Epidemiology of the Cutaneous Manifestations of Lupus Erythematosus

The cutaneous manifestations of LE have been classified by Gilliam and Sontheimer (Gilliam and Sontheimer 1981) into chronic cutaneous LE (CCLE), subacute cutaneous LE (SCLE), and acute cutaneous LE (ACLE). Any of these forms can appear in patients with LE confined to the skin or in patients with SLE. Overall, cutaneous manifestations appear in 72%-85% of patients with SLE (Dubois and Tuffanelli 1964). They can occur at any stage of the disease, but they are the first manifestations of SLE in 23%-28% of patients (Table 3.7).

CCLE includes classic localized discoid LE (DLE), generalized DLE, hypertrophic/ verrucous DLE, lupus panniculitis (lupus profundus), and chilblain LE. DLE is characterized by inflammatory plaques with scaling, follicular, plugging, atrophic scarring, central hypopigmentation, and peripheral hyperpigmentation. Approximately 5% of patients with isolated localized DLE subsequently develop SLE. It has been postulated that SLE is seven-fold more common than DLE. Chronic discoid lesions may be the initial manifestation of SLE in approximately 10% of patients, and they can occur during the course of the disease in 15%-30% of patients. It has been suggested that patients with SLE and discoid lesions may have a more benign clinical course, with less severe renal disease, than unselected patients with SLE (Callen 1985). In another study that included 136 patients with DLE (Le Bozec et al. 1994), the authors described the development of SLE in 11 of them. Interestingly, most of these patients developed SLE within 5 years of the onset of the cutaneous lesions, and 4 of the 11 had a poor prognosis, with renal or neurologic involvement.

SCLE occurs in 10%-15% of patients with SLE and includes the papulosquamo-sus (psoriasiform) and annular-polycyclic variants. These lesions are usually widespread, symmetrical, and nonscarring. They occur in a characteristic, photoexposed distribution, with an LE-specific histopathology. A few patients may have a combination of psoriasiform and annular lesions, although most predominantly have either one or the other subtype (Sontheimer 1989). Approximately, 50% of patients with isolated SCLE subsequently develop SLE. Patients in this subset of LE frequently have anti-Ro antibodies (Sontheimer et al. 1982) and are HLA-B8-DR3/2 positive (Johansson-Stephansson et al. 1989). SCLE may be associated with other systemic autoimmune diseases, and photoactive medications may induce lesions of SCLE.

ACLE appears in 30%-60% of patients with SLE. It includes localized (malar) erythema, widespread (face, scalp, neck, upper chest, shoulders, extensor arms, and back of hands) erythema, and bullous (toxic epidermal necrolysis-like) LE. Additionally, nonspecific but disease-related cutaneous manifestations can appear in patients with LE, including photosensitivity, alopecia, urticaria, livedo reticularis, dermal vasculi-tis, and Raynaud's phenomenon (Yell et al. 1996).

The prevalence of photosensitivity ranges from 28% to 71%. It is one of the major diagnostic criteria for SLE. Photosensitivity precedes the clinical onset of internal manifestations of SLE in about one third of patients. Some patients may not notice erythema after prolonged UV exposure, but hours or days later they may note increased arthralgia, malaise, or fever.

Table 3.7. Prevalence of cutaneous manifestations in systemic lupus erythematosus in several series

Cutaneous

Dubois and

Estes and

Lee et al.

Grigor et al.

Weinstein

Worral et al.

Pistiner

Yell et al.

Manifestation

Tuffanelli 1964

Christian 1972

1977

1978

et al. 1987b

1990

et al. 1991

1996

(«=520)

(«=150)

(«=110)

(«=375)

(«=84)

(«=100)

(«=464)

(«=73)

All types of skin

lesions (%)

72

81

NA

NA

82

90

55

100

Raynaud's

phenomenon (%)

18.4

21

46

32

NA

NA

25

60

Photo

sensitivity (%)

32.7

NA

50

28

NA

48

37

63

Mucous

membrane (%)

9.1

7

NA

34

34.5

36

19

55

Malar rash (%)

20.9

NA

NA

NA

21

NA

34

45

Urticaria (%)

6.9

13

5

NA

NA

NA

4

44

Alopecia (%)

21.3

37

38

64

NA

NA

NA

40

Chronic discoid

lesions (%)

28.6

14

28

22

11

NA

23

25

Butterfly blush (%)

36.7

39

36

68

NA

NA

NA

14

Vasculitis (%)

NA

21

NA

70

18

NA

NA

11

Bullae (%)

0.4

2

NA

NA

NA

NA

NA

8

Psoriasiform

rash (%)

NA

2

NA

NA

NA

NA

NA

NA, not applicable.

Hair loss is a common and characteristic finding in patients with SLE. The variability in recording alopecia resulted in its exclusion from the ACR criteria. It may be scarring, if preceded by DLE, or nonscarring.

Urticaria, angioedema, and Raynaud's phenomenon are common cutaneous vascular reaction patterns. Some patients with SLE described lesions suggestive of urticarial vasculitis, with prevalences ranging from 7% (Provost et al. 1980) to 22% (O'Loughlin et al. 1978). Dermal vasculitis has been reported in 18% to 70% of patients with SLE. Livedo reticularis maybe associated with the antiphospholipd syndrome and has been reported as an initial manifestation of SLE in many patients (Weinstein et al. 1987a). Other skin disorders have been occasionally reported in patients with SLE, such as sclerodactyly, erythromelalgia, erythema multiforme, chronic ulcers, splinter hemorrhages, rheumatoid nodules, and acanthosis nigricans.

Mucous membrane involvement was thought to be relatively uncommon in SLE. Dubois and Tuffanelli (Dubois and Tuffanelli 1964) found only a 9% incidence. However, on careful inspection, more than 50% of patients with SLE may have mucosal lesions, mainly oral ulcers (Jonsson et al. 1984).

How To Deal With Rosacea and Eczema

How To Deal With Rosacea and Eczema

Rosacea and Eczema are two skin conditions that are fairly commonly found throughout the world. Each of them is characterized by different features, and can be both discomfiting as well as result in undesirable appearance features. In a nutshell, theyre problems that many would want to deal with.

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