Autologous fat grafting to correct severe facial lipoatrophy secondary to subcutaneous panniculitis-like T-cell lymphoma: A case report with 2-year follow-up

Abstract

Subcutaneous panniculitis-like T-cell lymphoma is a rare type of T-cell lymphoma that causes multiple painless subcutaneous nodules over the body. Patients with subcutaneous panniculitis-like T-cell lymphoma may develop facial lipoatrophy that causes disfigurement. We report the case of a 53-year-old female patient with subcutaneous panniculitis-like T-cell lymphoma and severe facial lipoatrophy who underwent three sessions of autologous fat grafting. During the 2-year follow-up, she regained a good facial contour after treatment.

Introduction

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL), first reported by Gonzalez et al. in 1991, is a rare type of primary cutaneous T-cell lymphoma with clinical manifestations resembling those of panniculitis; it accounts for <1% of all non-Hodgkin lymphoma cases.^1–3 Patients with SPTCL usually present with multiple painless subcutaneous nodules or skin ulcers on the extremities and trunk. These lesions can be recurrent and self-healing. ⁴ Some patients may also show B symptoms including fever, weight loss, and night sweats as well as laboratorial abnormalities such as leukocytopenia and liver dysfunction. ⁵ The diagnosis of SPTCL relies on autopsy and histopathological examination. Under the microscope, lobular infiltration of atypical lymphoid cells—almost confined to subcutaneous fat tissue—can be observed. The neoplastic T-lymphocytes surround individual fat cells to form a typical ring-like structure. The T-lymphocytes are CD3⁺ and CD8⁺ along with CD4⁻ and CD56⁻. ⁵ It should be noted that SPTCL and lupus erythematosus panniculitis (LEP) may sometimes overlap. ⁶ They could represent two ends of a spectrum of T cell–mediated orbital lymphoproliferative diseases. ⁷ To distinguish between them, the following information can be used. SPTCL features a dominance of CD8⁺ lymphocytes, and fat necrosis is common, whereas LEP is indicated by the presence of CD20⁺ B lymphocytes and plasma cells. First-line treatment of SPTCL includes corticosteroids or immunosuppressive drugs. Patients who are unresponsive to first-line treatment may receive chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone—the CHOP regimen).

Patients with SPTCL occasionally present with facial or limb lipoatrophy, possibly resulting from local or distant CD8⁺ T-cell activation. ⁸ Few case reports have reported SPTCL patients with facial lipoatrophy. Autologous adipose tissue grafting is a promising method to treat lipoatrophy caused by autoimmune diseases. It is easy to perform, minimally invasive, effective, and economical. In this study, we present the case of a patient with facial lipoatrophy secondary to SPTCL, who achieved satisfactory aesthetic improvement without adverse effects after three sessions of autologous fat grafting.

Case presentation

A female in her early 50s was admitted to the Department of Plastic and Aesthetic Surgery, Peking Union Medical College Hospital in December 2019 with severe facial lipoatrophy secondary to SPTCL. Eleven years ago, she had experienced facial swelling with subcutaneous erythematous nodules of diameter of 1–2 cm. She had no fever, night sweats, weight loss, lymphadenopathy, or hepatosplenomegaly. Enhanced magnetic resonance imaging and positron emission tomography/computed tomography scans revealed soft tissue changes in the bilateral cheeks and mandibular regions, indicating lymphoma. The patient underwent facial skin biopsy; histopathological examination revealed atypical lymphocyte infiltration with rimming of adipocytes. Immunohistochemistry showed CD2⁺, CD3⁺, CD8⁺, CD4⁺, TIA-1⁺, CD56⁻, CD30⁻, L26⁻, KP-1⁻, and Ki-67(40%–60%). After examining rheumatologic parameters to exclude other rheumatic immune diseases, the diagnosis of SPTCL was established. The patient underwent four sessions of CHOP therapy. The SPTCL was well controlled but left disfigured facial lipoatrophy. Apparent soft tissue volume loss was observed on bilateral cheeks and temporal regions. Since the disease causes lipoatrophy and creates a local inflammatory environment, autologous fat grafting is believed to be the best treatment option. Adipose tissue was harvested from the lower abdomen, subjected to filtration, purification, and grafted in the form of microfat to the bilateral cheeks, nasolabial folds, and temporal regions. The patients underwent three sessions of autologous adipose tissue grafting at an 8-month interval. The grafted fat volume of each side of the face was 40 mL, 30 mL, and 17 mL, respectively. The facial contour remarkably improved after each treatment session, and the patient ultimately reached a satisfying facial aesthetic result (Figure 1). Informed consent was obtained from the patient for agreement for publication of the case.

Figure 1.

Facial images of a patient with facial lipoatrophy before treatment and after each session of autologous fat grafting. In the first session, 40 mL of purified autologous adipose tissue was injected into the cheek and the nasolabial region on each side. In the second session, 30 mL was injected into the midface region on each side. In the third session, 15 mL was injected into the midface region, and 2 mL was injected into the temporal region on each side.

Discussion

This study reported the case of a SPTCL patient with severe facial lipoatrophy who underwent three sessions of autologous fat grafting. Her facial contour showed significant improvement at the long-term follow-up. At present, the treatment of SPTCL focuses on systematic medication, including immunosuppressive therapy as the first-line treatment and chemotherapy as the second-line treatment. Although some previous reports mention lipoatrophy, very few studies have described the treatment and prognosis of facial lipoatrophy.

Lipoatrophy of subcutaneous tissue is not uncommon in patients with SPTCL. This may result from the activation of CD8⁺ T cells that infiltrate into the subcutaneous fat tissue and destroy the adipocytes. Extensive lipoatrophy involving affected or unaffected sites is a common clinical feature found in 29% of all SPTCL cases ⁸ ; however, it is unclear what percentage of patients experience facial lipoatrophy. In fact, facial lipoatrophy is associated with several autoimmune diseases including human immunodeficiency virus (HIV) infection, lupus erythematosus, and localized scleroderma. With progressive loss of subcutaneous fat tissue, the soft tissue becomes thinner, and the bone structure becomes more visible. Cheeks, temporal regions, periorbital area, and perioral area can be affected, ⁹ giving an aging, frustrated, and unpleasant look. Patients with facial lipoatrophy could have psychosocial dysfunction and low quality of life as dramatic changes in appearance severely impair self-esteem. Even if the primary disease is well controlled, the change in appearance is a consistent exposed reminder of their different appearance. Thus, adequate attention needs to be given to disfiguring secondary facial lipoatrophy.

Attempts have been made to correct facial lipoatrophy secondary to systematic diseases. Poly-L-lactic acid (PLLA), a biocompatible and biodegradable polymer, was approved by the US FDA in the name of Sculptra to treat HIV-associated lipoatrophy in 2004. Other dermal fillers, including hyaluronic acid, ¹⁰ calcium hydroxyapatite, ¹¹ and polymethylmethacrylate, ¹² have also been applied for volumetric correction of facial lipoatrophy and have yielded good aesthetic results. In addition to the above synthetic dermal fillers, autologous fat grafting is regarded as a promising tool to treat secondary facial lipoatrophy. It may offer similar or superior efficiency than dermal fillers. Dollfus et al. used autologous fat grafting for the treatment of six HIV-infected adolescents and reported good cosmetic results. ¹³ The authors believe that the dermal fillers were unsuitable for various reasons including the need of repeated injections, uncertainty of long-term side effects, and high cost. From the current viewpoint, dermal fillers are considered safe and effective; however, they need to be routinely injected to maintain good effect. The multiple injections and the consequent financial burden are major concerns. In comparison, autologous fat grafting is certainly more economically advantageous for patients. A study compared the cost of PLLA injection and autologous fat transfer and claimed that the average session cost of autologous fat transfer is approximately one-third of that of PLLA injection. ¹⁴ Though facial autologous adipose tissue grafting could lead to minor to severe adverse effects, the overall complication rates were low, as reported in the literature. ¹⁵

When it comes to autologous fat grafting, there is a long-time controversy on the fate of the grafted adipocytes after nonvascularized fat grafting. It is traditionally believed that the grafted adipocytes partially survive and stay alive for a long time. The grafted adipocytes can be integrated into the surrounding host fat tissue. The volume effect depends on the graft volume and the survival rate. However, in recent years, in vitro studies revealed that most of the grafted adipocytes soon died after transplantation and only a small number of adipocytes in the surrounding area with good nutrition and oxygen supply from plasma diffusion survived. The resident and grafted stem cells were activated to start regeneration to replace the necrotic tissue.^16,17 The debate between the traditional “cell survival theory” and the new “host replacement theory” may continue; however, autologous fat grafting is proven to be a promising treatment modality for facial lipoatrophy.

In recent years, there have been attempts to improve the retention rates of grafted fat. The practice of adding stem cells or platelet-rich plasma has been proven to facilitate adipose tissue survival.^18,19 High level of evidence is expected to prove the effectiveness of autologous adipose tissue with supplements.

Conclusion

To the best of our knowledge, this is the first report on successful multiple sessions of autologous adipose tissue grafting in a patient with facial lipoatrophy secondary to SPTCL. Awareness of this rare condition and appropriate treatment strategies are essential for reducing appearance-related anxiety in affected patients while improving their psychological health and quality of life.