Lupus Profundus

Overview

"Lupus panniculitis is a rare variant of lupus erythematosus. It may occur as a separate disease or coexist with systemic or discoid lupus erythematosus. It is characterized by persistent, tender and hard nodules localised on the face, arms, shoulders, breast and buttocks. Healing of lesions is associated with scarring, lipoatrophy and rarely ulceration. Treatment of lupus panniculitis depends on disease advancement or concomitance of additional lupus erythematosus symptoms." [1] 

"LEP occurs in 1% to 3% of patients with cutaneous lupus erythematosus, mostly affecting females of childbearing age, with a female to male ratio of 4.5:1. (3) LEP presents as persistent, often painful subcutaneous nodules, ranging from 1 to 5 cm in diameter, mainly involving the proximal extremities (lateral aspects of the arms and shoulders), thighs, buttocks, trunk, face, and scalp. Overlying skin may appear erythematous. Lesions may ulcerate, healing with atrophy, skin depression, dimpling, and scarring. LEP may present as the sole manifestation of the disease or associated with discoid and/or systemic lupus. Although spontaneous resolution may occur, LEP may follow a chronic course of remission and exacerbation persisting for months to years" [2]

Only 10% have SLE.

Lupus profundus (also known as lupus panniculitis) is an uncommon form of CCLE.

Coexistent DLE occurs in at least one-third of patients with lupus profundus; SLE is present in approximately 10 percent of patients [3]

Clinical manifestations

– Lupus profundus presents as indurated plaques or nodules with or without overlying cutaneous changes [57]. The plaques or nodules may appear on the scalp, face, upper arms, chest (particularly breasts), lower back, flank, upper thighs, or buttocks and are often tender or painful. Infrequently, patients develop ulceration or calcifications at sites of involvement. Upon resolution, lupus profundus may leave depressed areas of lipoatrophy (picture 11).

Lesions involving the breast may be initially concerning for breast malignancy, including an atypical appearance with calcification on mammography; this presentation has been referred to as "lupus mastitis" [3]

●Histopathology – Histopathologic examination reveals perivascular infiltrates of mononuclear cells plus panniculitis, manifested as hyaline fat necrosis with mononuclear cell infiltration and lymphocytic vasculitis (picture 12A-B). The presence of immune deposits in the dermal-epidermal junction on direct immunofluorescence offers support for the diagnosis [59]. (See 'Diagnosis' below.)

●Differential diagnosis – Nodules of lupus profundus on the breast may raise concern for a breast malignancy. The possibility of subcutaneous panniculitis-like T cell lymphoma, which often manifests with subcutaneous nodules or plaques on the trunk or extremities, also should be considered [60]. A biopsy will distinguish lupus profundus from these entities. (See 'Diagnosis' below.)

Treatment

Antimarials

Glucocorticoids

Immunosuppressives

In recurrence or more aggressive lupus panniculitis used drugs include:

• methotrexate
• cyclophosphamide
• cyclosporine A 
• Thalidomide may be used as alternative treatment in patients who do not respond to other forms of therapy. However, its application necessitates that the patient be strictly monitored with respect to possible neuropathies, which occur in 70% of cases and are considered to be irreversible
• Cases of lupus panniculitis improvement after disulone therapy were documented 
• dapsone
• belimumab