Cutaneous and subcutaneous inflammatory sclerosis syndromes

Abstract

Systemic scleroderma and localized scleroderma (morphea) show comparable changes on skin biopsy specimens, and a distinction has often been made on the basis of Raynaud's phenomenon, organ involvement, and laboratory abnormalities characteristically seen in systemic scleroderma. Critical evaluation not only of patients with localized scleroderma but also of those with eosinophilic fasciitis, morphea profunda, and acral pansclerotic morphea has disclosed Raynaud's phenomenon, organ involvement, and laboratory abnormalities typical of systemic scleroderma in a small percentage of patients. Histologically, all five conditions show similar inflammation and sclerosis of the skin, the primary difference being the depth at which these changes occur. These conditions may possibly be related, and the clinical and laboratory differences observed may result from variations in depth, nature, and intensity of the cutaneous and subcutaneous inflammatory sclerosis.

Localized Eosinophilic Fasciitis

“…1,2 The eosinophils may be absent from the tissues 6 and their presence or absence may be related to timing and the site of the biopsy. 2,7 An advancing margin of the lesion may be the site of early active disease.…”

“…However, it is becoming increasingly recognized that EF shares many features in common with scleroderma. 6,7 It may occur concomitantly with progressive systemic sclerosis 3 or coexist with multiple plaques of morphea. 8 The rarity of Raynaud's phenomenon and significant visceral changes, the absence of sclerodactyly and telangiectasia, help to differentiate EF from systemic scleroderma.…”

“…The histopathology of EF, localized and systemic scleroderma will show comparable changes, the major difference being the depth of the inflammatory process. 7 Moreover, the dense dermal thickening, replacement of panniculus by connective tissue, epidermal and adnexal structure atrophy and absence of thickened fascia help to differentiate these scleroderma syndromes from EF. Although EF and other scleroderma syndromes have a propensity for primary involvement at different levels in the skin, the considerable overlapping in the laboratory and histologic findings and concomitant occurrence in individual patients reinforces the notion that EF may be an early manifestation or variant of scleroderma.…”

“…Although EF and other scleroderma syndromes have a propensity for primary involvement at different levels in the skin, the considerable overlapping in the laboratory and histologic findings and concomitant occurrence in individual patients reinforces the notion that EF may be an early manifestation or variant of scleroderma. [7][8][9] Localized EF must be differentiated from other conditions which may present initially as localized erythematous induration of the skin. These conditions include morphea, scleroderma (morphea profunda), focal myositis or polymyositis, necrotizing fasciitis and nodular fasciitis.…”

“…2,9 Other organ system involvement 6 should be searched for as more cases are recognized; these patients should be followed closely for development of severe hematologic disorders, including lymphoid malignancy. 2,7 …”