American Journal of Dermatopathology最新文献

Background: Microvascular thrombosis is key to the pathogenesis of calciphylaxis. C5b-9-mediated microvascular injury reflective of complement pathway activation could be a key pathophysiologic event.

Methods: We conducted a retrospective multicenter study of 24 patients who have had biopsy-supported calciphylaxis from the 2010-2022 data base from Emory where C5b-9 immunohistochemistry (IHC) had not been conducted and the 2019-2023 data base from Cornell where C5b-9 IHC was done as part of the routine calciphylaxis work up. IHC for C5b-9 on lesional biopsy specimens was assessed and correlated with routine light microscopic findings and clinical features.

Results: Most of the patients in our study had uremic calciphylaxis associated with obesity, diabetes, dialysis, hypertension, hyperparathyroidism and elevated serum phosphorus. Most patients did not have defined procoagulant and/or hyperviscosity states. The vascular pathology was predominantly limited to the subcutaneous fat and ranged from a calcific intimal arteriopathy to microvascular thrombosis with endothelial injury with or without endothelial calcification. In most cases (ie, in excess of 80%), there was prominent deposition of C5b-9 within the vasculature including the microvasculature and arteries of the fat at least localized to injured vessels suggesting a causal association. In about 40% of cases, there was evidence of systemic complement pathway activation revealed by concurrent dermal microvascular C5b-9 deposition.

Conclusions: Calciphylaxis is characterized by subcuticular vascular changes that reflect an interplay between complement triggered endothelial cell injury, resultant vascular thrombosis, and subsequent abluminal calcification. Complement inhibition therapy defines a potential intervention that should be explored.

Abstract: Syphilis is a sexually transmitted disease that can present a wide variety of clinical and histopathological manifestations. We present the case of a 75-year-old patient with a persistent maculopapular rash on the trunk and extremities, in which the biopsy showed an exclusively perifollicular granulomatous infiltrate. In the immunohistochemical staining with anti- Treponema pallidum antibodies, spirochetes scattered within the perifollicular inflammatory infiltrate were identified. These findings together with the serological analysis allowed the diagnosis of secondary syphilis. Folliculotropic nonalopecic syphilis is rare, and the pathogenic mechanisms that cause this specific tropism are unknown. On the other hand, granulomatous inflammation is a pattern typically described in tertiary syphilis but also occasionally found in secondary syphilis. We present a case of secondary syphilis with the combination of both histopathological findings, an uncommon constellation for dermatopathologists to consider.

Abstract: Primary cutaneous spindle B-cell lymphoma is an uncommon subtype of cutaneous lymphoma characterized by a distinct spindled cytology of neoplastic B cells. Despite sharing clinical, histopathological, and phenotypical similarities with primary cutaneous follicle center lymphoma, an indolent form of B-cell lymphoma, it also exhibits certain features akin to primary cutaneous diffuse large B-cell lymphoma. Notably, in rare instances, a more aggressive clinical course has been observed. This report details a rare case of primary cutaneous spindle cell B-cell follicle center lymphoma, manifested as a prolonged solitary plaque of cicatricial alopecia. In addition, we provide a comprehensive review of existing cases documented in the literature.

Abstract: Poromas are benign adnexal neoplasms of eccrine origin, believed to arise from the outer layer of acrosyringia and upper dermal eccrine ducts, with a predilection for glabrous skin. They typically present as a pink or red papule with a surrounding thin moat on the palms and soles. We report a case of poroma with histopathologic features reminiscent of syringocystadenoma papilliferum (SCAP). A 70-year-old woman presented with a 2.0 cm pedunculated nodule on the left suprapubic abdomen. Histopathologically, the lesion predominantly displayed features of a conventional poroma but also included areas with endophytic invaginations lined by large, plump epithelioid cells with abundant eosinophilic cytoplasm and occasional decapitation secretion, alongside a stroma rich in plasma cells-characteristics suggestive of SCAP. However, definitive bilayers with myoepithelial cells were not observed. Immunohistochemical studies revealed that the tumor cells were positive for TRPS1 (particularly around SCAP-like areas) and CEA (indicating ductal differentiation), but negative for BRAF V600E and NUT. The diagnosis of poroma with apocrine differentiation mimicking SCAP was favored. This unusual morphologic variation in poromas is rare, with fewer than 5 cases documented in the literature. These SCAP-like features likely represent a variation within the morphologic spectrum of poromas rather than the presence of 2 synchronous tumors. Our case highlights the importance of recognizing such variations in poroid neoplasms to ensure accurate diagnosis.

Case presentation: We report a case of erythematoviolaceous cutaneous plaques in an uncommon location (abdomen and dorsum) in a patient with rheumatoid arthritis and without other disorders such as atherosclerotic disease, macromastia and smoking.

Discussion and conclusion: It is believed that ischemia or inflammation creates local hypoxia, leading to an increase in pro-angiogenic cytokines with subsequent endothelial proliferation and neovascularization. We report a case of a patient with rheumatoid arthritis (autoimmune disease) who presented with an asymptomatic cutaneous violaceous and roundish lesion in the trunk that may be related to diffuse dermal angiomatosis.

Abstract: Silicone granulomas, or "siliconomas," are the common foreign-body inflammatory responses to injected silicone material. In rare cases, siliconomas develop remotely from the original site of injection, secondary to silicone migration. If a history of silicone injection is not noted, such lesions risk misdiagnosis (possibly as infection or malignancy). Intraocular silicone oil is commonly used in retinal detachment surgery, with occasional reports of granulomatous responses after its application in this context. Here, we report a 66-year-old man who developed a periocular silicone granuloma years after scleral buckle surgery for a detached retina and summarize the literature of similar reports of migratory siliconoma.

Abstract: Squamomelanocytic tumor is a poorly defined term that indicates co-occurrence of malignant melanoma and squamous cell carcinoma within the same lesion. We present an unusual case of squamomelanocytic tumor in which both intermingling components showed predominantly spindle cell morphology and were confirmed with double staining for SOX-10 and AE1/AE3.