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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?

 
 
 

Discuss the skin changes of lupus erythematosus.

Skin changes occur very frequently in lupus erythematosus (LE) and are second in frequency only to musculoskeletal complaints in this condition, occurring in about 85% of patients. It is useful to classify the eruptions seen in LE as to their possible diagnostic and prognostic significance. Skin lesions that are diagnostic of LE have been called lupus-specific eruptions. Skin biopsies of these lesions show characteristic histopathologic changes of cutaneous LE. Further classification of the lupus-specific eruptions into subtypes of cutaneous LE is also useful, as some lesions of cutaneous LE are more strongly associated with systemic lupus erythematosus.

Lupus patients also develop many skin changes that are not specific for LE, termed lupus-nonspecific eruptions (Table 22-1). These eruptions do not help to establish a diagnosis of LE, but they may still be very important to note, as specific systemic findings may be associated with them. For example, cutaneous lesions of palpable purpura in a patient with LE are not lupus-specific, that is, such lesions may be seen in patients who do not have LE; however, they may be associated with vasculitic lesions of the kidney or central nervous system (CNS), and thus they have significance in the evaluation and treatment of lupus.


Walling HW, Sontheimer RD: Cutaneous lupus erythematosus: issues in diagnosis and treatment, Am J Clin Dermatol 10:365–381, 2009.
Table 22-1. Classification of Cutaneous Disease in Lupus Erythematosus
  LUPUS-SPECIFIC ERUPTIONS LUPUS-NONSPECIFIC ERUPTIONS
  Acute cutaneous lupus erythematosus (ACLE)
(Malar rash of lupus; macular or papular photodistributed eruption;
lupus hairs)