed not only in the limbs but also in the face, neck, and upper trunk. A 28-year-old female without a specific medical history presented in a hospital following the complaint of painful erythematous patches and pustules on her palms and soles. She had no previous history of palmoplantar pustulosis and other infections or malignancies. A skin biopsy showed diffuse dermal infiltration of neutrophils.Laboratory tests showed increased neutrophil count and erythrocyte sedimentation rate. After systemic corticosteroid administration was initiated, the lesions gradually disappeared. The patient was subsequently diagnosed with Sweet’s syndrome according to histology, clinical feature, and response to treatment. However, there have been few reports of Sweet’s syndrome confined to the individuals’ palms and soles. According to the literatures, although the dorsum of the hand is frequently affected, the palmoplantar involvement as in our case appears to be rare.
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Nivolumab (anti-PD-1) currently used in many cancers. With the usage of nivolumab increased, many cutaneous side effects were reported including maculopapular rash, lichenoid reactions, vitiligo, bullous disorders, psoriasis exacerbation, and alopecia areata (AA). Here, we report AA after nivolumab for treatment of hepatocellular carcinomas (HCC). A 55-year-old male presented with multiple hairless patch from 1 month ago. He suffered HCC and treated with nivolumab for 6 months after hepatectomy. He treated for hair loss with triamcinolone intra-lesional injection without improvement.We performed skin biopsy on the scalp. Histopathologic findings revealed decreased of hair follicles on the horizontal section with lymphocyte infiltration on the perifollicular area on the vertical section. Clinicopathologic findings were agreed with AA. Considering lack of previous history of AA and hairless patches with 6 months after nivolumab injection, we diagnosed him as nivolumab induced AA. Treatment included topical steroid, and minoxidil. No regrowth of hair was noted after 4 months of follow-up. Nivolimumab induced AA is rare side effect. Pathogenesis of nivolumab induced AA remain unclear. But our case is likely related to nivolumab, known to induce immune related adverse events, and given in the delay of a few months between introduction and the occurrence of the hair loss. Here, we reports nivolmumab induced AA; rare side effect.
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Sweet’s syndrome was first described as a reactive dermatosis characterized by sudden onset of fever, leukocytosis, and erythematous plaques infiltrated with neutrophils.Therefore, Sweet’s syndrome is also known as acute febrile neutrophilic dermatosis. However, subsequently, it became clear that fever and neutrophilia in Sweet’s syndrome vary depending on the case, and several other characteristics have been described. The lesions in Sweet’s syndrome are typically observed not only in the limbs but also in the face, neck, and upper trunk. A 28-year-old female without a specific medical history presented in a hospital following the complaint of painful erythematous patches and pustules on her palms and soles. She had no previous history of palmoplantar pustulosis and other infections or malignancies. A skin biopsy showed diffuse dermal infiltration of neutrophils.Laboratory tests showed increased neutrophil count and erythrocyte sedimentation rate. After systemic corticosteroid administration was initiated, the lesions gradually disappeared. The patient was subsequently diagnosed with Sweet’s syndrome according to histology, clinical feature, and response to treatment. However, there have been few reports of Sweet’s syndrome confined to the individuals’ palms and soles. According to the literatures, although the dorsum of the hand is frequently affected, the palmoplantar involvement as in our case appears to be rare.
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Many cutaneous adverse reactions including eczematoid dermatitis and lichenoid eruption have been reported with the increased usage of tumor necrosis factor-α inhibitors. However, there are very few reports of generalized pustular eruptions with adalimumab administration. A 44-year-old woman visited our clinic with multiple skin lesions scattered across her body. The patient developed these lesions three days after the initiation of adalimumab treatment. At the time of the patient’s visit, physical examination revealed multiple erythematous papules and pustules across the body. Histopathological findings revealed subcorneal neutrophilic microabscess formation with dense perivascular and perifollicular infiltration that was mainly composed of lymphocytes and neutrophils. We diagnosed the patient with cutaneous pustular eruptions due to adalimumab exposure and initiated treatment with prednisolone and discontinued adalimumab injections. After 1 month of treatment, the patient’s skin lesions improved. Herein, we report a case of generalized pustular eruptions, a rare cutaneous adverse reaction to adalimumab.
RESUMEN
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. We report a case of MF patient who showed atypical histological findings. A 76 year-old Asian male patient visited our dermatology clinic with multiple skin lesions on the trunk since 2 years ago. Histopathological findings showed hyperchromatic enlarged atypical lymphocytes perivascular infiltration. Immuno-phenotyping showed numerous dermal lymphocytes with positive for CD3, 4, 8. In T-cell receptor gene rearrangement study, which revealed monoclonality. Patient was finally diagnosed with MF by confirming the monoclonality of the TCR gene rearrangement. The patient was treated with MTX, NBUVB therapy. Skin lesions were mostly improved and patient remained in good condition for 15 months without recurrence. Diagnosis of MF in early stages is important. Clinically, when encountering refractory erythematous plaques with peripheral rims, dermatologist should consider the possibility of MF and histologic remission of epidermis due to previous treatments.
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Background@#Although alopecia areata (AA) is usually diagnosed based on the patient’s clinical manifestations, histologic features are the key to establishing the correct diagnosis. Moreover, it is possible to know about the progression of AA through understanding the various histologic characteristics of AA. Many studies have reported a lot of literatures related to AA, but studies investigating the correlation between histologic features and prognosis have not yet been reported. @*Objective@#This study aimed to establish the histologic features of AA in scalp biopsy specimens and sought to correlate between histopathologic features and clinical prognosis. @*Methods@#The pathology archives of Kyung Hee University Hospital at Gang-dong were searched for AA in the diagnostic field from the period of 2006 to 2016. The biopsy specimens were sectioned by Tyler technique. A total of 464 patients were included in the analysis. @*Results@#There were reduction of anagen hair count according to the severity of AA, and a higher percentage of telogen ratio and vellus hair counts were observed in the alopecia totalis and alopecia universalis. Total hair counts (9.9) including miniaturized hairs were decreased prominently at more than 5 years of current duration. There was a high ratio of peribulge lymphocytic infiltration (45%) in patients with ophiasis and acute diffuse type and those with total alopecia showed prominent small portions of follicular miniaturization (27%). @*Conclusion@#These histopathologic features mentioned above may shed light on the understanding of disease progression of AA, which have various clinical aspects. Using these histologic features, we will be able to predict the efficacy for therapy and prognosis of AA.