Sokumbi, Olayemi

AbstractSolid organ and hematopoietic stem cell transplantation may be complicated by the development of post‐transplant lymphoproliferative disorders (PTLDs). The World Health Organization categorizes PTLDs into four entities including non‐destructive, monomorphic, polymorphic, and classical Hodgkin lymphoma types. The most common PTLDs are B‐cell lymphomas, with T‐cell lymphomas accounting for only a few cases. Cutaneous T‐cell lymphomas are rarer still in post‐transplant patients with primary cutaneous peripheral T‐cell lymphoma being an extraordinarily rare subtype in this population. PTLDs may be aggressive and are often associated with high morbidity and mortality. Advances in medicine have led to increased awareness of PTLDs and improved diagnostic tools which assist in the diagnosis of these conditions. However, the clinical and histopathologic heterogeneity of PTLDs may make diagnosis a challenge. In the transplant patient population, the cutaneous manifestations of the lymphoproliferative disease may mimic other conditions, such as eczematous dermatitis and graft‐vs‐host disease. Herein, we report a case of post‐transplant primary cutaneous peripheral T‐cell lymphoma not otherwise specified (PTCL‐NOS) in a pediatric heart transplant patient and describe the clinical presentation and diagnostic histopathologic features.

AbstractBackgroundSpitzoid neoplasms in pediatric patients pose an interesting challenge for clinicians. More data on the clinical, histologic, and molecular characteristics of these lesions are necessary to distinguish features that may portend recurrence or malignant behavior to help determine future treatment guidelines in pediatric patients.MethodsInstitutional Review Board approval was obtained from Children's Hospital of Wisconsin to conduct a retrospective analysis of spitzoid neoplasms. Patients with biopsied or excised spitzoid neoplasms between 01/01/2000 and 08/01/2016 were included. Pertinent clinical and histologic data were collected. Atypical, unusual, or diagnostically uncertain lesions were selected for re‐review.Results266 lesions from 264 patients were included. 243 were classified as benign (91.35%), 22 as atypical (8.27%), and 1 as spitzoid melanoma (0.38%). No clinical or histologic variables were found to be statistically significant between the benign Spitz, atypical Spitz, and spitzoid melanoma cohorts. No known deaths occurred.ConclusionOur findings highlight the extreme variability of spitzoid neoplasms clinically and histologically. Importantly, this study demonstrates that the vast majority of spitzoid neoplasms in pediatric populations are benign and supports conservative management of spitzoid lesions in children.

Abstract: Common variable immunodeficiency disorder is a primary immunodeficiency disorder characterized by reduced levels of serum immunoglobulins and impaired antibody response. This condition may be associated with development of noninfectious granulomatous dermatitis of the skin which may be disfiguring and destructive. There are no published guidelines for the treatment of cutaneous granulomas in this patient population. In recent studies, rubella virus–positive cells in granulomas were localized to M2 macrophages which have an important role in wound healing and the secretion of immunoregulatory cytokines. We present a case of treatment-refractory, disfiguring common variable immunodeficiency disorder–associated granulomatous dermatitis. Immunofluorescence microscopy of the biopsy specimen confirmed the presence of rubella vaccine capsid proteins in M2 macrophages within the granuloma, a newly recognized phenomenon in this patient population. This knowledge may serve to identify future therapeutic targets or preventative strategies for granulomatous dermatitis in patients with primary immunodeficiency disorder.

AbstractPrimary cutaneous follicle center cell lymphoma is the most prevalent type of primary cutaneous B‐cell lymphoma and usually portends a favorable prognosis. Typically, the diagnosis can be rendered based on characteristic histopathologic features and immunohistochemical profile. Rarely, a diagnostically challenging variant with a predominant spindle morphology mimicking other malignant spindle cell neoplasms may be encountered. Even more unusual is the presence of a prominent myxoid stroma in this rare sarcomatoid variant of follicle center cell lymphoma. Herein, we present a case of a 52‐year‐old man with a slowly enlarging cyst‐like lesion on the chin with histopathologic examination revealing a malignant, predominantly spindled neoplasm within an abundant myxoid stroma. Following a broad panel of immunohistochemical stains, the strong positive staining of the spindle cells for LCA (CD45), CD20, and Bcl‐6 confirmed the diagnosis of follicle center cell lymphoma. We present this distinctive rare subtype of cutaneous follicle center cell lymphoma to increase awareness of this variant and to discuss challenging histopathologic mimics to consider while highlighting the utility of immunohistochemistry stains to avoid misdiagnosis.

Objective Epidemiologic studies comparing the incidence and prevalence of systemic lupus erythematosus (SLE) and isolated cutaneous lupus erythematosus (CLE) are few. Olmsted County, Minnesota provides a unique setting for such a study owing to resources of the Rochester Epidemiology Project. We sought to describe and compare the incidence and prevalence of SLE and CLE from 1993–2005. Methods SLE cases were identified from review of medical records and fulfilled the 1982 American College of Rheumatology classification criteria. CLE cases included patients with classic discoid lupus erythematosus, subacute CLE, lupus panniculitis, and bullous lupus erythematosus. Age‐ and sex‐adjusted incidence and prevalence were standardized to the 2000 US white population. Results The age‐ and sex‐adjusted incidence of SLE (2.9 per 100,000; 95% confidence interval [95% CI] 2.0–3.7) was similar to that of CLE (4.2 per 100,000; 95% CI 3.1–5.2, P = 0.10). However, the incidence of CLE was 3 times higher than SLE in men (2.4 versus 0.8 per 100,000; P = 0.009). The age‐ and sex‐adjusted prevalence of CLE on January 1, 2006 was higher than that of SLE (70.4 versus 30.5 per 100,000; P < 0.001). The prevalences of CLE and SLE in women were similar, but the prevalence of CLE was higher in men than in women (56.9 versus 1.6 per 100,000; P < 0.001). The incidence of CLE rose steadily with age and peaked at 60–69 years. Conclusion The incidences of CLE and SLE are similar, but CLE is more common than SLE in men and in older adults. These findings may reflect differences in genetic or environmental etiology of CLE.

AbstractBackgroundVarious components of the skin biopsy requisition form (SBRF) may contribute to accurate dermatopathologic interpretation.MethodsA search of electronic databases, including those of Ovid MEDLINE In‐Process & Other Non‐Indexed Citations, Ovid MEDLINE, Ovid EMBASE, the Cochrane Database of Systematic Reviews, the Cochrane Central Register of Controlled Trials, and Scopus, was conducted from inception to October 2011. Two authors independently screened all articles for eligibility. Inclusion criteria required material to represent original studies on skin biopsy and pathology requisition forms. Data abstracted from each article that met the inclusion criteria included details of the study characteristics, including the study location, type of pathology practice, specimen type, type of dermatoses, medical specialty of the requesting provider, suggested clinical components, and format of the SBRF.ResultsOf 32 titles and abstracts reviewed, seven articles were included. From these, we determined that dermatologists, general practitioners and surgeons completed SBRFs. Commonly included components were patient demographics and requesting clinician characteristics. Clinical information and differential diagnosis were provided in 4% (two of 48 surgeons) to 36% (18 of 50 dermatologists) of requisitions. Most SBRFs did not include information on specimen type, clinical morphology, photographs or clinical history.ConclusionsThe limited medical literature demonstrates variation in the content of SBRFs across clinicians and practices, and suggests an important target for improvement in the quality of communication and dermatologic care by requesting clinicians and pathologists.

BackgroundCutaneous granulomatous inflammation can occur in patients with T‐cell lymphoma and Hodgkin disease. We describe the unusual microscopic pattern of a granuloma annulare (GA)‐like eruption co‐existing with B‐cell chronic lymphocytic leukemia (B‐CLL).MethodsWe reviewed the histopathology and immunophenotype of skin biopsies from two patients with B‐CLL and cutaneous lesions resembling GA.ResultsBoth patients had symptomatic cutaneous lesions clinically resembling GA; one had lesions refractory to standard dermatologic therapy. Histopathology showed GA‐like palisaded histiocytic infiltration, with subtle collections of lymphocytes interspersed among the granulomatous inflammation. Immunohistochemistry showed strong expression of CD20 and CD79a, with aberrant CD5 co‐expression, confirming cutaneous involvement by B‐CLL.ConclusionsCo‐existence of a GA‐like infiltrate and cutaneous B‐CLL raises the possibility that granulomatous inflammation occurs as a secondary response to dermal infiltration by leukemic cells. Because histopathologic findings can be subtle, knowledge of this association is essential to avoid overlooking the diagnosis. Regardless of whether histopathology reflects a reactive or primary phenomenon, documentation of cutaneous involvement by B‐CLL may serve as a rationale for specific treatment of the underlying B‐CLL in patients with skin lesions unresponsive to dermatologic therapy and for whom there is no other justification for leukemia‐targeted therapy.