Localised scleroderma is ?
Ans. is 'b' i.e., Morphea Scleroderma Scleroderma is a chronic disease of unknown etiology that affects the microvasculature and loose connective tissue and is characterized by fibrosis and obliteration of vessels in the skin, lungs, gastrointestinal tract, kidney and hea. Scleroderma is classified into : - Cutaneous (localized) Scleroderma or Morphea. Systemic sclerosis (generalized scleroderma). Cutaneous scleroderma (Morphea or localized scleroderma) Morphea is characterized by excessive collegen deposition leading to thickening of dermis, subcutaneous tissue or both. Internal organs are not involved (in contrast to system sclerosis). Morphea presents as dark, purple or bluish, firm indurated plaques. The most impoant skin finding is the induration of skin (which is felt inside -dermal induration) and hence inability to pinch skin (hidebound skin). Morphea is divided into : ? Plaque-type -4 Most common. occurs most commonly on lower trunk followed by extremities. Face is spared. Generalized-type --> When plaque type morphea involves 3 or more major anatomical ragions of chest (sternum), abdomen, lower back, buttock and thighs. Linear type Involves most commonly lower extremities followed by upper extremities, frontal poion of head (forehead) and anterior trunk. Frontoparietal linear morphea involves frontoparietal aspect of face and scalp: it is referred to as en coup de sabre. Deep morphea (Morphea profundus or subcutaneous morphea) It affects the subcutaneous tissue in contrast to other variant of morphea where dermis is involved primarily. Systemic sclerosis (Generalized scleroderma) Systemic sclerosis is generalized form of scleroderma, which involves internal organs along with skin. So, systemic sclerosis is a multisystem disorder. It is characterized by diffuse sclerosis of Skin predominantly affecting the acral region, i.e., hands, feet, finger, toes and face. Which leads to sclerodactyly and contractures in acral areas. Other manifestations are : - Raynaud's phenomenon --> Most common & earliest manifestation. Mask like face (expression less) Beaking of nose Thinning of lips. Mat like telangiectasia Salt-pepper dyschromatosis Periungual telangiectasia Calcinosis cutis on elbow, knuckles and malleoli. Calcinosis cutis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia occur together in CREST syndrome a variant of sclerodermea. Extracutaneous manifestation are : GIT --> Reflux esophagitis is the most common systemic complaint. Other are esophageal dysmotility, Constipation, diarrhea, malabsorption. Musculoskeletal --> Ahritis, proximal muscle weakness. Respiratory -4 Pulmonary fibrosis, alveolitis Cardiac Pericarditis, conduction defect, pulmonary hypeension. Renal --> Renal failure.
Shagreen patch
Morphea
Heliotrope erythema
Gottron's papule