Visceral Scleroderma without Skin Involvement

Systemic sclerosis sine scleroderma: Demographic, clinical, and serologic features and survival in forty-eight patients

“…Rodnan and Fennel first coined the term "progressive systemic sclerosis sine scleroderma" in 1962 (6). Only 31 additional patients with this variant have been reported in detail (6)(7)(8)(9)(10)(11)(12)(13)(14). We describe a large group of patients with ssSSc and com-pare them with patients diagnosed as having lcSSc.…”

“…Horswell et al reported on a 59-year-old woman who had gastrointestinal involvement confirmed by histologic examination of a portion of the small intestine excised at laparotomy (8). Crown described a 20-year-old man with lung, heart, and gastrointestinal disease (9). All of the patients reported on by Rodnan and Fennel died from their disease, including 2 from cardiac involvement and 2 from infection (6).…”

Prescleroderma: A Distinct Stage of Systemic Sclerosis

“…Systemic sclerosis (SSc) sine scleroderma (ssSSc) was proposed as a distinct disease subtype in 1986, but no data were published to support a separate classification 3 . Until 2000, only 31 patients with this variant were reported in detail 2,4–12 ; since then, the demographic, clinical and laboratory features of 48 patients with ssSSc have been described 12 and criteria proposed for the diagnosis of ssSSc (Table). Preliminary criteria proposed for the diagnosis of ssSSc included: 1) Raynaud's phenomenon or a peripheral vascular equivalent; 2) positive antinuclear antibodies; 3) any of the following visceral involvement: distal esophageal hypomotility, small bowel hypomotility, pulmonary interstitial fibrosis, primary pulmonary arterial hypertension, cardiac involvement typical of scleroderma, or renal failure consistent with scleroderma renal crisis; and 4) no other defined connective tissue or other disease as a cause of any of the first three criteria.…”

Eosinophilic fasciitis. Case report and review of the literature.

“…Scleroderma occurs in conjunction with overlap "g4 syndromes (Tuffanelli and Winkelmann, 1961; f ' Dubois et al, 1971;Sharp et al, 1972;Dubois, 1974), calcinosis (Thibierge and Weissenbach, 1910;Brooks, 1934), visceral involvement without skin changes (Crown, 1961;Rodnan and Fennell, 1962), pneumonitis (Rodnan et al, 1967), malignancy pOUS (Tompkin, 1969), and childhood variants (Jaffe and x300. Winkelmann, 1961;Kass et al, 1966), as well as scleroderma-like changes in Werner's syndrome (Epstein et al, 1966), cutaneous amyloid (Loewenthal 'eosinoand Falkson, 1965) and carcinoid tumours (Fries et oids was al., 1973) (Table 2).…”