The single major criterion is presence of sclerodermatous
involvement proximal to the digits, affecting limbs, face, neck, or trunk usually in a bilateral and symmetrical pattern.; the minor criteria are sclerodactyly, digital pitted scars or tissue loss of volar pads of fingertips, and bibasilar pulmonary fibrosis.4 The diagnosis is based on presence of either the major criterion or at least two of minor criteria.
Conclusions: Data demonstrate ATYR1923 is efficacious in a murine model of sclerodermatous
chronic graft versus host disease when administered weekly at only 0.4 mg/kg.
Min, "Mesenchymal stem cells (MSCs) attenuate cutaneous sclerodermatous
graft-versus-host disease (Scl-GVHD) through inhibition of immune cell infiltration in a mouse model," The Journal of Investigative Dermatology, vol.
Aside from the acute gastric GVHD and mild transaminase elevation consistent with liver GVHD discussed above, the course was complicated only by ongoing and difficult-to-control diffuse chronic sclerodermatous
GVHD of the skin requiring several courses of immunosuppressive therapy.
Jin et al., "Donor-derived regulatory B cells are important for suppression of murine sclerodermatous
chronic graft-versus-host disease," Blood, vol.
Progeric children are normal at birth and within the first year of life develop severe growth deficiency with sclerodermatous
The affected demonstrates a characteristic features by displaying delayed growth, osteoporosis, cardiovascular ailments, alopecia, pinched nose, and sclerodermatous
Gilliam, "Murine sclerodermatous
graft-versus-host disease, a model for human scleroderma: cutaneous cytokines, chemokines, and immune cell activation," Journal of Immunology, vol.
or "tumored" scales having tumour (scleroma)-like hardened skin scales
Radiation-induced fibrosis (RIF), radiation recall dermatitis, post-irradiation sclerodermatous
panniculitis, and cancerous sclerosing recurrence are the main differential diagnoses.
changes are present, histology may reveal varying degree of collagen sclerosis resembling morphea.
Juvenile systemic sclerosis involves the skin, muscle, and visceral organs; therefore, the differential diagnosis should include other multiple-system organ diseases with skin involvement including juvenile dermatomyositis, mixed connective tissue disease, SLE, juvenile idiopathic arthritis, overlap syndrome, eosinophilic fasciitis, chronic graft versus host disease, nephrogenic systemic fibrosis, phenylketonuria with sclerodermatous
skin lesions, and syndromes of premature aging.