Dermatomyositis with Eosinophils.

Dermatomyositis is an idiopathic inflammatory myopathy that often presents with symmetric proximal skeletal muscle weakness and characteristic skin findings. Typical skin biopsy findings include vacuolar changes of the basal layer, increased dermal mucin, and a predominantly lymphocytic infiltrate. We report a case of dermatomyositis presenting as intensely pruritic papules and plaques, with initial histopathology being atypical of dermatomyositis due to the presence of eosinophils. The initial biopsy demonstrated a superficial dermatitis with eosinophils, initially thought to represent a drug eruption. A second biopsy of the same cutaneous manifestation was performed at a later time given high clinical suspicion for dermatomyositis and demonstrated a more classic vacuolar interface dermatitis with increased mucin and an absence of eosinophils. Notably, increased pruritus was specifically associated with the lesion that demonstrated tissue eosinophilia. The case illustrates the importance of considering tissue eosinophilia in the histologic presentation of dermatomyositis.

Acquired vulvar lymphangioma: risk factors, disease associations, and management considerations: a systematic review.

Acquired vulvar lymphangioma (AVL) is not well-characterized. Diagnosis is delayed and the condition is often refractory to therapy. Objective: The objective of this study was to provide a systematic review of AVL including risk factors, disease associations, and management options. Methods: A primary literature search was conducted using 3 databases: PubMed, CINAHL, and OVID, from all years to 2022. Results: In total, 78 publications with 133 patients (48 ± 17 years) were included. Most studies were case reports/series. The most common disease association was prior malignancy (70 patients, 53% of cases) and inflammatory bowel disease (6 patients, 5% of cases). The most common malignancy was cervical cancer (57 patients, 43% of cases). Most patients had prior radiation or surgery, with 36% (n = 48) treated with radiation, 30% (n = 40) with lymph node dissection, and 27% (n = 36) with surgical resection. Common presenting symptoms included discharge/oozing, pain, and pruritus. Most patients underwent surgical treatment for AVL with 39% treated with excision, 12% with laser therapy (the majority used CO2), and 11% with medical therapies. Most patients had failed prior therapies and there was a diagnostic delay. Limitations: Retrospective nature. Most studies were limited to case reports and case series, with interstudy variability and result heterogeneity. Conclusion: AVL is an underrecognized entity and should be considered in patients with a history of malignancy or radiation to the urogenital area. Treatment should include multidisciplinary care and address underlying lymphatic changes, manage any existing inflammatory conditions, and utilize skin-directed therapies and barrier agents while addressing symptoms of pruritus and pain. Prospective studies are needed to further characterize AVL and develop treatment guidelines.

Epithelioma Cuniculatum (Plantar Verrucous Carcinoma): A Systematic Review of Treatment Options.

Epithelioma cuniculatum (EC) is a subtype of verrucous carcinoma (VC) that affects the feet. Treatment involves complete tumor removal by wide local excision (WLE) or Mohs micrographic surgery (MMS). Extensive local destruction may require amputation. We sought to compare reported treatment methods for EC and determine their efficacy by assessing for tumor recurrence and treatment-associated complications. A systematic review of the literature spanning multiple databases was performed. To date, surgical excision is recommended as the standard of care for treatment of EC, with amputation considered in more advanced cases. Mohs micrographic surgery appears to be a promising treatment modality for EC and may have lower recurrence rates than WLE but requires further investigation.

Cutaneous T-cell lymphoma following dupilumab use: a systematic review.

In recent years, studies have associated dupilumab with unmasking or progression of cutaneous T-cell lymphoma (CTCL). The objective of this study was to synthesize reported cases of CTCL in the setting of dupilumab use. Two independent reviewers conducted a systematic review using PubMed and Embase databases. Twelve studies, of 27 total patients, were included. Dupilumab-associated CTCL was diagnosed on average 7.8 months following dupilumab initiation. Of reports that included body surface area (BSA), 100% had a BSA >50% prior to diagnosis, and the majority of patients were diagnosed at later stages of disease: stage III (4/18) or IV (6/18). Post-dupilumab biopsies revealed a greater density of cells and a predominant lichenoid pattern, compared to various histologic patterns and less cell density in pre-dupilumab biopsies. The majority of TCR gene rearrangement studies were equivocal (20%) or negative (60%), posing a potential diagnostic pitfall. Limitations included lack of standardized data collection and reporting and a small number of studies. We suggest that earlier biopsies, three to four months after initiation of dupilumab, may be helpful for earlier recognition of CTCL in patients who are not improving, progressing, or have morphologic changes on dupilumab.

Dupilumab-Associated Mycosis Fungoides with a CD8+ Immunophenotype.

Dupilumab is a humanized IgG4 monoclonal-antibody that is approved by the United States Food and Drug Administration (FDA) for the treatment of moderate-to-severe atopic dermatitis (AD) in patients aged 12 years and older. In recent years, several case studies have associated the unmasking or progression of cutaneous T-cell lymphomas (CTCL) with dupilumab treatment. To date, all reported cases of dupilumab-associated CTCL have shown a CD4+ T-helper-cell-predominant immunophenotype. Here, we report a case of a 72-year-old man who presented with a 2-year history of a diffuse, pruritic eruption, who was started on dupilumab for 9 weeks. He subsequently developed mycosis fungoides (MF) with a CD8+-predominant immunophenotype. Overall, cases of CD8+ mycosis fungoides are less common and relatively less understood than their CD4+ counterparts, with varied presentations and courses. We present a case of dupilumab-associated CD8+ MF to highlight this presentation for pathologists and providers.

Enfortumab Vedotin-Associated Toxic Epidermal Necrolysis-like Toxic Erythema of Chemotherapy.

ABSTRACT: Enfortumab vedotin is an antibody-drug conjugate used for treatment of urothelial cancer. It has recently been associated with several reports of Stevens Johnson syndrome/toxic epidermal necrolysis (TEN). In this report, we describe the case of a 63-year-old man who developed widespread, near full-thickness desquamation, clinically mimicking TEN but with histologic features of toxic erythema of chemotherapy. This distinction is significant because it may have implications for prognosis and treatment. Further investigation is needed to ascertain the incidence of true TEN versus clinical imitators in patients with enfortumab vedotin-related cutaneous toxicity.

Inflammatory Vulvar Dermatoses (Part I).

Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (part I) and benign and malignant vulvar neoplasms (part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.

Vulvar Neoplasms (Part II).

Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (Part I) and benign and malignant vulvar neoplasms (Part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.