Pseudolymphomatous Cutaneous Angiosarcoma Presenting With Persistent Firm Facial Edema in a Patient With Systemic Sclerosis.

ABSTRACT: Pseudolymphomatous cutaneous angiosarcoma (cAS) is a rare subtype characterized by a prominent lymphocytic infiltrate, posing diagnostic challenges due to its resemblance to lymphoid neoplastic processes. We present a novel case highlighting the clinical and histopathological features, notably its association with persistent firm facial edema in a patient with systemic sclerosis (SSc). A 47-year-old woman with a 21-year history of SSc presented with firm palpebral edema evolving to involve the entire face and cervical region over six months. Diagnostic imaging revealed inflammatory changes in orbital regions, supradiaphragmatic lymphadenopathies, and lytic lesions. Skin biopsy demonstrated a diffuse neoplasm with vascular channels and solid areas, accompanied by dense lymphocytic proliferation. Pseudolymphomatous cutaneous angiosarcoma, a rare malignant neoplasm, exhibits variable clinical presentations and rapid progression. Histologically, it manifests as irregularly shaped vascular channels lined by prominent endothelial cells. Immunohistochemistry, particularly markers such as v-ets erythroblastosis virus E26 oncogene homolog (avian) (ERG), aids in diagnosis. Notably, this case marks the first presentation of cAS with persistent facial edema in SSc, highlighting the association between SSc and cancer risk. This case underscores the diagnostic challenges posed by cAS and emphasizes the importance of early detection for optimal patient outcomes. Further understanding of its association with autoimmune disorders such as SSc is crucial for comprehensive management strategies.

Granulomatous Panniculitis as an Initial Manifestation of Sjögren Syndrome.

ABSTRACT: A 65-year-old woman presented with unexplained weight loss, recurrent fever, and a dermatosis with painful nodules on the extremities. Biopsies showed focal lobular panniculitis with neutrophilic microgranulomas. Comprehensive investigations ruled out infection and hematologic and solid organ neoplasms. Laboratory results showed anti-Ro/SSA and anti-La/SSB antibody positivity, and elevated inflammatory markers. Dry mouth and eye were confirmed. The diagnosis of Sjögren syndrome with cutaneous panniculitis was established. Prednisone treatment with 30 mg/d resulted in remission of fever and pain improvement. This case emphasizes Sjögren syndrome as an autoimmune disease with multiple cutaneous manifestations and highlights its association with granulomatous panniculitis.

Sweet Syndrome as a Herpetiform Mimic: A Diagnostic Challenge.

ABSTRACT: The authors present a singular case of Sweet syndrome (acute febrile neutrophilic dermatosis) manifesting with an unusual herpetiform clinical presentation, underscoring the imperative for its inclusion in differential diagnoses of herpetic infections. A 26-year-old female patient with a systemic lupus erythematosus history presented with facial edema, hyperthermia, cephalalgia, and polyarticular pain. Dermatological examination revealed clustered, vesicle-like papules on erythematous, edematous skin, mimicking herpetic infection. Elevated acute-phase reactants and urine anomalies were noted. Histopathology confirmed Sweet syndrome, characterized by superficial and deep neutrophilic dermatitis, karyorrhexis, and papillary dermal edema. The patient responded to corticosteroid therapy and a brief antibiotic course, resolving both systemic and cutaneous symptoms. This case is remarkable for its atypical herpetiform presentation, a clinical rarity in Sweet syndrome, challenging the conventional diagnostic process. It emphasizes the necessity of considering Sweet syndrome in differential diagnoses when encountering herpetiform lesions, particularly in patients with autoimmune backgrounds. This case contributes significantly to the understanding of Sweet syndrome's clinical variability and highlights the critical role of thorough clinicopathological evaluation in achieving accurate diagnosis in complex dermatological disorders.

Clinical, molecular, and histological characteristics of severely necrotic and fatal mpox in HIV-infected patients.

BACKGROUND: This case series of 5 patients with severely necrotic mpox highlights the predominantly necrotic nature of lesions seen in cases of severe mpox as shown by skin and lung biopsy, as well as the extensive dissemination of the infection, as shown by polymerase chain reaction (PCR) assessment in different body sites. CASE PRESENTATIONS: Patients were male, the median age was 37, all lived with HIV (2 previously undiagnosed), the median CD4+ cell count was 106 cells/mm3, and 2/5 were not receiving antiretroviral treatment. The most common complication was soft tissue infection. Skin and lung biopsies showed extensive areas of necrosis. Mpox PCR was positive in various sites, including skin, urine, serum, and cerebrospinal fluid. The initiation of antiretroviral treatment, worsened the disease, like that seen in immune reconstitution syndrome. Three patients died due to multiple organ failure, presumably associated with mpox since coinfections and opportunistic pathogens were ruled out. CONCLUSIONS: Severely necrotic manifestations of mpox in people living with advanced and untreated HIV are related to adverse outcomes.

Exposing the Great Imitator: Proposal for a Holistic Diagnosis of Oral Secondary Syphilis in People Living with HIV.

Oral secondary syphilis may mimic various infectious, neoplastic, or immune-mediated processes; hence, its diagnosis may represent a challenge. Early diagnosis of syphilis, a disease that has increased in recent decades, is essential for adequate management, particularly in people living with HIV (PLWH). This study aimed to comprehensively characterize oral secondary syphilis in a group of 47 PLWH. A group of PLWH with oral secondary syphilis attending four HIV-referral centers in Mexico City was included (2004-2021). Clinical and laboratory data were retrieved, and an exhaustive oral examination was performed following the established criteria. Demographic, clinicopathological, immunohistochemical, and serological features of the patients were analyzed. Approximately 11% of PLWH with oral secondary syphilis demonstrated negative Venereal Disease Research Laboratory tests. A noticeable feature was the absence of symptoms in 95.7% of cases, despite the clinically evident appearance of the lesions. In contrast to previous results, 18% of ulcerations were detected to be deep, crateriform, and infiltrative, and 22% of the mucous patches were highly keratotic lesions. Most samples (77.3%) showed superficial lymphoplasmacytic infiltrates in the superficial lamina propria, with perivascular and perineural patterns, and immunohistochemistry was positive in 66.7% of the cases. The "great imitator" appears not only clinically but also histopathologically and immunohistochemically, where features may be comparable with those of chronic inflammatory processes, deep infections, or malignant processes. Although not recommended as a routine assay, IHC could be a critical tool, particularly in PLWH with atypical clinical features or with negative and/or dubious serology.

Histopathological and Immunohistochemical Characterization of Skin Biopsies From 41 SARS-CoV-2 (+) Patients: Experience in a Mexican Concentration Institute: A Case Series and Literature Review.

ABSTRACT: The SARS-CoV-2 pandemic brought countless clinical and pathophysiological questions. Although mucocutaneous infections are the most visible, they are among the least studied. This article provides relevant information to characterize morphologically and immunohistochemically the dermatoses from patients with COVID-19, during the first year of the pandemic. Immunohistochemistry reactions against the spike protein were performed in 48 skin biopsies, and the positive cases were classified according to their histomorphology; at the end, 41 biopsies led us to identify 12 morphological patterns that mimic other skin pathologies, among which pityriasiform patterns predominate. For the literature review, we selected cases of SARS-CoV-2 dermatoses that included complete histopathological information and that were published during the same interval of time; after careful evaluation, 205 biopsies were selected and then classified into 8 groups according to previously published proposals. Dermatoses associated with SARS-CoV-2 are as diverse in their clinical expression as in their histopathology, mimicking entities totally unrelated to COVID-19. Furthermore, some of these groups are characteristically associated with an aggressive course of the disease. Undoubtedly, it is necessary to delve into the possibility that these findings are translatable into prognostic and therapeutic factors.

Primary meningococcal septic arthritis associated with joint calcium oxalate crystals: A case report and review of the literature.

Primary meningococcal septic arthritis (PMSA) is an extremely rare local infection by Neisseria meningitidis in the absence of meningitis or meningococcaemia syndrome. A 30-year-old healthy, immunocompetent man presented with arthralgia, fever, chest rash, and significant swelling of the right knee. On admission, a disseminated maculopapular and purpuric rash, oligoarthritis, neutrophilia, and elevated acute phase reactants were documented. Following arthrocentesis of the right knee, isolation of N. meningitidis and the presence of calcium oxalate crystals in the synovial fluid were reported. The diagnosis of PMSA was made. Histological analysis of the skin lesion showed leucocytoclastic vasculitis. He was treated with intravenous ceftriaxone plus open surgical drainage and ambulatory cefixime with adequate response. After 1 month, he presented resolution of the pathological process. We performed an extensive review of the literature, finding that the key elements supporting the diagnosis of PMSA are prodromal upper respiratory tract symptoms and skin involvement prior to or synchronous with the arthritis. Also, the most frequently involved joint is the knee. This report is the first case of a patient presenting with PMSA associated with calcium oxalate crystals in the synovial fluid. Herein, we discuss the most frequent clinical manifestations, the unusual histological features, the recommended treatment, and the reported prognosis of this rare entity.

Porocarcinoma with areas of mucinous differentiation suggesting multilineage differentiation.

Porocarcinoma is an infrequent malignant adnexal carcinoma, with some histopathological variants described, such as the clear cell, the sarcomatoid or the pigmented porocarcinoma. We report an invasive porocarcinoma showing areas of tumor cells floating in prominent dermal mucin, simulating mucinous carcinoma, that we consider a new histopathological variant of porocarcinoma. We report a 74-year-old male with previous history of multiple basal cell carcinomas that presented a nodule on his left temple. Histopathologic study showed a large ulcerated multilobular tumor composed of thickened cords of cells emanating from a hyperplastic epidermis and showing a mixed infiltrative and pushing pattern in the dermis. Poroid differentiation was observed in most of the neoplasm, both in intraepidermal and dermal invasive component. Within the neoplasm a prominent area where these small nests with clear formation of ducts were floating in mucinous pools with few septa intermingled was observed, simulating a primary cutaneous mucinous carcinoma. Cytology, immunohistochemistry and the presence of both neoplastic areas as closely related and with multiple points of connectivity favors the consideration of a composite tumor in this peculiar case. Other differentials are discussed.

Histopathology of Cutaneous Aging.

Aging is (so far) an inexorable and irreversible path in all species and organisms. In human beings, aging involving the skin has a special meaning because our appearance has become crucial for our social life in the modern world. Knowledge of the morphologic changes that happen during the aging process is crucial for understanding its pathogenesis, which in turn is necessary to approach it and even revert it. Skin aging happens because of 2 main sets of changes. Many -although not all-are the cause of exposure to external agents (extrinsic aging), of which the most important is solar exposure (also known as photoaging). In addition, skin also degenerates by mechanisms linked to genetically programed information (intrinsic aging). In this article, the histopathologic changes evident in exposed and nonexposed skin are examined.

Impact of early recognition of potentially malignant oral disorders on the prognosis in people living with HIV.

Despite the success of combination antiretroviral therapies, people living with HIV (PLWH) are at an increased risk of developing diverse malignancies, including oral cancer. We here present two cases of PLWH where the early diagnosis of potentially malignant disorders in the oral cavity impacted their treatment and survival, remaining free of disease after their complete elimination. These cases demonstrate the importance of oral examinations and tissue biopsies as a part of the close monitoring of PLWH.

Histopathology of aging of the hair follicle.

Hair follicles experience several changes with aging, the most noticeable of which is graying of the hair shaft due to loss of melanin. Additional changes in the diameter and length of the hair have contributed to the concept of senescent alopecia, which is different from androgenetic alopecia according to most. Graying happens in most individuals, although in different grades and starting at different ages. It is related to a decrease in the number and activity of the melanocytes of the hair bulb, which eventually completely disappear from the bulb of the white hair. Residual non-active melanocytes remain in the outer root sheath and in the bulge, which allows for repigmentation of the hair under certain stimuli or conditions.

Intramural schwannoma involving a vein.

Schwannoma involving the blood vessels is a rare phenomenon. So far, only three cases of intravascular schwannoma have been described (all of which were intraluminal), and the origin of the schwannoma in such cases is not yet completely understood. Here, we describe a very rare intramural venous schwannoma in the subcutaneous right prepatellar area of a 31-year-old man. The schwannoma grew by enlarging and thickening the blood vessel wall, between two preserved layers of the vein. In some areas, there was erosion of the luminal layer, with fibrin apposed to the tumor. The tumor expressed S100 and was negative for CD31, CD34, desmin, and smooth muscle actin. The expression of p16 was preserved. Endothelial markers such as CD31 and Factor VIII showed the endothelial lining (which was D2-40-negative) above the tumor. Although degenerative atypia was present, there were no mitotic figures or necrosis identified.

Superficial Solitary Fibrous Tumor: A Series of 26 Cases.

While superficial (cutaneous/subcutaneous) solitary fibrous tumor (SFT) have been described, definitive diagnosis is difficult due to overlapping features with other tumors. We describe the largest series to date of superficial SFT. For inclusion, SFT had to arise in dermis or subcutis. Twenty-six cases were identified. Patients ranged from 16 to 80 years (mean, 47 y) with a marked female predominance (19 F; 7 M). Tumors involved the head (11), thigh (7), back (3), shoulder (2), upper arm (1), ankle (1), and great toe (1). Mean size was 2.9 cm (range, 1.0 to 7.0 cm). The majority (n=19) had typical histologic features (cellular SFT) with irregular fascicles of spindled cells, staghorn-like blood vessels, and variable amounts of collagen. Necrosis was evident in 3 cases (all <25%). Mitotic activity ranged from 0 to 10 mitotic figures/10 high-power fields (mean, 2 mitotic figures/10 high-power fields). Seventeen of the 18 were positive for STAT6, whereas 21/22 expressed CD34. All were low risk (23/23) by proposed criteria (Demicco and colleagues), including 2 cases with malignant histology. Three could not be risk stratified due to lack of information on tumor size. Follow-up, available on 7 cases, showed no recurrence/metastasis (mean follow-up, 100 mo; range, 2 to 241 mo). Cutaneous SFT are more common in women and most often involve the head. They are usually low risk by proposed criteria and appear to behave in an indolent manner, though larger studies are needed to confirm this. Recognition that SFT may present as a superficial mass will avoid misclassification as other CD34-positive neoplasms that frequently arise in the skin and subcutaneous tissue.

Nutritional Deficiency-Related Oral and Cutaneous Lesions in an Obese Patient.

The cutaneous and oral lesions related to nutritional deficiencies are scarcely reported. Micronutrient deficiencies may significantly affect mouth mucosa and skin, causing great morbidity. We report an extraordinary case with detailed clinical and microscopic findings affecting the oral cavity and skin. Physicians must be familiar with these manifestations to suspect the diagnosis.

Polypoid Spitz Nevus With a Halo Reaction.

Approximately, 2% of Spitz nevi are polypoid; between 3.6% and 7.4% present with a halo reaction. In tandem, these low percentages make the presence of a polypoid Spitz nevus with a halo reaction uncommon; we have not found reports of any previous cases. In the current report, we present a polypoid Spitz nevus with a halo reaction on the back of a 10-year-old male and discuss the morphologic findings. The lesion showed preserved nuclear expression of BAP1. There was no immunohistochemical expression of BRAF and ALK, while the melanocytic cells expressed p16. Comparative genomic hybridization was performed, and no significant aberrations were found. Only 2 small losses were evidenced in chromosome 8. The patient has been followed now for 2 years with no recurrence.

Focal Epithelial Hyperplasia in Adult Patients With HIV Infection: Clearance With Topical Imiquimod.

Case 1 A 41-year-old man with human immunodeficiency virus (HIV) 1 diagnosed 16 years prior to his consultation was referred for an 8-month history of multiple painless lumps in his mouth. He had A2 status (CD4 cell count of 273 cells/mm3 and viral load of 43,000 copies/L) and was taking treatment with lamivudine/zidovudine (combivir) and efavirenz. Physical examination showed multiple small mucosal-colored and lobulated papulonodules located in the palate and lower gingiva and a whitish verrucous plaque on the lower labial mucosa (Figure 1a). The lesions were diagnosed clinically as focal epithelial hyperplasia (FEH) and further confirmed by classical histopathological findings (Figure 1b). He had previously received unspecified treatment; thus, topical 5% imiquimod cream was initiated every night. Mild erosion and ulceration developed in the upper labial mucosa, which were managed with lubrication (petrolatum ointment). After 2 weeks, all of the small lesions disappeared and the largest plaque resolved 1 week later (Figure 1c). A small residual mass in the area of biopsy, suggesting a scar, remained on the lower lip. The area was removed surgically and corresponded to fibrosis histologically, with no evidence of human papillomavirus (HPV) infection. CD4 cell count (694 cells/mm3) and viral load (<40 copies/L) did not show remarkable changes after imiquimod administration. No serious side effects were observed and the patient has remained free of disease after 1 year of follow-up.

Mucin as a diagnostic clue in dermatopathology.

Mucins are high-molecular weight glycoproteins typically found in normal skin in small amounts. There are several reports regarding different types of cutaneous mucinosis. In this report, we discuss mucins in dermatopathology as a diagnostic clue of some well-known entities and some less frequent cutaneous diseases. We also emphasize mucin as a sign in the differential diagnosis between conditions that show histopathological overlap. Lastly, we discuss the locations and circumstances in which mucin could be considered almost normal or physiological.

Pemphigus foliaceus with circinated plaques and neutrophil pustules.

Pemphigus is a group of autoimmune intraepidermal bullous diseases; being pemphigus foliaceus (PF) and pemphigus vulgaris (PV) the most common subtypes. Pustular variants are scarcely reported for both PV and PF. The purpose of this manuscript was to describe the clinical, microscopic and immunologic findings of an atypical case of PF presenting with pustules, including a review of the literature. PF is described as blisters and because this entity is rare, it is not known for the general medical community that they are other clinical features that can be seen as this one we present here with pustules.