Lupus Erythematosus and Localized Scleroderma Coexistent at the Same Sites: A Rare Presentation of Overlap Syndrome of Connective-Tissue Diseases

The first case was described in 1976 as annular atrophic plaques on the face and neck of a 48-year-old man.³ As in our case, there were overlapping features of DLE and localized scleroderma. The investigators postulated that the entity was an atypical form of DLE.³ There were 4 more cases described in 1978, but the majority of these patients were young women with linear plaques. Instead of calling the disease a new form of DLE, the investigators considered it to be an overlap syndrome.⁴ Many years passed before another similar case was described in the literature in 1990.⁵ Interestingly, the investigators performed multiple biopsies on this patient over several years and observed that the pathology changed from subacute cutaneous LE to an overlap of subacute cutaneous LE and localized scleroderma to localized scleroderma, suggesting that localized scleroderma was the end result of persistent inflammation from the cutaneous LE lesions. The investigators compared the evolution of subacute cutaneous LE to localized scleroderma in the patient to the evolution of acute graft-versus-host disease (GVHD) to chronic GVHD. Acute GVHD has a lichenoid tissue reaction that develops into sclerosis in the chronic form.⁵

Additionally, there were 3 cases in the literature showing an overlap of lupus panniculitis with localized scleroderma.^6,7 Stork and Vosmik⁶ described a case of a 22-year-old woman with lesions clinically suspicious for localized scleroderma, with lupus panniculitis demonstrated on histopathology. They discussed the difficulty in differentiating between lupus panniculitis and localized scleroderma but did not specify whether they believed the case represented a distinct entity or an overlap syndrome.⁶ Alternatively, Marzano et al⁷ reported 2 similar cases, which the investigators considered to be a specific new variant called sclerodermic linear lupus panniculitis.

In the last 10 years, there were 3 additional cases reported that described an overlap of DLE and localized scleroderma in the same anatomic location, similar to our patient.^8-10 Although Julia et al⁸ considered their case to be an example of the distinct entity called sclerodermiform linear LE, the investigators in the other 2 cases described the possibility of an overlap syndrome.^9,10

Based on reported cases, we found the following patterns in the overlap of cutaneous LE and localized scleroderma: predilection for young women, photodistributed lesions, DLE, linear morphology clinically, and positivity along the dermoepidermal junction on direct immunofluorescence. As in our case, the few affected men were older compared to affected women. Men ranged in age from 34 to 48 years compared to women who ranged in age from 7 to 29 years. We did not find a pattern in the laboratory findings in these patients. Most patients had a good response to antimalarials, topical steroids, or systemic steroids.

Conclusion

All 12 previously reported cases showed some form of overlap of cutaneous LE and localized scleroderma. As previously discussed, overlap syndromes are common in patients with CTDs. We postulate that our case represents a rare form of overlap syndrome, with the overlap occurring at the same clinical sites.