Adolescence Pseudolymphoma / Pseudolinfoma na adolescência

Objective: report an uncommon case of cutaneous pseudolymphoma in teenage years, undiagnosed for approximately 8 years old. Methodology: data were taken from medical records, patient interviews, photographic records of the injuries, diagnostic methods, and literature review. The paper was subjected and approved by the Research Ethics Committee (REC), under the number 4.952.193, authorized by the patient and their legal sponsor. Final Conclusions: the related case shows the importance of reliable and differential diagnoses since the patient carried the injury through approximately eight years without getting any diagnosis and/or treatment. Furthermore, the unusual age and the location of the injuries make the information presented here fundamental to helping other professionals and contributing to the Public Health System (AU).

Objetivo: Relatar um caso incomum de Pseudolinfoma Cutâneo na adolescência, não diagnosticado por aproximadamente oito anos. Método: As informações foram obtidas pela revisão do prontuário, entrevista com o paciente, registro fotográfico das lesões e dos métodos diagnósticos e revisão de literatura. O trabalho foi submetido e aprovado pelo Comitê de Ética em Pesquisa (CEP), número de aprovação 4.952.193, com autorização do paciente e seus responsáveis legais. Considerações Finais: o caso relatado evidencia a importância de um diagnóstico fidedigno e dos diagnósticos diferenciais, uma vez que a paciente apresentou a lesão por aproximadamente oito anos, sem receber nenhum diagnóstico e/ou tratamento. Além disso, não só a faixa etária é incomum, mas também a localização da lesão e por esta razão, as informações são fundamentais para auxiliar outros profissionais, com benefício deste estudo para o Sistema de Saúde (AU).

Ultrasonography of cutaneous nodular pseudolymphoma at 18 and 71 MHz.

Cutaneous pseudolymphomas are reactive lymphoproliferations. The most frequent type is nodular pseudolymphoma, and to date, their ultrasonographic appearance has not been reported. We reviewed the ultrasound images of histologically confirmed nodular types of pseudolymphomas studied with 18 and 71 MHz linear probes. All lesions were predominantly hypoechoic and presented prominent vascularity. Seventy percent of cases involved dermis and hypodermis, and 30% were only dermal. Seventy percent of cases showed internal hypoechoic globules, and 100% presented a teardrop sign, more clearly detected at 71 MHz. Ultrasound can support the diagnosis, assessment of the extent, and degree of vascularity of cutaneous nodular pseudolymphomas.

Foliculitis pseudolinfomatosa de McNutt (localización nasal) / McNutt's pseudolymphomatous folliculitis (nasal located)

La foliculitis pseudolinfomatosa, descripta por McNutt en 1986, es una afección de etiología desconocida y poco frecuente, que simula un linfoma cutáneo tanto por su clínica como por su histología. Se presenta como una lesión nodular solitaria, eritematosa, de 0,5 hasta 3cm, de crecimiento rápido, sobre todo en la cara, en personas de 40 a 60 años, con una histopatología caracterizada por un infiltrado linfocitario B yT perifocular, y células dendríticas positivas en la inmunohistoquímica para S100yCD1a. Su curso es benigno, muchas veces autolimitado. Se expone el caso de una paciente con una particular forma clínica de pseudolinforma.

Pseudolymphomatous folliculitis, described by McNutt in 1986, is a non-frequent entity of unknown etiology that simulates a cutaneous lymphoma, both clinically and histologically. It shows as a solitary erythematous nodular lesion of 0.5 to 3 cm, with a rapid growth, mainly on the face, in people aged 40 to 60 years, and histopathology characterized by a perifollicular B and T lymphocytic infiltrate, and positive dendritic cells for immunohistochemistry S100 and CD1a. Its course is benign, often self-limited. The case of a patient with a particular clinical form of pseudolymphoma is presented.

Shall we exclude parapsoriasis from the medical vocabulary?

Since the first description of parapsoriasis more than 100 years ago, parapsoriasis has been a questionable condition and occasionally considered a precursor of cutaneous lymphoma. The name "parapsoriasis" is related to a heterogenous group of diseases that show a distinct clinical presentation; however, the histopathological criteria are not strongly specific. Pathologists do not consider parapsoriasis as a possible histopathological diagnosis, but dermatologists use the term as clinical hypothesis. We aim to provide an historical review of parapsoriasis focusing on histopathological criteria, considering its possible relation with cutaneous skin lymphoma, based on articles from PubMed and standard dermatopathological books. Parapsoriasis does not have well-defined histopathological criteria, so its use should be avoided. Being aware of parapsoriasis complexity, a consensus meeting can help to create a guideline regarding this topic.

Rosettes in T-cell pseudolymphoma: a new dermoscopic finding

Abstract Rosettes are small white structures visible with polarized light dermoscopy, whose exact morphological correlation is not yet defined. These small shiny structures are found in several conditions such as scarring, dermatofibroma, molluscum contagiosum, squamous cell carcinoma, basal cell carcinoma, melanoma, melanocytic nevus, discoid lupus erythematosus, and papulopustular rosacea. In this novel report, the authors describe the presence of rosettes in a T-cell pseudolymphoma lesion.

Rosettes in T-cell pseudolymphoma: a new dermoscopic finding.

Rosettes are small white structures visible with polarized light dermoscopy, whose exact morphological correlation is not yet defined. These small shiny structures are found in several conditions such as scarring, dermatofibroma, molluscum contagiosum, squamous cell carcinoma, basal cell carcinoma, melanoma, melanocytic nevus, discoid lupus erythematosus, and papulopustular rosacea. In this novel report, the authors describe the presence of rosettes in a T-cell pseudolymphoma lesion.

Parotid follicular lymphoid hyperplasia - a rare entity: the challenges in differential diagnosis / Hiperplasia linfoide folicular em parótida - uma rara entidade: os desafios no diagnóstico diferencial

ABSTRACT A 61-year-old female patient presented a nodular lesion located in the right buccal mucosa with a 3-month evolution. Clinical hypotheses of salivary duct cyst and mucocele were proposed, and the patient underwent excisional biopsy. Microscopically, a well-circumscribed and encapsulated lymphoid aggregate fragment was observed, characterized by layers of well-differentiated small lymphocytes and collections of reactive lymphoblasts. These findings, associated with immunohistochemistry, established the diagnosis of follicular lymphoid hyperplasia. Currently, the patient is well, under follow-up after six months.

RESUMEN Paciente del sexo femenino de 61 años de edad exhibió lesión nodular localizada en mucosa yugal derecha con tiempo de evolución de tres meses. Se establecieron las hipótesis clínicas de quiste del ducto salival y mucocele, y la paciente se sometió a una biopsia excisional. Microscópicamente, se observó un fragmento de agregado linfoide bien circunscrito y encapsulado, caracterizado por capas de linfocitos pequeños bien diferenciados y colecciones de linfoblastos reactivos. Esos hallazgos, asociados al estudio inmunohistoquímico, basaron el diagnóstico de hiperplasia folicular linfoide. Al presente, la paciente se encuentra bien, bajo seguimiento seis meses después.

RESUMO Paciente do sexo feminino, 61 anos de idade, exibiu lesão nodular localizada em mucosa jugal direita com evolução há três meses. As hipóteses clínicas de cisto do ducto salivar e mucocele foram estabelecidas, e a paciente foi submetida à biópsia excisional. Microscopicamente, foi observado fragmento de agregado linfoide bem circunscrito e encapsulado, caracterizado por camadas de pequenos linfócitos bem diferenciados e coleções de linfoblastos reativos. Esses achados, associados ao estudo imuno-histoquímico, estabeleceram o diagnóstico de hiperplasia linfoide folicular. Atualmente, a paciente encontra-se bem, sob proservação após seis meses.

Case for diagnosis. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Abstract: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease, with an indolent evolution and benign course. The classic presentation is a solitary nodule on the face or trunk. The disorder's rarity and clinical and histopathological characteristics, can make the diagnosis difficult. We present the case of a 36-year-old Caucasian woman with a purplish erythematous nodule, hardened, shiny, asymptomatic, on the left nasal ala, which had grown progressively for 45 days. Histopathological examination and immunohistochemistry panel demonstrated alterations consistent with primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was complete remission of the condition within 60 days of treatment with potent occlusive corticosteroids.

Rare case report of an aggressive follicular lymphoid hyperplasia in maxilla.

Follicular lymphoid hyperplasia is a very rare though benign reactive process of an unknown pathogenesis that may resemble a follicular lymphoma, clinically and histologically. Oral reactive follicular hyperplasia (RFH) has been described on the hard or soft palate and at the base of the tongue. We describe here the first case of RFH presenting as an aggressive tumor on the right posterior side of the maxilla in a 24-year-old male patient. The lesion had a clinical evolution of 18 months and was noticed after the surgical extraction of the right third molar, although we cannot assume a cause-effect relation with that surgical event whatsoever. His medical history was unremarkable. Following an incisional biopsy, histological examination revealed lymphoid follicles comprised by germinal centers surrounded by well-defined mantle zones. The germinal centers were positive for Bcl-6, CD10, CD20, CD21, CD23, CD79a, and Ki-67, while negative for Bcl-2, CD2, CD3, CD5, and CD138. The mantle and interfollicular zones were positive for Bcl-2, CD2, CD3, CD5, CD20, and CD138. Both areas were diffusively positive for kappa and lambda, showing polyclonality. The patient underwent a vigorous curettage of the lesion with no reoccurrences at 36 months of follow-up. This case report demonstrates that morphologic and immunohistochemical analyses are crucial to differentiate RFH from follicular lymphoma, leading to proper management.

Lymphocytoma cutis on the inguinal region: report of a rare case of benign lymphoproliferative disorder

Abstract Lymphocytoma cutis, or benign reactive lymphoid hyperplasia, is an inflammatory skin lesion that mimics clinically and histologically malignant lymphoma. Most cases are idiopathic, but they may also be triggered by multiple factors, such as insect bites, tattoos, injections and herpes zoster. Clinically, the lesions are erythematous, soft papules, plaques or nodules, usually located on the upper limbs and face. The diagnosis is mainly based on histopathology and immunohistochemistry. Corticosteroid injections, cryosurgery, PUVA therapy, radiotherapy and surgery can be therapeutic options in cases requiring immediate treatment. To demonstrate an atypical presentation of this tumor, a case lymphocytoma skin on the groin will be reported, describing its diagnosis and treatment.

Red nodule on the face with "spontaneous" regression

Abstract Pseudolymphomatous folliculitis is a rare entity included among the cutaneous pseudolymphomas. A 32-year-old man, with an unremarkable medical history, presented with a two-month history of an asymptomatic solitary nodule on his left cheek. Histopathological examination demonstrated a dense nodular and diffuse dermal lymphocytic infiltrate with numerous histiocytes and dendritic cells that surrounded hypertrophic hair follicles. Pseudolymphomatous folliculitis commonly presents in the fourth decade of life, with no sex predominance, as an asymptomatic, rapidly growing and solitary red dome-shaped nodule on the face. It has a benign clinical course as the lesions usually resolve with surgical excision or regress spontaneously after incisional biopsy. Although there is no report of pseudolymphomatous folliculitis progressing into lymphoma in the literature, follow-up of these patients is recommended.

Pseudolinfoma post herpes zoster: Fenómeno isotópico / Pseudolymphoma post herpes zoster: Isotopic phenomenon

El pseudolinfoma es una patología que debe su nombre, a la similitud de sus lesiones desde el punto de vista clínico, histopatológico o ambos, al linfoma. El fenómeno isotópico, describe la aparición de nuevas lesiones en la misma localización, de otro cuadro dermatológico, en este caso un herpes zoster, ya resuelto. Presentamos una paciente de sexo femenino de 66 años de edad, que consultó por lesiones tumorales en región frontal, donde había presentado un kerpes zoster un mes antes. Se realizó biopsia con diagnóstico de pseudolinfoma. Las lesiones remitieron espontáneamente sin tratamiento, a la espera del informe histopatológico.

Pseudolymphoma is a pathology which owes its name to the similarity of their injuries, from clinically histologically or both to lymphoma. Isotopic phenomenon describes the appearance of new lesions in the same location of other dermatological disease, in this case a herpes zoster, already solved. We present a 66 years old female that consulted for tumoral lesions in frontal region, where she had present a herpes zoster, one month before. It was performed biopsy with diagnosis pseudolymphoma. The lesions disappeared spontaneously without treatment, awaiting the histopahology report.

Approach to Cutaneous Lymphoid Infiltrates: When to Consider Lymphoma?

Cutaneous lymphoid infiltrates (CLIs) are common in routine dermatopathology. However, differentiating a reactive CLI from a malignant lymphocytic infiltrate is often a significant challenge since many inflammatory dermatoses can clinically and/or histopathologically mimic cutaneous lymphomas, coined pseudolymphomas. We conducted a literature review from 1966 to July 1, 2015, at PubMed.gov using the search terms: Cutaneous lymphoma, cutaneous pseudolymphoma, cutaneous lymphoid hyperplasia, simulants/mimics/imitators of cutaneous lymphomas, and cutaneous lymphoid infiltrates. The diagnostic approach to CLIs and the most common differential imitators of lymphoma is discussed herein based on six predominant morphologic and immunophenotypic, histopathologic patterns: (1) Superficial dermal T-cell infiltrates (2) superficial and deep dermal perivascular and/or nodular natural killer/T-cell infiltrates (3) pan-dermal diffuse T-cell infiltrates (4) panniculitic T-cell infiltrates (5) small cell predominant B-cell infiltrates, and (6) large-cell predominant B-cell infiltrates. Since no single histopathological feature is sufficient to discern between a benign and a malignant CLI, the overall balance of clinical, histopathological, immunophenotypic, and molecular features should be considered carefully to establish a diagnosis. Despite advances in ancillary studies such as immunohistochemistry and molecular clonality, these studies often display specificity and sensitivity limitations. Therefore, proper clinicopathological correlation still remains the gold standard for the precise diagnosis of CLIs.

Linfoma ocular de células B da zona marginal: relato de caso e revisão da literatura / Ocular B-cell marginal zone lymphoma: case report and literature review

RESUMO Paciente feminina, 70 anos, apresentou-se com queixas de prurido ocular à esquerda, lacrimejamento e hiperemia há 2 meses. Ao exame físico, apenas hiperemia conjuntival discreta. Foi solicitada biópsia, que sugeriu a hipótese de pseudolinfoma pela insuficiência de critérios classificatórios. Após um ano a paciente retornou com os mesmos sintomas e proptose ocular à esquerda, associado a proliferação de aspecto linfático em conjuntiva bulbar, com presença de vasos sanguíneos, ocupando toda região superior e maior parte da medial, se estendendo até o limbo, sem oclusão do eixo visual e mobilidade ocular extrínseca preservada. Nova biópsia e imuno-histoquímica, foram compatíveis com linfoma de células B da zona marginal. Foram realizadas tomografias computadorizadas (TC) de crânio, pescoço e órbitas, revelando aumento do volume do músculo reto superior esquerdo, com intensa impregnação pelo contraste, acometendo ventre e tendão, com borramento de gordura adjacente. Foram programados 6 ciclos de quimioterapia com ciclofosfamida, doxorrubicina, vincristina e prednisona, CHOP, a cada 21 dias. Houve melhora dos sintomas oculares após o primeiro ciclo. Após o quarto, nova TC de órbitas evidenciou regressão do espessamento do músculo reto superior esquerdo, ainda melhor na TC realizada após o sexto ciclo. Os linfomas MALT são responsáveis por 5-17% de todos os casos de LNH, respondem por mais de 90% dos linfomas que acometem os anexos oculares, mas podem se originar em diversos tecidos. Devido a raridade da doença, carecem estudos prospectivos randomizados que definam um consenso terapêutico. A literatura sugere que o tratamento deve ser individualizado.

ABSTRACT Female patient, 70 years old, presented with complaints of left eye pruritus, tearing and redness, for 2 months. Physical examination revealed only mild conjunctival hyperemia. Biopsy was ordered, and suggested the hypothesis pseudo lymphoma by insufficient classification criteria.After a year, the patient returned with the same symptoms, and left ocular proptosis associated with lymphatic aspect of proliferation in bulbar conjunctiva, with the presence of blood vessels, occupying the entire upper region and most of the medial, extending into the limbo without occlusion the visual axis, extrinsic ocular motility preserved.Another biopsy and immunohistochemistry were compatible with a B cell marginal zone lymphoma. Tomographic studies of cranium, neck and orbits were performed, and the orbit showed enlargement of the left superior rectus muscle volume, with intense contrast uptake, affecting belly and tendon and blurring of adjacent fat. Six cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone, CHOP, were done every 21 days. There was improvement in ocular symptoms after the first cycle. After the fourth, new scans from the orbits revealed regression of thickening of the left superior rectus muscle, and even better after the sixth cycle. The MALT lymphomas account for 5-17% of all cases of NHL, accounting for over 90% of lymphomas affecting the eye attachments, but can originate in different tissues. Due to the rarity of the disease, there are no randomized prospective studies to define a therapeutic consensus. The literature suggests that treatment should be individualized.

Cutaneous primary B-cell lymphomas: from diagnosis to treatment

AbstractPrimary cutaneous B-cell lymphomas are a heterogeneous group of mature B-cells neoplasms with tropism for the skin, whose biology and clinical course differ significantly from the equivalent nodal lymphomas. The most indolent forms comprise the primary cutaneous marginal zone and follicle center B-cell lymphomas that despite the excellent prognosis have cutaneous recurrences very commonly. The most aggressive forms include the primary cutaneous large B-cell lymphomas, consisting in two major groups: the leg type, with poor prognosis, and others, the latter representing a heterogeneous group of lymphomas from which specific entities are supposed to be individualized over time, such as intravascular large B-cell lymphomas. Treatment may include surgical excision, radiotherapy, antibiotics, corticosteroids, interferon, monoclonal antibodies and chemotherapy, depending on the type of lymphoma and on the type and location of the skin lesions. In subtypes with good prognosis is contraindicated overtreatment and in those associated with a worse prognosis the recommended therapy relies on CHOP-like regimens associated with rituximab, assisted or not with local radiotherapy. We review the primary cutaneous B-cell lymphomas, remembering the diagnostic criteria, differential diagnosis, classification, and prognostic factors and presenting the available therapies.

Possibly drug-induced palpable migratory arciform erythema

Palpable migratory arciform erythema is an entity of unknown etiology, with few published cases in the literature. The clinical and histopathological features of this disease are difficult to be distinguished from those of Jessner’s lymphocytic infiltration of the skin, lupus erythematous tumidus and the deep erythema annulare centrifugum. We describe here the first two Brazilian cases of palpable migratory arciform erythema. The patients presented with infiltrated annular plaques and erythematous arcs without scales. These showed centrifugal growth before disappearing without scarring or residual lesions after a few days. They had a chronic course with repeated episodes for years. In addition, these cases provide evidence of a drug-induced etiology.

T-cell-predominant lymphoid hyperplasia in a tattoo

Cutaneous lymphoid hyperplasia (CLH) can be idiopathic or secondary to external stimuli, and is considered rare in tattoos. The infiltrate can be predominantly of B or T-cells, the latter being seldom reported in tattoos. We present a case of a predominantly T CLH, secondary to the black pigment of tattooing in a 35-year-old patient, with a dense infiltrate of small, medium and scarce large T-cells. Analysis of the rearrangement of T-cells receptor revealed a polyclonal proliferation. Since the infiltrate of CLH can simulate a T lymphoma, it is important to show that lesions from tattoos can have a predominance of T-cells.

Pseudolinfoma cutâneo: relato de caso / Cutaneous pseudolymphoma: case report

Os pseudolinfomas cutâneos representam um grupo heterogêneo de reações linfoproliferativas benignos que podem simular clinica e histologicamente linfomas cutâneos. O objetivo deste estudo foi relatar a apresentação anatomoclínica de um caso de pseudolinfoma cutâneo para o aperfeiçoamento do seu diagnóstico diferencial com linfomas cutâneos. Paciente do gênero masculino, 66 anos procurou atendimento médico relatando história de nódulos em região superior do dorso há seis anos. Ao exame físico foram observados sete nódulos com aproximadamente 0,5 cm de diâmetro e sem sinais de linfonodomegalias. A biópsia de pele mostrava um denso infiltrado de linfócitos e histiócitos na derme. A análise imuno-histoquímica revelou uma população mista de linfócitos B (CD 20) e linfócitos T (CD 3). O diagnóstico diferencial entre as lesões benignas e malignas é a principal prioridade nesses casos. Tanto o pseudolinfoma, quanto o linfoma cutâneo se manifestam como nódulos solitários, pápulas e placas decorrentes de infiltração linfocitária. Para o diagnóstico diferencial, estão indicadas a avaliação imuno-histoquímica e técnicas de biologia molecular...

Cutaneous pseudolymphoma represents heterogeneous groups of benign lymphoproliferative reactions that may simulate clinically and histologically cutaneous lymphomas. The aim of this study was to report a case of cutaneous pseudolymphoma. Male patient, 66-year-old, came to us reporting a 6-year history of nodules on the upper back. On physical examination we noticed 7 nodules with 0.5 cm of diameter and no sign of adenopathy. The skin biopsy showed a dense infiltrate of lymphocytes and histiocytes in the dermis. The immunohistochemical analysis revealed a mixed population of B lymphocytes (CD 20) and T lymphocytes (CD 3). Differential diagnosis between benign and malignant lesions is the main concern in these cases. In some cases, pseudolymphoma manifests as solitary nodules, papules and plaques that are clinically indistinguishable from cutaneous lymphomas. The differentiation process can be further facilitated by immunohistochemical and molecular biological techniques...

Acral pseudolymphomatous angiokeratoma: case report and literature review / Angioqueratoma pseudolinfomatoso acral: relato de caso e revisao da literatura

The authors describe a case of a female patient with Acral Pseudolymphomatous Angiokeratoma of Children, known as APACHE. It is a rare benign cutaneous disease, of unknown etiology, characterized by multiple, asymptomatic erythematous-violaceous papules and nodules, usually located unilaterally with acral distribution. Today, this denomination is questionable, since there are published reports of this disease in adults and in different locations. Clinically, it is similar to an angiokeratoma, whereas hystologically, it corresponds to a distinct type of pseudolymphoma. The immunohistochemical study is required to distinguish APACHE from cutaneous lymphoma.

Relata-se o caso de uma paciente com diagnóstico de angioqueratoma pseudolinfomatoso acral, conhecido por sua sigla em inglês APACHE - Acral Pseudolymphomatous Angiokeratoma of Children. É uma doença cutânea benigna, rara, de etiologia desconhecida, caracterizada por múltiplas pápulas e nódulos eritêmato-violáceos assintomáticos, de localização geralmente unilateral e acral. Atualmente, questiona-se esta denominação, já que há relatos na literatura do quadro em adultos e em outras localizações. Clinicamente, é similar a um angioqueratoma, porém, histologicamente, corresponde a um tipo distinto de pseudolinfoma. O estudo imuno-histoquímico é necessário para diferenciação dos linfomas cutâneos.