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| IMAGES IN MEDICINE |
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| Year : 2017 | Volume
: 19
| Issue : 2 | Page : 151-152 |
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Subcutaneous panniculitis like T-Cell lymphoma
Gurjeet Singh Chowdhary
INHS Asvini, Mumbai, Maharashtra, India
| Date of Web Publication | 13-Feb-2018 |
Correspondence Address:
Dr. Gurjeet Singh Chowdhary
Oic Oncology Center, INHS Asvini, Colaba, Mumbai - 400 005, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jmms.jmms_62_17
Facial swellings could arise symmetrically due to underlying congenital; or acquired disorders such as renal, cardiac, endocrine, or storage disorders, mostly the manifestations of systemic illness in the face. It requires a high index of clinical suspicion by an alert clinician to distinguish the emergency and diffuse inflammatory causes as in lymphedema, hay fever, etc., or an abscess or dental etiologies which are mostly well lateralized from long-standing causes which may be vascular malformations, fibrous dysplasias or slowly progressive and sinister swellings due to malignancies. These cause significant distress to both the patient and the treating clinician. This case highlights the need to look beyond the usual or common causes since these underlying diseases may be sinister yet highly treatable and ameliorate the patients' symptoms dramatically. This case highlights a mature T-cell cutaneous lymphoma who presented repeatedly with little relief from treatment initially offered.
Keywords: Rimming of fat cells, subcutaneous panniculitis-like, T-cell lymphoma
How to cite this article:
Chowdhary GS. Subcutaneous panniculitis like T-Cell lymphoma. J Mar Med Soc 2017;19:151-2 |
| Introduction | |  |
The causes of long-standing facial swelling are usually due to cardiac, renal, storage, or endocrine etiologies which resolve by treating the primary disorder. Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) Initially described by Gonzalez et al. in 1991 is a rare cutaneous lymphoma that involves the face, legs or trunk in young adults. It should be suspected in case of atypical or non-resolving dermatological manifestations.
| Case Report | | |
A 39-year-old male never smoker, with no known comorbid illness presented with a history of on and off joint pains with periorbital swelling for 4 weeks. Examination revealed a diffuse right hemifacial plaque 7 cm × 5 cm and oval plaque 5 cm × 4 cm over a region of the left forehead. He also had a right upper lip necrotic scar [Figure 1]. Positron emission tomography (PET)-computed tomography scan showed fluorodeoxyglucose-avid lesions in teres minor, and serratus anterior skin biopsy confirmed primary cutaneous T-cell lymphoma, and he was staged to T2N0M0. After receiving oral prednisolone for over 15 months to which he had no meaningful response, he was given CHOP Regimen includes intravenous Cyclophosphamide, Hydroxyldaunorubicin (Doxorubicin), ONCOVIN ® (Vincristine) and oral Prednisone chemotherapy and is showing gradual improvement clinically and on PET imaging [Figure 1] and [Figure 2]. The rare diagnosis of primary cutaneous (alpha-beta subtype CD4−, CD8+, and CD56− on malignant lymphoid cells) has over 85% 5 years overall survival.[1],[2] This patient has likely progressed from an undifferentiated mixed connective tissue disorder.[4] | Figure 2: The eyes of the patient may be covered – before and after clinical treatment and positron emission tomography-computed tomography images
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| Discussion | | |
Subcutaneous panniculitis type of T-cell cutaneous lymphoma is a very rare disease with an indolent course which requires a multidisciplinary approach to treatment by oncologist and dermatologist.[3] Since the diagnosis may easily be missed by the untrained eye, therefore, the immunohistiochemistry and morphological pattern would correctly diagnose this condition. Most cutaneous lymphomas occur due to chronic irritation or inflammation as by underlying vasculitis. These are mostly CD3 and 8 positive and CD4 and 56 negative with an alpha-beta T-cell phenotype.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. Subcutaneous panniculitis-like T-cell lymphoma: Definition, classification, and prognostic factors: An EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood 2008;111:838-45.  [ PUBMED] |
| 2. | Salhany KE, Macon WR, Choi JK, Elenitsas R, Lessin SR, Felgar RE, et al. Subcutaneous panniculitis-like T-cell lymphoma: Clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. Am J Surg Pathol 1998;22:881-93. |
| 3. | Ghobrial IM, Weenig RH, Pittlekow MR, Qu G, Kurtin PJ, Ristow K, et al. Clinical outcome of patients with subcutaneous panniculitis-like T-cell lymphoma. Leuk Lymphoma 2005;46:703-8. [ PUBMED] |
| 4. | Cassis TB, Fearneyhough PK, Callen JP. Subcutaneous panniculitis-like T-cell lymphoma with vacuolar interface dermatitis resembling lupus erythematosus panniculitis. J Am Acad Dermatol 2004;50:465-9. [ PUBMED] |
[Figure 1], [Figure 2]
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