No abstract available.
Carcinoma, Basal Cell ; Osteogenesis

Clear cell sarcoma of kidney(CCSK) is a rare pediatric neoplasm characterized by a predominating component of clear cells, a predilection for metastases to bone, and a poor prognosis. The incidence of CCSK peaks during the 2nd year of life and adult cases are very rare. We report a case of CCSK encountered in the right kidney of a 39-year-old man. Grossly, it was a lobulated mass showing infiltrative margin, measured 7x5.5x5cm and had a homogeneous gray-tan color with a soft, fish-flesh consistency. Microscopically, about half of the tumor revealed the classic pattern of CCSK, having tumor cell cords or nests separated by the characteristic alveolar capillary networks. The tumor cells had clear pale cytoplasm, bland looking round nuclei and inconspicuous nucleoli. The other half showed the epithelioid-trabecular pattern forming pseudorosette or cord-like structures. Immunohistochemically, there was only a focal positive reaction to vimentin. Ultrastructurally, the tumor cells showed the primitive nephrogenic mesenchymal differentiation such as electron lucent cytoplasm, a small amount of organelles, scanty heterochromatin, inconspicuous nucleoli, and a lack of flocculant basal lamina material around the cytoplasmic membrane. We consider that this is a case of CCSK occuring in the oldest patient ever reported, confirmed by both immunohistochemistry and electron microscopy.
Adult ; Male ; Female ; Humans ; Incidence ; Neoplasm Metastasis

Background: Pityriasis lichenoides et varioliformis acuta (PLEVA), a type of pityriasis lichenoides disease, is an uncommon inflammatory cutaneous disorder of unknown etiology. PLEVA presents as acute inflammatory papules that progressively develop into erosions or necrotic crusts all over the body. Objective: This study aimed to analyze the clinicopathological findings of 39 patients with PLEVA at a tertiary referral center in Seoul, Korea. Methods: This was a retrospective and single-center study with a longitudinal section. Medical records and telephone interviews were used to review the data of patients diagnosed with PLEVA between 2006 and 2019. Results: This study included 39 patients with PLEVA (male: female=2.9:1). The mean age of onset was 30.1 years, and 20/39 (51%) patients were children or young adults (＜30 years old). The mean duration of the disease course was 8.9 months. The disease was associated with a wide spectrum of clinical features ranging from erythematous inflammatory papules to diffusely progressing ulcers or crusted lesions. Treatment modalities involved topical corticosteroids (67%), low-dose systemic corticosteroids (44%), systemic antibiotics (33%), narrow-band ultraviolet B treatment (18%), and observation (13%). Except in one case, there were no reports of intolerable comorbidity or severe side effects within the follow-up period. Conclusion PLEVA showed a male predominance and primarily occurred during childhood or young adulthood in our study cohort. PLEVA could be associated with cutaneous T-cell lymphoma. Various treatment modalities may be used according to the clinical severity, and most patients exhibited a good response to treatment.

Neutrophilic urticarial dermatosis (NUD) clinically resembles urticaria but histopathologically represents a neutrophilic dermatoses. Affected patients develop erythematous macules, papules or slightly raised plaques lasting 24 to 48 hours. Histopathologic examination reveals an intense neutrophilic infiltration in the dermis with leukocytoclasia, but without fibrinoid necrosis of vessel walls. Clinical manifestations of NUD are similar to urticaria or Sweet’s syndrome, but it differs in that accompanying systemic symptoms like fever or arthralgia, and histologic features of dense neutrophilic infiltration in the dermis. A 69-year-old female patient presented with a one-day history of pruritic skin rashes and fever. Skin rashes appear erythematous patches and plaques on the whole body accompanied with multiple vesiculobullous lesions on both lower legs. Skin biopsy of lesion displayed interstitial neutrophilic infiltration in Indian file between collagen fiber, and leukocytoclasia. There were no clinical and laboratory findings that suspect of associated diseases. Herein, we report the first case of NUD developing vesiculobullous lesions.

Neutrophilic urticarial dermatosis (NUD) clinically resembles urticaria but histopathologically represents a neutrophilic dermatoses. Affected patients develop erythematous macules, papules or slightly raised plaques lasting 24 to 48 hours. Histopathologic examination reveals an intense neutrophilic infiltration in the dermis with leukocytoclasia, but without fibrinoid necrosis of vessel walls. Clinical manifestations of NUD are similar to urticaria or Sweet’s syndrome, but it differs in that accompanying systemic symptoms like fever or arthralgia, and histologic features of dense neutrophilic infiltration in the dermis. A 69-year-old female patient presented with a one-day history of pruritic skin rashes and fever. Skin rashes appear erythematous patches and plaques on the whole body accompanied with multiple vesiculobullous lesions on both lower legs. Skin biopsy of lesion displayed interstitial neutrophilic infiltration in Indian file between collagen fiber, and leukocytoclasia. There were no clinical and laboratory findings that suspect of associated diseases. Herein, we report the first case of NUD developing vesiculobullous lesions.