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Year : 2016  |  Volume : 59  |  Issue : 1  |  Page : 81-83
Type D lymphomatoid papulosis simulating aggressive epidermotropic cytotoxic lymphoma

1 Department of Dermatology, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India

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Date of Web Publication9-Mar-2016


Three histological subtypes of lymphomatoid papulosis (LyP), type A (histiocytic), type B (mycosis fungoides like) and type C (anaplastic large cell lymphoma like) are well recognized. Two new histological variants, type D (simulating an aggressive epidermotropic cytotoxic lymphoma) and type E (angioinvasive type) has been described recently. We describe a 27-year-old man presented with a history of asymptomatic erythematous papules on both upper and lower limbs noted since 10 years of age. There were no systemic symptoms. Biopsy revealed an atypical dermal lymphoid infiltrate with epidermotropism, and the immunohistochemical markers showed a diffuse positivity for CD3, CD8, CD56, T1A and granzyme B with the focal positivity of CD30. All other relevant tests were normal. In this case report of a recently described delineated variant of LyP we emphasize the indolent course of this entity although the histology would suggest a more aggressive disease.

Keywords: CD30 lymphomas, cutaneous lymphoma, lymphomatoid papulosis

How to cite this article:
Mani V, George R, Vijayakumar K, Nair S. Type D lymphomatoid papulosis simulating aggressive epidermotropic cytotoxic lymphoma . Indian J Pathol Microbiol 2016;59:81-3

How to cite this URL:
Mani V, George R, Vijayakumar K, Nair S. Type D lymphomatoid papulosis simulating aggressive epidermotropic cytotoxic lymphoma . Indian J Pathol Microbiol [serial online] 2016 [cited 2021 Oct 25];59:81-3. Available from: https://www.ijpmonline.org/text.asp?2016/59/1/81/174823

   Introduction Top

Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative disorder characterized clinically by chronic recurrent self-healing papulonodular or papulonecrotic lesions. LyP and cutaneous anaplastic large cell lymphoma form a spectrum of CD30+ lymphoproliferative disorders which is usually differentiated by clinical appearance, course, and histological criteria. LyP usually persists for years or decades with an excellent prognosis. [1] The three well recognized histological subtypes of LyP are (i) type A, characterized by a mixed infiltrate containing large atypical CD30+ cells intermingled with small lymphocytes, histiocytes, neutrophils and or eosinophils, (ii) type B, characterized by an epidermotropic infiltrate of small atypical lymphoid cells with cerebriform nuclei and histologically resembles mycosis fungoides, and (iii) type C, characterized by large CD30+ cells with relatively few admixed inflammatory cells which mimics anaplastic large T-cell lymphomas but with an indolent clinical course. [1] Recently, two new clinicopathological variants have been described. They include LyP type D in which the histology simulates an aggressive epidermotropic CD8 positive cytotoxic T-cell lymphoma but clinically follows an indolent course and type E, characterized by angioinvasive infiltrate of small to large atypical lymphoid cells which are CD30+, CD8+ and T-cell intracellular antigen-1 (TIA-1)+ which also follows an indolent course with an excellent prognosis. [2],[3] In this report, we describe the clinical course and histopathological features of LyP type D in an adult Indian male which manifested first at 10 years of age.

   Case report Top

A 27-year-old man presented with a history of asymptomatic erythematous papules on both upper and lower limbs which were first noted at the age of 10. The lesions healed in 7-10 days with varioliform scars. He then had a long period of quiescence. He was first seen in our hospital at the age of 26 years in November 2011 with a history of recurrence of lesions since 3 years. The lesions had progressed to involve the trunk as well as the limbs. He also had occasional lesions on the palms and soles. There was a history of exacerbation during summer. Face, oral mucosa, and scalp were never affected, and there were no associated systemic lesions. A biopsy of a representative lesion was done at that visit, but the patient was lost to follow-up for another 16 months when he returned in March 2013 because of the progressive nature of the symptoms.

On examination, he had erythematous crusted papules on left forearm, dorsum of the right hand, left thigh and dorsal aspect of the feet [Figure 1]a and b. He also had few small erythematous papules on abdomen along with varioliform scars on the back [Figure 1]c]. The general and physical examination were within normal limits. Two skin biopsies were done; the first one was performed when he presented in November 2011. The skin biopsy done at that time from a lesion on the left leg showed parakeratotic crust and superficial and deep perivascular and periadnexal moderate to dense chronic inflammatory infiltrates composed of lymphocytes and histiocytes. There were neither epidermotropism nor atypical lymphoid cells. On immunostaining, the inflammatory infiltrate showed a reactive pattern of staining with CD3 positive T-lymphocytes, CD20 positive B-lymphocytes, and the MIB-1 labeling index was <1%. Immunostaining for CD30 was negative. The second biopsy done in March 2013 from the abdominal wall showed parakeratosis, lymphocytic exocytosis, epidermotropism, superficial and deep dense perivascular, periadnexal and interstitial infiltrate composed of lymphocytes, histiocytes, plasma cells and atypical lymphoid cells with irregularly contoured nuclei showing coarse chromatin and moderate amounts of cytoplasm. Angiocentricity was also present. Immunohistochemistry revealed diffuse positivity of atypical lymphoid cells for CD3, CD8, CD56, TIA-1 and granzyme B with CD30 positivity in a few large atypical lymphoid cells [Figure 2]a-c. CD5, CD7, and CD20 were negative, and CD4 showed focal positivity. Ki67 proliferative index (MIB-1) was around 30%. Other relevant investigations including hemoglobin, total and differential counts, platelet count, blood picture, liver function tests, creatinine, lactate dehydrogenase, and uric acid were within normal limits. An x-ray of chest and ultrasonography of abdomen did not reveal any systemic involvement. A diagnosis of LyP type D was made based on the clinical and histological features. The patient was staged as T3b based on ISCL/EORTC proposal on TNM classification of cutaneous lymphoma other than MF/SS. [4] He continues to have crops of self-healing lesions with no other untoward symptoms.
Figure 1: Erythematous crusted papules on right forearm (a) and left foot (b). Varioliform scars on back (c)

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Figure 2: (a) H and E section of skin showing atypical lymphoid cells in epidermis (epidermotropism) and parakeratotic crust with serum and exudate (×100). (b) CD8 immunostaining highlighting epidermotropism (×100). (c) CD30 immunostaining of atypical lymphoid cells (×400)

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   Discussion Top

Our patient had the typical clinical features of LyP type D of recurrent relatively asymptomatic self-healing erythematous papules with a tendency to ulceration associated with histological features of CD8 positive cytotoxic T-cell lymphoma. [2] There was a gradual progression of the disease, affecting the trunk in the last 3 years. The disease course was punctuated with long periods of quiescence. Interaction between CD30 and CD30L (CD30 ligand) was postulated to play an important role in pathomechanism of CD30+ lymphoproliferative disorders and in particular, overexpression of CD30L might have a major role in self-regression of skin lesions, which is the most distinctive clinical feature of LyP. [5]

LyP, part of the spectrum of CD30+ lymphoproliferative disorders generally occurs in adults with male predominance but may occur in children as well. It usually follows an indolent course and has an excellent prognosis. However, a cohort study has shown that 4% of patients develop systemic lymphoma, and 2% of patients die of the systemic disease over a median follow-up period of 77 months. [6]

The diagnosis of LyP may be difficult due to its high histological variability and overlapping features with other cutaneous lymphomas. Clinicopathological correlation plays an important role in diagnosis and appropriate management in such cases. The clinical and morphological features of LyP type D mimics primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma which is clinically characterized by the presence of localized or disseminated papular or nodular eruptions with central ulceration and necrosis or by superficial hyperkeratotic patches and plaques which may disseminate to other visceral organs including lungs, testis, central nervous system or oral mucosae often sparing lymph nodes. However, the course of primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma is aggressive with a median survival of 32 months and warrants chemotherapy. [7] In a report by Saggini et al., 8 patients with LyP type D were followed up for a mean period of 84 months (range 1-303 months) highlighting the benign nature of the disease. [2] Available treatment modalities for LyP including low-dose oral methotrexate (5-20 mg/week) or psoralen with ultraviolet A light (PUVA) therapy are neither curative nor affects the natural course of the disease. [6] Our patient was managed with topical fluticasone ointment.

   Conclusion Top

We have described a newly delineated variant of (LyP type D) which histologically simulates an aggressive epidermotropic cytotoxic lymphoma of which only a few cases of this entity have been reported in the literature. This case highlights the indolent course of this entity although the histology would suggest a more aggressive disease. Clinicopathological correlation is vital for the correct diagnosis of these cases. The patients, however, should remain under follow-up as some patients can develop systemic lymphoma.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 1
Saggini A, Gulia A, Argenyi Z, Fink-Puches R, Lissia A, Magaña M, et al. A variant of lymphomatoid papulosis simulating primary cutaneous aggressive epidermotropic CD8+cytotoxic T-cell lymphoma. Description of 9 cases. Am J Surg Pathol 2010;34:1168-75.  Back to cited text no. 2
Kempf W, Kazakov DV, Schärer L, Rütten A, Mentzel T, Paredes BE, et al. Angioinvasive lymphomatoid papulosis : A new variant simulating aggressive lymphomas. Am J Surg Pathol 2013;37:1-13.  Back to cited text no. 3
Kim YH, Willemze R, Pimpinelli N, Whittaker S, Olsen EA, Ranki A, et al. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome : A proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood 2007;110:479-84.  Back to cited text no. 4
Mori M, Manuelli C, Pimpinelli N, Mavilia C, Maggi E, Santucci M, et al. CD30-CD30 ligand interaction in primary cutaneous CD30(+) T-cell lymphomas: A clue to the pathophysiology of clinical regression. Blood 1999;94:3077-83.  Back to cited text no. 5
Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, et al. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders : a0 report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood 2000;95:3653-61.  Back to cited text no. 6
Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior. Am J Pathol 1999;155:483-92.  Back to cited text no. 7

Correspondence Address:
Renu George
Department of Dermatology, Christian Medical College, Vellore - 632 008, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.174823

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