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Pathology Quiz Case 2: Diagnosis

Pathology Quiz Case 2: Diagnosis Diagnosis: Dermatopathic lymphadenitis (DL) Dermatopathic lymphadenitis characteristically presents as a single or multiple enlarged axillary or inguinal lymph nodes. The lymph nodes are usually discrete, superficial, nontender, firm, and freely movable.1 Presentation as an enlarged neck mass is exceedingly rare. The disease is typically associated with a benign pruritic skin disorder. It is frequently noted as one of many findings in a systemic disease (eg, chronic lymphocytic leukemia or AIDS). It may also be associated with a cutaneous neoplasm (eg, mycosis fungoides or Sézary syndrome) or discovered as an isolated finding. Dermatopathic lymphadenitis was first described as lipomelanotic reticulosis because of the obvious accumulation of lipids and melanin pigment in the cortical region of lymph nodes.2 Further research that elucidated a relationship between the characteristic appearance of the lymph nodes and pruritic skin diseases led to the name dermatopathic lymphadenitis.2 The histologic and immunohistochemical features of DL have been well described. The lymph node paracortex is expanded by pale-staining phagocytic histiocytes, Langerhans cells, and interdigitating reticulum cells. The cells have pale, occasionally vacuolated cytoplasm with elongated folded nuclei.1,3 Macrophages may contain intracytoplasmic melanin, but this finding is inconsistent. Lymphocytes with irregular to cerebriform nuclei accompany the proliferation of histiocytes. The Langerhans and interdigitating reticulum cells are related antigen-presenting cells that express HLA-DR, OKT6, and S100 protein, which is sometimes used as a diagnostic marker for DL. However, it has been shown that S100 protein is not sensitive or specific for DL and does not correlate with disease severity. In one series, only 10% to 60% of lymph nodes stained positive in cases in which the histopathologic characteristics of DL were present.3 In an attempt to provide a minimal definition for the histologic characteristics of DL, Gould et al3 noted a histologic continuum for the paracortical changes. They described the continuum with 4 grades, ranging from sparse histiocytes or dendritic cells (grade 1) to sheets of these cells obscuring the germinal centers (grade 4). The higher the grade, the higher the incidence of concomitant skin disease. As suggested by the name, DL is usually accompanied by generalized acute or chronic pruritic skin diseases such as psoriatic erythroderma. It has been hypothesized that this disorder is a nonspecific reactive change due to barrier disruption of dependent skin.4 Melanin pigment was noted to be phagocytosed by melanophores in the disrupted dermis and transported to the lymph nodes. There was no evidence of increased production of melanin. Furthermore, DL has been described in the absence of clinical skin disease. The incidence of DL associated with skin disease at the time of diagnosis is estimated to be between 66% and 91%.3 It has been speculated that this discrepancy is accounted for by a skin disease within the previous few years. Since Langerhans cells, histiocytes, and melanin have the potential to stay in lymph nodes for years, a lymph node indicative of DL can reflect a resolved skin disease or the cumulative effect of many minimal dermatologic abnormalities. Also, DL has been described in patients with pruritus without any identifiable skin disease, reinforcing the idea that this finding is due to barrier disruption of dependent skin.3 A more ominous association of DL has been noted in patients with mycosis fungoides, chronic lymphocytic leukemia, Sézary syndrome, and AIDS. It is unfortunately difficult to distinguish DL that is associated with mycosis fungoides from DL without mycosis fungoides. Much effort has been given to analysis of fine-needle aspiration cytology (FNA) for this differentiation. As mycosis fungoides develops, the histopathologic features may be indistinguishable from those of DL. Both entities demonstrate diffuse pale cells, stain positive for S100 protein, and have cerebriform lymphocytes.3-5 The earliest distinguishing feature is an increase in the size of the cerebriform lymphocytes within the paracortical areas. As the cells enlarge and multiply, they invade the medullary cords and sinus and form monomorphic infiltrates, defining a malignant neoplasm.1 It has been noticed that patients with AIDS seem to have a higher incidence of DL than the normal population. A few hypotheses have been suggested to explain this increased incidence. Patients with AIDS are known to have a wide variety of skin diseases that can range from minor to severe and may cause more frequent DL-like changes. A second explanation may be that DL reflects an intrinsic part of the AIDS disease process. Langerhans cells are decreased in the epidermis of patients with AIDS and are thought to migrate to the paracortical region of lymph nodes, simulating the DL histopathologic features.3 Because DL has the potential to be associated with life-threatening disease, a thorough investigation should be undertaken to discover the underlying cause. Any history of skin disease, itching, human immunodeficiency virus, or other constitutional symptoms should be elucidated. Other causes of head and neck masses should be ruled out. Thorough examinations of the head and neck and skin should be performed. If the cause remains unknown, a dermatologic evaluation may prove beneficial. Fine-needle aspiration of the mass is the simplest means to make a diagnosis and to exclude other causes of lymphadenopathy.4,5 In a study to determine the FNA morphological criteria for DL, Iyer et al5 found that the presence of melanin-laden macrophages, large histiocytic clusters with central blood vessels, and histiocytes with elongated, crumpled, vesicular nuclei and pseudonucleoli, as well as positivity for S100 protein and negativity for CD68, all aid in the diagnosis. Unfortunately, none of these criteria is absolutely diagnostic of DL, and the results of FNA are not always accurate. Excisional lymph node biopsy is therefore the procedure of choice for definitive diagnosis and treatment because the cerebriform cells may be present only locally and are often missed on FNA. Excisional biopsy is indicated in all cases in which mycosis fungoides is suspected. Dermatopathic lymphadenitis rarely presents as a neck mass and is therefore infrequently seen by otolaryngologists. However, early recognition and accurate histopathologic diagnosis are essential to appropriate treatment. With increased awareness of this cutaneous disease and its potential relationship with severe life-threatening illnesses, otolaryngologists can play a central role in ensuring satisfactory patient outcomes. Article Submissions Residents and fellows in otolaryngology are invited to submit quiz cases for this section and to write letters to the Archives commenting on cases presented. Quiz cases should follow the patterns established. See Instructions for Authors (http://archotol.ama-assn.org/misc/ifora.dtl). Material for CLINICAL PROBLEM SOLVING: PATHOLOGY should be submitted electronically via the online submission and review system at http://manuscripts.archoto.com. Reprints are not available from the authors. References 1. Weaver DK Atypical lymphadenopathies of the head and neck. Crit Rev Clin Lab Sci 1981;15 (1) 1- 24PubMedGoogle ScholarCrossref 2. Steffen C Frederic Woringer: Pautrier-Woringer disease (lipomelanotic reticulosis/dermatopathic lymphadenitis. Am J Dermatopathol 2004;26 (6) 499- 503PubMedGoogle ScholarCrossref 3. Gould EPorto RAlbores-Saavedra JIbe MJ Dermatopathic lymphadenitis: the spectrum and significance of its morphologic features. Arch Pathol Lab Med 1988;112 (11) 1145- 1150PubMedGoogle Scholar 4. Sudilovsky DCha I Fine needle aspiration cytology of dermatopathic lymphadenitis. Acta Cytol 1998;42 (6) 1341- 1346PubMedGoogle ScholarCrossref 5. Iyer VKKapila KVerma K Fine needle aspiration cytology of dermatopathic lymphadenitis. Acta Cytol 1998;42 (6) 1347- 1351PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology–Head & Neck Surgery American Medical Association

Pathology Quiz Case 2: Diagnosis

Archives of Otolaryngology–Head & Neck Surgery , Volume 133 (7) – Jul 1, 2007

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Publisher
American Medical Association
Copyright
Copyright © 2007 American Medical Association. All Rights Reserved.
ISSN
0886-4470
DOI
10.1001/archotol.133.7.730-b
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Abstract

Diagnosis: Dermatopathic lymphadenitis (DL) Dermatopathic lymphadenitis characteristically presents as a single or multiple enlarged axillary or inguinal lymph nodes. The lymph nodes are usually discrete, superficial, nontender, firm, and freely movable.1 Presentation as an enlarged neck mass is exceedingly rare. The disease is typically associated with a benign pruritic skin disorder. It is frequently noted as one of many findings in a systemic disease (eg, chronic lymphocytic leukemia or AIDS). It may also be associated with a cutaneous neoplasm (eg, mycosis fungoides or Sézary syndrome) or discovered as an isolated finding. Dermatopathic lymphadenitis was first described as lipomelanotic reticulosis because of the obvious accumulation of lipids and melanin pigment in the cortical region of lymph nodes.2 Further research that elucidated a relationship between the characteristic appearance of the lymph nodes and pruritic skin diseases led to the name dermatopathic lymphadenitis.2 The histologic and immunohistochemical features of DL have been well described. The lymph node paracortex is expanded by pale-staining phagocytic histiocytes, Langerhans cells, and interdigitating reticulum cells. The cells have pale, occasionally vacuolated cytoplasm with elongated folded nuclei.1,3 Macrophages may contain intracytoplasmic melanin, but this finding is inconsistent. Lymphocytes with irregular to cerebriform nuclei accompany the proliferation of histiocytes. The Langerhans and interdigitating reticulum cells are related antigen-presenting cells that express HLA-DR, OKT6, and S100 protein, which is sometimes used as a diagnostic marker for DL. However, it has been shown that S100 protein is not sensitive or specific for DL and does not correlate with disease severity. In one series, only 10% to 60% of lymph nodes stained positive in cases in which the histopathologic characteristics of DL were present.3 In an attempt to provide a minimal definition for the histologic characteristics of DL, Gould et al3 noted a histologic continuum for the paracortical changes. They described the continuum with 4 grades, ranging from sparse histiocytes or dendritic cells (grade 1) to sheets of these cells obscuring the germinal centers (grade 4). The higher the grade, the higher the incidence of concomitant skin disease. As suggested by the name, DL is usually accompanied by generalized acute or chronic pruritic skin diseases such as psoriatic erythroderma. It has been hypothesized that this disorder is a nonspecific reactive change due to barrier disruption of dependent skin.4 Melanin pigment was noted to be phagocytosed by melanophores in the disrupted dermis and transported to the lymph nodes. There was no evidence of increased production of melanin. Furthermore, DL has been described in the absence of clinical skin disease. The incidence of DL associated with skin disease at the time of diagnosis is estimated to be between 66% and 91%.3 It has been speculated that this discrepancy is accounted for by a skin disease within the previous few years. Since Langerhans cells, histiocytes, and melanin have the potential to stay in lymph nodes for years, a lymph node indicative of DL can reflect a resolved skin disease or the cumulative effect of many minimal dermatologic abnormalities. Also, DL has been described in patients with pruritus without any identifiable skin disease, reinforcing the idea that this finding is due to barrier disruption of dependent skin.3 A more ominous association of DL has been noted in patients with mycosis fungoides, chronic lymphocytic leukemia, Sézary syndrome, and AIDS. It is unfortunately difficult to distinguish DL that is associated with mycosis fungoides from DL without mycosis fungoides. Much effort has been given to analysis of fine-needle aspiration cytology (FNA) for this differentiation. As mycosis fungoides develops, the histopathologic features may be indistinguishable from those of DL. Both entities demonstrate diffuse pale cells, stain positive for S100 protein, and have cerebriform lymphocytes.3-5 The earliest distinguishing feature is an increase in the size of the cerebriform lymphocytes within the paracortical areas. As the cells enlarge and multiply, they invade the medullary cords and sinus and form monomorphic infiltrates, defining a malignant neoplasm.1 It has been noticed that patients with AIDS seem to have a higher incidence of DL than the normal population. A few hypotheses have been suggested to explain this increased incidence. Patients with AIDS are known to have a wide variety of skin diseases that can range from minor to severe and may cause more frequent DL-like changes. A second explanation may be that DL reflects an intrinsic part of the AIDS disease process. Langerhans cells are decreased in the epidermis of patients with AIDS and are thought to migrate to the paracortical region of lymph nodes, simulating the DL histopathologic features.3 Because DL has the potential to be associated with life-threatening disease, a thorough investigation should be undertaken to discover the underlying cause. Any history of skin disease, itching, human immunodeficiency virus, or other constitutional symptoms should be elucidated. Other causes of head and neck masses should be ruled out. Thorough examinations of the head and neck and skin should be performed. If the cause remains unknown, a dermatologic evaluation may prove beneficial. Fine-needle aspiration of the mass is the simplest means to make a diagnosis and to exclude other causes of lymphadenopathy.4,5 In a study to determine the FNA morphological criteria for DL, Iyer et al5 found that the presence of melanin-laden macrophages, large histiocytic clusters with central blood vessels, and histiocytes with elongated, crumpled, vesicular nuclei and pseudonucleoli, as well as positivity for S100 protein and negativity for CD68, all aid in the diagnosis. Unfortunately, none of these criteria is absolutely diagnostic of DL, and the results of FNA are not always accurate. Excisional lymph node biopsy is therefore the procedure of choice for definitive diagnosis and treatment because the cerebriform cells may be present only locally and are often missed on FNA. Excisional biopsy is indicated in all cases in which mycosis fungoides is suspected. Dermatopathic lymphadenitis rarely presents as a neck mass and is therefore infrequently seen by otolaryngologists. However, early recognition and accurate histopathologic diagnosis are essential to appropriate treatment. With increased awareness of this cutaneous disease and its potential relationship with severe life-threatening illnesses, otolaryngologists can play a central role in ensuring satisfactory patient outcomes. Article Submissions Residents and fellows in otolaryngology are invited to submit quiz cases for this section and to write letters to the Archives commenting on cases presented. Quiz cases should follow the patterns established. See Instructions for Authors (http://archotol.ama-assn.org/misc/ifora.dtl). Material for CLINICAL PROBLEM SOLVING: PATHOLOGY should be submitted electronically via the online submission and review system at http://manuscripts.archoto.com. Reprints are not available from the authors. References 1. Weaver DK Atypical lymphadenopathies of the head and neck. Crit Rev Clin Lab Sci 1981;15 (1) 1- 24PubMedGoogle ScholarCrossref 2. Steffen C Frederic Woringer: Pautrier-Woringer disease (lipomelanotic reticulosis/dermatopathic lymphadenitis. Am J Dermatopathol 2004;26 (6) 499- 503PubMedGoogle ScholarCrossref 3. Gould EPorto RAlbores-Saavedra JIbe MJ Dermatopathic lymphadenitis: the spectrum and significance of its morphologic features. Arch Pathol Lab Med 1988;112 (11) 1145- 1150PubMedGoogle Scholar 4. Sudilovsky DCha I Fine needle aspiration cytology of dermatopathic lymphadenitis. Acta Cytol 1998;42 (6) 1341- 1346PubMedGoogle ScholarCrossref 5. Iyer VKKapila KVerma K Fine needle aspiration cytology of dermatopathic lymphadenitis. Acta Cytol 1998;42 (6) 1347- 1351PubMedGoogle ScholarCrossref

Journal

Archives of Otolaryngology–Head & Neck SurgeryAmerican Medical Association

Published: Jul 1, 2007

References