Erythema nodosum-like cutaneous lesions of sarcoidosis showing livedoid changes in a patient with sarcoidosis and Sjögren's syn

ARTICLE

Auteur(s) : Hideo Takenoshita¹, Toshiyuki Yamamoto²

¹Department of Dermatology, Shirakawa Kosei General Hospital, 961-0005 Shirakawa, Japan
²Department of Dermatology, Fukushima Medical University, Hikarigaoka 1, Fukushima 960-1295, Japan

A 30-year-old Japanese woman complained of scattered erythema on the lower legs. Ophthalmological examination revealed uveitis. A physical examination showed erythematous patches with mild tenderness scattered on the anterior aspects of the bilateral lower legs (figure 1A). Erythema nodosum associated with Behçet's disease was suspected, but histological examination revealed epithelioid granulomas containing giant cells scattered throughout the dermis and the subcutaneous tissues (figure 1B). The lumina of the vessels were narrowed by fibrinoid degeneration (figure 1C). Lymphocyte infiltration was scarce, and no caseation necrosis was noted. She also complained of sicca. Laboratory examination showed increased serum levels of lysozyme (19.4 μg/mL, normal; 5.0-10.2) and angiotensin converting enzyme (45.3 U/L, normal; 8.3-21.4), positive anti-nuclear antibody (1:40, homogeneous & speckled), elevated IgG levels (2,503 mg/dL), SS-A antibody (78.5 IU/mL), and SS-B antibody (36.1 IU/mL). Gum test showed over 10 mL/10 min. She refused a salivary gland biopsy. Schirmer test was bilaterally positive (5 mm in both eyes), but she had negative keratoconjunctivitis sicca. Sialoscintigraphy revealed a mild impairment (grade 2). Chest X-ray and computed tomography (CT) showed bilateral hilar lymphadenopathy. She was diagnosed with sarcoidosis and subclinical Sjögren's syndrome (SjS). Investigation for respiratory and cardiac function revealed normal findings, and she was thereafter only monitored, without systemic prednisolone. After 6 months, cutaneous lesions on the anterior and posterior aspects of the lower limbs, mimicking livedo, showed enlargement (figure 1D), and 1 year after the onset, the skin lesions spontaneously regressed (figure 1E).

Erythema nodosum is one of the non-specific manifestations associated with sarcoidosis, which histologically shows panniculitis in the septal region of subcutaneous tissues, without granulomas. On the other hand, an erythema nodosum-like eruption in sarcoidosis is a specific form of cutaneous sarcoidosis, histologically characterized by sarcoidal granuloma in the dermis to subcutis [1]. This subtype is occasionally seen in Japan, and predominantly occurs in women with a high frequency of ocular involvement [1]. The most interesting aspect of our case is the fact that the erythema nodosum-like eruption gradually spread to the upper limbs and posterior aspects of the limbs bilaterally, assuming livedoid changes. Cases of cutaneous sarcoidosis showing livedoid changes have been reported [2, 3]; however, a shift in clinical appearance from erythema nodosum-like lesions to livedoid lesions has never been previously reported. Histological examination of the cases showing livedoid changes revealed sarcoidal granuloma and fibrinoid degeneration around the vessel walls in the lower dermis to subcutaneous tissues, as were noted in our case, which are supposed to cause disturbances of local circulation. Cutaneous lesions of sarcoidosis with livedo disappeared with the administration of systemic predonisolone [2] and topical corticosteroids [3]. In our case, complete spontaneous regression occurred 12 months after onset. Spontaneous regression of cutaneous sarcoidosis is occasionally seen, and the erythema nodosum-like lesions are transient and spontaneously disappear in the majority of cases [1].

Another interesting point in this case is the association with SjS. Coexistence of sarcoidosis with autoimmune disorders, such as scleroderma and SjS, has occasionally been observed. Sarcoidosis and primary SjS share pathogenic, immunogenetic, and clinical features. The occurrence of sarcoidosis in patients with SjS has been estimated in only 1% to 2% of cases [4, 5]. Numerous cytokines, such as tumor necrosis factor-α, interferon-γ, interleukin-12, and interleukin-18, which are produced by activated macrophages and T-cells, are thought to play a pivotal role in the pathogenesis of granuloma formation in sarcoidosis [6]. Those cytokines may also play a role in the induction of SjS. In conclusion, our case indicates that erythema nodosum-like lesions and livedoid lesions are consecutive, and few of the cases presenting erythema nodosum-like lesions are thought to progress to the livedoid changes.

Acknowledgments

References

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