33111 New onset scrotal lesions in a patient with herpes simplex virus treated with foscarnet

Introduction: Intravenous (IV) foscarnet is used for the treatment of acyclovir-resistant herpes simplex virus (HSV). Foscarnet-induced penile ulcerations, thought to be due to an irritant contact dermatitis, can occur where urine contacts the skin, such as the glans penis adjacent to the urethral meatus. We present a gentleman who developed serpiginous papules on the scrotum from foscarnet therapy. Case Presentation: A 53-year-old African-American male with a history of HIV was admitted to the hospital for IV foscarnet treatment of HSV of the penile shaft recalcitrant to oral valacyclovir. Nine days after admission, the patient developed new onset burning and painful lesions on the scrotum. On physical examination, grouped serpiginous pink papules coalescing into a plaque on the left scrotum were noted. HSV PCR was negative. Skin biopsy demonstrated an erosive dermatitis with epidermal necrosis, supportive of an irritant contact dermatitis secondary to foscarnet. Subsequently, the patient did endorse urine coming into contact with his scrotum during urination. One week after stopping foscarnet, the scrotal lesions subsided without further treatment. Discussion: Penile ulcerations are a known but uncommonly encountered side effect of foscarnet therapy. Nearly 90% of a foscarnet dose is excreted in the urine, and it is thought that these ulcerations are due to an irritant contact dermatitis from urine. Scrotal lesions presenting as serpiginous papules have less commonly been reported. Foscarnet-induced irritant contact dermatitis should be considered in any patient on foscarnet presenting with new onset lesions in the groin

A Slow Growing Verrucous Plaque on the Scalp

A 73 year-old Caucasian male with a history of multiple non-melanoma skin cancers presented to dermatology for evaluation of a growth on the left scalp. He endorsed that the lesion had been present for many years but was slowly growing larger and thicker and had recently bled spontaneously and become intermittently painful. The lesion had been evaluated several times over a span of 3 years and was previously thought to most likely represent a verrucous seborrheic keratosis. The patient was otherwise in a good state of health and had no known history of immunosuppression.On the left parietal scalp there was a pink-brown verrucous plaque with filiform projections and focal hemorrhagic crust. The lesion measured 5.2 cm x 3.8 cm.Saucerization biopsy of the most exophytic portion of the lesion was performed and revealed a broad, atypical melanocytic proliferation within a larger verrucous keratosis with features consistent with a ‘verrucous pseudonevoid melanoma of the scalp. The Breslow depth was measured at 1mm.The patient was treated with a wide local excision with 1 cm margins. The re-excision specimen showed focal residual melanoma in-situ and clear margins. A sentinel lymph node biopsy was performed for staging and was found to be negative for metastatic disease.Nevoid melanoma is a rare entity which presents diagnostic difficulty on both clinical and histopathological grounds. On physical examination this tumor can be mistaken clinically for a verruca, benign melanocytic or epidermal nevus, or seborrheic keratosis. There are two major architectural variants: verrucous subtype and a dome-shaped variant (resembles a Meissner or Spitz nevus). The verrucous-subtype (as seen in our case) has been reported to have the following features which may distinguish it from a papillomatous nevus: (1) broad, exophytic growth pattern with a verrucous epidermal hyperplasia, (2) continuous proliferation of melanocytes along the dermal-epidermal junction, (3) confluent sheets of uniform, monomorphic without evidence of true maturation, and (4) occasional dermal mitoses.Mortality related to nevoid melanomas is thought to be consistent with that of traditional melanomas of the same Breslow depth. However, nevoid melanomas are commonly more advanced at the time of diagnosis given propensity for initial clinical and/or histologic misdiagnosis. Therefore, heightened awareness of this entity is critical to better ensure earlier diagnosis.https://scholarlycommons.henryford.com/merf2019caserpt/1011/thumbnail.jp

EBV+ B-cell polymorphic lymphoproliferative disorder of the lip in a patient with advanced chronic lymphocytic leukemia

Introduction: Polymorphic B-cell lymphoproliferative disorders (B-LPDs) are a rare, morphologically heterogeneous, and diagnostically challenging group of neoplasms that occur in the setting of immunosuppression. Associated lesions are almost uniformly Epstein-Barr virus-positive (EBV+) and can cause destructive masses that may mimic more aggressive lymphomas. Clinical outcomes are highly variable, ranging from resolution with withdrawal of immunosuppression to fatal dissemination of disease. Clinical case: A 77-year-old white male with a history of advanced chronic lymphocytic leukemia (CLL) presented with a six-week history of a tender sore on his right lower lip. He had previously been treated with acyclovir without improvement. Physical exam revealed a 1 cm firm tender nodule with overlying crusted erosion on the right lower lip. A deep saucerization biopsy was performed which showed a dense lymphoplasmacytic infiltrate composed primarily of plasmacytoid cells extending into the subcutis. In-situ hybridization for EBV (EBER) was diffusely positive, while CD30, CD20, and Pax-5 immunostains showed only rare clusters of positive cells. The kappa/lambda ratio was 1:10, consistent with lambda light chain restriction. IGH clonality assay failed to detect a monoclonal population. On the basis of these results, a diagnosis of EBV+ polymorphic B-LPD was favored. Unfortunately, the patient expired a few weeks after presentation as a result of complications from his CLL. Conclusion: We report a rare case of cutaneous EBV+ polymorphic B-LPD. Prompt and accurate diagnosis of this entity is important to avoid pursuit of unnecessarily aggressive therapies, prompt workup for undiagnosed immunodeficiency, and minimize the risk of fatal progression.https://scholarlycommons.henryford.com/merf2020caserpt/1108/thumbnail.jp

33714 Palisaded neutrophilic and granulomatous dermatitis in the setting of SRSF2-mutated chronic myelomonocytic leukemia: Case report and review of the literature

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a rare cutaneous histopathologic reaction pattern associated with several underlying disorders. Few cases of PNGD have been associated with hematologic malignancies, in particular with chronic myelomonocytic leukemia (CMML), a malignant hematopoietic disorder with features of myeloproliferative neoplasm and myelodysplastic syndrome. CMML is characterized by peripheral blood monocytosis and bone marrow dysplasia, and can be supported by an acquired clonal cytogenetic abnormality most commonly in TET2, SRSF2, ASXL1, RUNX1, NRAS, and CBL. We present a patient with a papulosquamous rash on the neck, chest, and shoulders with histomorphological features on the spectrum of PNGD. Subsequent lab workup demonstrated a persistent mild monocytosis, raising concern for CMML. The patient was referred to hematology-oncology for a bone marrow biopsy, which ultimately led to her diagnosis. Cytogenic studies of the bone marrow biopsy demonstrated mutations in SRSF2, IDH2, and ASXL1, which were strongly supportive of this diagnosis. After discussion at a multidisciplinary tumor board, treatment directed at the skin eruption alone was recommended. She was started on prednisone taper and demonstrated marked clinical improvement. PNGD in the context of CMML has been scarcely reported, with only 4 prior reports in the literature. Our patient is the fifth reported case, and the fourth case with confirmed underlying SRSF2 mutation. This is likely a novel and reproducible clinical-histopathologic-molecular subtype of reactive granulomatous disease. The findings in this case strengthen the previously made association between PNGD and SRSF2-mutated CMML, and may help better define a unique recognizable subtype for dermatopathologists

Delayed drug hypersensitivity reaction to secukinumab in a patient with hidradenitis suppurativa

A woman in her 30s presented to the dermatology clinic with widespread, pruritic, red papules and plaques involving the ears, trunk and extremities. The rash developed a few days after receiving her second injection of secukinumab, which was initiated for recalcitrant Hurley stage III hidradenitis suppurativa. Investigations revealed a psoriasiform drug hypersensitivity reaction secondary to secukinumab. In this report, we describe the clinical course, histopathological correlation and treatment of this rarely documented reaction

p-16: immunohistochemical staining to differentiate an inflamed atypical nevus

In the monitoring of patients who have had metastatic melanoma, repeat skin exams at specific intervals is a crucial screening tool to prevent recurrence. At many of these visits, suspicious melanocytic lesions are biopsied to determine if they represent a return of the patient’s melanoma. Here, we present a case of a suspicious atypical melanocytic nevus discovered during a skin exam following diagnosis of metastatic melanoma to a lymph node from an unknown primary lesion. To determine whether this lesion was melanoma, p16 immunohistochemical staining was performed of both the lymph node biopsy and the nevus, and provided a reliable means for determining the nature of the nevus. This information would be helpful to readers who care for patients with a history of melanoma who require differentiation of atypical nevi from recurrence of melanoma.https://scholarlycommons.henryford.com/merf2020caserpt/1012/thumbnail.jp

Congenital Midline Nodules on the Chin and Sternum

History: A 5-day old black male full-term neonate born via vacuum-assisted delivery for non-reassuring fetal heart rate presented with congenital presentation of two asymptomatic midline lesions which appeared asymptomatic. There was no history of seizures, ophthalmologic findings, abnormalities in head circumference, height, weight or limb size. Newborn screening examination was unremarkable. Examination: On the midline submental chin there was a soft, brown dome-shaped plaque measuring 0.8-centimeters with a circumferential ring of light brown pigmentation; on the midline upper chest there was a light brown 2-millimeter dome-shaped papule. Course and Therapy: Ultrasound of the submental chin lesion revealed a 0.5 x 0.8 x 0.4-centimeter heterogeneously hypoechoic structure with a peripheral soft tissue rind. Punch biopsies of the submental chin and the midline upper chest revealed haphazardly arranged striated muscle fibers in the dermis, some of which inserted directly into the epidermis. The muscle fibers were highlighted by Masson’s trichrome and myogenin. Alcian blue revealed increased dermal mucin. Discussion: Striated muscle hamartomas (SMH) are rare, benign congenital skin tumors characterized by haphazard arrangement of mature striated skeletal muscle, collagen, nerve bundles, and adipose tissue in the dermal and subcutaneous tissue. Although a rare entity, it is important to recognize this benign hamartoma as a congenital midline defect. Conservative management with clinical monitoring is recommended if cosmetically acceptable, as spontaneous regression over a period of years has been reported. Surgical excision may be pursued; however, the hamartoma may recur.https://scholarlycommons.henryford.com/merf2020caserpt/1133/thumbnail.jp

Clinical Outcomes Associated With Melanocytic Lesions Assessed Via Ancillary Gene Expression Profiling (GEP)

Aims/Objectives: Compare GEP assay prediction of 434 melanocytic lesions with dermatopathologist interpretation. Methods: Sensitivity and specificity of assay were calculated based on disagreement of assay prediction with dermatopathologist interpretation. Histologic features were recorded in disagreeing cases. Results: Eighty-five percent of lesions (369/434) had sufficient RNA for scoring. 74.2% 274/369 lesions were classified as “benign”, 11.9% (44/369) “indeterminate”, and 13.8% (51/369) “malignant”. 38/51 of lesions rendered “malignant” by dermatopathologists were classified “malignant” by assay (sensitivity = 74.5%). Lesions rendered by assay as “benign” but “malignant” by dermatopathologists were more likely to have rarer cytologic features. (13/51) lesions rendered “malignant” by dermatopathologists were classified by assay as “benign,” (4/13) or “indeterminate” (9/13). 270/318 lesions rendered “benign” by dermatopathologists were “benign” by assay (specificity = 84.9%). Of 44/369 “indeterminate” lesions, dermatopathologists rendered 9/44