LEP, historically referred to as "Kaposi-Irgang disease" or also known as "lupus pan-niculitis", is a rare variant of CCLE in which pathologic changes occur primarily in the lower dermis and subcutaneous tissue. In 1883, subcutaneous nodules associated with LE were first described by Kaposi (Kaposi 1883), but the term "lupus profundus" was coined by Irgang (Irgang 1940) in 1940. Subsequently, different authors reported new cases and contributed to define the clinical and histopathologic characteristics of this disease (Arnold 1956, Sanchez et al. 1981, Tuffanelli 1971, Winkelmann 1970). Middle-aged women are predominantly affected; however, in a recent study it has been shown that LEP in Asian patients is more frequent in a younger age group compared with the Caucasian population (Ng et al. 2002). The course of this subtype of CCLE is usually chronic and characterized by periods of remission and exacerbation. The major morbidity is usually disfigurement and disability related to pain, and the lesions finally resolve, leaving deep atrophic scars (Kuhn et al. 2000c, Mascaro et al. 1997). LEP can be found in approximately 2%-10% of patients with SLE and other autoimmune diseases; however, patients with LEP present more commonly without further or only mild signs of systemic manifestations. In some extensive cases, association of LEP with serious systemic disease or a lethal outcome has been reported, although there is a relatively low incidence of renal involvement (Grossberg et al. 2001). Serologically, ANAs may be found to be low in titer or nonexistent, and hyper-gammaglobulinemia has been reported in some cases of LEP along with low total complement and C4 levels (Martens et al. 1999). Approximately 70% of patients with LEP also have other lesions of CLE, particularly discoid or hypertrophic lesions, often overlying the panniculitis lesions (Balabanova et al. 1992, Suss et al. 1994). When LEP exists in the absence of other LE-specific lesions, the diagnosis has been questioned. Therefore, it is important to obtain biopsy specimens to confirm the diagnosis because a number of cases of subcutaneous lymphoma have given a clinical appearance of lupus profundus (Magro et al. 1997, Ng et al. 2002). Physical factors, such as trauma, may often be directly related to the lesions of LEP (Tuffanelli 1971); however, the relevance of photosensitivity in this rare subtype of CCLE is unknown (Fabbri et al. 2003).
Single or multiple sharply defined, persistent, asymptomatic or sometimes painful subcutaneous plaques or nodules of varying sizes are the typical lesions of LEP (Costner et al. 2003, Peters and Su 1989). The overlying skin ultimately becomes attached to the firm lesions, producing a deep depression into the subcutis with a normal or erythematous, inflammatory surface (Fig. 6.16). Dystrophic calcifications or ulcerations within older lesions of LEP, leaving atrophic scars or sometimes resembling lipatrophy, may occur and at times can be a prominent clinical feature of the disease requiring surgical excision. In addition, LEP may produce breast nodules that can mimic carcinoma, clinically and radiologically (Holland et al. 1995, Peters 2000), and linear involvement of the extremities or the scalp has also been observed (Nagai et al. 2003, Tada et al. 1991). Most lesions of LEP are usually found in areas of increased fat deposition, such as the trunk, buttocks, and proximal upper and lower extremities, but the shoulders and thighs are further sites of predominant involvement (Martens et al. 1999). LEP may also develop on the scalp clinically simulating alopecia areata (Kossard 2002) and in unusual zones on the face, such as the parotid
Fig. 6.16. Lupus erythematosus profundus (LEP). Subcutaneous nodules leaving extensive depressed, atrophic areas on the upper arm with hyperpigmented borders
region. Interestingly, periorbital edema as an initial symptom of LEP has been described in several patients as the only clinical manifestation of the disease (Franke et al. 1999, Lodi et al. 1993, Magee et al. 1991).
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