Lymphoplasmacytic plaque in children: Case report and literature review.

We report a case of benign lymphoplasmacytic plaque (LPP) in a child. These asymptomatic erythematous papulonodular lesions are an emerging clinicopathological entity. Herein, we describe a previously unreported site for LPP lesions, namely, the volar wrist and the distal ipsilateral palm.

Approach to dermal-based lymphoid infiltrates and proliferations.

The histopathological diagnosis of dermal-based lymphoid infiltrates and proliferations is often challenging due to the vast list of biologically diverse entities that archetypally or occasionally center in the mid-dermis, especially because significant overlap exists in their clinical, histopathologic, and immunophenotypic features. The differential diagnosis includes reactive infiltrates in common and rare inflammatory dermatoses, benign conditions that may mimic lymphoid neoplasms (pseudolymphomas), and true clonal proliferations arising either primarily in the skin or rarely in extracutaneous tissues with secondary cutaneous dissemination. While numerous histopathological and immunophenotypic features have been reported to support a definitive diagnosis, no single ancillary test is sufficient for their distinction. Therefore, in this review we advocate a stepped histopathological approach for dermalbased lymphoid infiltrations, employing as key elements the general lymphocytic composition (relative B- versus T-cell ratio), coupled with the predominant cytomorphology (cell size) present. Following this strategy, the relative incidence of cutaneous involvement by each disease should always be considered, as well as the notion that a definitive diagnosis must be founded on a multiparameter approach integrating all clinical, histopathologic, immunophenotypic, and-in selected cases-molecular features.

Treatment of Cutaneous Pseudolymphoma: A Systematic Review.

Cutaneous pseudolymphoma (CPL) is a reactive polyclonal T- or B-cell lymphoproliferative process. CPL may appear as localized or disseminated skin lesions. While most cases of CPL are idiopathic, they may also occur as a response to, for example, contact dermatitis, arthropod reactions, and bacterial infections. CPL can be classified based on its clinical features, but all variants have similar histopathological patterns of either predominantly B-cell infiltrates, T-cell infiltrates, or mixed T/B-cell infiltrates. The prognosis of CPL is good, but the underlying disease process should be taken into account. If an antigenic stimulus is identified, it should be removed. In patients with idiopathic CPL, a close follow-up control strategy should be adopted. The aim of this systematic review is to summarize all reported treatments for CPL. The review was based on articles from the PubMed database, using the query "skin pseudolymphoma treatment", English and German, about "human" subjects, and published between 1990 and 2015 documenting adequate treatment and/or aetiology. Mainly individual case reports and small case series were found. Treatment options include topical and intralesional agents, systemic agents, and physical modalities. The final part of the review proposes a treatment algorithm for CPL according to each aetiology, based on the literature of the last 25 years. Future research should focus on randomized controlled trials and studies on long-term outcomes, which were not identified in the current review.

The impact of IgG-4-ROD on the diagnosis of orbital tumors: A retrospective analysis.

This study was to determine the prevalence of immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD) among patients who have previously undergone biopsy and were diagnosed to have idiopathic orbital inflammatory disease (IOID) or orbital lymphoproliferative disease (OLD), namely, lymphoma and benign reactive lymphoid hyperplasia (BRLH). This is a retrospective cross-sectional study. The charts and slides of all patients who underwent biopsies and were histopathologically diagnosed to have either IOID or OLD were reviewed. Demographics, clinical features, initial histopathological diagnoses, treatment received, and final outcome were noted. Using the diagnostic criteria for diagnosis for IgG4 disease, those cases that would classify as "possible IgG4-related disease (IgG4-RD)" were reviewed, reclassified, and reassigned a diagnosis of IgG4-ROD. We reviewed 105 patients' clinical charts. Of these 105 patients, upon reviewing the histopathology, 18 (17.15%) patients were found to fit the diagnostic criteria for possible IgG4-ROD. Of these 18 patients who were now reassigned the diagnosis of IgG4-ROD, the most common previous histopathological diagnosis was found to be IOID, for eight patients (44%), then BRLH, which was noted in five patients (27.8%), followed by lymphoma, which was noted in two patients (11.1%). Previously diagnosed cases of IOID and OLD were found to fulfill the criteria for IgG4-ROD. Given the advent of recent diagnostic and histopathological techniques, all cases of suspected IOID and OLD should be screened for IgG4-ROD and all previously diagnosed cases must be closely followed up, given the systemic implication of IgG4-RD. Histopathological reassessment of previously diagnosed cases may be considered.

Lymphocytic infiltrations of face.

The immune system protects our organism and, of course, our skin from harmful factors. One of the key elements of the immune system is lymphocytes. Lymphocytes play a role in the pathogenesis of various skin diseases. Lymphocytic infiltrates are seen in many skin diseases. Some of the skin diseases characterized by lymphocytic infiltration show up in specific anatomic locations, whereas other entities can be placed in all areas of the body. The course of lymphocytic infiltrations of the face is variable and unpredictable, most often lasting from months to years. The most important diseases with lymphocytic infiltration of the face are pseudolymphomas. This review discusses various types of cutaneous pseudolymphomas and other diseases with lymphocytic infiltration mainly involving the face.

Severe DRESS syndrome managed with therapeutic plasma exchange.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but increasingly described phenomenon of immune activation and organ dysfunction in association with a wide variety of medications. This reaction shows a broad spectrum of clinical presentation and severity, ranging from mild to lethal. Treatment strategies of immune suppression appear be helpful in some cases, but treatment failures occur frequently with reported mortality rates of 5% to 10%. We present a pediatric case of DRESS syndrome associated with either lamotrigine or bupropion, leading to multiorgan involvement and life-threatening complications of respiratory failure and cardiac arrest. After failing to improve with removal of these medications and administration of systemic corticosteroids, our patient showed dramatic, sustained clinical response to therapeutic plasma exchange. To our knowledge, this is the first reported case of therapeutic plasma exchange used for life-threatening DRESS syndrome in a pediatric patient. This case suggests needed research for this therapeutic option in life-threatening DRESS syndrome resistant to high-dose steroids.

Hiperplasia cutánea linfoide (Linfocitoma cutis) secundaria a fármacos: hallazgos clínicos y morfológicos / Cutaneous lymphoid hyperplasia (lymphocytoma cutis) due to drugs: clinical and morphological findings

Pseudolinfoma cutáneo es el término utilizado para indicar un grupo de trastornos linfoproliferativos benignos de la piel, siendo el linfocitoma cutis una forma de seudolinfoma de células B. Clínicamente se manifiesta como nódulos o placas solitarias, generalmente localizados en la cara de mujeres jóvenes. Su etiopatogenia estaría relacionada con una respuesta inmunológica local exagerada ante diversos desencadenantes tales como, picadura de insectos, acupuntura, vacunas, tatuajes, medicamentos e infecciones. Se presentan dos casos de pacientes con diagnóstico de linfocitoma cutis realizado en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez de Temuco.

Cutaneous pseudolymphoma is the term used to indicate a group of benign lymphoproliferative disorders of the skin, being the cutaneous lymphomas a form of B-cell pseudolymphoma. Clinically it manifests as solitary nodules or plaques, usually located on the face of young women. Its pathogenesis could be related to an exaggerated local immune response to various causes such as insect bites, acupuncture, vaccinations, tattoos, drugs and infections. We present two cases of patients diagnosed with skin linfocitoma made in the Pathology Unit in Hernán Henríquez Hospital of Temuco.

Clinical evaluation for lymphoproliferative disease prompted by finding of IgM warm autoanti-IT in two cases.

Anti-IT is an unusual specificity originally described as a naturally occurring cold agglutinin. The antibody reacts strongly with cord RBCs, weakly with adult I RBCs, and most weakly with the rare adult i RBCs. IgG anti-IT in patients with hemolytic anemia has been associated with Hodgkin's lymphoma. Difficulties in blood grouping tests and the presence of a warm reactive agglutinin in samples from two patients with hemolytic anemia led to further serologic studies and the identification of anti-IT. In both cases, the anti-IT was a rarely encountered IgM warm reactive agglutinin; in one case, the IgG component was also anti-IT, whereas in the second case the IgG antibody was broadly reactive. The unusual serologic finding of anti-IT prompted further clinical evaluation for lymphoproliferative disease in these two patients.

[Cutaneous pseudolymphomas and pseudoclonality of lymphoid infiltrates]. / Pseudolymphomes cutanés et pseudoclonalité des infiltrats lymphoïdes.

Cutaneous pseudolymphomas represent an heterogeneous group of tumours resembling lymphomas by some clinical and histological aspects. They are distinguished, in particular, by their benign evolution. The histological and immunohistological examinations combined with molecular biology help to elucidate any diagnostic uncertainty. In these diseases, monoclonality and pseudoclonality must be distinguished in the lymphoid infiltrates. Searching for any specific etiology is important because removing the cause is likely to clear the lesions.

Hyperplasia of hair follicles and other adnexal structures in cutaneous lymphoproliferative disorders: a study of 53 cases, including so-called pseudolymphomatous folliculitis and overt lymphomas.

We studied 53 cutaneous lymphoproliferative disorders, all of which manifested hair follicle hyperplasia. There were 42 cases conforming to the description of pseudolymphomatous folliculitis (PLF) and 11 cases of authentic lymphomas including mycosis fungoides, CD30+ anaplastic large cell lymphoma, diffuse large B-cell lymphoma, B-cell small cell lymphoma/leukemia, and peripheral T-cell lymphoma, not otherwise specified. All patients with PLF clinically presented with a solitary nodule preferentially involving the face. Beside hair follicle hyperplasia, the typical features were a dense infiltrate of small well-differentiated lymphocytes, lymphoplasmacytoid cells, plasma cells, and epithelioid histiocytes forming tiny granulomas. Some unusual or worrisome features recognized included eccrine/apocrine duct hyperplasia, subcutis/muscle infiltration, lymphocyte "smudging," single file infiltration, and large atypical cells. Immunohistochemically, T-cell predominant cases dominated in the series. All 34 tested cases revealed a polyclonal pattern of kappa and lambda immunoglobulin (Ig) light chain expression. In 4 cases, scattered CD30+ cells were identified. Monoclonal rearrangements of T-cell receptor (TCR) and IgH genes were detected in 19 and 3 cases respectively, including 1 case with dual T-cell receptor/IgH rearrangement. Three of 30 tested cases proved positive for herpes simplex virus-1, whereas herpes simplex virus-2 always tested negative. Of 31 cases tested for Borrelia burgdorferi, 30 specimens were negative. In 9 cases, fluorescent in situ hybridization for t(11;18) and t(14;18) revealed none of the above translocations. The most common treatment modality was surgical removal. Forty patients with a mean follow-up of 3.7 years included 39 patients with no evidence of disease and 1 individual with local recurrence. The comparison of "clonal cases of PLF" and those with polyclonal population or in which clonality remained undetermined revealed no differences between the 2 groups in the clinical presentation, pathologic, and immunohistochemical features. We conclude that hyperplasia of hair follicles and other adnexa can be seen not only in the condition currently known as PLF, but also in genuine cutaneous lymphomas and may be just a happenstance secondary to a basic pathologic process.

[Nodular lymphoid hyperplasia--underestimated problem of gastrointestinal tract pathology in children]. / Guzkowy przerost limfoidalny--niedoceniony problem w patologii przewodu pokarmowego u dzieci.

Nodular lymphoid hyperplasia (NLH) is a rare condition with unknown etiology frequently associated with common variable immunodeficiency (CVID) and selective immunoglobulin A deficiency. NLH is a common cause of gastrointestinal bleeding. The risk of malignancy has been well recognized in subjects with NLH. The present knowledge about the nodular lymphoid hyperplasia (NLH) was described.

Reactive hyperplasias,precancerous and malignant lesions of the oral mucosa.

The oral cavity contains many organs and tissues compressed in a small area. Accordingly oral tumors have a wide variety of appearances. Reactive hyper-plastic lesions include epulis,morsicatio,traumatic ulcer or palatal hyperplasia. These benign lesions must be separated clinically and histologically from precancerous and neoplastic lesions. In leukoplakia,the individual risk can be estimated by clinical signs. Nevertheless histopathology is mandatory because precancerous lesions usually precede or accompany most oral cancers. Amalgam tattoo,oral nevi and melanoacanthoma have to be considered as differential diagnoses of oral melanoma. Accurate clinico-pathological diagnosis is mandatory to insure appropriate therapy. Oral soft tissue tumors such as Kaposi sarcoma and multiple mucosal neuromas in MEN 2b require interdisciplinary management. Diseases affecting the minor salivary glands which may be encountered by dermatologists include mucocele, necrotizing sialometa-plasia,and tumors such as pleomorphic adenoma.

Lymphoproliferative diseases of the ocular adnexa in Korea.

OBJECTIVES: To evaluate the clinical features, treatments, and outcomes of patients with ocular lymphoproliferative disease classified according to the World Health Organization classification and to determine prognostic factors of this disease in South Korea. METHODS: Between March 1, 1995, and December 31, 2005, 128 cases of patients with ocular lymphoid tumors treated at the Samsung Medical Center were retrospectively reviewed. RESULTS: The mean patient age was 46.3 years (range, 1-87 years). The major histopathologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma in 96 patients (75.0%), lymphoid hyperplasia in 11 (8.6%), diffuse large B-cell lymphoma in 6 (4.7%), and mantle cell lymphoma in 4 (3.1%). Ocular lymphoproliferative lesions were located in the conjunctiva (53 patients), the eyelid (33 patients), and the orbit (42 patients). Twenty patients had tumor relapses (15.6%), and 9 died of lymphoma during follow-up (7.0%). Regarding the analysis of prognostic factors, most patients with MALT lymphoma evidenced local disease, required local treatment, and exhibited a superior prognosis. CONCLUSIONS: Lymphomas of the MALT type constitute most ocular adnexal lymphoproliferative diseases and occur more frequently in South Korea than in Western countries. Patients with MALT lymphoma have favorable outcomes compared with patients with other types of lymphoma.

Cutaneous lymphoid hyperplasia: a case report and brief review of the literature.

Cutaneous lymphoid hyperplasia (CLH) is considered a benign lymphoid reactive process that results from various antigenic stimuli and may have potential for progression to overt lymphoma. CLH lesions may closely resemble lymphoma both clinically and histologically. We present a case of a 54-year-old woman who spontaneously developed lesions of unknown cause consistent with CLH. We also review the literature and discuss the etiology, clinical features, diagnosis, and management of CLH.

Drug-associated lymphoma and pseudolymphoma: recognition and management.

This article discusses ways to recognize and manage lymphomas and pseudolymphomas associated with drug exposure. Over the last 30 years, the classification of pseudolymphomas and lymphomas has undergone significant change, especially following the application of sophisticated immunostaining and gene rearrangement analysis. The term cutaneous pseudolymphomas (CPL) is a nonspecific term for a heterogeneous group of benign reactive T- or B-cell lymphoproliferative processes that simulate cutaneous lymphomas clinically or histologically. While pseudolymphomas are relatively rare diseases, their clinical and histological heterogeneity has led to multiple systems of categorization based on immunological factors, causative agents, presentation, and clinical course.