Differences in Clinical Responses to Ustekinumab Treatment among Body Regions: Results from a Real-World Prospective, Observational, and Multi-Center Study in Korea

Background@#In psoriasis treatment, not all body regions improve simultaneously after clinical interventions. @*Objective@#This study was aimed at evaluating clinical responses across body regions, which may differentially influence patient treatment plans. @*Methods@#This prospective, observational, and multi-center study was conducted in Koreans who adhered to ustekinumab treatment based on criteria per local label and reimbursement guidelines. A total of 581 were included in this analysis. @*Results@#The mean (±standard deviation) psoriasis area severity index (PASI) score at baseline, age, disease duration, and body surface area (%) were 18.9±9.69, 44.2±13.29 years, 11.3±9.65 years, and 27.8±17.83, respectively. Across the head and neck, upper extremities, trunk, and lower extremities, the correlation between the PASI sub-scores for the upper and lower extremities was the highest (r=0.680). The mean PASI sub-score for the lower extremities was the highest at baseline. PASI90 and PASI100 scores were the highest for the head and neck region, indicating the highest response rates, while those for the lower extremities were consistently low at all visits. @*Conclusion@#We found differences in regional ustekinumab responses, with the lower extremities being the most difficult to treat. These findings should be considered in psoriasis treatment.

Efficacy and Safety of Oral Alitretinoin in Various Refractory Dermatologic Diseases: A Retrospective Study / 대한피부과학회지

Background@#Patients with dermatologic diseases refractory to conventional treatments including corticosteroids, immunosuppressants, and phototherapy require new treatment options. Limited studies have investigated the efficacy of alitretinoin for various diseases. @*Objective@#This study aimed to evaluate the efficacy and safety of oral alitretinoin for various refractory dermatologic diseases. @*Methods@#A total of 168 patients treated with oral alitretinoin for various dermatologic diseases such as psoriasis, contact dermatitis, hand eczema, and pityriasis rubra pilaris were reviewed. Treatment response was measured according to the Physician’s Global Assessment scale and adverse events (AEs) based on medical records. @*Results@#Patients with a mean age of 46.6±14.7 years were treated with oral alitretinoin for 26.1±27.6 weeks. The overall response rate (very good or excellent) was 49.5%. The response rate was the highest (74.4%) in the eczema disease group. Overall, 80 patients (74.8%) experienced AEs, and headache (46.7%) was the most common AE, followed by decreased serum free T4 (16.8%) and elevated triglycerides (12.1%). Most AEs were tolerable, except for one case of benign intracranial hypertension. @*Conclusion@#Despite its limited approval for severe chronic hand eczema, oral alitretinoin may be a relatively safe and effective option for various refractory dermatologic diseases.

Syringocystadenocarcinoma Papilliferum in Situ Secondary to Scalp Nevus Sebaceus / 대한피부과학회지

Syringocystadenocarcinoma papilliferum in situ is extremely rare. A 51-year-old female presented with a solitary yellowish patch on the scalp, accompanied by hair loss and a protruding mass. Histopathological evaluation revealed a large number of sebaceous glands and mild papillomatosis without hair follicles. The tumor contained many cystic spaces filled with amorphous material and a connection to the epidermis. The tumor cells showed papillomatous projections and were multilayered with nuclear atypia but no dermal involvement or lymphovascular invasion. On immunohistochemical analysis, the tumor cells showed immunopositivity for carcinoembryonic antigen, Ki-67, epithelial membrane antigen, and gross cystic disease fluid protein-15. Therefore, the patient was diagnosed with syringocystadenocarcinoma papilliferum in situ originating from a nevus sebaceus. The tumor was removed by wide excision, and no recurrence was observed. Our case report supports the multistep pathogenesis of syringocystadenocarcinoma papilliferum originating from a nevus sebaceus.

Syringocystadenocarcinoma Papilliferum in Situ Secondary to Scalp Nevus Sebaceus / 대한피부과학회지

Syringocystadenocarcinoma papilliferum in situ is extremely rare. A 51-year-old female presented with a solitary yellowish patch on the scalp, accompanied by hair loss and a protruding mass. Histopathological evaluation revealed a large number of sebaceous glands and mild papillomatosis without hair follicles. The tumor contained many cystic spaces filled with amorphous material and a connection to the epidermis. The tumor cells showed papillomatous projections and were multilayered with nuclear atypia but no dermal involvement or lymphovascular invasion. On immunohistochemical analysis, the tumor cells showed immunopositivity for carcinoembryonic antigen, Ki-67, epithelial membrane antigen, and gross cystic disease fluid protein-15. Therefore, the patient was diagnosed with syringocystadenocarcinoma papilliferum in situ originating from a nevus sebaceus. The tumor was removed by wide excision, and no recurrence was observed. Our case report supports the multistep pathogenesis of syringocystadenocarcinoma papilliferum originating from a nevus sebaceus.

Clinicopathological Characteristics of H-Zone and Non-H-Zone Basal Cell Carcinoma in the Head and Neck Region / 대한피부과학회지

Background@#Basal cell carcinoma (BCC) is the most common type of skin cancer. However, limited comparative data on the characteristics and prognosis of BCC in the H-zone and non-H-zone exist. @*Objective@#We aimed to compare the clinical, histopathological, and surgical characteristics of BCCs in the head and neck region between the H- and non-H-zones. @*Methods@#We retrospectively reviewed the clinical and histopathological characteristics of 292 head and neck BCC lesions in 275 patients and the characteristics of Mohs micrographic surgery (MMS) of 252 BCC lesions in 239 patients. @*Results@#In the H-zone, 226 lesions (77.4%) were found. Clinically, patients with H-zone BCC, compared to those with non-H-zone, were significantly older (71.6±11.4 years vs. 64.6±13.5 years) and smaller in diameter (10.1±7.1 mm vs. 12.4±9.9 mm). The noduloulcerative type was more frequent in the H-zone, while the superficial and morpheaform types in the non-H-zone. Histopathologically, the infiltrative subtype was particularly noted in the H-zone than the non-H-zone (7.5% vs. 1.5%). Out of 37 BCC patients with prior treatment history, 31 (83.8%) were in the H-zone. Two out of 252 lesions (0.8%) recurred after MMS at our institution. The mean stages of MMS were comparable between the H- and non-H-zones (1.59 vs. 1.45, p=0.135). @*Conclusion@#H-zone BCCs were associated with older age and smaller tumor size compared to the non-H-zone BCCs. The noduloulcerative clinical and nodulocystic pathologic subtypes were the most common in both H-zone and non-H-zone BCCs.

The KAAACI/KDA Evidence-Based Practice Guidelines for Chronic Spontaneous Urticaria in Korean Adults and Children: Part 1. Definition, Methodology and First-line Management

Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.

The KAAACI/KDA Evidence-Based Practice Guidelines for Chronic Spontaneous Urticaria in Korean Adults and Children: Part 1. Definition, Methodology and First-line Management

Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.

A Case of Angiomatoid Spitz Nevus with High Cellularity and Lymphovascular Tumor Emboli-like Features / 대한피부과학회지

A Spitz nevus is an uncommon benign tumor that often occurs in children. Angiomatoid Spitz nevus is a rare and distinct variant of the Spitz nevus that exhibits a spindle and/or epithelioid nevus between angioma-like, densely arranged small blood vessels. We present a case of angiomatoid Spitz nevus with high cellularity and lymphovascular tumor emboli-like features. In our case, tumor cells with high cellularity were observed throughout the lesion, and lymphovascular tumor emboli-like nests were present.

A Typical Case of Mycoplasma pneumoniae-induced Rash and Mucositis Confused with Stevens-Johnson Syndrome / 대한피부과학회지

No abstract available.

Clinco-pathologic Features of Alopecia in a Patient of Cronkhite-Canada Syndrome / 대한피부과학회지

Although hair loss in Cronkhite-Canada syndrome (CCS) developed frequently, its exact mechanism has not been elucidated. Thus, we attempted to investigate the histopathologic features of hair loss with scalp biopsy in one case of CSS. The patient complained of persistent diarrhea and diffuse hair loss of the scalp 1 month prior to the hospital visit. CCS has been already diagnosed through colonoscopy and medical examination. Scalp biopsy was performed to evaluate hair loss of the patient, and pathologic examination showed increased telogen hairs (anagen to telogen is 7:8) without specific dermal inflammation and miniaturization of hair follicles. The patient was treated with topical and systemic steroids, and hair loss has almost completely recovered in 5 months. In this case, we investigated the clinical and pathological features of hair loss through scalp biopsy in one case of CCS and reported them with a review of the literature.

Shiitake Dermatitis due to Song-hwa Mushroom (Lentinula edodes var.) / 대한피부과학회지

No abstract available.

A Case of SAPHO Syndrome Associated with Lytic Bone Lesions Resembling Metastases / 대한피부과학회지

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.

Treatment of Hair Loss in the Trichorhinophalangeal Syndrome

No abstract available.

The Clinical Profile of Patients with Psoriasis in Korea: A Nationwide Cross-Sectional Study (EPI-PSODE)

BACKGROUND: Psoriasis is an immune-mediated, chronic inflammatory disease affecting multiple aspects of patients' lives. Its epidemiology varies regionally; however, nationwide epidemiologic data on psoriasis depicting profile of Korean patients has not been available to date. OBJECTIVE: To understand nationwide epidemiologic characteristics and clinical features of adult patients with psoriasis visited university hospitals in Korea. METHODS: This multicenter, non-interventional, cross-sectional study recruited 1,278 adult patients with psoriasis across 25 centers in Korea in 2013. Various clinical data including PASI, BSA, DLQI, SF-36 and PASE were collected. RESULTS: A total of 1,260 patients completed the study (male:female=1.47:1). The mean age was 47.0 years with a distribution mostly in the 50s (24.9%). Early onset (<40 years) of psoriasis accounted for 53.9% of patients. The mean disease duration was 109.2 months; mean body mass index was 23.9 kg/m²; and 12.7% of patients had a family history of psoriasis. Plaque and guttate types of psoriasis accounted for 85.8% and 8.4%, respectively. Patients with PASI ≥10 accounted for 24.9%; patients with body surface area ≥10 were 45.9%. Patients with DLQI ≥6 accounted for 78.8%. Between PASI <10 and PASI ≥10 groups, significant difference was noted in age at diagnosis, disease duration, blood pressure, waist circumference of female, and treatment experiences with phototherapy, systemic agents, and biologics. CONCLUSION: This was the first nationwide epidemiologic study of patients with psoriasis in Korea and provides an overview of the epidemiologic characteristics and clinical profiles of this patient population.

Primary Extramammary Paget's Disease with Lymphatic Invasion Confirmed by D2-40 Immunostain / 대한피부과학회지

No abstract available.

Secondary Cutaneous Amyloidosis in a Patient with Mycosis Fungoides

Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Finally, she reached complete remission of mycosis fungoides. However, new yellowish lesions started to appear 1 year after discontinuing the phototherapy. A physical examination revealed multiple yellowish plaques on both extremities. The plaques were well circumscribed and slightly elevated. All laboratory tests were normal. A biopsy specimen showed multiple nodular deposits of eosinophilic amorphous material in papillary dermis and upper reticular dermis. The deposits represented apple green birefringence on Congo red stain viewed under polarized light. Acellular small nodules in the upper dermis consisted of randomly oriented, non-branching, 6.67~12.7 nm thick amyloid fibrils on electron microscopy. We report an interesting and rare case of secondary cutaneous amyloidosis after narrow-band UVB therapy and PUVA therapy in a patient with mycosis fungoides.

Secondary Cutaneous Amyloidosis in a Patient with Mycosis Fungoides

Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Finally, she reached complete remission of mycosis fungoides. However, new yellowish lesions started to appear 1 year after discontinuing the phototherapy. A physical examination revealed multiple yellowish plaques on both extremities. The plaques were well circumscribed and slightly elevated. All laboratory tests were normal. A biopsy specimen showed multiple nodular deposits of eosinophilic amorphous material in papillary dermis and upper reticular dermis. The deposits represented apple green birefringence on Congo red stain viewed under polarized light. Acellular small nodules in the upper dermis consisted of randomly oriented, non-branching, 6.67~12.7 nm thick amyloid fibrils on electron microscopy. We report an interesting and rare case of secondary cutaneous amyloidosis after narrow-band UVB therapy and PUVA therapy in a patient with mycosis fungoides.

Multiple Skin Colored Nodules on both Legs in Patient with Positive QuantiFERON®-TB Gold Test

Nodular tuberculid (NT) was originally described by Jordaan et al. in 2000 in 4 patients from South Africa. It appeared as nodules on the legs; the pathologic changes were situated in the deep dermis and adjacent subcutaneous fat. A 34-year-old woman visited our hospital with subcutaneous skin-colored or slightly erythematous round to oval nodules. Skin biopsies revealed granulomatous inflammation at the dermo-subcutaneous junction with vasculitis. Chest X-ray, tuberculosus (TB)-polymerase chain reaction and TB culture of the skin specimen were normal. A QuantiFERON®-TB Gold test (QUIAGEN, Germany) was positive, which suggested a diagnosis of latent TB infection. The patient was treated with anti-TB medication and her condition has not recurred. Herein, we report a case of a patient with latent TB diagnosed by a positive QuantiFERON®-TB Gold test whose skin lesions had the clinical and histopathologic features of NT.

Multiple Skin Colored Nodules on both Legs in Patient with Positive QuantiFERON®-TB Gold Test

Nodular tuberculid (NT) was originally described by Jordaan et al. in 2000 in 4 patients from South Africa. It appeared as nodules on the legs; the pathologic changes were situated in the deep dermis and adjacent subcutaneous fat. A 34-year-old woman visited our hospital with subcutaneous skin-colored or slightly erythematous round to oval nodules. Skin biopsies revealed granulomatous inflammation at the dermo-subcutaneous junction with vasculitis. Chest X-ray, tuberculosus (TB)-polymerase chain reaction and TB culture of the skin specimen were normal. A QuantiFERON®-TB Gold test (QUIAGEN, Germany) was positive, which suggested a diagnosis of latent TB infection. The patient was treated with anti-TB medication and her condition has not recurred. Herein, we report a case of a patient with latent TB diagnosed by a positive QuantiFERON®-TB Gold test whose skin lesions had the clinical and histopathologic features of NT.

Pili Annulati with Multiple Fragile Hairs

No abstract available.