Update on Management of Panniculitis Associated with Connective Tissue Disorders

Introduction

The panniculitis can occur with other underlying diseases. The connective tissue panniculitis is lupus erythematosus panniculitis (LEP) and lupus profundus. The management of panniculitis causes death, deep scars, cosmetic disfigurement, and psychiatric sequelae. Topical therapies may not penetrate enough to be effective. Panniculitis refers to the inflammation of subcutaneous tissue. It can be seen in many diseases, including trauma, neoplasm, vascular and enzymatic insufficiency. It is characterized by deep and painful nodules. The clinical finding helps correctly diagnose connective tissue panniculitis. Panniculitis has different cell changes. The treatment of panniculitis

Precision and prompt identification are crucial as the focus of treatment should target the initial inflammatory stage, given that subsequent atrophy is irreversible and challenging to address. Since the inflammation is situated in the subcutaneous adipose layer, topical treatments might not sufficiently penetrate for efficacy, necessitating the use of systemic agents.

What Is Panniculitis?

Kaposi first described lupus erythematosus panniculitis in 1883. Panniculitis is defined as the involvement of the subcutaneous fat in lupus erythematosus. If there are changes in discoid lupus, for example, erythema, scaling, and follicular plugging, then it is called lupus profundus. It is subcutaneous tissue inflammation and can be seen in many disease processes, such as trauma, infection, neoplasm, vascular and enzymatic insufficiency, and connective tissue diseases. Lupus erythematosus panniculitis and lupus profundus mainly affect young women in their 30s and early 40s.

What Are the Clinical Features Of Panniculitis?

There are many similarities in the clinical features and histological appearances of the connective tissue panniculitis. Clinical findings help to diagnose connective tissue panniculitis correctly. Histological features are also required as the patients suffer from underlying diseases. Some of the clinical features are erythema, scaling, follicular plugging, and changes in discoid lupus. There is a presence of tenderness at deep subcutaneous nodules or plaque that may sometimes crop with overlying hyperpigmentation. The nodules are mainly present at the proximal extremities: the upper arms, shoulders, buttocks, trunk, breast, face, and scalp. Sometimes, the legs are also involved, but it is unusual. In children, there is facial involvement. In the case of untreated lesions of panniculitis, it may be ulcerated.

What Is the Diagnosis of Panniculitis?

The histological features and clinical features of panniculitis have been organized into proposed criteria by Peters and Su. It can be diagnosed by major criteria, such as the presence of hyaline fat necrosis, lymphocytic aggregates and lymphoid follicle formation, and calcification.

What Are the Management of Panniculitis Associated with Connective Tissue Disorders?

Simplified Information on Treatment for Lupus Panniculitis (LEP):

1. Challenges in Studying LEP:

• Low prevalence makes treatment strategies hard to study.

• The disease has relapsing and remitting patterns.

• The lack of appropriate outcome measures complicates research.

2. Antimalarials as First-Line Therapy:

• Hydroxychloroquine is a key player.

• Typically given at <6.5 mg/kg/day.

• Slow-acting, may take up to 3 months for results.

3. Mechanism of Antimalarials:

Effects include antigen presentation, cytokine inhibition, photoprotection, and more.

4. Alternatives if Antimalarials Are not Effective:

• Chloroquine and hydroxychloroquine can be used.

• Quinacrine (compounded) can be added for better results.

5. Topical Steroids and Other Options:

• Reports of success with Clobetasol proprionate 0.05 % ointment.

• Oral steroids used cautiously due to potential long-term side effects.

6. UV Protection as a Therapeutic Approach:

• Even if unclear, sun protection is recommended for all forms of CLE.

• Lesion distribution argues against UV triggers.

7. Immunomodulators as Alternatives:

• Thalidomide and Dapsone are used if antimalarials fail.

• Thalidomide is limited by side effects; it requires careful monitoring.

• Dapsone is successful at 25 to 75 mg daily.

8. Systemic Immunomodulators for Severe Cases:

• Azathioprine, Methotrexate, Cyclophosphamide, and Cyclosporin A reported effectiveness.

• Used in cases with underlying systemic lupus erythematosus.

9. Other Treatment Options:

• IVIG and photopheresis were reported in limited cases.

• Rituximab was used successfully in one patient.

10. Managing Calcification: Diltiazem, a calcium-channel blocker, proposed for calcified variants of panniculitis.

What Are the Update On the Management of Panniculitis Associated with Connective Tissue Disorders?

1. Unraveling the Complex Tapestry: Connective tissue disorders pose unique challenges, with manifestations extending beyond the joints and muscles to involve the skin's intricate layers. Recent research shows light on other updates on management other than medicine.

2. Precision Medicine at the Forefront: One of the striking advancements in the management of panniculitis associated with connective tissue disorders is the increasing role of precision medicine. The treatments are based on individual patient profiles, genetic markers, and specific disease manifestations allowing for a more targeted and effective therapeutic approach. This shift from a one-size-fits-all model to personalized medicine marks a significant stride forward.

3. Emerging Therapies and Treatment Modalities: From immunomodulatory agents to targeted biologics, the arsenal of available treatments is expanding. The use of these therapies in the management opens new ways for improved patient outcomes.

4. Diagnostic Advancements: Accurate and timely diagnosis is pivotal in guiding effective management strategies. Recent diagnostic advancements, including advanced imaging techniques and molecular biomarkers, enhance our ability to differentiate between various subtypes of panniculitis and tailor interventions accordingly. This diagnostic precision facilitates early intervention and targeted therapy.

5. Holistic Patient-Centric Care: The patient-centric approach to management is focused on the management of panniculitis. Addressing the psychosocial aspects of living with panniculitis and connective tissue disorders is integral to comprehensive care. Support networks, patient education, and shared decision-making contribute to a more empowered patient experience.

Conclusion

In summary, the comprehensive update on the management of panniculitis linked to connective tissue disorders underscores a paradigm shift towards precision medicine and patient-centric care. Through a synthesis of emerging therapies, diagnostic tools, and a deeper understanding of disease mechanisms, this review has highlighted the strides made in enhancing treatment efficacy and patient outcomes. Through all studies, stand at the intersection of medical innovation and compassionate healthcare, the evolving landscape offers optimism for those grappling with these intricate conditions, paving the way for a more nuanced and effective approach to managing panniculitis in the context of connective tissue disorders.