INTRODUCTION
Calcinosis cutis (CC) is a very rare and poorly characterized finding in systemic lupus erythematosus (SLE).1 In this retrospective study, we present our experience of 10 individuals with SLE who developed CC, describing the epidemiology, diagnosis, and clinical characteristics of this rare entity.
The study was approved by the institutional review board of Massachusetts General Hospital. We selected subjects with concurrent diagnoses of SLE and CC by searching in our institutional research patient database. A total of 148 cases were found, of which 10 subjects had validated diagnoses via medical chart review of CC and SLE without other concurrent autoimmune connective tissue diseases such as mixed connective tissue disease (MCTD), dermatomyositis, or scleroderma. All data were extracted from chart review.
The mean (SD) age of subjects was 53 (17.4) years, and all were female. Seven (70%) of the subjects were white. In all cases, the development of CC occurred years, in some cases, decades after the diagnosis of SLE was established, and was not associated with SLE flare. The lower extremities were the most common area of involvement of CC, as seen in 8 of the 10 subjects, and other locations involved the upper extremities, abdomen, back, and scalp. Seven of the 10 subjects experienced multiple lesions of CC. All patients were confirmed to have at least an ANA 1:160, and 8 of the 10 subjects had high-titer ANAs ranging from 1:640 to 1:10240. Notably, 7 of the 10 subjects were anti-RNP positive, and 6 were anti-Smith positive. All anti-RNP positive subjects were also anti-Smith positive with the exception of one subject who had low-titer RNP antibodies. Seven (70%) subjects were anti-Ro antibody positive, and 7 (70%) subjects were anti-dsDNA positive (Table 1). Four (40%) subjects had lupus nephritis, and 8 (80%) reported Raynaud’s disease. Three (30%) subjects had definite biopsy-proven lupus panniculitis with CC occurring at the sites of lupus panniculitis, while an additional 2 (20%) subjects had possible lupus panniculitis on biopsy and examination.
Treatment of CC lesions included surgical excision, topical and intralesional steroids, topical and intralesional sodium thiosulfate, calcium channel blockers, and colchicine; with surgical excision as the most common and the most successful treatment modality.
In this retrospective study, we aim to characterize the clinical presentation and raise awareness of this rare finding in patients with SLE. As previously reported,1,2 the diagnosis of CC followed the diagnosis of SLE by years and was not associated with SLE flare. Most individuals had multiple CC lesions, and the most common location was the lower extremities. Although CC was associated with lupus panniculitis in up to 5 of the 10 subjects (50%), CC also appeared at body sites that did not exhibit other stigmata of CLE. Interestingly, the antibody profile of those with CC and SLE favors positive RNP, anti-Smith, anti-Ro, and anti-dsDNA antibodies. The high rates of concurrent Raynaud's disease and RNP antibodies suggest that CC appears in SLE patients who, though they do not meet the diagnostic criteria of MCTD, do share some features of the latter.
The study was approved by the institutional review board of Massachusetts General Hospital. We selected subjects with concurrent diagnoses of SLE and CC by searching in our institutional research patient database. A total of 148 cases were found, of which 10 subjects had validated diagnoses via medical chart review of CC and SLE without other concurrent autoimmune connective tissue diseases such as mixed connective tissue disease (MCTD), dermatomyositis, or scleroderma. All data were extracted from chart review.
The mean (SD) age of subjects was 53 (17.4) years, and all were female. Seven (70%) of the subjects were white. In all cases, the development of CC occurred years, in some cases, decades after the diagnosis of SLE was established, and was not associated with SLE flare. The lower extremities were the most common area of involvement of CC, as seen in 8 of the 10 subjects, and other locations involved the upper extremities, abdomen, back, and scalp. Seven of the 10 subjects experienced multiple lesions of CC. All patients were confirmed to have at least an ANA 1:160, and 8 of the 10 subjects had high-titer ANAs ranging from 1:640 to 1:10240. Notably, 7 of the 10 subjects were anti-RNP positive, and 6 were anti-Smith positive. All anti-RNP positive subjects were also anti-Smith positive with the exception of one subject who had low-titer RNP antibodies. Seven (70%) subjects were anti-Ro antibody positive, and 7 (70%) subjects were anti-dsDNA positive (Table 1). Four (40%) subjects had lupus nephritis, and 8 (80%) reported Raynaud’s disease. Three (30%) subjects had definite biopsy-proven lupus panniculitis with CC occurring at the sites of lupus panniculitis, while an additional 2 (20%) subjects had possible lupus panniculitis on biopsy and examination.
Treatment of CC lesions included surgical excision, topical and intralesional steroids, topical and intralesional sodium thiosulfate, calcium channel blockers, and colchicine; with surgical excision as the most common and the most successful treatment modality.
In this retrospective study, we aim to characterize the clinical presentation and raise awareness of this rare finding in patients with SLE. As previously reported,1,2 the diagnosis of CC followed the diagnosis of SLE by years and was not associated with SLE flare. Most individuals had multiple CC lesions, and the most common location was the lower extremities. Although CC was associated with lupus panniculitis in up to 5 of the 10 subjects (50%), CC also appeared at body sites that did not exhibit other stigmata of CLE. Interestingly, the antibody profile of those with CC and SLE favors positive RNP, anti-Smith, anti-Ro, and anti-dsDNA antibodies. The high rates of concurrent Raynaud's disease and RNP antibodies suggest that CC appears in SLE patients who, though they do not meet the diagnostic criteria of MCTD, do share some features of the latter.
DISCLOSURES
The authors declare no conflicts of interest or funding sources.
IRB Approval Status: Approved by the Mass General Brigham
IRB.
REFERENCES
1. Kim MS, Choi KC, Kim HS, Song IG, Shin BS. Calcinosis cutis in systemic lupus erythematosus: a case report and review of the published work. J Dermatol. 2010;37(9):815-818. doi:10.1111/j.1346-8138.2010.00894.x
2. Adriana T. Lopez B, Marc E. Grossman MF. Facial calcinosis cutis in a patient with systemic lupus erythematosus: a case report of tissue injury owing to photosensitivity as the cause of dystrophic calcification. JAAD Case Reports. 2017;3(5):460-463. doi:10.1016/j.jdcr.2017.06.018
2. Adriana T. Lopez B, Marc E. Grossman MF. Facial calcinosis cutis in a patient with systemic lupus erythematosus: a case report of tissue injury owing to photosensitivity as the cause of dystrophic calcification. JAAD Case Reports. 2017;3(5):460-463. doi:10.1016/j.jdcr.2017.06.018
AUTHOR CORRESPONDENCE
Flavia Fedeles MD [email protected]
