ABSTRACT
Cowden syndrome is caused by mutations in the phosphatase and tensin homolog (PTEN) gene and is part of the PTEN hamartoma tumor syndrome. Skin lesions including trichilemmomas, acral keratosis, mucocunateous neuromas and oral paillomas are the most prevalent feature found in patients with Cowden syndrome. It also possesses an increased risk of developing malignancies including breast, thyroid, endometrial, and colorectal cancers.Due to the increased risk of cancer, early diagnosis and regular surveillance are important for Cowden syndrome patients. Herein, we report a case of Cowden syndrome with diverse cutaneous manifestations and thyroid cancer.
ABSTRACT
The treatment of pathological scars, such as keloids and hypertrophic scars, can be challenging for dermatologists. The first-line treatment is intralesional corticosteroid injection, especially when patients complain of pain or discomfort. Laser treatment can also be used in patients with keloids and hypertrophic scars. However, even after multiple sessions of intralesional corticosteroid injections and laser treatment, desirable outcomes may not be achieved, and recurrence is common. Recent studies on the efficacy of intralesional bleomycin injection (BLMILI) in treating keloids and hypertrophic scars have suggested that a significant improvement is observed after BLMILI. However, there is limited research on the effectiveness of BLMILI for patients who do not respond to other treatments, such as intralesional corticosteroid injection or laser treatment. Here, we report four cases of BLMILI in keloids and hypertrophic scars that were unresponsive to previous intralesional corticosteroid injection and/or laser treatment.
ABSTRACT
Onychogryphosis is a disorder of nail plate growth, which most commonly involves the toenails. It is characterized by opaque, yellow-brown thickening of the nail plate with associated marked convexity and elongation. Treatment for onychogryphosis can be conservative or operative depending on the cause and medical status of the patient. A 30-year-old male presented with onychogryphosis of the right and left toenails. Since the patient had shown recurrence after simple nail avulsion several years ago, the inverted T incision method and fusiform excision of the hypertrophic hyponychium were performed. No recurrence was observed during the 3-year follow-up period. Our results showed that nail avulsion combined with traction osteophyte removal is a suitable surgical method for treating onychogryphosis.
ABSTRACT
Background@#Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is rare Epstein-Barr virus (EBV)-associated disease. The classic form of HVLPD is a self-resolving disease, whereas the systemic form can progress to malignant lymphoma, resulting in fatal outcomes. However, the prognostic factors remain unclear. Objective: This study aimed to evaluate the clinical characteristics of HVLPD and the association between whole blood EBV DNA and clinical outcomes. @*Methods@#We retrospectively reviewed our 25-year experience involving 11 patients with HVLPD from a single tertiary center in South Korea and evaluated the clinical characteristics of HVLPD and the correlation between whole blood EBV DNA and clinical outcomes. @*Results@#Of the total 11 patients, 54.5% (6/11) manifested classic HVLPD that resolved with conservative treatment, while 45.5% (5/11) patients had systemic HVLPD, four of whom died of progressive disease or hemophagocytic syndrome. Five patients with systemic HVLPD manifested severe skin lesions such as prominent facial edema, deep ulcers and necrotic skin lesions involving sun-protected areas. Median EBV DNA levels at initial diagnosis were higher in three dead patients than in those alive (2,290 vs. 186.62 copies/μl). @*Conclusion@#When EBV DNA levels were high, patients showed severe skin lesions and when EBV DNA levels were low, skin lesions tended to improve. Thus, patients with high EBV DNA levels showed an increased risk of severe skin lesions and disease progression.
ABSTRACT
Background@#We recently discovered the presence of specialized nail mesenchyme below the nail matrix and designated it as onychomatricodermis. @*Objective@#We did further research to characterize the histologic, histochemical, immunohistochemical and ultrastructural features of the onychomatricodermis containing onychofibroblasts in the nail unit. @*Methods@#Ten polydactyly nail unit specimens and 8 nail matrix biopsies were included. H&E-stained slides were reviewed. We did Alcian blue staining and Masson Trichrome staining, as well as immunohistochemical staining for type Ⅰ collagen, CD10, CD13 and CD34. In addition, polydactyly nail units were examined by transmission electron microscopy. @*Results@#In H&E staining, the specialized mesenchyme called onychomatricodermis was observed to be slightly distant from the undersurface of the nail matrix and be less eosinophilic area. Onychomatricodermal onychofibroblasts showed light purple abundant cytoplasm.Masson Trichrome staining revealed fewer collagen fibers within the onychomatricodermis. In Alcian blue staining the onychomatricodermis showed mucin deposition within the onychofibroblasts and around them. Immunohistochemically, type Ⅰ collagen was expressed much less in the onychomatricodermis while it was strongly expressed elsewhere in the nail unit. In nail matrix biopsy specimens onychomatricodermal onychofibroblasts expressed CD10 and CD13 strongly, and expressed CD34 as well. Ultrastructurally, collagen fibrils were found sparsely within the onychomatricodermis, whereas collagen fibrils were densely distributed in the dermis of other parts of the nail unit. @*Conclusion@#We demonstrated that there was less collagen expression in the onychomatricodermis containing onychofibroblasts. In addition, we found morphological and immunohistochemical features of onychomatricodermal onychofibroblasts (onychofibroblasts of Dongyoun). These findings support the presence of onychomatricodermis containing onychofibroblasts in the nail unit.
ABSTRACT
Background@#Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is rare Epstein-Barr virus (EBV)-associated disease. The classic form of HVLPD is a self-resolving disease, whereas the systemic form can progress to malignant lymphoma, resulting in fatal outcomes. However, the prognostic factors remain unclear. Objective: This study aimed to evaluate the clinical characteristics of HVLPD and the association between whole blood EBV DNA and clinical outcomes. @*Methods@#We retrospectively reviewed our 25-year experience involving 11 patients with HVLPD from a single tertiary center in South Korea and evaluated the clinical characteristics of HVLPD and the correlation between whole blood EBV DNA and clinical outcomes. @*Results@#Of the total 11 patients, 54.5% (6/11) manifested classic HVLPD that resolved with conservative treatment, while 45.5% (5/11) patients had systemic HVLPD, four of whom died of progressive disease or hemophagocytic syndrome. Five patients with systemic HVLPD manifested severe skin lesions such as prominent facial edema, deep ulcers and necrotic skin lesions involving sun-protected areas. Median EBV DNA levels at initial diagnosis were higher in three dead patients than in those alive (2,290 vs. 186.62 copies/μl). @*Conclusion@#When EBV DNA levels were high, patients showed severe skin lesions and when EBV DNA levels were low, skin lesions tended to improve. Thus, patients with high EBV DNA levels showed an increased risk of severe skin lesions and disease progression.