Autoimmune Disease

Rheumatoid Arthritis

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Rheumatoid Nodule - Fitzpatrick's Dermatology
Rheumatoid arthritis is an autoimmune, systemic inflammatory disease characterized by a debilitating polyarthritis.  There are many manifestations besides joint dysfunction, including internal organ involvement, and cutaneous involvement.  Some of the skin conditions associated with RA include rheumatoid nodules, pyoderma gangrenosum, granulomatous dermatitis, and vasculitis.  The most common finding is the rheumatoid nodule.  The nodules though benign can lead to complications, such as infection, ulceration, and fistula. The following list and images demonstrate many of the common skin conditions associated with rheumatoid arthritis:

Palisading granulomas
--Rheumatoid nodule
--Superficial ulcerating rheumatoid necrobiosis
Vascular reactions
--Vasculitis
--Bywater's lesions (small ulcers or petechiae  on the fingers and digital pulp papules)
Neutrophilic dermatoses
--Pyoderma gangrenosum
--Rheumatoid neutrophilic dermatosis (Rare, but can occur in severe RA.  Erythematous, urticaria-like plaques and papules that are sharply marginated.)
Sweet's syndrome

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Bywaters Lesions - FItzpatrick's Dermatology
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Rheumatoid Neutrophilic Dermatosis - Fitzpatrick's Dermatology

Systemic Lupus Erythematosus

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'Butterfly Rash' of SLE - Fitzpatrick's
Systemic lupus erythematosus (SLE) is an autoimmune disease in which cells and organs are damaged by tissue-binding autoantibodies and immune complexes.  This disease affects women much more than men, approximately in a 9:1 female to male ratio.  Lupus is sometimes referred to as a disease spectrum, as patients can have varying levels of disease and systemic involvement. 

SLE is a complex disease, but skin conditions can be a key concern, and useful for diagnosis and monitoring.  SLE skin lesions are typically classified into the three main categories listed below.  It is important to note that a patient may not fall completely within one category, and it is not unusual to see lesions of multiple categories on one patient. 





Acute Cutaneous Lupus Erythematosus
  • Known for the "butterfly rash" or "malar rash" of the face.  Symmetric erythema and edema over the malar eminences and nose, while sparing the nasolabial folds.  See the picture on the left for an example.
  • May also present with generalized lesions on the hands and arms (extensor side) that spare the knuckles
  • ACLE is usually exacerbated by exposure to UV light
Subacute Cutaneous Lupus Erythematosus
  • Presents as widespread erythematous macules and papules, which then evolve to become hyperkeratotic plaques
  • Lesions are characteristically photosensitive, and occur in sun-exposed areas
Chronic Cutaneous Lupus Erythematosus (Classic Discoid Lupus Erythematosus)
  • Begin as rep/purple macules, papules, or small plaques
  • Develop a hyperkeratotic surface, and are well demarcated, coin shaped, erythematous plaques
  • Commonly found on the face, scalp, ears, and neck

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Subacute Cutaneous Lupus Erythematosus - Fitzpatrick's Dermatology
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Classic Discoid Lupus (CCLE) - Fitzpatrick's Dermatology

Scleroderma

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Localized Cutaneous Sclerosis
Scleroderma is a multisystem autoimmune rheumatic disease associated with a high mortality rate.  The major components of the disease include inflammation, vasculature, connective-tissue producing cells, and genetic factors.  Changes in the microvasculature leads to findings such as the Raynaud phenomenon (vasospasm of the extremities, leading to changes in circulation and color of the hands and feet).  Almost all patients with scleroderma have symptoms of the Raynaud phenomenon and skin sclerosis. 

Scleroderma is divided into two major categories.  The findings of each are listed below:

Localized Cutaneous Systemic Sclerosis
  • Long history of Raynaud phenomenon
  • Sclerosis of the skin spares proximal areas
  • Classic CREST findings:  Calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia
  • Visceral disease, including pulmonary hypertension
  • Pulmonary fibrosis and GI tract involvement
Diffuse cutaneous Systemic Sclerosis
  • Proximal skin sclerosis (no sparing of the trunk and upper arms)
  • Onset of Raynaud phenomenon is associated with the onset of skin sclerosis
  • First two years of disease has many prominent inflammatory features
  • Pulmonary fibrosis, cardiac involvement, and scleroderma renal crisis
  • Often Scl-70 or anti-RNAPIII test positive