TOPICS

Nodular Scleroderma Revisited: Systemic Sclerosis Presenting as Annular Keloidal Sclerotic Plaques

aDavid N. Lortscher, MD; aPhilip R. Cohen, MD; bCarolyn A. Bangert, MD; aTaraneh Paravar, MD aThe Division of Dermatology, University of California San Diego, San Diego, California; bThe Department of Dermatology, University of Texas Health Science Center at Houston, Houston, Texas

Disclosure: The authors report no relevant conflicts of interest.


Abstract

Background: Nodular scleroderma, also known as keloidal scleroderma, is a rare variant of systemic sclerosis. Purpose: The clinical features, pathologic findings and postulated pathogenesis of nodular scleroderma are discussed. Methods: A woman with previously undiagnosed systemic sclerosis who presented with nodular scleroderma is described. Using the PubMed database, a literature search was performed on keloidal scleroderma, nodular scleroderma, and systemic sclerosis. Results: Nodular scleroderma is characterized by firm plaques or nodules, which can mimic a keloid, that are typically located on the anterior or posterior upper trunk and the arms; they show pathologic changes of scleroderma, keloid, or hypertrophic scar. A keloidal response of inflamed skin that is involved in an active fibrotic process inherent to systemic sclerosis, in individuals who are genetically predisposed to keloid formation, is the hypothesized pathogenesis. Conclusion: Nodular scleroderma is rare. The authors’ patient presented with diarrhea, dysphagia, fatigue, Raynaud’s phenomenon, shortness of breath, and annular keloidal plaques of morphea whose biopsy showed features of hypertrophic scar; additional studies confirmed the diagnosis of the nodular scleroderma variant of systemic sclerosis. The possibility of systemic sclerosis should be entertained in patients who present with nodular or keloidal plaques that morphologically resemble morphea and have histologic findings of a scar or a keloid—especially if there are associated symptoms suggestive for systemic sclerosis. (J Clin Aesthet Dermatol. 2016;9(6):56–57.)


Nodular scleroderma is a rare variant of systemic sclerosis. Patients have not only nodular lesions of morphea, but also systemic involvement of the disease. The authors report the case of a woman with previously undiagnosed systemic sclerosis who presented with annular sclerotic plaques of keloidal scleroderma on her upper back and breasts.

Case report

A 53-year-old woman presented with itchy firm lesions of eight years duration on her upper back and breasts. There was no prior history of trauma to the sites. She also had diarrhea, dysphagia, fatigue, Raynaud’s phenomenon, and shortness of breath.

Clinical examination revealed symmetrically distributed, hyperpigmented, indurated annular plaques with raised borders on her upper back and breasts (Figure 1). Dermatoscopic evaluation of her proximal nailfolds showed dilatation and dropout of the capillary loops. There was no calcinosis, sclerodactyly, or telangiectasia; diffuse induration of the facial, extremity, or truncal skin was absent.

Microscopic examination of biopsies from both the right upper back and the left breast showed similar changes. There was increased deposition of collagen bundles extending from the mid-reticular dermis to the dermal-subcutaneous junction; the papillary dermis was spared and there were no pathologic changes in the epidermis (Figure 2). There was a marked increase in spindled fibroblasts within the fibrous tissue. The cells did not stain with antibody to CD34, factor XIIIa, S100 protein, and smooth muscle actin. The pathology findings were initially interpreted as a hypertrophic scar.

Subsequent laboratory investigation revealed anemia and moderate renal insufficiency. Her anti-nuclear antibody titer was elevated (>1:640) and demonstrated an anti-centromere pattern. Antibodies against topoisomerase I (Scl-70), double-stranded DNA, Smith, Sjogren’s syndrome A/Ro, and Sjogren’s syndrome B/La were negative.

Additional work-up was performed. Upper endoscopic examination revealed a thickened esophageal wall and absent peristalsis of the distal esophagus. Pulmonary function tests demonstrated mild restrictive lung disease. However, there was no evidence of increased pulmonary artery pressure on her echocardiogram.

Correlation of her clinical presentation, pathology findings, and additional studies established the diagnosis of nodular scleroderma. There was no change following intralesional therapy with 0.5mL of 10mg/mL of triamcinolone into one of her skin lesions. Other treatment options for her skin lesions, including topical agents and phototherapy, were declined.

She was referred to a rheumatologist for management of her systemic sclerosis. Initial treatment with methotrexate was discontinued because of nausea and vomiting. Thereafter, hydroxychloroquine, lisinopril, and mycophenolic acid were started; this treatment has been well-tolerated and maintained.

Discussion

Nodular scleroderma, also known as keloidal scleroderma, is an uncommon type of systemic sclerosis. It is characterized by firm plaques or nodules that are typically located on the anterior or posterior upper trunk and the arms; often the morphology of the clinical lesions mimics a keloid.[1–3] Histologic evaluation of the clinical lesions can show pathologic changes of scleroderma, keloid, or hypertrophic scar.[2],[4],[5]

The pathogenesis of nodular scleroderma lesions is considered to be a keloidal response of inflamed skin that is involved in an active fibrotic process inherent to systemic sclerosis. It occurs in individuals who are genetically predisposed to keloid formation. In these patients, the skin lesions occur at sites that are at high risk for the development of keloid and hypertrophic scar formation, such as the trunk.[6],[7]

The classification of patients with keloidal lesions of scleroderma depends on the presence or absence of systemic involvement. Patients with such lesions who have systemic involvement should be designated as having nodular scleroderma.[3],[6] In contrast, individuals with the same lesions, but without systemic involvement, are classified as having nodular morphea.[1],[5],[8]

The patient’s cutaneous lesions were consistent with those of morphea with an annular morphology; remarkably, they showed pathologic changes suggestive of a hypertrophic scar. In addition, she had no other skin findings of limited or diffuse systemic sclerosis. However, her additional symptoms (such as Raynaud’s phenomenon and dysphagia along with shortness of breath and fatigue) prompted us to perform a thorough laboratory and organ-directed evaluation. Correlation of the clinical, pathologic, laboratory, and investigative findings allowed us to establish the diagnosis of nodular scleroderma.

Conclusion

Nodular scleroderma is a rare type of systemic sclerosis. The authors’ patient presented with annular plaques of morphea that demonstrated features consistent with hypertrophic scar on pathologic examination. Her associated symptoms prompted them to consider systemic involvement of her condition; an elevated anti-nuclear antibody with an anticentromere pattern associated with abnormal evaluation of her esophagus and lungs confirmed the diagnosis of keloidal systemic sclerosis. In conclusion, the possibility of systemic sclerosis should be considered in those individuals who present with nodular or keloidal plaques that clinically resemble morphea and have histologic findings of either a scar or a keloid—especially if there are associated symptoms suggestive for systemic sclerosis.

References

1. Krell GM, Solomon AR, Glavey CM, Lawley TJ. Nodular scleroderma. J Am Acad Dermatol. 1995;32:343–345.

2. Labandeira J, León-Mateos A, Suárez-Peñaranda JM, et al. What is nodular-keloidal scleroderma? Dermatology. 2003;207:130–132.

3. Heath CR, David JN, Taylor SC. Nodular scleroderma presenting as multiple spontaneous keloidal scars. J Am Acad Dermatol. 2012;66:e245–e246.

4. Wriston CC, Rubin AI, Elenitsas R, Crawford GH. Nodular scleroderma: a report of 2 cases. Am J Dermatopathol. 2008;30:385–388.

5. Kauer F, Simon JC, Sticherling M. Nodular morphea. Dermatology. 2009;218:63–66.

6. Melani L, Caproni M, Cardinali C, et al. A case of nodular scleroderma. J Dermatol. 2005;32:1028–1031.

7. James WD, Berger TG, Butler DF, Tuffanelli DL. Nodular (keloidal) scleroderma. J Am Acad Dermatol. 1984;11: 1111–1114.

8. Micalizzi C, Parodi A, Rebora A. Morphoea with nodular lesions. Br J Dermatol. 1994;131:298–300.

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