Among the cutaneous features (Table 1), fingertip ulceration was found in 36 (66.7%), sclerodactyly
in 48 (88.8%) (Figure 1), digital pitted scars in 30 (55.5%) (Figure 2), digital gangrene in 3 (5.6%), calcinosis cutis in 4 (7.4%), microstomia in 48 (88.8%) (Figure 3), radial furrows around mouth in 18 (33.3%) (Figure 4), pinched nose in 33 (61.1%), diffuse alopecia in 10 (18.5%) and nail changes in 10 (18.5%) cases.
anic's Dysphagia Fever Diagnosis Follow-up Hands (c) 1 - - - Sjogren Alive, 12 y 2 + UCTD Alive, lung transplant, 18-y follow-up, 7 y after transplant 3 + - - PM Alive, 7 y 4 UCTD Alive, lung transplant, 10-y follow-up, 5 y after transplant 5 - - - UCTD Alive, 8 y 6 - - + PM Dead, unknown 7 - + + Overlap RA Alive, 15 y and SSc 8 - - - UCTD Alive, lung transplant, 18-y follow-up, 11 y after transplant 9 - - + SSc Alive, 9 y 10 - - - UCTD Alive, 13 y 11 + - + PM Alive, 6 y 12 - - + PM Alive, 20 y Abbreviations: PM, polymyositis;RA, rheumatoid arthritis; SD, sclerodactyly
;SSc, systemic sclerosis;UCTD, undifferentiated connective tissue disease.
Scleroderma was judged as a reasonable diagnosis based on her puffy fingers that developed into sclerodactyly
, abnormal nail fold capillaries, Raynaud's phenomenon, interstitial lung disease, and lower esophageal dysmotility .
, which presents as tightening of the skin of the fingers or toes, and
However, there have been only few reports of AlTDs associated with CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly
and Telangiectasias), a limited form of scleroderma .
History of gastroesophageal reflux disease, Raynaud's disease, and sclerodactyly
may suggest existing disease.
Skin of the extremities (sclerodactyly
) and face were thickened.
The most common clinical feature of the disease was Raynaud's phenomenon (100%) followed by microstomia (98%) sclerodactyly
(91%) digital pitting (89%) digital ulcers (82%) and arthralgias (73%).
There was no skin nodule, skin rash, lymphadenopathy, hepato-splenomegaly, puffy fingers, sclerodactyly
, or no history of Raynaud's phenomenon, dysphagia, hemoptysis or any bleeding episodes, muscle weakness, seizures or abnormal behavior.
The CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly
, and telangiectasia) is one clinical subtype of limited cutaneous systemic sclerosis.
(hardening of skin of the fingers or toes)