Amrita Goyal

PD‐1, S‐100 and CD1a expression in pseudolymphomatous folliculitis, primary cutaneous marginal zone B‐cell lymphoma (MALT lymphoma) and cutaneous lymphoid hyperplasia

Pseudolymphomatous folliculitis is a lymphoid proliferation that clinically and histopathologically mimics primary cutaneous extranodal marginal zone lymphoma of mucosa‐associated lymphoid tissue (MALT lymphoma). In this study, we assessed the diagnostic value of three immunohistochemical markers, programmed death‐1 (PD‐1), CD1a and S100.

Granulomatous reaction to liquid injectable silicone for gluteal enhancement: review of management options and success of doxycycline

Liquid injectable silicone (LIS) for cosmetic purposes has been widely available and commonly used for almost half a century. An increase in buttock augmentation procedures because of patients wanting to emulate the look of some celebrities has recently highlighted cases in the media of unsafe administration of liquid silicone injections. Severe complications including death have been reported. In this article, we report a case in which liquid silicone was injected in the patients gluteal tissue resulting in granulomatous reactions in the surrounding tissues. The patient responded well to doxycycline, presumably because of both its antimicrobial properties and immunomodulatory effects.

Igg4 Expression in Primary Cutaneous Marginal Zone Lymphoma: A Multicenter Study

Background: Primary cutaneous marginal zone lymphoma (PCMZL) is the second most common B-cell lymphoma of the skin. A recent study has demonstrated a strikingly high prevalence of immunoglobulin (Ig)G4 expression in PCMZL with plasmacytic differentiation. Objective: The objective was to investigate the incidence of IgG4 expression in PCMZL, and its correlation with clinical and immunophenotypic features. Materials and Methods: Multicenter study that utilized immunohistochemistry and in-situ hybridization to evaluate the expression of IgG4, Ig light (&kgr; and &lgr;), and heavy chains (IgM, IgG), and the ratio of T (CD3+) and B (CD20+) cells in biopsy specimens from 30 patients with PCMZL and to correlate these findings with the clinical features. Results: IgG4 expression was observed in 4 out of 30 patients (13%) with PCMZL. Patients with IgG4-positive lymphomas were 57 to 77 years of age (mean, 69) at biopsy. The lesions were solitary in 2 patients with IgG4-positive lymphomas, and were most commonly located on the trunk. Patients with IgG4-negative lymphomas experienced earlier disease onset at an average age of 53 years. The majority of the IgG4-negative cases presented with localized disease, on the trunk and upper extremities. There was no significant difference in the IgG4-positive versus negative cases for the following parameters: Ig &kgr; or &lgr; restriction, B-cell or T-cell predominance, and site of the lesions. Conclusions: IgG4 expression was observed in a minority of PCMZL patients. We did not identify significant clinical or immunophenotypic differences between IgG4 positive and negative cases.

Cytokeratin 17 is highly sensitive in discriminating cutaneous lymphadenoma (a distinct trichoblastoma variant) from basal cell carcinoma.

Cutaneous lymphadenoma (CL) is rare neoplasm that clinically and histologically resembles basal cell carcinoma (BCC). CL, composed of dermal basaloid epithelial islands with prominent admixed lymphocytes, characteristically contains cytokeratin 20 (CK20)‐positive Merkel cells (MCs). However, CK20 may be of limited use because of low MC density in small samples. CK17 is expressed diffusely throughout BCC. We investigated the discriminatory utility of CK17 and CK20 in CL and BCC.

Adult T-Cell Leukemia/Lymphoma

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive peripheral T-cell lymphoma caused by long-term infection with human T-lymphotropic virus 1 (HTLV-1). Patients with ATLL have an extremely poor prognosis, their disease complicated by multidrug resistance of malignant cells, infection due to impaired T-cell mediated immunity, hypercalcemia, and multiple organ failure. Half of patients develop cutaneous manifestations. The cutaneous lesions of ATLL are highly varied and include erythroderma, purpuric papules, and plaques. This chapter addresses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, and molecular characteristics of this disease. It closes with a differential diagnosis and clinical case.

Differential Expression of Hedgehog and Snail in Cutaneous Fibrosing Disorders: Implications for Targeted Inhibition

OBJECTIVES To examine Hedgehog signaling in cutaneous fibrosing disorders for which effective approved therapies are lacking, expand our knowledge of pathophysiology, and explore the rationale for targeted inhibition. METHODS Stain intensity and percentage of cells staining for Sonic hedgehog (Shh), Indian hedgehog (Ihh), Patched (Ptch), glycogen synthase kinase 3 β (GSK3-β), β-catenin, and Snail were evaluated in human skin biopsy specimens of keloid, hypertrophic scar (Hscar), scleroderma, nephrogenic systemic fibrosis (NSF), scar, and normal skin using a tissue microarray. RESULTS Ihh, but not Shh, was detected in a significantly larger proportion of cells for all case types. Ptch, GSK3-β, and β-catenin showed a gradient of expression: highest in NSF and keloid; moderate in normal skin, scar, and Hscar; and lowest in scleroderma. Snail expression was binary: low in normal skin but high in all fibrosing conditions studied. CONCLUSIONS Differential overexpression of Hedgehog and Snail in cutaneous fibrosing disorders demonstrates a role for targeted inhibition. Ptch, GSK3-β, and β-catenin can help differentiate scleroderma from NSF in histologically subtle cases. Differences in expression between keloid and hypertrophic scar support the concept that they are pathophysiologically distinct disorders. Our findings implicate Snail as a target for the prevention of fibrogenesis or fibrosis progression and may offer a means to assess response to therapy.

Introduction to the B-Cell Lymphomas

The human body contains a vast array of B cells, with phenotypes ranging from the naive B cell to the centrocyte, centroblast, immunoblast, plasmablast, and plasma cell. Each of these B-cell subsets has different functional, proliferative, and migratory capacities, and the diversity of cutaneous B-cell lymphoma (CBCL) reflects the variety of non-neoplastic B-cell counterparts. The WHO-EORTC classification [1] recognizes four cutaneous B-cell lymphomas: primary cutaneous marginal zone lymphoma (pcMZL or MALT lymphoma), primary cutaneous follicle-center lymphoma (pcFCL), primary cutaneous diffuse large B-cell lymphoma, leg-type (pcDLBCL), and intravascular large B-cell lymphoma (ivLBCL). Here we offer a schema for understanding and distinguishing these lymphomas. Each lymphoma is addressed individually in subsequent chapters.

Basaloid Carcinoma of the Breast Mimicking Cutaneous Basaloid Neoplasms.

Basaloid carcinoma of the breast (BCB) is a rare, triple-negative aggressive primary breast tumor that can closely mimic cutaneous basal cell carcinoma (BCC), neuroendocrine tumors, adnexal neoplasms, and other primary breast tumors. Accurate diagnosis of this tumor is critical for appropriate clinical management. We add to the literature 2 female patients with BCB presenting with a nipple mass. Histopathologic findings from both patients showed dermal nests and cords of atypical basaloid cells with epidermal involvement, closely resembling cutaneous BCC. A panel of immunohistochemical stains, including the novel use of CK17, is essential for differentiating BCB from mimickers. BCB is a rare primary breast tumor that follows an aggressive clinical course and closely mimics many basaloid neoplasms, including cutaneous BCC clinicopathologically. Increased awareness of BCB among dermatologists and dermatopathologists is critical for accurate diagnosis and patient care.

Very low-dose versus standard dose radiation therapy for indolent primary cutaneous B-cell lymphomas: A retrospective study

Limitations include the fact that roughly 90% of US allopathic dermatologists are members of the American Academy of Dermatology; our results may underestimate provider counts. Our ideal dermatologist:population ratio of 3.5 per 100,000 people is somewhat arbitrary, given that an appropriate ratio has never been validated. Patients in rural, poor, and high-minority areas lack an acceptable level of access to dermatologists. Minority physicians are more likely to care for patients of their own race and practice in underserved areas. Sadly, only 3% and 4.1% of US dermatologists are African American and Latin American, respectively. Physicians with rural backgrounds and clinical experience are more likely to practice in those areas, as evidenced by Michigan State University’s Rural Physician Program. Initiatives to train more dermatologists from rural, poor, or high-minority areas, and methods to incentivize practice in these areas, such as loan forgiveness, should be considered. The AccessDerm teledermatology initiative has expanded access to dermatologic care by helping patients unable to receive in-person consultation. However, teledermatology alone without access to definitive treatments, including obtaining biopsy specimens, surgical services, and medication monitoring, will not adequately serve the needs of these populations. In addition, dermatologists often serve as advocates in their communities for skin health education. Residency-trained dermatologists are leaders of important public health initiativeswhen they are active participants in the communities they serve.

Introduction to Cutaneous Lymphomas

The term cutaneous lymphoma encompasses an array of neoplasms that vary widely in their clinical presentation, prognosis, histopathology, immunohistochemistry, and molecular biology. The World Health Organization–European Organization for Research and Treatment of Cancer (WHO/EORTC) classification system recognizes 15 primary cutaneous lymphomas (Table 2.1). These lymphomas are divided into three overarching categories: T-cell lymphomas, B-cell lymphomas, and precursor neoplasms. Each chapter in this book is dedicated to one of these cutaneous lymphomas and offers a clinical and histopathologic description, differential diagnosis, and one or more clinical cases. Our intent in writing this book is to familiarize the reader with the most recent classifications of the cutaneous lymphomas, offer relevant clinical and histopathologic characteristics, and provide direction in navigating the often difficult task of distinguishing the various forms of cutaneous lymphoma.