Clinical characteristics and courses of Korean patients with giant cell arteritis: a multi-center retrospective study

Objective@#Giant cell arteritis (GCA) is a large-vessel vasculitis that primarily affects elderly individuals. However, data regarding Korean patients with GCA are scarce owing to its extremely low prevalence in East Asia. This study aimed to investigate the clinical characteristics of Korean patients with GCA and their outcomes, focusing on relapse. @*Methods@#The medical records of 27 patients with GCA treated at three tertiary hospitals between 2007 and 2022 were retrospectively reviewed. @*Results@#Seventeen (63.0%) patients were females, and the median age at diagnosis was 75 years. Large vessel involvement (LVI) was detected in 12 (44.4%) patients, and polymyalgia rheumatica (PMR) was present in 14 (51.9%) patients. Twelve (44.4%) patients had fever at onset. The presence of LVI or concurrent PMR at diagnosis was associated with a longer time to normalization of the C-reactive protein level (p=0.039) or erythrocyte sedimentation rate (p=0.034). During follow-up (median: 33.8 months), four (14.8%) patients experienced relapse. Kaplan-Meier analyses showed that relapse was associated with visual loss (p=0.008) and the absence of fever (p=0.004) at onset, but not with LVI or concurrent PMR. @*Conclusion@#Concurrent PMR and LVI were observed in approximately half of Korean patients with GCA, and the elapsed time to normalization of inflammatory markers in these patients was longer. The relapse rate in Korean GCA is lower than that in Western countries, and afebrile patients or patients with vision loss at onset have a higher risk of relapse, suggesting that physicians should carefully monitor patients with these characteristics.

A Case of Overlap Syndrome of Systemic Sclerosis and Cryoglobulinemic Vasculitis With Central Nervous System Involvement

Mixed cryoglobulinemic vasculitis (CV) is occasionally caused by autoimmune diseases including systemic sclerosis. Multiorgan involvement such as skin, kidney, and peripheral nerve involvement is common in mixed CV. However, central nervous system (CNS) involvement is extremely rare. Here, we report a case of overlap syndrome of limited cutaneous systemic sclerosis and mixed cryoglobulinemic vasculitis with CNS involvement. The neurologic deficits and systemic symptoms improved promptly after steroid and cyclophosphamide therapy.

A Case of Overlap Syndrome of Systemic Sclerosis and Cryoglobulinemic Vasculitis With Central Nervous System Involvement

Mixed cryoglobulinemic vasculitis (CV) is occasionally caused by autoimmune diseases including systemic sclerosis. Multiorgan involvement such as skin, kidney, and peripheral nerve involvement is common in mixed CV. However, central nervous system (CNS) involvement is extremely rare. Here, we report a case of overlap syndrome of limited cutaneous systemic sclerosis and mixed cryoglobulinemic vasculitis with CNS involvement. The neurologic deficits and systemic symptoms improved promptly after steroid and cyclophosphamide therapy.

Neutrophilic Dermatosis of the Dorsal Hands / 대한피부과학회지

Neutrophilic dermatosis of the dorsal hands (NDDH) is a rare, localized variant of Sweet syndrome, and it was first described by Galaria et al. in 2000. It usually occurs in middle-aged women and it is characterized by erythematous papules, plaques, pustules and hemorrhagic bullae that are generally located on the dorsal hands. The histopathological findings show prominent papillary dermal edema and a dense dermal neutrophilic infiltration. A neutrophilic infiltrate can also cause a variable degree of vascular damage: endothelial cell swelling, RBC extravasation, leukocytoclasia and fibrin deposition within the walls of vessels. We report here on a case of NDDH in a 50-year-old man who was treated with oral corticosteroid and the NDDH recurred after 9 months.

Porokeratotic Eccrine Ostial and Dermal Duct Nevus Showing Partial Remission by Topical Photodynamic Therapy

Porokeratotic eccrine ostial and dermal duct nevus (PEODDN) is an uncommon, benign dermatosis and is characterized by asymptomatic grouped keratotic papules and plaques with a linear pattern on the extremities. Various treatments, including topical steroids, topical calcipotriol, topical 5-fluorouracil, retinoid, cryotherapy, and carbon dioxide laser ablation have been tried for PEODDN, but the results were unsatisfactory. Recently, topical photodynamic therapy (PDT) has been shown to be effective for various cutaneous disorders. We report a case of PEODDN showing partial remission with PDT using topical 5-aminolevulanic acid in a 4-year-old girl.

Congenital Syphilis Presenting with a Generalized Bullous and Pustular Eruption in a Premature Newborn

Congenital syphilis occurs when Treponema pallidum crosses the placenta during pregnancy or from contact with an infectious genital lesion during delivery. Cutaneous manifestations of congenital syphilis are relatively common, occurring in approximately 30% to 70% of patients. Maculopapular lesions, vesiculobullous lesions, condylomata lata lesions, annular lesions, and erythema multiforme-like targetoid lesions have been reported. We report on a premature newborn with congenital syphilis who presented with generalized bullous and pustular eruption and desquamation at birth.

Lichen Sclerosus et Atrophicus with Multiple Distribution and Hemorrhagic Change / 대한피부과학회지

Lichen sclerosus et atrophicus (LSA) is a benign, chronic inflammatory mucocutaneous disease of unknown etiology with a predilection for the female genital area, which is highly bothersome. It may also involve other areas, mainly the upper trunk, axillae and buttocks and clinicians often make a misdiagnosis of these extragenital lesions due to their rarity and lack of symptoms. We herein report the case of a 69-year-old female patient with multiple extragenital LSA presenting with hemorrhagic change. Histopathologic evaluation showed follicular plugging, atrophy of stratum malpighii with hydropic degeneration of basal cells, prominent upper dermal edema and lichenoid lymphocytic infiltration in the mid dermis which was consistent with LSA. We remind clinicians of including LSA in the differential diagnoses of multiple cutaneous hemorrhagic lesions.

Cyst-like Combined Dermatofibroma: Aneurysmal and Atypical Variants / 대한피부과학회지

Dermatofibroma is a common benign fibrohistiocytic tumor that mainly presents as a brown papule or nodule. However, it can sometimes present with diverse clinical forms such as a cyst. Combined dermatofibroma is defined as a tumor that comprises two or more variants of dermatofibroma in a single lesion. We herein report on a case of combined dermatofibroma in a 40-year-old woman who presented with a cyst form on her lateral neck. The histological findings included the combined features of the aneurysmal and atypical variants of dermatofibroma such as cleft-like spaces with hemorrhage, some pleomorphic cells and multinucleated giant cells, as well as a feature of typical dermatofibroma.

A Case of Pachydermoperiostosis / 대한피부과학회지

Pachydermoperiostosis is a very rare genodermatosis that is characterized by pachydermia, digital clubbing and periostosis. It usually occurs during adolescence and the disease is inherited in an autosomal dominant manner. Although the pathogenesis of this disease is still a matter of debate, it is associated with abnormal proliferation of fibroblasts, osteoblasts and endothelial cells and altered proteoglycan synthesis. We report here on a case of pachydermoperiostosis in a 21-year-old man who was diagnosed by the clinical features, skin biopsy and radiological examinations.

Detection of Treponema pallidum in the Skin Lesions of Primary and Secondary Syphilis: An Immunohistochemical Study / 대한피부과학회지

BACKGROUND: Syphilis is an infection caused by Treponema pallidum (T. pallidum), and this disease is increasing in incidence. However, making the diagnosis of syphilis is sometimes still challenging because of the variable clinical and histopathologic findings and there are cases with negative serologic findings, and especially in the setting of HIV infection and immunosuppressive therapy. OBJECTIVE: In this study, we specifically evaluated the number and distribution patterns of T. pallidum in the skin lesions from patients with primary or secondary syphilis. METHODS: orty eight skin biopsy specimens with the clinical and/or serological diagnosis of syphilis were evaluated by immunohistochemistry (IHC) using primary polyclonal antibodies against T. pallidum. RESULTS: Overall, T. pallidum was identified in 45 specimens (94%). The IHC of the 22 specimens from the patients with primary syphilis were all positive (100%). Twenty three (88%) out of 26 specimens from the patients with secondary syphilis showed positive results. T. pallidum was also identified in three patients with negative VDRL. Although the density of T. pallidum was higher in the specimens from the patients with primary syphilis than in those from the patients with secondary syphilis, the differences were not statistically significant (p=0.32). An epitheliotropic pattern was more frequently observed in the specimens from the patients with secondary syphilis (81%) than in those from the patients with primary syphilis (50%) (p=0.01). The density and distribution patterns of T. pallidum didn't show any correlation with the duration of skin lesions or the VDRL titer. CONCLUSION: IHC using a polyclonal antibody against T. pallidum could be an effective method for making the diagnosis of primary and secondary syphilis.

A Case of Multiple Eruptive Dermatofibromas in a Patient with Systemic Lupus Erythematosus / 대한피부과학회지

Dermatofibromas are common benign fibrohistiocytic tumors that occur primarily as a solitary lesion, while multiple eruptive dermatofibromas (MEDFs) are a rare presentation of dermatofibromas and MEDFs are frequently associated with altered immunity, such as autoimmune diseases and immunosuppression. It is still unclear why MEDFs occur in association with these conditions, but this relationship may suggest that MEDFs are the result of an abortive immunoreactive process. We herein report on a case of MEDF that developed in a patient who was diagnosed with systemic lupus erythematosus.

A Case of Tuberculosis Cutis Orificialis with Perianal Involvement

Tuberculosis cutis orificialis (TCO) is a rare manifestation of cutaneous tuberculosis that is caused by auto-inoculation of mycobacteria in patients with advanced internal tuberculosis. TCO occurs in oral, perianal, or genital mucosa and adjacent skin. The tongue is the most frequently affected site, but the perianal area can also be affected. A 39-year-old male presented with a 4-month history of painful ulcers on the perianal area. The histopathologic findings revealed granulomatous infiltrates composed of epithelioid cells and Langhans-type giant cells in the dermis, and a few acid-fast bacilli noted on Ziehl-Neelsen staining. The polymerase chain reaction (PCR) was positive for Mycobacterium tuberculosis and the chest X-ray showed findings consistent with active pulmonary tuberculosis in both upper lung zones. The skin lesion showed complete resolution 2 months after the start of treatment with antituberculosis agents. We report a case of TCO with perianal involvement in a patient with underlying active pulmonary tuberculosis.

Epigallocatechin-3-gallate Suppresses Galactose-alpha1,4-galactose-beta1,4-glucose Ceramide Expression in TNF-alpha Stimulated Human Intestinal Epithelial Cells Through Inhibition of MAPKs and NF-kappa B

Intestinal epithelial cells (IECs) have been known to produce galactose-alpha1,4-galactose-beta1,4-glucose ceramide (Gb3) that play an important role in the mucosal immune response. The regulation of Gb3 is important to prevent tissue damage causing shiga like toxin. Epigallocatechin-3-gallate (EGCG) has been studied as anti-carcinogenic, anti-oxidant, anti-angiogenic, and anti-viral activities, and anti-diabetic. However, little is known between the expressions of Gb3 on IECs. The aim of this study was to examine the inhibitory effect of EGCG, a major ingredient of green tea, on Gb3 production via mitogen-activated protein kinases (MAPKs) and nuclear factor-kappa B (NF-kappa B) in the TNF-alpha stimulated human colon epithelial cells, HT29. To investigate how Gb3 is regulated, ceramide glucosyltransferase (CGT), lactosylceramide synthase (GalT2), and Gb3 synthase (GalT6) were analyzed by RT-PCR in HT 29 cells exposed to TNF-alpha in the presence or absence of EGCG. EGCG dose-dependently manner, inhibits TNF-alpha induced Gb3 expression by blocking in both the MAPKs and NF-kappaB pathways in HT29 cells. TNF-alpha enhanced CGT, GalT2 and GalT6 mRNA levels and EGCG suppressed the level of these enzymes enhanced by TNF-alpha treatment.