Chilblain Lupus Erythematosus

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CHLE, a rare manifestation of CCLE distinguished by Hutchinson in 1888 (Hutchinson 1888), is strongly influenced by environmental factors (Breathnach and Wells 1979, Doutre et al. 1992, Helm and Jones 2002, Rowell 1987, Uter et al. 1988). This subtype seems to be more frequent in women and, interestingly, very uncommon in the United States, as Tuffanelli and Dubois (Tuffanelli and Dubois 1964) failed to detect such lesions among 520 patients; however, these patients were collected for the most part from the warm Southern California area. In contrast, Millard and Rowell (Millard and Rowell 1978) detected 17 cases with this subtype in a review of 150 patients with CLE (11.3%). Four of these patients demonstrated erythema multiforme-like lesions, and 3 of these 17 patients subsequently developed features of SLE. One further reported case of CHLE was induced by pregnancy and disappeared after delivery (Stainforth et al. 1993). The pathogenesis is unknown, but microvascular injury secondary to exposure to cold, damp weather or a drop in temperature and possible

Fig. 6.14. Telangiectoid variant of discoid lupus erythematosus (DLE). Multiple erythematous plaques and reticulate telangiectasia on the face that produce large areas of disfigurement on confluence

Chilblain Lupus Erythematosus

hyperviscosity from immunologic abnormalities may play a role (Mascaro et al. 1997, Yell et al. 1996). In most reported cases, patients with CHLE present a polyclonal hypergammaglobulinemia, increased serum immunoglobulin levels, and a positive rheumatoid factor. In addition, anti-double-stranded DNA or anti-Ro/SSA antibodies have often been detected, but laboratory examinations usually fail to reveal evidence of cryoglobulins, cryofibrinogens, or cold agglutinins (Su et al. 1994). In a few patients, CHLE has been described in association with antiphospholipid syndrome (Allegue et al. 1988, De Argila Fernandez-Auran et al. 1996). The evolution of lesions in patients with CHLE is usually chronic, and sometimes these lesions precede other manifestations of SLE (Doutre et al. 1992). The risk of developing SLE is estimated to be approximately 20%, but in this rare form of CCLE, only a few studies have been reported; nevertheless, long-term follow-up of these patients is warranted (Viguier et al. 2001). Most patients progressing to SLE have arthralgia, manic depressive psychoses, or a reduced creatinine clearance, presumably from renal involvement. Furthermore, most patients with CHLE have or have had typical DLE lesions on the face; however, the evolution of both types of lesions is different, and chilblain manifestations usually persist when classic DLE disappears.

Clinically, CHLE is characterized by symmetrically distributed, circumscribed, sometimes infiltrated, pruriginous or painful areas of livid and purple plaques that appear and exacerbate during cold, damp weather periods (Fig. 6.15). There is only a slight tendency to central regression, and the lesions, in their evolution, may ulcerate or present firmly adherent hyperkeratosis (Kuhn et al. 2000c, Sontheimer and Provost 2003). The lesions of CHLE involve mostly the dorsal and lateral parts of the hands and feet, the ears, the nose, the elbows, the knees, or the calves (Helm and Jones 2002, Su et al. 1994). On toes and fingers, the lesions develop on the back or on the pads (Doutre et al. 1992, Fisher and Everett 1996), and fissuring of the knuckles as well as

Fig. 6.15. Chilblain lupus erythematosus (CHLE). Red-purple patches on the finger end joints that are precipitated by cold, damp climates

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accompanying hyperhidrosis are common, producing a great deal of discomfort (Costner et al. 2003). Ulceration is frequent in digital pulp lesions, and they easily become necrotic on the soles (Mascaro et al. 1997). When located in the periungual zone, the nail plate may develop mild to severe dystrophy.

Because CHLE lesions are highly reminiscent of simple chilblains or pernio lesions (Viguier et al. 2001), one could question whether such patients have simple pernio that in the predisposed individual produces a Koebner's phenomenon resulting in DLE. The terms "chilblain lupus" and "perniotic lupus" have been used to describe such lesions. Unfortunately, the term "lupus pernio" has also been used for such lesions, although this term is more properly used to designate a form of cutaneous sarcoidosis (James 1992). For a positive diagnosis of CHLE, it has been proposed to establish two groups of major and minor criteria (Su et al. 1994). Major criteria include (a) cold-induced or cold-aggravated lesions in acral locations and (b) evidence of LE on histopathology or direct immunofluorescence. Minor criteria include (a) the coexistence of SLE or other manifestations of CLE, (b) positive response to LE therapy, and (c) negative results of cryoglobulin and cold agglutinin studies. The diagnosis of CHLE may be affirmed if the patient fulfills both major criteria and at least one of the minor criteria.A chronic form of CHLE occurs especially in older persons who have underlying vascular abnormalities, such as acrocyanosis, Raynaud's phenomenon, atherosclerosis, or erythrocyanosis. In such patients, this subtype of CCLE can last for several months and tends to recur annually, sometimes with hem-orrhagic blisters, erosions, or ulcers.

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