Association of Cutaneous Lupus Erythematosus with Other

CHAPTER 10 Association of Cutaneous Lupus Erythematosus 10 with Other Dermatological Diseases Kyrill Pramatarov,Nikolai Tsankov Lupus erythematosus (LE) may be associated with various systemic and dermato- logic diseases. In some cases, this association may be explained by autoimmune mechanisms or by the similarity of the clinical features; in other cases, the possible coexistence of diseases is coincidental. Psoriasis and Lupus Erythematosus The coincidence of LE and psoriasis seems to be rare. Based on their prevalence in the population, the coexistence of psoriasis with all forms of lupus seems to be less than expected. Dubois (Dubois 1974) reported that 0.6% of 520 patients with systemic LE (SLE) had concurrent psoriasis. Tumarkin et al. (Tumarkin et al. 1971) described 637 patients with discoid LE (DLE), and only 1 had coexistent psoriasis. In 1927, O’Leary (O’Leary 1927) described one of the first cases of coexistent psoriasis and LE. Throughout the years, several explanations concerning this coexistence have been developed. Schaumann (Schaumann 1928) postulated that the combination of LE and psoriasis – disorders with different “affinity” to the ground on which they appear – must be sought in the etiologic factors determining their pathogenesis. Louste et al. (Louste et al. 1939) focused on the “endocrine deficiency.” Charpy et al. (Charpy et al. 1952) proposed that both disorders (in combination with arteriitis and hypertonia) be considered “disorders of adaptation.” Kocsard (Kocsard 1974) considered the association of cutaneous LE and psoriasis vulgaris for a best explanation of the frequency of pseudopelade in patients with psoriasis. Millns and Muller (Millns and Muller 1980) think that LE and psoriasis could appear independently in the same patient without there being a causal relationship between the two disorders. Kulick et al. (Kulick et al. 1983) found that the frequency of antibodies to Ro/SSA is increased in patients with psoriasis and LE. They suggested that this might be a spe- cific serologic marker for the LE-psoriasis overlap. It is well-known that the same antibodies occur in the antinuclear antibody (ANA)-negative, highly photosensitive group of patients with SLE. Accordingly, patients with psoriasis and LE may in fact have an increased risk for photosensitivity. Forty-three percent of all patients with SLE are photosensitive. On the contrary, the subset of “photosensitive psoriasis” is very small and comprises approximately 5.5% of all cases. These patients have a sig- nificantly higher prevalence of skin type I, and 50% have preceding polymorphous light eruption rising into psoriasis. Psoriasiform or annular lesions are also typical for patients with subacute cutaneous LE (SCLE). In such cases, histologic examina- 134 Kyrill Pramatarov, Nikolai Tsankov tion, immunofluorescence, and serology tests are necessary to distinguish SCLE from psoriasis. Some of these patients, especially those with circulating anti-Ro/SSA antibodies, may be exclusively photosensitive. The action spectrum for LE is generally considered to be ultraviolet (UV)B, but some patients may flare after UVA exposures received in tanning salons or from sunlight filtered through window glass. The control of SLE often requires systemic administration of steroids, especially for renal and central nervous system involvement. A rebound flare of psoriasis is always possible on withdrawal of steroid therapy. Administration of antimetabolites used as steroid-sparing agents may prevent this rebound flare and improve psoriasis. Phototherapy is contraindicated in patients with cutaneous LE. On the contrary, psoralen-UVA exposure is indicated in psoriatic individuals and in those with severe “photosensitive psoriasis.” Screening for ANAs, including anti-Ro/SSA and anti- La/SSB antibodies, is necessary before treating any photosensitive patient with UV light. Psoriasis could coexist with other photosensitive disorders, such as vitiligo, por- phyria, drug-induced photodermatitis, polymorphous light eruption, chronic actinic dermatitis, solar urticaria, actinic prurigo, and the so-called “fair skin type”. Some psoriasis patients develop LE (subacute cutaneous, chronic cutaneous, or systemic) after psoralen-UVA therapy (Dowdy et al. 1989). Zalla and Muller (Zalla and Muller 1996) studied 9420 patients with psoriasis, and 65 (0.69%) had concomitant photosensitive disorders. Of these, 23 (35%) had psoriasis and nonlupus-related photosensitivity and 42 (65%) had psoriasis and LE with or without photosensitivity. The conclusion is that the coexistence of psoriasis with LE or other photosensitive disorders is rare. These studies may explain the coexistence of LE in patients with psoriasis after UV therapy. However, this possibility does not help explain the coexistence of LE in patients with psoriasis who have not had significant exposure to UV light or in those in whom LE develops before the onset of psoriasis. The explanation probably resides in the multifactorial etiology of both diseases. The hypothesis that patients with LE and psoriasis have common serologic markers (anti-Ro/SSA antibodies) is not convincing (Baselga et al. 1994, Hays et al. 1984, Kobayashi et al. 1995, Kulick et al. 1983). Both disorders may appear independently in the same patient with no causal relationship between them (Millns and Muller 1980, Wlashev et al. 1986). Antimalarials are now the drugs of choice for treatment of the cutaneous and joint manifestations of LE. It is well known that the use of chloroquine and hydroxychloro- quine may aggravate or precipitate psoriasis (Nicolas et al. 1988). We suggest that in patients treated with antimalarials in which LE precedes the development of psoriasis, drug-induced psoriasis could be possible (Tsankov et al. 1990). Large-scale prospective studies of the general population for the coexistence of diseases such as LE and psoriasis aim at the same scientific goal: elucidation of the etiology and pre- scription of the the most appropriate therapy (Rongioletti et al. 1990a). Erythema Multiforme and Lupus Erythematosus: Rowell’s Syndrome In 1963, Rowell et al. (Rowell et al. 1963) described four patients with the clinical picture of chronic DLE associated with erythema multiforme (EM). Besides the skin Association of Cutaneous Lupus Erythematosus with Other Dermatological Diseases 135 changes compatible with both diseases, the patients had characteristic immunologic findings consisting of the speckled type of ANAs, the anti-SjT type of precipitating antibody to saline extract of human tissues and rheumatoid factor. In some patients, perniotic-like lesions were also observed. Later it turned out that SjT is identical to anti-La/SSB or anti-Ro/SSA antibodies. Several cases with this unusual picture have been reported subsequently, and this distinctive subset of LE is called “Rowell’s syndrome.” Twenty-seven cases of the syndrome had been reported by 1989. Our group observed a 30-year-old man who had DLE, EM-like lesions, and ANAs in a low titer (Pramatarov et al. 1983). SjT antibodies were not studied. Four additional cases were reported later. Parodi et al. (Parodi et al. 1989), in 1989, described a 62-year-old man with DLE and EM.They reviewed the previous reported cases and suggested that most are cases of coincidental association of LE and EM. Their review revealed that none of the cases reported after that described by Rowell covered the diagnostic criteria of the syndrome.In 1995,Fiallo et al.(Fiallo et al.1995) described a 19-year-old man with SLE and annular polycyclic lesions on the cheeks, upper trunk, back, and arms. They believed that Rowell’s syndrome was a distinct entity and that their patient was additional evidence for its existence. In 1996, Fitzgerald et al. (Fitzgerald et al. 1996) described a 47-year-old woman with a long history of EM-like eruptions in association with LE.The patient had a speckledANA pattern,and the authors believed that she met the criteria for Rowell’s syndrome and that this syndrome is a distinct clinical and immunologic entity. In 1996, Chua et al. (Chua et al. 1996) described a 9-year-old girl with SLE and ANA titer of 1:640 who also had necrotizing lymphadenitis. In 1999, Shteyngarts et al. (Shteyngarts et al. 1999) described a 34-year-old woman with a history of SLE with concomitant EM lesions.The case was compared with other cases with Rowell’s syndrome. Their belief is that the coexistence of LE and EM does not impart any unusual characteristics to either disease and that the immunologic disturbances in such patients are probably coincidental. In 2000, Roustan et al. (Roustan et al. 2000) described a 27-year-old woman with EM-like lesions. They also reviewed the cases of Rowell’s syndrome reported previously and declared that the main clinical and immunologic findings are not distinctive and could be detected in various subtypes of LE. Roustan et al. believed that their patient might be included in so-called Rowell’s syndrome but with the clinical picture of SCLE. In 1999, Marzano et al. (Marzano et al. 1999) described a woman with LE and long-standing vesiculobullous EM-like lesions and typical laboratory findings of the antiphospholipid syndrome. The case could be consistent with the diagnosis of Rowell’s syndrome, if the latter is regarded as a clinical entity.In conclusion,the existence of Rowell’s syndrome is still disputable since few, if any,cases met the criteria of the originally reported cases by Rowell,but coexistence of LE and EM is possible and well documented. EM-like lesions can appear in patients with DLE, as it was in the original article by Rowell, they can also appear in patients with SLE, and they might be a clinical picture of SCLE. Moreover, in 1989, Sontheimer (Sontheimer 1989) stressed that the annular polycyclic changes may resemble EM. Lupus Erythematosus and Lichen Planus LE and lichen planus (LP) possess different clinical and histologic pictures and immunologic findings. Copeman et al. (Copeman et al. 1970) reported first in 1970 136 Kyrill Pramatarov, Nikolai Tsankov the coexistence of both diseases in four patients.

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