Clinical Appearance Classification

Proven Lupus Treatment By Dr Gary Levin

Proven Lupus Treatment System by Dr. Gary Levin

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Several distinctly different clinical entities can be allocated to the group of CCLE (Table 2). They may be found as one and only manifestation or associated with other forms of CCLE, localized or disseminated, both with and without systemic manifestations and varying relation to SLE. The most common

Table 2. Specific LE Skin Symptoms

Acute cutaneous lupus erythematosus (ACLE) Localized


Subacute cutaneous lupus erythematosus (SCLE) Annular

Papulosquamous Chronic cutaneous lupus erythematosus (CCLE) Discoid (DLE)

Localized (above neck) Generalized (above and below neck) follicular Hypertrophic DLE Mucosal LE Lupus panniculitis

Lupus profundus (Panniculitis and DLE) Lupus tumidus Chilblain lupus Papulous mucinosis form is chronic discoid lupus erythematosus (CDLE or DLE). It is character zed by persistent, sharply demarcated, elevated erythematous plaques with adherent scales which may rarely ulcerate (Fig. 1A). Early stages are characterized by erythema and hyperpigmentation. The characteristic painful sensation upon touching is caused by follicular plugging resulting in the so-called carpet-tack sign. Apart from that, atrophy and scarring can be found in the center of untreated lesions and may result in considerable disfiguration particularly when present in the face (Fig. 1B). A characteristic pitted, acneiform scarring is also a common residual feature of the perioral area including the lips.

DLE lesions predominantly occur in the light-exposed areas of skin like face, ears, neck and arms, but may be found in sun-protected areas as well as inguinal folds, palmo-plantar locations and the scalp. At the latter location, DLE may even be the only cutaneous manifestation in 10% of cases and thus presents a classical differential diagnosis of scarring alopecia. Altogether involvement of the scalp can be found in about 60% of DLE patients (Sontheimer and Provost 1996) (Fig. 1C). With a distribution above the neck, the so-called localized form of DLE can be separated from a generalized DLE if it is present above and below the neck. Small, follicularly orientated erythematous papules of less than 1 cm in diameter present as follicular DLE at the elbows, but may occur at any other part of the body as well. Precipitation of DLE lesions by physical trauma (Kobner phenomenon) has already been mentioned above and may explain occurance at unusual locations.

If DLE lesions are distinctly proliferative, they are referred to as hypertrophic or verrucous DLE. They have to be separated from spinous cell carcinoma, keratoacanthoma and lichen planus by histology and immunohistochemistry (Perniciaro et al. 1995; Uy et al. 1999).

Lupus Profundus
Fig. 1. A. Clinical presentation of chronic discoid lupus erythematosus. Typical sharply demarcated elevated erythematous plaque. B. Chronic atrophic lesions of the face lead to disfiguration. C. Involvement of the scalp leads to cicatricial alopecia

Another rare manifestation of CCLE is lupus profundus or lupus panniculitis, also referred to as Kaposi-Irgang disease (Caproni et al. 1995; Watanabe and Tsuchida 1996; Kundig et al. 1997; Uy et al. 1999; Martens et al. 1999). It can be found in about 2% of SLE patients, but presents more commonly without further or only mild signs of systemic manifestation in about 50% of patients. Severe nephritis is an uncommon event. In contrast to DLE, mainly women are affected by painful, firm subcutaneous nodules of red-bluish colour and up to 1-3 cm diameter. Their main locations are the upper arms, buttocks and thighs, but chest, head and neck can be affected as well (Fig. 2A).

Lupus Profundus

Fig. 2. A. Rare manifestations of chronic cutaneous lupus erythematosus. Lupus profundus or lupus panniculitis lead to saucer-like depressions resembling lipatrophy. B. Chilblain or perniotic lupus is characterized by bluish-red patches and plaques located at the acra, such as the ears

Fig. 2. A. Rare manifestations of chronic cutaneous lupus erythematosus. Lupus profundus or lupus panniculitis lead to saucer-like depressions resembling lipatrophy. B. Chilblain or perniotic lupus is characterized by bluish-red patches and plaques located at the acra, such as the ears

The inflammatory process in the deeper dermis and subcutaneous tissue results in saucer-like depressions sometimes resembling lipathrophy. When DLE lesions are found on the overlying skin as is the case in 70% of patients, it is referred to as lupus profundus.

Chilblain or perniotic lupus is characterized by bluish-red patches and plaques at acral locations like nose, ears, fingers, toes, knees and elbows (Su et al. 1994; Fisher and Everett 1996). These are painful upon pressure, especially when located at heels and knuckels where fissuring is quite common

Pictures Ulcers From Lupus
Fig. 3. Ulcers of the buccal mucosa in chronic cutaneous lupus erythematosus

(Fig. 2B). It mainly occurs in cold climates and is possibly caused by a Köbner phenomenon. During further disease process, typical DLE lesions may appear and patches may ulcerate. As in lupus profundus, mild systemic symptoms like arthralgia can be found to fulfil three to four ACR criteria for diagnosis of SLE in up to 20% of patients. Due to its rare occurence, no definite association to SLE can be made. The term lupus pernio is often used synonymously for this entity, should however be restricted to cutaneous sarcoidosis as an important differential diagnosis.

Another rare manifestation of CCLE, lupus tumidus, is characterized by excessive dermal mucin deposition resulting in urticarial plaques (Ruiz and Sanchez 1999; Dekle et al. 1999; Kühn et al. 2003). Compared to other CCLE types, it is characterized by distinct photosensitivity which explains its main occurence at sun-exposed areas and induction of lesions upon photoprovocation. With a male preponderance its peak incidence is around the 30-40th year of age. Differential diagnosis to lymphocytic infiltration and polymorphous light eruption can be very difficult both clinically and histologically (Sontheimer and Provost 1996).

Mucous membrane involvement (mucosal DLE) can be found among CCLE patients in up to 25% (Burge et al. 1989; Botella et al. 1999). It does not necessarily reflect systemic manifestation, specific or high antibody titers or high disease activity. It is, however, included in the list of 11 diagnostic criteria for SLE defined by the ACR. Oral, mainly buccal manifestations are most common (Fig. 3), but nasal, conjunctival and anogenital mucous membranes may be affected as well. They present as painful erythematous patches which may ulcerate and cause atrophy in the long run. Sometimes mucosal DLE resembles lichen planus with honeycomb appearance. Squamous cell carcinoma as a long-term complication should be suspected and excluded in any case of asymmetrical induration of either mucosal or cutaneous lesions. Affliction of the lips (vermillion border, diffuse cheilitis especially of lower lip) can cause considerable discomfort and disfiguration. Mucosal DLE of the nose may result in nasal septum perforation especially in association with SLE. Similarly, ocular affections which are mainly located at the palpebral conjunctiva and lower eyelids can cause permanent loss of eye lashes, ectropion and stromal keratitis (Uy et al. 1999).

Annular erythema has in the past been regarded as a rare manifestion of CCLE. It will not be dealt with in this chapter since it is generally associated with SCLE and Sjogren's syndrome (Watanabe et al. 1997).

Papular mucinosis may occur as early or only manifestation of CCLE and shows similarities to lupus tumidus in respect to dermal mucin deposition (Kanda et al. 1997; Williams and Ramos-Caro 1999, Sonntag et al. 2003, Kuhn et al. 2003). In contrast to lupus tumidus, more papular than plaque appearance and lack of inflammatory erythema can be found. Asymptomatic isolated or multiple lesions are located at the trunk, upper limbs and the face with less apparent photosensitivity than in lupus tumidus. Diagnosis may be only made upon immunohistochemical detection of linear or granular depositions of IgG, IgM and complement C3 at the dermo-epidermal junction whereas other histological traits of LE are absent. Accordingly, differential diagnosis to other cutaneous mucinoses can be difficult.

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