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|Year : 2014 | Volume
| Issue : 3 | Page : 335-337
Subcutaneous Panniculitis like T Cell Lymphoma associated with erythromelalgia
J Thomas, BV Maramattom, PM Kuruvilla, J Varghese
Department of Rheumatology, Lourdes Hospital, Kochi, Kerala, India
|Date of Web Publication||14-Aug-2014|
Dr. J Thomas
Department of Rheumatology, Lourdes Hospital, Kochi, Kerala
Source of Support: None, Conflict of Interest: None
Erythromelalgia is a rare disorder that simulates a small fiber neuropathy and patients often have painful erythematous extremities during episodes. It is of two types: A primary or inherited form that is sometimes associated with a Na channel mutation or a secondary disorder associated with an underlying systemic disorder. We present a 19-year-old boy who presented to us with erythromelalgia and a febrile illness with systemic rash. Detailed work-up revealed another unusual condition: Subcutaneous panniculitis like T cell lymphoma (SPTCL). This is the first report of an association of erythromelalgia with SPTCL.
Keywords: Erthromelalgia, secondary erythromelalgia, SPTCL, subcutaneous panniculitis like T cell lymphoma
|How to cite this article:|
Thomas J, Maramattom B V, Kuruvilla P M, Varghese J. Subcutaneous Panniculitis like T Cell Lymphoma associated with erythromelalgia. J Postgrad Med 2014;60:335-7
|How to cite this URL:|
Thomas J, Maramattom B V, Kuruvilla P M, Varghese J. Subcutaneous Panniculitis like T Cell Lymphoma associated with erythromelalgia. J Postgrad Med [serial online] 2014 [cited 2020 Feb 17];60:335-7. Available from: http://www.jpgmonline.com/text.asp?2014/60/3/335/138827
| :: Introduction|| |
Erythromelalgia is a rare condition that presents with episodic painful hyperemic extremities simulating a small fiber neuropathy. It occurs in a primary form and a secondary form. Although treatment options are limited, it is important to recognize the disorder, because it may herald an underlying systemic disorder. We present the first report of erythromelalgia associated with subcutaneous panniculitis like T cell lymphoma (SPTCL).
| :: Case Report|| |
A 19-year-old boy presented to the rheumatology department with severe pricking pain in both legs of 6 months duration. He described the pain as intermittent (4-5 per week), excruciating, located in a stocking distribution and compared the pain to his nails being pulled out. Some of these episodes were associated with redness of his legs predominantly below the knees and soles. His legs had recently become hyperpigmented after repetitive episodes. During exacerbations his pain forced him to limp. In the interim, he was asymptomatic. His past history was remarkable for repeated febrile episodes with skin rashes of 1 year duration. He had a marfanoid habitus; painful pitting pedal edema; and nodular erythematous rashes of the face, abdominal wall, chest, and thighs. Outside investigations included hemoglobin (Hb) 9.3 g/dl, total count of 1,800 cells, polymorphs of 67%, lymphocytes 21%, monocytes 6%, an ESR of 40 mm/h, and a platelet count of 1.7 lakhs. Vasculitic work up, viral markers, and Mantoux test were negative. His peripheral smear had shown bicytopenia (microcytic hypochromic anemia with leukopenia). Serum ferritin level was 1,329 and C-reactive protein (CRP) was 0.54. Arterial Doppler studies of both legs were normal. A bone marrow examination had shown histiocytic proliferation with some phagocytosis of hematopoietic cells suggestive of hemophagocytic syndrome. Ultrasound abdomen had shown splenomegaly. He had been treated with steroids and antibiotics with a provisional diagnosis of hemophagocytic syndrome; possibly secondary to adult onset Still's disease. He had two more episodes of documented hemophagocytic syndrome which were treated outside with steroids.
Presently, on examination, he had a leonine facies; tender nodules over the chest, abdomen, and thighs; scaly hyperpigmented lesions over both shins; and erythema over both soles during an acute episode with hyperesthesia and allodynia in a stocking distribution over the legs with absent ankle jerks [Figure 1], [Figure 2], and [Figure 3]. Nerve conduction velocities showed absent sural nerve (sural sensory nerve action potentials (SNAPs)). A skin biopsy revealed subcutaneous fat with a predominantly lobular infiltration of atypical lymphoid cells [Figure 4]. The fat lobules showed interstitial infiltrate of pleomorphic, small to medium and medium to large atypical lymphoid cells with irregular nuclei having dense chromatin. Characteristically, there was rimming of individual fat cells by surrounding neoplastic lymphoid cells. Mitotic figures and karyorrhectic debris were noted. Histiocytes were seen amidst these lymphoid cells. "Bean bag cells" were also noted in some areas. Immunohistochemistry showed atypical lymphoid cells which were CD3 positive, CD5 positive, CD8 positive, perforin positive, granzyme positive, CD56 negative, and CD 20 negative; confirming the diagnosis of SPTCL. He was initiated on chemotherapy with the CHOP regimen (cyclophosphamide, hydroxydaunorubicin hydrochloride (doxorubicin hydrochloride), vincristine, and prednisone).
|Figure 1: Multiple nodules seen on the trunk and legs with distal hyperpigmentation|
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|Figure 2: Multiple nodules seen on the trunk and legs with distal hyperpigmentation|
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|Figure 4: Histopathological examination (HPE) showing subcutaneous fat with a lobular infiltration of atypical lymphoid cells. Characteristically rimming of individual fat cells by surrounding neoplastic lymphoid cells is seen|
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| :: Discussion|| |
Erythromelagia is a rare condition with episodic redness, swelling, and severe pain of the extremities. Population based incidence studies show a low rate of 1.3 per 100,000 people per year.  Some attacks are precipitated by temperature changes and the color change can extend proximally. The attacks are mostly symmetrical and involve the lower extremities more than the upper extremities. Primary erythromelagia (PEM) is a form of small fiber neuropathy and is sometimes associated with a gain of function missense mutation in the voltage gate Na (V) 1.7 channels which are preferentially located in the dorsal root ganglion (DRG) and sympathetic ganglia.  The mutation results in hyperpolarization of activation, slow inactivation, and activation by minimal stimulation.  Familial PEM can be inherited in an autosomal dominant fashion. 
Secondary erythromelalgia (SEM) can be seen in association with polycythemia vera, myeloproliferative disorders, vasculitis, or secondary to medications. SPTCL was described in 1991 by Gonzalez and colleagues as a group of lymphomas localized primarily in the subcutaneous tissue with features of panniculitis and hemphagocytic syndrome.  It was first included as a provisional entity in the Revised European-American Lymphoma (REAL) classification as SPTCL.  SPTCL is most common in young adults and clinically patients present with fever and multiple subcutaneous nodules, most commonly on the extremities and trunk.  This is the first case report of SPTCL with erythromelalgia.
| :: References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]