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E-IJD CASE REPORT
Year : 2019  |  Volume : 64  |  Issue : 4  |  Page : 338
Erythema gyratum repens associated with diffuse b-cell lymphoma-report of a rare case


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

Date of Web Publication5-Jul-2019

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


DOI: 10.4103/ijd.IJD_228_17

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   Abstract 


Erythema gyratum repens (EGR) is a rare paraneoplastic dermatosis known to be associated with internal malignancy. Herein, we report a case of EGR with diffuse large B-cell lymphoma (DLBCL) in a young farmer who presented with generalized massive lymphadenopathy and wood-grain pattern dermatosis. Lymph node biopsy with immunohistochemistry established the diagnosis of DLBCL. He was managed by the oncologist with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) regimen which alleviated his symptoms considerably after completion of three cycles and was under follow-up.


Keywords: Diffuse large b-cell lymphoma, erythema gyratum repens, wood-grain pattern


How to cite this article:
Rao AG, Farheen SS, Amit K, Reddy UD, Aparna K, Kranthi J, Hukkani R. Erythema gyratum repens associated with diffuse b-cell lymphoma-report of a rare case. Indian J Dermatol 2019;64:338

How to cite this URL:
Rao AG, Farheen SS, Amit K, Reddy UD, Aparna K, Kranthi J, Hukkani R. Erythema gyratum repens associated with diffuse b-cell lymphoma-report of a rare case. Indian J Dermatol [serial online] 2019 [cited 2019 Jul 17];64:338. Available from: http://www.e-ijd.org/text.asp?2019/64/4/338/262166





   Introduction Top


Erythema gyratum repens (EGR) is a figurate erythema known to be a paraneoplastic process characterized by concentric erythematous bands forming wood-grain pattern. It is known to be associated with a variety of malignancies such as lung cancer, esophageal cancer, and breast cancer.[1] Moreover, it is considered as the most characteristic cutaneous manifestation of solid tumors.[2]

Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma. It usually starts as a mass in a lymph node in the body, such as in the chest or abdomen, or in a lymph node in the neck or armpit. It can also start in other areas such as the bone, intestines, or the brain or spinal cord.


   Case Report Top


A 36-year-old farmer presented with painless massive swellings over the neck and inguinal region of 3-year duration, followed by a rash all over the body a year later. Initially, a small swelling was noticed in the right inguinal region which increased gradually in size. Similar swellings were noticed in the left inguinal, both submandibular, cervical, and axillary regions. One year later, he developed an asymptomatic rash all over the body. There was no history of intake of immunosuppressive drugs. There were no associated breathlessness, cough, or loss of weight. Examination revealed massive enlargement of lymph nodes involving both submandibular, cervical, axillary, and inguinal groups and were matted, nontender, freely mobile, firm in consistency. Three subcutaneous firm nodules were also palpable along the left posterior axillary fold. Skin-colored papules and annular plaques were distributed on the front and back of the trunk, upper and lower limbs. Plaques ranged between 1 and 2 cm in size, round to irregular shape, hyperpigmented center with hypopigmented scaly border giving the appearance of wood grain [Figure 1]. Collarette scaling was evident in some plaques. Palms and soles were spared. Moderate hepatosplenomegaly was noticed. He was provisionally diagnosed as EGR with lymphoma. Routine investigations including complete blood picture, chest, and skull skiagram were unremarkable. Serology for human immune deficiency virus and venereal diseases research laboratory were nonreactive. Lactic dehydrogenase level was normal. Ultrasonography revealed hepatosplenomegaly. Mantoux's test was negative. Bone marrow aspiration showed cellular aspirate with a normal erythroid myeloid ratio. Cerebrospinal fluid analysis revealed normal study. Computed tomography showed massive lymphadenopathy of bilateral cervical, axillary, and inguinal groups. Fine-needle aspiration cytology from the axillary lymph node showed a monotonous population of cells with hyperchromatic nuclei and scanty cytoplasm suggestive of non-Hodgkin lymphoma [Figure 2]a. Lymph node biopsy showed replacement of lymph node architecture with diffuse sheets of lymphoid cells with irregular nuclear contours and fine chromatin suggestive of DLBCL [Figure 2]b and [Figure 2]c. Cutaneous deposits also showed lymphoid cells with irregular nuclear contours and fine chromatin suggestive of DLBCL [Figure 2]d. Skin biopsy showed basal cell degeneration with pigment incontinence and perivascular lymphohistiocytic infiltrate in the dermis. Immunohistochemistry study revealed CD 20 diffuse +++ positivity [Figure 3]a, CD 10 was ++ [Figure 3]b focal CD3+ positivity [Figure 3]c, and CD5, CD23, CD 43, and CD103 were negative, consistent with non-Hodgkin DLBCL. He was placed in Stage IV of Ann Arbor Classification of Lymphoma and was referred to oncologist for further management. He was managed with R-CHOP regimen [3] consisting of rituximab 375 mg IV on day 1 plus cyclophosphamide 750 mg IV on day 1 plus doxorubicin 50 mg IV on day 3 plus vincristine 1.4 mg IV on day 3 plus prednisone 40 mg daily on days 1–5. The cycle was repeated every 21-day for 6 cycles. The EGR regressed completely and lymphadenopathy was reduced considerably on completion of three cycles of R-CHOP regimen [Figure 4]a and [Figure 4]b.
Figure 1: Annular, irregularly oval scaly plaques resembling wood grain on trunk

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Figure 2: (a) Fine-needle aspiration cytology from axillary lymph node showing a monotonous population of cells with hyperchromatic nuclei and scanty neoplasm (H and E, x40) (b) Histopathology of lymph node (H and E ×40) showing replacement of lymph node architecture by diffuse sheets of lymphoid cells. (c) Histopathology of lymph node showing sheets of large lymphoid cells with irregular nuclear contours and fine chromatin (H and E, ×100). (d) Histopathology of cutaneous deposit showing large lymphoid cells with irregular nuclear contours and fine chromatin (H and E, ×40)

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Figure 3: (a) Immunohistochemistry, the cells showing CD 20 diffuse +++ positivity (b) Immunohistochemistry, the cells showing CD 10++ positivity. (c) Immunohistochemistry, the cells showing CD3+ positivity (x10)

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Figure 4: (a) Massive bilateral inguinal lymphadenopathy. (b) Complete regression of erythema gyratum repens and considerable decrease of lymphadenopathy following three cycles of R-CHOP therapy

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   Discussion Top


EGR is seen most often among Caucasians, with a male-to-female ratio of 2:1. An underlying neoplasm can be detected in 82% of cases of EGR. Moreover, Rongioletti et al. while reviewing 120 cases of EGR detected neoplasm in 70% of them.[4] It may precede or occur simultaneously or may follow malignancy.[5] However, the interval between the development of malignancy and EGR is not known. EGR manifested after a gap of 1 year of the development of DLBCL in the index case. Moreover, it has been reported that removal of the carcinoma results in clearance of the EGR, validating the causal relationship. In concert with this, the EGR also disappeared in the index case following three cycles of R-CHOP therapy.

The cause of DLBCL is unknown; however, certain factors are known to increase the risk, such as immunosuppression, pesticides, ultraviolet radiation, hair dyes, and diet.[6] Incidentally, the index case, farmer by occupation, had regular exposure to pesticides and ultraviolet radiation which might have potentially increased the risk for the development of lymphoma.

The characteristic pattern of skin rash, wood grain, or fractal-like geometrics is said to be due to change in body chemistry as a result of excretion by tumor cells or immune response.[7] Moreover, Forrester found elevated levels of glutamine in concentric rings of EGR and attributed to hungry neoplastic cells which thrive on transport of glutamine into mitochondria.[8] Furthermore, EGR has been reported in association with other conditions such as cryptogenic organizing pneumonia,[9] linear immunoglobulin A dermatosis, hypereosinophilic syndrome, pulmonary tuberculosis, ichthyosis, palmoplantar keratoderma, pemphigus vulgaris, ulcerative colitis, systemic lupus erythematosus, and drugs.

A literature search failed to reveal the association of EGR with DLBCL, and this may be the 1st case of association of EGR with DLBCL. However, Mantle B-cell non-Hodgkin lymphoma [10] and anaplastic large cell lymphoma have been associated with erythema annulare centrifugum.[11]

Management of DLBCL depends on the stage of the disease, and various regimes used are R-CHOP(rituximab, cyclophosphomide, doxorubicin, vincristine, and prednisone), RICE(rituximab, ifosfamide, carboplatin, etoposide), and DHAP (rituximab, cisplatin, cytarabine, and dexamethasone), local radiation therapy and autologous stem cell transplantation. Prognosis is usually guarded in DLBCL; however, it is not highly malignant in the index case as he has been enduring the ailment for the last 3 years.

Management of EGR includes recognition and treatment of underlying malignancy. Various therapies used are systemic steroids, Vitamin A, topical steroid, and azathioprine; however, they failed to relieve the symptoms of EGR.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Eubanks LE, McBurney E, Reed R. Erythema gyratum repens. Am J Med Sci 2001;321:302-5.  Back to cited text no. 1
    
2.
Kleyn CE, Lai-Cheong JE, Bell HK. Cutaneous manifestations of internal malignancy: Diagnosis and management. Am J Clin Dermatol 2006;7:71-84.  Back to cited text no. 2
    
3.
Feugier P, Van Hoof A, Sebban C, Solal-Celigny P, Bouabdallah R, Fermé C, et al. Long-term results of the R-CHOP study in the treatment of elderly patients with diffuse large B-cell lymphoma: A study by the group on lymphomas in adults. J Clin Oncol 2005;23:4117-26.  Back to cited text no. 3
    
4.
Rongioletti F, Fausti V, Parodi A. Erythema gyratum repens is not an obligate paraneoplastic disease: A systematic review of the literature and personal experience. J Eur Acad Dermatol Venereol 2014;28:112-5.  Back to cited text no. 4
    
5.
Boyd AS, Neldner KH, Menter A. Erythema gyratum repens: A paraneoplastic eruption. J Am Acad Dermatol 1992;26:757-62.  Back to cited text no. 5
    
6.
Blinder V, Fisher SG; Lymphoma Research Foundation, New York. The role of environmental factors in the etiology of lymphoma. Cancer Invest 2008;26:306-16.  Back to cited text no. 6
    
7.
Cócera M. Characterisation of skin states by non-crystalline diffraction. Soft Matter 2011;7:8605-11.  Back to cited text no. 7
    
8.
Forrester DM. Self-assembled multi-ring formations of glutamine and a possible link to erythema gyratum repens. Med Hypotheses 2015;85:10-6.  Back to cited text no. 8
    
9.
Samotij D, Szczech J, Bencal-Kusinska M, Reich A. Erythema gyratum repens associated with cryptogenic organizing pneumonia. Indian J Dermatol Venereol Leprol 2016;82:212-3.  Back to cited text no. 9
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10.
Carlesimo M, Fidanza L, Mari E, Pranteda G, Cacchi C, Veggia B, et al. Erythema annulare centrifugum associated with mantle B-cell non-Hodgkin's lymphoma. Acta Derm Venereol 2009;89:319-20.  Back to cited text no. 10
    
11.
Ural AU, Ozcan A, Avcu F, Kaptan K, Taştan B, Beyan C, et al. Erythema annulare centrifugum as the presenting sign of CD 30 positive anaplastic large cell lymphoma – Association with disease activity. Haematologia (Budap) 2001;31:81-4.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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