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Autoimmune Connective Tissue Diseases

»Discuss the skin changes of lupus erythematosus.
»What is acute cutaneous lupus erythematosus (ACLE)?
»Are there any common skin eruptions that may be confused with acute cutaneous lupus erythematosus?
»What is subacute cutaneous lupus erythematosus (SCLE)?
»Do patients with SCLE have SLE?
»How do you make a diagnosis of SCLE?
»What is the initial workup of SCLE?
»How is SCLE managed?
»What is chronic cutaneous lupus erythematosus?
»Describe the skin changes of discoid lupus erythematosus.
»Do patients with DLE develop systemic lupus erythematosus?
»How is discoid lupus erythematosus treated?
»What is minocycline-induced lupus?
»What is lupus panniculitis?
»Describe the bullous eruption of SLE.
»How is the bullous eruption of systemic lupus erythematosus treated?
»What is neonatal lupus erythematosus (NLE)?
»Which tests should be done in an infant with suspected NLE?
»Once a diagnosis of NLE is made, what workup should be done?
»What is the lupus band test?
»What is scleroderma?
»What is the CREST syndrome?
»Describe the early cutaneousfindings in progressive systemic sclerosis (PSS).
»What is dermatomyositis?
»Are there skin changes diagnostic of dermatomyositis?
»How do you diagnose dermatomyositis?
»Are any diseases associated with dermatomyositis?
»What is the antisynthetase syndrome?
»What is an overlap syndrome?
»What is mixed connective tissue disease?
»What is the antiphospholipid antibody syndrome?
»What are some other connective tissue diseases with cutaneous manifestations?
»What autoantibodies are associated with the different autoimmune connective tissue diseases?

 
 
 

Are there skin changes diagnostic of dermatomyositis?

Dermatomyositis. A, Characteristic violaceous erythema over dorsal knuckles in a patient with associated breast cancer. B, Characteristic photodistributed erythema with heliotrope of upper eyelids.  (Courtesy of James E. Fitzpatrick, MD.)
Fig. 22.9 Dermatomyositis. A, Characteristic violaceous erythema over dorsal knuckles in a patient with associated breast cancer. B, Characteristic photodistributed erythema with heliotrope of upper eyelids. (Courtesy of James E. Fitzpatrick, MD.)
Two cutaneous findings have been described as pathognomonic of dermatomyositis: Gottron’s papules and Gottron’s sign. Gottron’s papules are erythematous to purplish flat papules on the extensor surfaces of the interphalangeal joints. Gottron’s sign consists of symmetrical violaceous erythema, sometimes with edema, over the dorsal knuckles of the hands, elbows, knees, and medial ankles (Fig. 22-9A). Other skin findings that are characteristic of dermatomyositis are periorbital edema with a lilac-colored erythema (heliotrope, Fig. 22-9B), periungual telangiectasia with cuticle dystrophy, and a photodistributed violaceous erythema of the forehead; also, sun-exposed areas of the neck, upper chest, shoulders, dorsal arms, forearms, and hands. A diagnostic clue favoring dermatomyositis over lupus erythematosus is the violaceous erythema or papules over the knuckles. Lupus, on the other hand, shows erythema over the dorsal phalanges, but often spares the knuckles.

Callen JP, Wortmann RL: Dermatomyositis, Clin Dermatol 24:363–373, 2006.