Ethical and professionalism issues in dermatopathology: A cross-sectional survey of American Society of Dermatopathology Members.

BACKGROUND: Data regarding ethical/professional issues affecting dermatopathologists are lacking despite their importance in establishing policy priorities and educational content for dermatopathology. METHODS: A 14-item cross-sectional survey about ethical/professional issues in dermatopathology was distributed over e-mail to members of the American Society of Dermatopathology from June to September 2019. RESULTS: Two hundred sixteen surveys were completed, with a response rate of 15.3%. Respondents ranked appropriate and fair utilization of healthcare resources (n = 83 or 38.6%) as the most often encountered ethical/professional issue. Conflict of interest was ranked as the most urgent or important ethical/professional issue (n = 83 or 39.3%). One hundred thirty-three (61.6%) respondents felt "somewhat" or "not at all" well equipped to handle ethical dilemmas in practice and 47 (22.8%) respondents identified a major or extreme burden (eg, have considered resigning/retiring) due to ethical challenges. CONCLUSIONS: Areas of priority in ethics and professionalism issues can guide future policy and educational content in dermatopathology.

Acute inflammatory Demodex-induced pustulosis in an immunocompetent patient related to topical steroid use.

Demodex spp. mites are a common colonizer of sebaceous adult skin. Though usually clinically insignificant, demodicosis may be associated with a wide spectrum of skin diseases in immunocompetent hosts, such as erythematotelangiectatic and papulopustular rosacea, Demodex folliculorum, and blepharitis. We present a case of a healthy 9-year-old boy with an exuberant, inflammatory, Demodex-associated pustular eruption of the face, induced by the use of a high-potency topical steroid and successfully treated with oral ivermectin.

Histiocytoid Sweet syndrome harboring an isocitrate dehydrogenase 1 mutation: A case report and retrospective analysis of 29 cases of histiocytoid Sweet syndrome.

Histiocytoid Sweet syndrome (HSS) is a rare histopathologic variant of Sweet syndrome that demonstrates dermal and/or subcutaneous infiltrate with a prominent component of myeloid cells resembling histiocytes. It has been known to occur in association with hematologic neoplasms, including myelodysplastic syndrome (MDS) and acute myelogenous leukemia, but whether it confers an increased risk of such neoplasms is controversial. Here, we describe a case of a HSS that led to the diagnosis of MDS with an isocitrate dehydrogenase 1 (IDH-1) mutation and a corresponding study looking for additional cases of IDH-1 mutations in biopsies of histiocytoid and conventional Sweet syndrome.

Cryptococcus-like changes in the setting of vasculitis.

Cutaneous vasculitis has many underlying causes, and the clinical and histological findings often overlap. Inflammatory vasculitis can mimic infection; however, distinction is critical for the timely institution of appropriate therapy. We present two patients who had generalized polymorphous eruptions whose cutaneous pathology showed vasculitis with unusual haloed yeast-like cells within the inflammatory infiltrate, mimicking Cryptococcus. The unusual cells stained negatively with Gomori methenamine silver and periodic acid-Schiff fungal stains, but positively for CD68 and had cytoplasmic reactivity with antibody to myeloperoxidase (MPO). Both patients had positive serum anti-MPO antibodies. The first patient experienced a rapidly fatal course, whereas the second patient improved with prompt initiation of systemic corticosteroids. Interestingly, the second case had prior biopsy showing Sweet syndrome with crypotoccoid-appearing cells. Cryptococcoid cells have been described previously in association with neutrophilic dermatoses, but not in the setting of vasculitis as was seen in our patients. Our cases add to the existing literature on crypotoccoid mimickers, and are the first to be reported in association with vasculitis.

Acanthamoeba endophthalmitis during treatment for cutaneous disease in a renal transplant patient.

Acanthamoeba infections are difficult to diagnose and treat. We present a renal transplant patient who developed Acanthamoeba endophthalmitis on therapy with posaconazole and miltefosine for cutaneous acanthamobiasis. The patient was maintained on intracameral voriconazole injections, and oral azithromycin, fluconazole, and flucytosine. This case highlights novel presentations and treatments for acanthamoebic infection.

Eyelid syringocystadenoma papilliferum: A novel presentation with major review.

A major review of the literature of syringocystadenoma papilliferum's (SCAP's) presentation and management is presented. In addition, a case report of this unique diagnosis presenting as a corneal abrasion in a 66-year-old-male is included. This benign adnexal tumor of the apocrine glands is most commonly found in the face and neck. When found on the eyelids, these lesions are commonly misdiagnosed as basal cell carcinoma or cysts. Diagnosis is made based on histopathology. Treatment is complete excision of the lesion and this has a low recurrence rate. While predominantly benign, there have been cases of basal cell carcinoma development or other malignant transformations. Of the 26 reported cases of SCAP of the eyelids, none have caused a corneal abrasion. The authors present the only known presentation of eyelid SCAP, causing corneal abrasions, and provide a review of literature with discussion of clinical presentation, natural history, diagnosis, and treatment of this rare, eyelid lesion with potential for malignant transformation.

Acquired Epidermodysplasia Verruciformis and Its Relationship to Immunosuppressive Therapy: Report of a Case and Review of the Literature.

INTRODUCTION: Epidermodysplasia verruciformis (EV) is a rare inherited dermatosis characterized by increased susceptibility to human papilloma virus infection. Acquired EV occurs in patients with compromised cell-mediated immunity, such as patients with HIV and transplant recipients. Optimal management of acquired EV has not yet been established, as cases are rare and are due to a variety of underlying conditions. Additionally, no distinctions have been made between different immunosuppressive medications and their respective link to EV. METHODS AND RESULTS: We report a patient with systemic lupus erythematosus who developed EV while on azathioprine and prednisone. The patient's lesions resolved completely after she was switched from azathioprine to mycophenolate mofetil. Her lesions recurred when her immunosuppressive regimen was again changed from mycophenolate mofetil to methotrexate. A review of the literature revealed azathioprine to be related to other cases of acquired EV. DISCUSSION: This case indicates a possible link between specific immunosuppressive drugs and the development of EV, allowing for new EV treatment considerations. In this case and previous cases, azathioprine is indicated as being particularly linked with the development of EV, while mycophenolate mofetil may be an immunosuppressive option that is less likely to induce EV in patients predisposed to this condition. J Drugs Dermatol. 2017;16(7):701-704.

Development of Halo Nevi in a Lung Cancer Patient: A Novel Immune-Related Cutaneous Event from Atezolizumab.

Immunotherapy-induced vitiligo is an immune-related adverse event (irAE) observed in metastatic melanoma patients treated with immune checkpoint inhibitors that target the cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) and programmed cell death-1 (PD-1) pathways. To date, the development of leukoderma, poliosis, and halo nevi during immunotherapy has largely been reported in metastatic melanoma patients. We report a case of immunotherapy-induced leukoderma presenting as halo nevi in a patient with non-small cell lung cancer (NSCLC) treated with atezolizumab, a programmed cell death ligand (PD-L1) antibody. Immunotherapy-induced vitiligo in metastatic melanoma patients may be associated with improved survival, but it remains to be determined whether its occurrence in non-melanoma cancers has the same prognostic significance.

J Drugs Dermatol. 2017;16(10):1047-1049.

Eruptive angiokeratomas and porokeratosis in the setting of sclerodermatous graft-vs.-host disease.

Porokeratosis and angiokeratomas are both seen in the setting of chronic graft-vs.-host disease (GVHD), but rarely occur together. We present a case of a patient with lichen planus-like (LPL) and sclerodermatous chronic GVHD manifesting after allogeneic bone marrow transplant with concomitant eruptive angiokeratomas and porokeratosis.

Detection of Rickettsia rickettsii, Rickettsia parkeri, and Rickettsia akari in skin biopsy specimens using a multiplex real-time polymerase chain reaction assay.

BACKGROUND: Rickettsia rickettsii, Rickettsia parkeri, and Rickettsia akari are the most common causes of spotted fever group rickettsioses indigenous to the United States. Infected patients characteristically present with a maculopapular rash, often accompanied by an inoculation eschar. Skin biopsy specimens are often obtained from these lesions for diagnostic evaluation. However, a species-specific diagnosis is achieved infrequently from pathologic specimens because immunohistochemical stains do not differentiate among the causative agents of spotted fever group rickettsiae, and existing polymerase chain reaction (PCR) assays generally target large gene segments that may be difficult or impossible to obtain from formalin-fixed tissues. METHODS: This work describes the development and evaluation of a multiplex real-time PCR assay for the detection of these 3 Rickettsia species from formalin-fixed, paraffin-embedded (FFPE) skin biopsy specimens. RESULTS: The multiplex PCR assay was specific at discriminating each species from FFPE controls of unrelated bacterial, viral, protozoan, and fungal pathogens that cause skin lesions, as well as other closely related spotted fever group Rickettsia species. CONCLUSIONS: This multiplex real-time PCR demonstrates greater sensitivity than nested PCR assays in FFPE tissues and provides an effective method to specifically identify cases of Rocky Mountain spotted fever, rickettsialpox, and R. parkeri rickettsiosis by using skin biopsy specimens.

A surprising case of Mycobacterium avium complex skin infection in an immunocompetent patient.

An acute inflammatory nodule of unknown etiology can pose a formidable diagnostic challenge. Here, we highlight the importance of including Mycobacterium avium intracellulare complex (MAC) and other atypical mycobacterial infections in the differential diagnosis of a cutaneous nodule in an immunocompetent individual. We also explore the implications of eczema in the development of a mycobacterial infectious process. We report a case of MAC skin infection in an immunocompetent individual. The patient is a 49-year-old male with a history of dyshidrotic eczema presenting with a fluctuant, non-draining nodule on his right forearm for 2 to 3 weeks, identified by tissue DNA probe to be a cutaneous MAC infection without systemic complications, as serologies and chest X-ray were unremarkable. MAC should be included in the broader differential diagnosis of deep fungal vs atypical mycobacterial skin infections. Nucleic acid-based assays are an important tool in making a definitive diagnosis, allowing for utilization of appropriate therapy for the specific etiologic pathogen. Given the patient's preceding diagnosis of eczema, it is possible that the compromised skin barrier and dampened cytotoxic Th1 activity predisposed the patient to this infection, typically appreciated in the immunosuppressed, warranting further investigation into the relative risk for atypical mycobacterial infections in the setting of eczema.

Chromoblastomycosis Caused by Fonsecaea monophora Mimicking Lichen Planus.

Chromoblastomycosis is a rare fungal infection acquired by traumatic inoculation of pigmented fungi from an environmental source. The polymorphic presentation of chromoblastomycosis may mimic other dermatologic conditions, leading to delays in diagnosis. Thus, histopathology is critical in identifying the presence of fungi and confirming the diagnosis. We present a case of chromoblastomycosis caused by the organism Fonsecaea monophora mimicking a lesion of lichen planus to highlight the importance of histopathology in the diagnosis of this condition.