ABSTRACT
Nevus sebaceus is a hamartomatous lesion characterized by epidermal, follicular, sebaceus, and apocrine gland abnormalities. Approximately 25% of affected individuals may develop benign or malignant secondary neoplasms within the preceding nevus sebaceus. Primary cutaneous apocrine carcinoma (PCAC) is a rare malignant skin tumor affecting elderly adults in their sixth decade of life. Histologically, PCAC appears as a dermal tumor displaying apocrine differentiation with decapitation secretion and malignant features. Secondary malignancy arising from nevus sebaceus is a rare complication, especially for apocrine carcinoma. To date, approximately 200 cases of PCAC have been reported in the literature, and only a few cases have developed PCAC on the scalp. Very few cases (approximately only 12) of PCACs developing in nevus sebaceus have been reported. Here, we report an extremely rare case of the coexistence of PCAC and syringocystadenoma papilliferum arising within nevus sebaceus of the scalp.
ABSTRACT
Pilar leiomyoma or piloleiomyoma is a benign neoplasm of the smooth muscle arising from the arrector pili muscle. It manifests as brown to red firm papulonodules with sites of predilection being the face, trunk, and extensor surfaces of the extremities. Histologically, the lesions exhibit ill-defined dermal tumors with interlacing fascicles of spindle cells. Some genodermatoses are characterized by the development of visceral tumors and cutaneous leiomyomatosis such as Reed’s syndrome, and hereditary leiomyomatosis and renal cell cancer (HLRCC). A 55-year-old male presented with reddish-brown papules and nodules on the face and upper back, accompanied by sharp episodic pain on the face. He had undergone nephrectomy for renal cancer 9 years ago, and his younger brother had similar cutaneous manifestation. Histopathologic findings were consistent with pilar leiomyoma, showing bundles of smooth muscle tumors in the dermis. Based on the clinical information including clinical features, past medical history, and family history, HLRCC was highly suspected.To confirm the diagnosis, whole exome sequencing was performed using peripheral blood, which revealed a novel point mutation (c.739G>A, p.Glu247Lys) in the fumarate hydratase (FH) gene. We describe a confirmed case of HLRCC, which is a genetic disorder with a potential to cause visceral cancers, which dermatologists might overlook as a benign condition.
ABSTRACT
Rubinstein-Taybi syndrome (RSTS) is an extremely rare genetic disorder affecting multi-organ systems. A tendency to form keloid is one of the common dermatologic manifestations.We describe a 23-year-old female presented with extensive keloids which developed spontaneously. She had typical facial features, broad thumbs, and dental defects, which were suspicious features of genetic syndrome. Direct sequencing for cyclic-AMP-regulated enhancer binding protein revealed a novel mutation. So far, 23 cases of RSTS have been reported in Korean literature. To the best of our knowledge, this is the first report in Korea to describe confirmed case of RSTS with extensive keloids as a chief manifestation.
ABSTRACT
Background@#Although patients with psoriasis have an increased risk of cancers, little is known about the risk of psoriasis in cancer patients. @*Objective@#We aimed to comparatively analyze the incidence and risk factors of psoriasis in gastric cancer patients who underwent gastrectomy and in the general population. @*Methods@#A nationwide retrospective cohort of 52,608 gastric cancer survivors (2007~2015) was compared to 123,438 matched controls from the general population to estimate the incidence and hazard ratio (HR) of new-onset psoriasis. We also calculated the HRs for psoriasis according to adjuvant cancer treatment, obesity, and vitamin B12 supplementation in gastric cancer survivors. @*Results@#During a mean follow-up of 6.85 years, 645 of the 52,608 gastric cancer patients developed psoriasis, while 1,806 in the 123,438 matched control group developed psoriasis. Gastric cancer patients had a decreased risk of psoriasis (HR, 0.86; 95% confidence interval, 0.79~0.94), especially those who underwent subtotal gastrectomy. We found that vitamin B12 supplementation for more than 3 years had an additive effect on decreasing the risk of psoriasis in gastric cancer patients who underwent subtotal gastrectomy. Total gastrectomy, radio/chemotherapy, and obesity did not affect the risk of psoriasis in gastric cancer survivors. @*Conclusion@#The incidence of psoriasis is slightly lower in gastric cancer survivors than in the general population. Our results suggest that the development of psoriasis may be reduced by removing the source of systemic inflammation caused by Helicobacter pylori infection through subtotal gastrectomy in gastric cancer survivors.
ABSTRACT
Background@#Cyclosporine (CsA), an immunosuppressant that specifically regulates T-lymphocytes, has long been used in dermatology practice. However, nephrotoxicity, a well-known adverse effect associated with high-dose (≥5 mg/kg/d) and long-term administration (≥2 years) has limited the use of CsA. @*Objective@#We investigated the association between the cumulative dose of CsA and renal dysfunction, as well as the long-term effects of low-dose CsA on renal function in patients who presented with dermatological conditions. @*Methods@#The study included 697 patients who received CsA at an outpatient dermatology clinic between January 2015 and May 2019. Logistic regression analysis was performed to determine the association between the cumulative dose of CsA and renal dysfunction. @*Results@#Compared with patients who received the lowest cumulative dose (˂7,000 mg), those who received the highest cumulative dose of CsA (≥30,600 mg) showed a 64% higher risk of renal dysfunction; however, the difference was not statistically significant (odds ratio 1.64, 95% confidence interval 0.39∼6.84). @*Conclusion@#Among patients who receive a low-dose CsA regimen, those who are treated with cumulative doses higher than the critical value may be predisposed to renal dysfunction, whereas those treated with a cumulative dose lower than the critical value are unlikely to develop nephrotoxicity.
ABSTRACT
Background@#Cyclosporine (CsA), an immunosuppressant that specifically regulates T-lymphocytes, has long been used in dermatology practice. However, nephrotoxicity, a well-known adverse effect associated with high-dose (≥5 mg/kg/d) and long-term administration (≥2 years) has limited the use of CsA. @*Objective@#We investigated the association between the cumulative dose of CsA and renal dysfunction, as well as the long-term effects of low-dose CsA on renal function in patients who presented with dermatological conditions. @*Methods@#The study included 697 patients who received CsA at an outpatient dermatology clinic between January 2015 and May 2019. Logistic regression analysis was performed to determine the association between the cumulative dose of CsA and renal dysfunction. @*Results@#Compared with patients who received the lowest cumulative dose (˂7,000 mg), those who received the highest cumulative dose of CsA (≥30,600 mg) showed a 64% higher risk of renal dysfunction; however, the difference was not statistically significant (odds ratio 1.64, 95% confidence interval 0.39∼6.84). @*Conclusion@#Among patients who receive a low-dose CsA regimen, those who are treated with cumulative doses higher than the critical value may be predisposed to renal dysfunction, whereas those treated with a cumulative dose lower than the critical value are unlikely to develop nephrotoxicity.