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Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 837
Idiopathic cutaneous pseudolymphoma

Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India

Date of Web Publication23-Feb-2023

Correspondence Address:
Angoori Gnaneshwar Rao
Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_518_22

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How to cite this article:
Rao AG, Naresh M, Sindhuja B, Pranaya B, N Sindhu V S, Shreeja V. Idiopathic cutaneous pseudolymphoma. Indian J Dermatol 2022;67:837

How to cite this URL:
Rao AG, Naresh M, Sindhuja B, Pranaya B, N Sindhu V S, Shreeja V. Idiopathic cutaneous pseudolymphoma. Indian J Dermatol [serial online] 2022 [cited 2023 Jun 6];67:837. Available from:


A 45-year-old lady was presented to the dermatology department with a complaint of asymptomatic swelling over the left side of her nose for 2 months. The patient was apparently alright 2 months ago, and then she developed a small swelling over the left side of her nose, which gradually increased in size to attain the present size. No history of insect bite, trauma, or drug intake. No history of epiphora. She denied any history of chronic liver disease, borrelia infection, or tattoos. Examination revealed a single erythematous oval nodule of 2.5 × 2 cm in size over the left side of the nose, soft in consistency and nontender [Figure 1]. The local temperature was not raised. The surface of the nodule showed a conglomeration of tiny papules. There was no regional lymphadenopathy. She was provisionally diagnosed as having angiolipoma. Nodular basal cell carcinoma, neurofibroma, and myxoma were considered in the differential diagnosis. Routine hematological and biochemical investigations were within normal limits. A chest skiagram, abdominal ultrasonography, and positron emission tomography scan revealed no evidence of a tumour. Serology for human immunodeficiency virus (HIV), herpes simplex virus, venereal research laboratory test, hepatitis C virus, and hepatitis B virus antigen (HbsAg) was nonreactive. Histopathological examination of a biopsy taken from the nodule showed hyperkeratotic and parakeratotic epidermis, and the dermis showed intense lymphocytic infiltration, most of which were small lymphocytes [Figure 2]. Occasional lymphocytes showed mild large nuclei with cleaved nuclei [Figure 3]. Lymphocytic infiltration was also seen in the perifollicular area. The histopathological features were consistent with pseudolymphoma. To further characterize, immunohistochemistry was performed, which revealed positivity for CD3 [Figure 4], CD4 [Figure 5], CD8 [Figure 6], CD20 [Figure 7], and partial positivity for CD10 [Figure 8] and negativity for CD30 [Figure 9]. KI 67 (labelling index) was <5% [Figure 10]. The histopathological and immunohistochemical studies assisted in corroborating the diagnosis of pseudolymphoma. The patient was managed with intralesional corticosteroid therapy, which resulted in a complete resolution of the lesion.
Figure 1: (Original) Erythematous oval nodule over the left side of the nose

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Figure 2: (Original) Histopathological examination of a biopsy of the nodule. The epidermis is hyperkeratotic and parakeratotic, and the dermis shows intense lymphocytic infiltration. Most of them are small lymphocytes. (H and E stain, ×10)

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Figure 3: (Original) Histopathological examination of a biopsy of the nodule; intense lymphocytic infiltration; most of them are small lymphocytes. Occasional lymphocytes show mild large nuclei with cleaved nuclei. (H and E stain, ×40)

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Figure 4: (Original) Immunohistochemistry showing CD3 positivity (×40)

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Figure 5: (Original) Immunohistochemistry showing CD4 positivity (×40)

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Figure 6: (Original) Immunohistochemistry showing CD8 positivity (×40)

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Figure 7: (Original) Immunohistochemistry, positive for CD20 (×40)

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Figure 8: (Original) Immunohistochemistry, partially positive for CD10 (×40)

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Figure 9: (Original) Immunohistochemistry, negative for CD30 (×40)

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Figure 10: (Original) Immunohistochemistry, Ki (labelling index) positivity <5% (×40)

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The term cutaneous pseudolymphoma (C-PSL) was first described by Kaposi under the term sarcomatosis cutis. It neither refers to a specific disease nor implies any aetiology but simply implies the accumulation of lymphocytes in the skin in response to a variety of stimuli.[1] It is a reactive lymphoproliferative disorder predominantly involving B-cells or T-cells. It may be localized or disseminated and is classified into B-cell pseudolymphoma (B-PSL) and T-cell pseudolymphoma. C-PSL affects all age groups and presents as an erythematous nodule mainly involving the face and chest. However, multiple lesions have also been reported. Multiple agents known to cause C-PSL are physical, chemical agents, infections, drugs, tattoos, vaccinations, insect bites,[2] hair dye,[3] secondary syphilis, and viral infections, such as orf, milker's nodule, herpes simplex, herpes zoster. Various drugs known to cause C-PSL include phenytoin, carbamazepine, lamotrigine, phenobarbital, flucloxacillin, dapsone, cefixime, nitrofurantoin, penicillin, sulfonamides, amitriptyline, doxepin, fluoxetine, lithium. Drug-induced pseudolymphoma has a more insidious onset and usually appears several weeks after the beginning of the drug. Many a time, the cause may not be identified and is termed idiopathic. Similarly, the cause of C-PSL in the index case could not be established.

The C-PSL in the index case is composed of both T-cells and B-cells. Hence, low-grade T-cell neoplasm (mycosis fungoides) is unlikely because these T-cell neoplasms are composed of T-cells and their nuclei are cerebriform, both of which were not seen in the present case. Moreover, T-cell-rich B-cell lymphoma is unlikely because the B-cells did not have as much atypia as seen in T-cell-rich B-cell lymphoma.

In the present case, the C-PSL was positive for both CD4 and CD8 cells. Dual positivity is extremely rare and is commonly associated with HIV infection. However, the index case is immunocompetent.[4]

Single lesions of C-PSL can be treated with high-potent topical steroids, intralesional steroids, surgical excision, cryotherapy, and local radiation. Incidentally, the C-PSL was managed with intralesional steroids as it was a single lesion. Disseminated lesions can be treated with systemic steroids, tetracyclines, interferon alpha, photochemotherapy, hydroxychloroquine, and cytotoxic agents.[5],[6]

The clinical course of C-PSL is usually variable. Some lesions regress after biopsy, while some others persist for months to years. Rarely, C-PSL can progress to cutaneous lymphoma, but what triggers such progression is not well understood. However, certain clinical features such as multiple lesions and lymphadenopathy should alert a clinician to the onset of cutaneous lymphoma.[7] Interestingly, the transformation of lymphoid hyperplasia into gastric mucosa-associated lymphoid tissue lymphoma due to persistent antigenic stimulation induced by Helicobacter pylori infection was reported by Chen et al.[8] Hence, effort should be made to unearth the cause of pseudolymphoma so as to prevent the onset of lymphoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given her consent for images and other clinical information to be reported in the journal. The guardian understands that her names and initials will not be published and due efforts will be made to conceal the patient's identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Kerl H, Ackerman AB. Inflammatory diseases that simulate lymphomas: Cutaneous pseudolymphomas. In: Fitzpatrick TB, Isen AZ, Wolf K, Freedberg IM, Austin KF, editors. Dermatology in General Medicine. New York: McGraw-Hill; 1993. p. 1315-27.  Back to cited text no. 1
Burge G, Wood G, Kempt W, Schmit U, Sander CA, Cogliatti S. Lymphoid infiltrates of the skin mimicking lymphoma (cutaneous pseudolymphoma). In: LeBoit PE, Burg G, Weedon D, Saracin A. editors. World Health Organisation Classification of Tumors Tumor Pathology and Genetics of Tumors. Lyon: IARC Press; 2006. p. 212–4.  Back to cited text no. 2
Sequeira FF, Jayasheelan E, Stephen J. Paraphenylenediamine-induced cutaneous pseudolymphoma. Indian J Dermatol 2014;59:424.  Back to cited text no. 3
[PUBMED]  [Full text]  
Bergman, R. Pseudolymphoma and cutaneous lymphoma: Facts and controversies. Clin Dermatol 2010;28:568-74.  Back to cited text no. 4
Romero-Perez D, Martinez M, Encabo-Duran B. Pseudolymphomas cutaneous. Acta Dermosifiliogr 2016;107:640-51.  Back to cited text no. 5
Zhou LL, Mistry N. Cutaneous lymphoid hyperplasia (pseudolymphoma). CMAJ 2018;190:E398.  Back to cited text no. 6
Miguel D, Peckruhn M, Elsner P. Treatment of cutaneous pseudo lymphoma: A systematic review. Acta Derm Venereol 2018;98:310-7.  Back to cited text no. 7
Chen XY, Liu WZ, Shi Y, Zhang DZ, Xiao SD, Tytgat GNJ. Helicobacter pylori associated disease and lymphoid tissue hyperplasia in gastric antral mucosa. J Clin Pathol 2002;55:133-7.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]


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