After infection with borrelia burgdorferi via a tick bite, the body undergoes a series of events in an attempt to destroy the microorganism or limit its spread to other sites. The prevalence of borrelia lymphocytoma cutis has been reported to be from 0. Discussion cutis laxa is a rare disease which may be hereditary or acquired. Lymphocytoma cutis lc is one of the most common types of cutaneous b cell pseudolymphoma. It occurs both in adults and children, with a slight female predominance 2. The patient reported having similar episodes dating back to his early 20s. Department of pathology, j n medical college, amu, aligarh, uttar pradesh, india. Acrodermatitis chronica atrophicans aca is a slowly progressive lesion located primarily on the extensor. The clinical impression was that of lymphocytoma cutis and the lesions were treated with intralesional steroids resulting in prompt resolution leaving behind only flat discoloured areas. Lymphocytoma cutis is a rare, chronic, benign cutaneous bcell lymphoproliferative condition usually presenting with redpurple papules, nodules or plaques predominantly on the head and neck. Courtesy of stuart lessin, md diagnosis confirmation. Various stimuli can induce lymphocytoma cutis, but in most cases the cause is not known. It is suggested that the cases described represent different parts of a spectrum running from lymphocytoma cutis.
Characteristic histopathology of cutaneous lymphoplasia. Histopathologic patterns of erythema migrans and borrelial. The serum calcium dosage was applied, as a hypercalcemia suggested the diagnosis of sarcoidosis. Cutaneous presentation of orbital follicular lymphoma. Lymphocytoma cutis with conjunctival lesions british. As of 2016, the current approach to classification uses the who classification scheme. Lymphocytoma cutis, or spieglerfendt sarcoid, is classed as one of the pseudolymphomas, referring to inflammatory disorders in which the accumulation of lymphocytes on the skin resembles.
Chapter 1 introducton histopathology definition it is a branch of pathology which deals with the study of disease in a tissue section. Lymphocytoma cutis, or spieglerfendt sarcoid, is classed as one of the pseudolymphonas, 1 referring to inflammatory disorders in which the accumulation of lymphocytes on the skin resemble, clinically and histopathologically, cutaneous lymphomas. Gottlieb, md a classic reference tool to help users identify basic histopathologic patterns of inflammatory skin diseases. Lymphocytoma cutis, or benign reactive lymphoid hyperplasia, is an.
Clinically it is manifested as asymptomatic, indolent, nodular lesions of different sizes varying between 2 and 5 cm, usually solitary, mainly on exposed area of the. A 34yearold man presented to the outpatient dermatology clinic with 3 groups of mildly pruritic, erythematous papules and plaques on the right posterior shoulder, left midback, and left serratus anterior muscle. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. One hundred fifteen cases of cutaneous lymphoplasia lymphocytoma were examined histologically. Large cell lymphocytomaa clinicopathological study, clinical. A diffuse lymphoid infiltrate is seen throughout the entire cutis. Borreliaassociated bcell pseudolymphoma lymphocytoma cutis and lymphadenosis cutis benigna often are synonymously used. A characteristic histologic feature common to all 115 cases was the presence of large pale reticulum cells containing one or more round polychrome bodies. Lymphocytoma cutis cutaneous lymphoid hyperplasia, cutaneous bcell pseudolymphoma, spieglerfendt sarcoid is an entity encompassing a spectrum of benign bcell lymphoproliferative diseases that share clinical and histopathologic features. Welldemarcated plaque, 4 x 5 cm in size consisting of grouped erythematous shiny papules and nodules. Lymphocytoma cutis primary care dermatology society uk.
It was reported as a case of pseudoleukemic infiltration of the skin. Clh has had a wide variety of names, including spieglerfendt sarcoid, lymphocytoma cutis, and lymphadenosis benigna cutis. Pseudolymphomas can be categorized according to predominant cell type b cell, t cell and mixed and pattern of inflammation including bandlike and. Erythema migrans and borrelial lymphocytoma are early signs of lyme borreliosis. It is a process that simulates a cutaneous lymphoma blood cell cancer developing in the skin, but it behaves in a harmless manner. Grouped erythematous papules and plaques on the trunk. In 1923, bilerstein coined term lymphocytoma cutis term lymphadenosis benigna cutis was introduced by bafverstedt in 1943 in 1967, lever introduced term pseudolymphoma of spiegler and fendt subsequently,caro and helwig in. Borrelial lc occurs most commonly in areas endemic for ixodes ricinus tick in europe, and it is rare in. It has a worldwide distribution and affects all races and ethnic groups equally. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. However, histology showed a nodular dermal lymphoid infiltrate of centrocytelike cells with only scanty scattered blasts. The term pseudolymphoma has been used to classify cases of benign lymphoid infiltrates that mimic lymphoid malignancy.
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. Sarcomatosis cutis, lymphocytoma cutis, lymphadenosis benigna cutis, pseudolymphoma of spiegler and fendt, and actinic reticuloid are other accepted terminologies to describe cpl 1, 2. Histopathology remains one of the major tools of diagnosis in mycology20 table 3. Cutaneous lymphoma has a worse prognosis and treatment response. Lymphocytoma cutis case report 129 more unlikely hypotheses were. Kimu ras disease is characterized by the marked increase. Editor, lymphocytoma cutis is a benign lymphoid hyperplasia which typically occurs over the head and neck.
Case report lymphadenosis benigna cutis or cutaneous. Typically, the lesions are small, superficial, translucent, firm, nontender, pinkishred. Bernard ackerman, md, almut boer, md, bruce bennin, md, geoffrey j. It presents as red or violaceous nodules or plaques and is commoner in females. Jun 26, 2016 historical aspects cutaneous pseudolymphomacpl was first described under the term sarcomatosis cutis by kaposi in 1891. Lymphocytoma cutis, also known as cutaneous b cell pseudolymphoma, represents a spectrum of disease that shares similar clinical and histological features and simulates cutaneous lymphoma clinically and histologically. Clinical findings the localized form of bpsl presents with a solitary nodule, measuring up to 4 cm in most of the cases. In cutaneous pseudolymphoma, resemblance to lymphoma is usually most apparent histologically, but some examples may also mimic lymphoma clinically. In 1906 jadassohn 1 presented before the ninth german dermatological congress the first case of what was later to be known as lymphocytoma cutis. Inflammatory dermatoses mimicking malignancy pseudolymphoma. Hence, it was thought worthwhile to report the same, focusing attention, in particular, to its diagnostic clinical criteria, supported by histopathology, immunohistochemistry ihcprevalent nomenclature, and its differential diagnosis. The major advantages of histopathology are speed, low cost and the ability to provide a presumptive identification of the infecting fungus as well as demonstrating the tissue reaction. Erythema migrans em is an acute, selflimited lesion, often located in or near the groin, axilla, or popliteal fossa. Cutaneous lymphoid hyperplasia refers to a groups of benign cutaneous disorders characterized by collections of lymphocytes, macrophages, and dendritic cells in the skin 725 conditions included in this groups are 725 cutaneous lymphoid hyperplasia with nodular pattern, a condition of the skin characterized by a solitary or localized cluster of asymptomatic erythematous to violaceous.
Case report lymphadenosis benigna cutis or cutaneous lymphoid. Haemogram, urinalysis, biochemical parameters, ultrasonography. About 8590% of nonhodgkin lymphomas are derived from b cells, whereas the remaining lymphomas are derived from t cells or nk cells. In some cases extracutaneous sites are involved including the lung and gastrointestinal tract histology of cutis laxa. In 1922, when discussing bibersteins 2 case, jadassohn commented that his original patient was still living, that the skin lesions were unchanged and that no. Pdf editor, lymphocytoma cutis is a benign lymphoid hyperplasia which typically occurs over the head and neck. The tissue undergoes a series of steps before it reaches the examiners desk to be thoroughly examined microscopically to arrive at a particular diagnosis. Cutaneous bcell pseudolymphoma bpsl is often also referred as lymphocytoma cutis or cutaneous lymphoid hyperplasia. It clinically presents as skincolored to darkred papules, nodules, or infiltrative plaques similar in appearance to cutaneous malignant lymphoma. In europe, cutaneous lymphoid hyperplasia lymphocytoma cutis has been associated with borrelia infection and is said to be the most common cause in endemic areas. Lymphocytoma as a diagnostic term has been used since 1923 to designate cutaneous proliferations of lymphoid cells without detectable extracutaneous involvement.
In areas endemic for the tick, ixodes ricinus, this disease is more common. We report a case of lymphocytoma cutis with conjunctival lesions. Borrelial lymphocytoma bl is a subacute lesion, typically on the ear or breast, that eventually resolves 4, 26. Cutaneous pseudolymphoma refers to a heterogenous group of benign reactive tcell or bcell lymphoproliferative processes of diverse causes that simulate cutaneous lymphomas clinically and histologically. Nonhodgkin lymphoma includes a diverse spectrum of cancers of the immune system. The clinical differential diagnosis in each variety is difficult and includes numerous infiltrative disorders. Pseudolymphomas are also denominated cutaneous lymphoid hyperplasia clh, which better describes their benign clinical course. Liver and intrahepatic bile ducts tumor reactive lymphoid hyperplasia pseudolymphoma. Histopathology is an important tool in the diagnosis of cpl, and is necessary to distinguish cpl from reactive cutaneous inflammatory cell infiltrates, and lymphoma. By immunological and enzyme cytochemical methods the cells were identified as t lymphocytes. Pseudolymphoma is not a specific disease but rather an inflammatory response to known or unknown stimuli that results in a lymphomatousappearing but benign accumulation of inflammatory cells.
Due to this vast array of characteristics, the lesion is most. Histologic features help differentiate lymphocytoma cutis from cutaneous malignant lymphoma. The diagnosis is mainly based on histopathology and immunohistochemistry. Cutaneous b cell pseudolymphomas previously called lymphadenosis benigna cutis, lymphocytoma cutis, pseudolymphoma of spieglerfendt, or cutaneous lymphoid hyperplasia represent a reactive bcell proliferation response to a variety of antigenic stimuli, including arthropod bites, gold pierced earrings, tattoo dyes, and drugs. Clh lesions may closely resemble lymphoma both clinically and histologically. Borrelial lymphocytoma the term lymphadenosis benigna cutis labc was intro duced by bafverstedt in 1943 and applies to lesions that may appear in a solitary or a disseminated form labc dispersa. Historical aspects cutaneous pseudolymphomacpl was first described under the term sarcomatosis cutis by kaposi in 1891. Nov 28, 2012 lymphocytoma cutis, or spieglerfendt sarcoid, is classed as one of the pseudolymphonas, 1 referring to inflammatory disorders in which the accumulation of lymphocytes on the skin resemble, clinically and histopathologically, cutaneous lymphomas. In most cases, the causation is unknown, but a variety of stimuli are known to induce lymphocytoma cutis, many of which are potentially relevant to. The many faces of lymphocytoma cutis van vloten 2003.
Biopsies taken from patients soon after a tick bite show the tick head and neighboring skin with edema, vascular dilation, extravasation of. Pdf we describe a clinical case involving a 62yearold white male, diagnosed with lymphocytoma cutis spieglerfendt sarcoid in the cephalic. Dermatopathology reference describes cutis laxa histopathology including histologic features and provides links to additional medical references. The terms lymphocytoma cutis and cutaneous lymphoid hyperplasia have also been used historically and come under the same umbrella. In cutis laxa, dermal elastic fibres are both altered and reduced in. In most cases the infiltrate is located in the papillary dermis top heavy. The many faces of lymphocytoma cutis van vloten 2003 journal. Cutaneous lymphoid hyperplasia an overview sciencedirect. The many faces of lymphocytoma cutis wiley online library. Pseudolymphomas may arise in response to a wide variety of foreign antigens, but most are idiopathic.
Lymphadenosis benigna cutis, lymphocytoma cutis, pseudolymphomas. Abstract lymphadenosis benigna cutis or cutaneous lymphoid hyperplasia or lymphocytoma cutis or pseudolymphoma is classified as one of the inflammatory disorder in which accumulation of lymphocytes on skin resemble, clinically and histologically as, cutaneous lymphomas. Two cases of disseminated lymphocytoma cutis are reported. Various terms have been used for the benign processes which clinically and histologically resemble.
Subcutaneous lymphatic follicle formation and eosinophilic infiltration are observed histopathologically. Jul 31, 20 dermatopathology reference describes lymphocytoma cutis spiegler fendt sarcoid histopathology including histologic features and provides links to additional medical references. This form of the disease is a response to an infection due to borrelia burgdorferi subsp afzelius that is transmitted by a tick bite. In most cases, the causation is unknown, but a variety of stimuli are known to induce lymphocytoma cutis, many of which are potentially relevant to everyday diagnostic practice. Depending on aetiology, cbpl is also known as cutaneous lymphoid hyperplasia, lymphocytoma cutis, sarcomatosis cutis of spieglerfendt and bafverstedt syndrome. Lymphocytoma cutis is a rare pseudolymphoma that has also been known as cutaneous lymphoid hyperplasia or lymphadenosis benigna cutis. Histopathology of skin revealed fragmentation of and diminution in the elastic fibres without any evidence of calcification. Interpretation of cutaneous lymphoid infiltrates is one of the most troublesome areas of diagnostic dermatopathology.
Major advances have been made in the histologic classification, immunohistochemistry, and. Sarcomatosis cutis to denote benign formation resembling a sarcoma. Lymphocytoma cutis is a benign lymphocyte hyperplasia bcell reaction to an antigen such as a red tattoo, borrelia burgdorferi, injected drug, vaccine that prefers the head and neck region,and can be associated with sunlight sensitivity. The diagnosis is based on histology, the clinical context history of tick bite and localization at predilection site, serologic findings, andor detection of borrelia burgdorferi species dna in the tissue by pcr. We present a case of a 54yearold woman who spontaneously developed lesions of unknown cause consistent with clh. Pdf lymphocytoma cutis, also known as cutaneous b cell. Excised one month prior to presentation with recurrence.
What you should be alert for in the history solitary persistent lesion on the skin sometimes several grouped lesions generalized scattered lesions are uncommon either itchy or asymptomatic common triggers. The disease is rarely seen in india and may cause diagnostic difficulties for dermatologist residing in parts of the world that are not endemic for lyme disease. Localized lymphocytoma cutis, the most common form, may occur in both children and adults and has a short course. The accurate diagnosis of cutaneous pseudolymphoma depends on critical elements from the clinical history, physical exam, histologic, andor adjunctive studies that make the particular case fall short of the diagnosis of confirmed cutaneous lymphoma. There may be isolated lesions or regionalareas of localization. Borrelial lc occurs most commonly in areas endemic for ixodes ricinus tick in europe, and it is rare in north america. Apr 29, 2016 lymphocytoma cutis is a subtype of cutaneous pseudolymphoma involving the face, chest, and upper extremities.
Lymphocytoma cutis is the name for a group of skin disorders defined as benign. Dermatopathology reference describes lymphocytoma cutis spiegler fendt sarcoid histopathology including histologic features and provides links to additional medical references. The areas of predilection are the ear lobe, nipple and areola, and scrotal area. Lymphocytoma cutis lcc is a cutaneous bcell pseudolymphoma representing a wide variety of disorders that share clinical and histologic features. Lymphocytoma cutis, or spieglerfendt sarcoid, is classed as one of the. In those instances, the lesions presented without an inciting incident, became more pronounced, and.
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