Lupus Erythematosus Profundus

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LE profundus is a variant of LE described by Kaposi (Kaposi 1883) in 1883 and in more detail by Irgang in 1940 (Irgang 1940). LE profundus is a panniculitis, also termed "lupus panniculitis". Clinically, LE profundus has a predilection for the face, neck, proximal extremities, trunk, and lower back (Tuffanelli 1985).

Histologically, the epidermis and dermis may be involved, with vacuolar degeneration and dermal lymphocytic infiltration (Fig. 21.3). However, the key feature is the prominent lobular panniculitis. There is a diffuse lymphocytic infiltrate with nuclear dust (Sanchez et al. 1981), and fat necrosis may develop later on in hyalinization of adipose lobules. Areas with fat necrosis have plasma cells, histiocytes, and neutrophils. Eosinophils may be present in approximately 25% of cases (Peters et al. 1991). Vascular involvement includes arteries and veins with endothelial cell hypertrophy, edema, and, in some cases, thrombosis or calcification. Perivascular fibrosis (onion-skin configuration) can be observed in the surroundings of vessels.

Lupus Onion Skin
Fig. 21.3A, B. Lupus erythematosus profundus. Patchy lymphocytic infiltrates in the dermis and subcutaneous tissue

Another characteristic feature is the presence of reactive lymphoid follicles. Lymphoid follicles are rare in panniculitis but may be seen in rare cases of erythema nodosum, erythema induratum, and morphea (Harris et al. 1979). These entities are also part of the differential diagnosis of LE profundus. However, erythema induratum is usually on the calves of women, a location that is not common in LE profundus. In morphea, there are thickened collagen bundles, which are not present in LE profundus. Erythema nodosum usually does not display the histologic changes in the epidermis that may be seen in LE profundus. Another important differential diagnosis is subcutaneous panniculitis-like T-cell lymphoma, which is a cytotoxic T-cell malignancy. The neoplastic cells infiltrate the subcutaneous tissue and rim individual fat cells. Angioinvasion and necrosis may be prominent. T-cell receptor gene rearrangement studies are useful in diagnosis, as benign panniculitis does not show a monoclonal population (Sander et al. 2001).

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