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Joseph M Jaworski,1,2 Vanlila K Swami,1 Rebecca C Heintzelman,1 Carrie A Cusack,3 Christina L Chung,3 Jeremy Peck,3 Matthew Fanelli,3 Micheal Styler,4 Sanaa Rizk,4 J Steve Hou1 1Department of Pathology and Laboratory Medicine, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA; 2Department of Pathology, Mercy Fitzgerald Hospital, Darby, PA, USA; 3Department of Dermatology, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA; 4Department of Hematology/Oncology, Hahnemann University Hospital/Drexel University College of Medicine, Philadelphia, PA, USA Abstract: Blastic plasmacytoid dendritic cell neoplasm is an uncommon malignancy derived from precursors of plasmacytoid dendritic cells. Nearly all patients present initially with cutaneous manifestations, with many having extracutaneous disease additionally. While response to chemotherapy initially is effective, relapse occurs in most, with a leukemic phase ultimately developing. The prognosis is dismal. While most of the clinical and pathologic features are well described, the association and possible prognostic significance between peripheral blood absolute monocytosis (>1.0 K/µL) and blastic plasmacytoid dendritic cell neoplasm have not been reported. We report a case of a 68-year-old man who presented with a rash for 4–5 months. On physical examination, there were multiple, dull-pink, indurated plaques on the trunk and extremities. Complete blood count revealed thrombocytopenia, absolute monocytosis of 1.7 K/µL, and a negative flow cytometry study. Biopsy of an abdominal lesion revealed typical features of blastic plasmacytoid dendritic cell neoplasm. Patients having both hematologic and nonhematologic malignancies have an increased incidence of absolute monocytosis. Recent studies examining Hodgkin and non-Hodgkin lymphoma patients have suggested that this is a negative prognostic factor. The association between blastic plasmacytoid dendritic cell neoplasm and absolute monocytosis has not been described and may, in fact, have prognostic value. Keywords: blastic, plasmacytoid, dendritic, monocytosis

Skin cancer is the most common malignancy affecting solid organ transplant recipients (SOTR), and SOTR experience increased skin cancer-associated morbidity and mortality. There are no formal multidisciplinary guidelines for skin cancer screening after transplant, and current practices are widely variable. We conducted three rounds of Delphi method surveys with a panel of 84 U.S. dermatologists and transplant physicians to establish skin cancer screening recommendations for SOTR. The transplant team should risk stratify SOTR for screening, and dermatologists should perform skin cancer screening by full-body skin examination. SOTR with a history of skin cancer should continue regular follow-up with dermatology for skin cancer surveillance. High-risk transplant patients include thoracic organ recipients, SOTR age 50 and above, and male SOTR. High-risk Caucasian patients should be screened within 2 years after transplant, all Caucasian, Asian, Hispanic, and high-risk African American patients should be screened within 5 years after transplant. No consensus was reached regarding screening for low-risk African American SOTR. We propose a standardized approach to skin cancer screening in SOTR based on multidisciplinary expert consensus. These guidelines prioritize and emphasize the need for screening for SOTR at greatest risk for skin cancer., (© 2019 Steunstichting ESOT.)

Background: Because most of the US population will consist of nonwhite individuals by the year 2043, it is essential that both physicians and patients are educated about skin cancer in nonwhite persons., Objective: To update the epidemiology, investigate specific risk factors, and facilitate earlier diagnosis and intervention of keratinocyte carcinoma in nonwhite individuals., Methods: Institutional review board-approved retrospective chart review of all nonwhite patients who had received a biopsy-proven diagnosis of skin cancer at Drexel Dermatology during June 2008-June 2015., Results: Squamous cell carcinoma (SCC) was the most commonly diagnosed skin cancer in black and Asian populations, and basal cell carcinoma was the most common skin cancer in Hispanics. Black persons exhibited the majority of their SCC lesions in sun-protected areas, particularly the anogenital area. On average, current smokers received skin cancer diagnoses 12.27 years earlier than former smokers and 9.36 years earlier than nonsmokers., Limitations: Single-center design and interpractitioner variability of skin examination., Conclusion: The importance of lesions in photoprotected areas in nonwhite individuals should not go overlooked. However, emphasis should also be placed on active examination of sun-protected areas in nonwhite persons and recognition of the relationship between human papillomavirus and genital SCC lesions. Smoking cessation should be integrated in dermatologic counseling of all patients. Interventions tailored to each of these ethnic groups are needed., (Copyright © 2019 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Sclerosing perineuromas are rare, benign peripheral nerve sheath tumors classically reported on the fingers and palms of young men. We present the case of a 12-year-old boy with a slow-growing nodule on his right knee. Excision was performed, and pathology was consistent with a sclerosing perineuroma. This case highlights an atypical presentation of a rare lesion and provides useful knowledge of the clinical scenarios in which sclerosing perineuromas should be included in a differential diagnosis.

Background: Moonlighting refers to the practice of medicine outside one's training institution in exchange for financial compensation. High medical debt-to-income ratios drive residents to seek additional compensation during residency., Objective: To gather information to establish the current practices of moonlighting and to better understand the thoughts and experiences of dermatology residency program directors regarding moonlighting., Methods: All allopathic and osteopathic dermatology residency program directors in the United States and Puerto Rico received a blinded survey between February 1, 2017 and April 1, 2017 through an email link., Results: Response rate was 47.0%. Of the programs that responded, 63.16% allowed moonlighting. In three regions, 100% of programs allowed moonlighting. The geographic area with the lowest percentage of programs permitting moonlighting was New England with 25%., Limitations: This survey only reflects the field of dermatology and beliefs/policies of program directors., Conclusion: This survey highlighted that training programs allowing moonlighting tend to have a more positive outlook on the practice than programs who do not. Results revealed trends that suggest that states in regions with less access to dermatologic care were more inclined to allow moonlighting.

Importance: The risk for skin cancer has been well characterized in white organ transplant recipients (OTRs); however, most patients on the waiting list for organ transplant in the United States are nonwhite. Little is known about cutaneous disease and skin cancer risk in this OTR population., Objective: To compare the incidence of cutaneous disease between white and nonwhite OTRs., Design, Setting, and Participants: This retrospective review of medical records included 412 OTRs treated from November 1, 2011, through April 22, 2016, at an academic referral center. Prevalence and characteristics of cutaneous disease were compared in 154 white and 258 nonwhite (ie, Asian, Hispanic, and black) OTRs. Clinical factors of cutaneous disease and other common diagnoses assessed in OTRs included demographic characteristics, frequency and type of cancer, anatomical location, time course, sun exposure, risk awareness, and preventive behavior., Main Outcomes and Measures: Primary diagnosis of malignant or premalignant, infectious, and inflammatory disease., Results: The 412 patients undergoing analysis included 264 men (64.1%) and 148 women (35.9%), with a mean age of 60.1 years (range, 32.1-94.3 years). White OTRs more commonly had malignant disease at their first visit (82 [67.8%]), whereas nonwhite OTRs presented more commonly with infectious (63 [37.5%]) and inflammatory (82 [48.8%]) conditions. Skin cancer was diagnosed in 64 (41.6%) white OTRs and 15 (5.8%) nonwhite OTRs. Most lesions in white (294 of 370 [79.5%]) and Asian (5 of 6 [83.3%]) OTRs occurred in sun-exposed areas. Among black OTRs, 6 of 9 lesions (66.7%) occurred in sun-protected areas, specifically the genitals. Fewer nonwhite than white OTRs reported having regular dermatologic examinations (5 [11.4%] vs 8 [36.4%]) and knowing the signs of skin cancer (11 [25.0%] vs 10 [45.4%])., Conclusions and Relevance: Early treatment of nonwhite OTRs should focus on inflammatory and infectious diseases. Sun protection should continue to be emphasized in white, Asian, and Hispanic OTRs. Black OTRs should be counseled to recognize the signs of genital human papillomavirus infection. Optimal posttransplant dermatologic care may be determined based on the race or ethnicity of the patients, but a baseline full-skin assessment should be performed in all patients. All nonwhite OTRs should be counseled more effectively on the signs of skin cancer, with focused discussion points contingent on skin type and race or ethnicity.

The authors present the immunopathologic findings of Toll-like receptor 2 in psoriasis. This novel work shows positive staining within the dermal capillaries in psoriatic lesions. Neither normal skin nor lesional skin in eczema showed similar staining. The authors postulate how the activation of this innate immune system reactant plays a significant role in psoriasis and show how it may be associated with a cascade of events that begins with streptococcus and ends with psoriasis.

Importance: Organ transplant recipients have a higher incidence of skin cancer. This risk is magnified over time and with continued exposure to immunosuppression. Skin cancer in nonwhite patients is associated with greater morbidity and mortality owing to diagnosis at a more advanced stage, which suggests that nonwhite organ transplant recipients are at even higher risk., Objective: To describe demographic and clinical factors and the incidence of skin cancer in nonwhite organ transplant recipients., Design, Setting, and Participants: We performed a retrospective medical record review of patients who were organ transplant recipients (154 were white and 259 nonwhite [black, Asian, Hispanic, Pacific Islander]) seen from November 1, 2011, to April 18, 2016 at an academic referral center., Main Outcomes and Measures: Variables were analyzed and compared between racial groups, including sex, age, race/ethnicity, Fitzpatrick type, type and location of skin cancer, type of organ transplanted, time to diagnosis of skin cancer after transplantation, and history of condyloma acuminata and/or verruca vulgaris., Results: Most of the 413 patients (62.7%) evaluated were nonwhite organ transplant recipients; 264 were men, and 149 were women. Their mean (SD) age was 60.09 (13.59) years. Nineteen skin cancers were identified in 15 patients (5.8%) representing 3 racial/ethnic groups: black (6 patients), Asian (5), and Hispanic (4). All squamous cell carcinomas in blacks were diagnosed in the in situ stage, located on sun-protected sites, and occurred in patients whose lesions tested positive for human papilloma virus (HPV) and/or who endorsed a history of condyloma acuminata or verruca vulgaris. Most skin cancers in Asians were located on sun-exposed areas and occurred in individuals who emigrated from equatorial locations., Conclusions and Relevance: Nonwhite organ transplant recipients are at risk for developing skin cancer posttransplantation. Follow-up in a specialized transplant dermatology center and baseline total-body skin examination should be part of posttransplantation care in all organ transplant recipients, including nonwhite patients. A thorough inspection of the groin and genitalia is imperative in black organ transplant recipients. History of HPV infection, particularly in black organ transplant recipients, and sun exposure/emigration history in Asian organ transplant recipients should be documented. Vigilant photoprotection may be of lesser importance in the prevention of skin cancer in black organ transplant recipients. Risk factors for nonwhite organ transplant recipients differ between races/ethnicities and warrant further study in efforts to better counsel and prevent skin cancer in these patients.

Importance: Immunosuppressed patients with solid organ transplants have an increased risk for nonmelanoma skin cancer. Vismodegib has been reported to be effective for select locally advanced or metastatic basal cell carcinomas. However, there is no data documenting the use and safety of vismodegib in immunosuppressed organ transplant patients., Observations: We describe a 78-year-old white man with a history of orthotopic heart transplant, immunosuppressed with low-dose cyclosporine, who presented to a specialty dermatology transplant clinic with multiple, recurrent, locally aggressive facial basal cell carcinomas. Through a multidisciplinary approach, the patient was started on vismodegib therapy. The pharmacokinetics of cyclosporine in the setting of vismodegib administration and weekly monitoring of cyclosporine levels ensured that therapeutic immunosuppression levels were achieved without toxic effects., Conclusions and Relevance: To our knowledge, this is the first report that details vismodegib use in an immunosuppressed heart transplant patient receiving cyclosporine therapy. With a growing immunosuppressed organ transplant population at high risk for basal cell carcinoma, therapeutic options for locally advanced or metastatic disease are limited. Vismodegib appears to be a safe option for patients receiving cyclosporine therapy with routine monitoring. Future research is needed to evaluate the safety profile of vismodegib with other immunosuppressive agents.

The purpose of this article is to review the common neoplasms, infections, and inflammatory dermatoses that may present around or near the mouth. Dental professionals are well positioned to evaluate perioral skin conditions, further contributing to patients' general health. This article includes a review of seborrheic keratosis, warts, actinic keratoses, actinic cheilitis, and squamous cell carcinoma, among several other perioral cutaneous lesions., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Importance: Atopic dermatitis (AD) is thought to be a double-hit phenomenon with an unknown environmental component and a genetic abnormality likely centered on the filaggrin gene. Biologically, the presence of Staphylococcus aureus in AD was reported more than 2 decades ago, but the relationship to AD has been elusive., Objective: To explore the bacteria that produce the biofilms in the lesions of AD and the response of the innate immune system to these biofilm occlusions of the sweat ducts by specifically evaluating Toll-like receptor 2., Design, Setting, and Participants: University hospital dermatologic clinic study involving the environmental component related to the characterization, correlation, and impact of staphylococci and their biofilms in AD. We processed routine skin swabs from lesional and nonlesional skin from 40 patients with AD and performed scrapings and biopsies. We also obtained 20 samples from controls (10 inflamed skin samples and 10 normal skin samples)., Exposures: Gram staining, bright-field microscopy, hematoxylin and eosin, periodic acid-Schiff, Congo red, and light microscopy., Main Outcomes and Measures: Association of staphylococcal biofilms with AD pathogenesis., Results: All AD-affected samples contained multidrug-resistant staphylococci, with S aureus (42.0%) and Staphylococcus epidermidis (20.0%) as the predominant species. All isolates were positive for extracellular polysaccharide and biofilm (85.0% strong biofilm producers and 15.0% moderately to weakly positive). Polymerase chain reaction revealed the biofilm-mediating icaD (93.0%) and aap (12.5%) genes in the isolates (some contained both). We also examined tissues for microbial identification, extracellular biomass formation, biofilm formation, and staphylococcal biofilm in skin tissues. Occlusion of sweat ducts with periodic acid-Schiff-positive and Congo red-positive material was noted on microscopic tissue examination. Toll-like receptor 2 was shown to be activated in AD lesional skin (immediately proximal to the sweat ducts), which likely led to the initiation of proteinase-activated receptor 2-mediated pruritus and MyD88-mediated spongiosis., Conclusions and Relevance: Biofilm formation by AD-associated staphylococci almost certainly plays a major role in the occlusion of sweat ducts and leads to inflammation and pruritus. We believe the environmental hit in AD relates to staphylococci and their biofilms, which occlude sweat ducts.

Pigmentary demarcation lines are common, especially in individuals with darker skin types. Type B pigmentary demarcation lines (Futcher lines) involve the posterior lower extremities and can be associated with pregnancy, often with spontaneous resolution after delivery. The pathogenesis of pigmentary demarcation lines remains unknown; however, neurogenic inflammation and/or mosaicism are thought to play a role in their development. We report a case of type B pigmentary demarcation lines in pregnancy and provide a review of the literature.

Importance: In 1947, Sulzberger and colleagues published a micrograph of a blocked acrosyringium in a patient with atopic dermatitis (AD), believing that it had a large role in the disease process. Lacking appropriate probes, they could not confirm the finding., Objective: To confirm the observations by Sulzberger et al on the blockage of sweat ducts in AD in pathologic specimens., Design and Setting: Biopsy specimens diagnostic of various inflammatory diseases and with a secondary differential diagnosis of eczema were evaluated at an academic medical center., Exposures: Evidence of ductal obstruction in each specimen was examined following staining with hematoxylin-eosin, periodic acid-Schiff, and Gram stain., Main Outcomes and Measures: Comparison of biopsy specimens with control specimens and additional controls consisting of noninflamed skin., Results: Using 36 biopsy specimens, this study confirmed the observations by Sulzberger et al on the blockage of sweat ducts in AD. Blocked acrosyringia were noted in each specimen on routine staining with hematoxylin-eosin. The study also confirmed the findings by earlier investigators about the blockage of sweat ducts in miliaria, showing eosinophilic material in the ducts that was positive for periodic acid-Schiff. Previous researchers also observed bacteria in the blockages, and this study demonstrated the same findings in AD, rather than miliaria., Conclusion and Relevance: Subclinical miliaria may be the earliest change in AD and likely initiates the process that causes intense pruritus.

Coma blisters (CBs) are self-limited lesions that occur in regions of pressure during unconscious states classically induced by barbiturates. We report a case of CBs sans coma that were histologically confirmed in a 41-year-old woman who developed multiple tense abdominal bullae with surrounding erythema following a transatlantic flight. Interestingly, the patient was fully conscious and denied medication use or history of medical conditions. A clinical diagnosis of CBs was confirmed by histopathologic findings of eccrine gland necrosis, a hallmark of these bulIous lesions.

Cutaneous and systemic plasmacytosis (C/SP), human herpes virus-8 (HHV8), negative multicentric plasmacytic Castleman disease (MPCD), and idiopathic plasmacytic lymphadenopathy are polyclonal plasma cell proliferations of unknown etiology that predominantly affect Asian individuals. Herein, we present our experience with a Vietnamese man with typical C/SP limited to the skin but, after 10 years, may have developed perirenal involvement, and with a white man with human immunodeficiency virus and HHV8 negative MPCD with involvement of skin, lymph nodes, and kidneys at presentation, and who later succumbed to gastric carcinoma. Based on a review of the literature, we suggest that C/SP, cutaneous MPCD, and idiopathic plasmacytic lymphadenopathy with skin involvement are part of a continuum rather than distinct entities and, as such, may be regarded as variants of HHV8-negative MPCD. Although the majority of patients with C/SP run a chronic benign course, special attention should be given to monitoring for pulmonary and renal involvement. We hypothesize that long-lived plasma cells originate and survive in the environment of the skin akin to other stromal "survival" niches due to the local production of interleukin 6 and that such patients might respond to agents that interfere with interleukin-6 activity., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Palisaded and neutrophilic granulomatous dermatitis (PNGD) has been associated with many conditions including rheumatoid arthritis, systemic lupus erythematosus, systemic vasculitis, and other diseases with circulating immune complexes. Lymphoproliferative conditions, bacterial endocarditis, and various drugs can also induce this condition. Many patients also have symmetric polyarthritis with various serological abnormalities. We present a case of a 46-year-old female who presented with painful erythematous annular plaques and nodules on her legs. The lesions started a week prior to visit and increased in number over the course of the week. The patient had an established history of sarcoidosis with past episodes of uveitis and erythema nodosum. The histopathological findings included a diffuse pandermal infiltrate mostly composed of neutrophils, nuclear debris, and strands of deeply eosinophilic degenerated collagen. Vasculitis was not present. No significant increase in dermal mucin was detected. Based on the clinical and pathological findings, the patient was diagnosed with late-stage PNGD. To our knowledge, this is the first case of PNGD described in an adult patient of sarcoidosis., (Copyright © 2010 John Wiley & Sons A/S.)

Background: Human papillomavirus (HPV) has been implicated in the development of digital squamous cell carcinoma (SCC). Case reports in the literature mostly identify HPV type 16 present in tumors, but HPV types 2, 31, 34, 35, and 73 have also been isolated., Methods: Two cases of digital SCC associated with HPV 16 in young African-American men are presented., Results and Conclusions: Digital SCC associated with HPV may be difficult to evaluate and treat, particularly in African-Americans and patients with human immunodeficiency virus (HIV). We discuss the need for careful evaluation, treatment, and follow-up of these individuals.

Actinic lichen nitidus is a unique photoinduced lichenoid eruption that displays histologic features of classic lichen nitidus, with some clinical similarity. It is seen most commonly in deeply pigmented patients (Fitzpatrick skin types V and VI), in photodistributed areas, and during the summer months. Sun avoidance and topical corticosteroids are the mainstay of therapy; however, seasonal recurrences are common. Actinic lichen nitidus has unique properties that differentiate it from classic lichen nitidus. Confusion exists in the literature regarding the nomenclature of this entity, and it appears to be an underrecognized disease in the United States.

Dermatitis herpetiformis (DH) is a chronic pruritic cutaneous eruption associated with gluten-sensitive enteropathy (celiac disease [CD]) and immunoglobulin A (IgA) deposition in the skin. While the disease is not uncommon among adolescents, DH is rarely seen in prepubertal patients. Children with DH present similarly to adults; however, uncommon skin findings have been reported. Because of an increased risk for autoimmune diseases and lymphoma, accurate diagnosis and treatment are imperative. We present a case of DH in a 6-year-old Latino boy previously diagnosed with atopic dermatitis and recurrent urticaria. Our aim is to highlight the various cutaneous presentations of DH and encourage clinicians to consider this diagnosis in young patients with recalcitrant atypical skin disease.

Paroxetine (Paxil) is a widely prescribed antidepressant of the selective serotonin reuptake inhibitor (SSRI) class for which multiple cutaneous adverse effects have been reported, including urticaria, ecchymoses, vasculitis, and pruritus. In contrast, fluoxetine (Prozac) is the only SSRI previously reported to cause urticarial vasculitis. Urticarial vasculitis is a chronic disorder marked by recurrent episodes of erythematous, indurated wheals that histologically manifest the features of leukocytoclastic vasculitis. This case represents the first reported incidence of paroxetine-induced urticarial vasculitis, and highlights the need to consider the entire SSRI class as s as a potential cause of the condition.

Wells syndrome, also known as eosinophilic cellulitis, is an uncommon condition whose etiology often remains a mystery. Patients present with recurrent cutaneous swellings that are often cellulitic in appearance. Histopathologic evaluation of the skin lesions reveals a dense dermal eosinophilic infiltrate, marked edema, and characteristic "flame figures". Notably, the picture is devoid of vasculitis. Therapy with low-dose systemic steroids has proven variably successful. Clinical evidence lending support for the efficacy of other medications has been, for the most part, anecdotal. We present a case of Wells syndrome, review the literature, and discuss therapeutic options.

Background: Polymorphous light eruption (PMLE) is the most common chronic idiopathic photodermatosis usually manifesting as a papular eruption (3-6 mm), with several other morphological variants described., Methods: Between June 1998 and August 2001, nine patients presented with complaints of a pruritic pinpoint papular eruption associated with sun exposure. A detailed history and complete skin examination were performed along with a skin biopsy if active lesions were present. Phototesting to ultraviolet-A (UV-A), ultraviolet-B (UV-B) and visible light was performed in four patients. Antinuclear antibody (ANA) testing was performed in three patients. The diagnosis of PMLE was made based on the history, morphology of the lesions, results of phototesting and skin biopsy if available., Results: In all patients, pinpoint papules (1-2 mm) were observed on sun-exposed areas, sparring the face and flexural surfaces. All patients were African American women with skin type IV-VI and a mean age of 39.3 years (range 21-52 years). Phototest results were normal in three patients; one patient, who was on glyburide, had a decreased minimal erythema dose to UV-A. ANA testing was negative. Two histopathologic patterns were observed: (i) focal lichenoid and perivascular lymphohistiocytic infiltrate with red blood cell extravasation in four specimens and (ii) superficial and deep interstitial lymphocytic infiltrate with papillary dermal edema in the remaining three specimens. All patients responded to topical corticosteroids, broad-spectrum sunscreens and antihistamines., Conclusion: Recognition of this pinpoint papular variant of PMLE in dark-skinned individuals is important in the evaluation and management of these patients.