Journal of Dermatology 2014; 41: 421–423
doi: 10.1111/1346-8138.12438
CONCISE COMMUNICATION
Systemic sclerosis with sarcoidosis: Case report and review of the published work Satoko SENDA,* Ken IGAWA,* Megumi NISHIOKA, Hiroyuki MUROTA, Ichiro KATAYAMA Department of Dermatology, Graduate School of Medicine, Osaka University, Osaka, Japan
ABSTRACT Sarcoidosis and systemic sclerosis (SSc) rarely coexist. Here, we report a Japanese female SSc patient who developed systemic sarcoidosis. Her SSc was a limited type negative for anti-Scl-70 antibody and positive for anticentromere antibody (ACA). Moreover, we performed a review of the English-language published work that described cases of concurrent SSc and sarcoidosis. Then, we found that most SSc and sarcoidosis concurrent patients positive for anti-Scl-70 antibody were male (77.8%). On the other hand, most patients positive for ACA were female (87.5%). These results suggest some relationships between autoantibody profiles and sex in SSc and sarcoidosis concurrence.
Key words:
autoantibody, concurrent, sarcoidosis, sex, systemic sclerosis.
INTRODUCTION (a) Sarcoidosis is a multisystem granulomatous disorder of unknown etiology.1 On the other hand, systemic sclerosis (SSc) is a multisystem connective tissue disorder characterized by fibrosis and sclerosis of the skin and internal organs with various degrees of vascular damage. To date, these disorders have rarely been observed concurrently.2 Herein, we present a female case of limited SSc that developed systemic sarcoidosis in her clinical course. In addition, we present a review of previous reports of concurrent SSc and sarcoidosis.
CASE REPORT A 59-year-old woman visited our department because of finger stiffness. Physical examination revealed sclerodactyly, sclerosis of the dorsum manus, extension of the cuticle, bleeding of the nail fold and erythema of the perionychia (Fig. 1a). Raynaud’s phenomenon during the winter seasons had gradually developed in the last 20 years. Two years prior, she had noticed bleariness in her left eye and visited an ophthalmologist, who diagnosed this as uveitis. We considered a diagnosis of SSc and/or systemic sarcoidosis and further examinations, including skin biopsy, were performed. Skin biopsy from her dorsum manus revealed swelling and degeneration of collagen fibers in the middle and lower dermis (Fig. 1b). Moreover, so-called naked epithelioid cell granuloma was obvious in the upper dermis (Fig. 1c). Autoantibody screening results were positive for antinuclear (1:1280) and an-
(b)
(c)
(d)
Figure 1. (a) Clinical appearance of swollen fingers, sclerosis of the dorsum manus and extension of cuticles. (b,c) Epithelioid cell granuloma in the upper dermis (hematoxylin–eosin [HE], original magnifications: [b] 940; [c] 9100). (d) Degeneration of collagen fibers in the middle and lower dermis (HE, 9200).
ticentromere antibodies (ACA). Anti-SS-A and anti-SS-B antibodies were not observed. The angiotensin-converting enzyme level was higher than the normal score (32.5 IU/L; normal,
doi: 10.1111/1346-8138.12438
CONCISE COMMUNICATION
Systemic sclerosis with sarcoidosis: Case report and review of the published work Satoko SENDA,* Ken IGAWA,* Megumi NISHIOKA, Hiroyuki MUROTA, Ichiro KATAYAMA Department of Dermatology, Graduate School of Medicine, Osaka University, Osaka, Japan
ABSTRACT Sarcoidosis and systemic sclerosis (SSc) rarely coexist. Here, we report a Japanese female SSc patient who developed systemic sarcoidosis. Her SSc was a limited type negative for anti-Scl-70 antibody and positive for anticentromere antibody (ACA). Moreover, we performed a review of the English-language published work that described cases of concurrent SSc and sarcoidosis. Then, we found that most SSc and sarcoidosis concurrent patients positive for anti-Scl-70 antibody were male (77.8%). On the other hand, most patients positive for ACA were female (87.5%). These results suggest some relationships between autoantibody profiles and sex in SSc and sarcoidosis concurrence.
Key words:
autoantibody, concurrent, sarcoidosis, sex, systemic sclerosis.
INTRODUCTION (a) Sarcoidosis is a multisystem granulomatous disorder of unknown etiology.1 On the other hand, systemic sclerosis (SSc) is a multisystem connective tissue disorder characterized by fibrosis and sclerosis of the skin and internal organs with various degrees of vascular damage. To date, these disorders have rarely been observed concurrently.2 Herein, we present a female case of limited SSc that developed systemic sarcoidosis in her clinical course. In addition, we present a review of previous reports of concurrent SSc and sarcoidosis.
CASE REPORT A 59-year-old woman visited our department because of finger stiffness. Physical examination revealed sclerodactyly, sclerosis of the dorsum manus, extension of the cuticle, bleeding of the nail fold and erythema of the perionychia (Fig. 1a). Raynaud’s phenomenon during the winter seasons had gradually developed in the last 20 years. Two years prior, she had noticed bleariness in her left eye and visited an ophthalmologist, who diagnosed this as uveitis. We considered a diagnosis of SSc and/or systemic sarcoidosis and further examinations, including skin biopsy, were performed. Skin biopsy from her dorsum manus revealed swelling and degeneration of collagen fibers in the middle and lower dermis (Fig. 1b). Moreover, so-called naked epithelioid cell granuloma was obvious in the upper dermis (Fig. 1c). Autoantibody screening results were positive for antinuclear (1:1280) and an-
(b)
(c)
(d)
Figure 1. (a) Clinical appearance of swollen fingers, sclerosis of the dorsum manus and extension of cuticles. (b,c) Epithelioid cell granuloma in the upper dermis (hematoxylin–eosin [HE], original magnifications: [b] 940; [c] 9100). (d) Degeneration of collagen fibers in the middle and lower dermis (HE, 9200).
ticentromere antibodies (ACA). Anti-SS-A and anti-SS-B antibodies were not observed. The angiotensin-converting enzyme level was higher than the normal score (32.5 IU/L; normal,
