Abstract:

A 27-year-old female presented with a 1-year history of a facial rash and skin tightening. Review of systems was notable for dysphagia, Raynaud phenomenon, muscle weakness, and dyspnea. Physical examination demonstrated microstomia, swollen fingers, violaceous erythema on the cheeks, forehead and eyelids, indurated violaceous and hyperpigmented plaques on the neck, chest, and abdomen, and firm linear plaques in the bilateral axillae. Skin biopsy revealed dermal proliferation of fibroblasts and keloidal collagen in a transverse and haphazard array, with superficial to deep perivascular and peri-eccrine lymphoplasmacytic inflammation. Verhoeff-Van Gieson stain showed decreased and fragmented elastic fibers. Immunohistochemical studies revealed decreased CD34 and increased SMA expression within the fibrotic stroma. Laboratory studies were remarkable for elevated ANA, anti-smooth muscle antibody, rheumatoid factor, anti-SS-A, ESR, aldolase, and CK. The patient was ultimately diagnosed with keloidal scleroderma and inflammatory myopathy. Nodular or keloidal scleroderma is a rare variant of scleroderma characterized by hypertrophic scar-like plaques or nodules in association with localized morphea or systemic sclerosis. The terms nodular and keloidal scleroderma are used interchangeably. Some authors suggest that the term keloidal scleroderma be reserved for cases with clinical and histologic features of keloid. The etiology of keloidal scleroderma remains unclear but may involve an interplay of genetic factors and inflammatory reactions resulting in a keloidal response. On histology, features of classic scleroderma, hypertrophic scar or keloid may be present which may pose a diagnostic challenge. Clinicians and dermatopathologists should be aware of this rare variant of scleroderma and the importance of clinicopathologic correlation.

Financial Disclosure:
No current or relevant financial relationships exist.

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Victoria Lee, PhD