Review the following visual approach to rashes in systemic rheumatic diseases.

Important Note: Always rule out potential infectious or drug etiologies before assuming the rash is systemic in origin.

Left photo shows “butterfly”/malar rash (pink/red scaly rash over the cheeks and nasal bridge). Right photo of hands shows rash in between the knuckles.

Photo showing young girl with the characteristic malar rash seen in lupus. The rash tends to spare the eyelids and nasolabial folds.

Rashes occur in parallel with other organ disease activity in systemic lupus erythematosus

Clinical Description

Flat or raised pink/red scaly rash over the cheeks and nasal bridge – “butterfly”/malar rash; can also be generalized

  • Spares nasolabial folds
  • Non-scarring
  • Rash in between the knuckles

Brief  Histopathologic Description

  • Interface dermatitis with disrupted dermo-epidermal junction

Differential Diagnosis

  • Rosacea: presence of red papules and telangiectasia; triggered by spicy foods
  • Seborrheic dermatitis: pink-red, scaly and not itchy rash involving primarily the scalp
  • Dermatomyositis: does not spare nasolabial folds; rash overlies the knuckles
  • Cutaneous lymphoma
  • ANA, dsDNA, anti-Smith, complement levels
  • Urinalysis with sediment and protein creatinine ratio
  • Skin biopsy may be required.

Left photo shows well demarcated coin shaped, red, and scaly papules or plaques on a male patient's nose and face. The right photo shows the atrophic (central) scarring and dyspigmentation (borders) resulting from such a rash.

Clinical Description

  • Well demarcated coin shaped, red, and scaly papules or plaques in sun–exposed areas
  • Result in atrophic (central) scarring and dyspigmentation (borders)

Brief Histopathologic Description

  • Interface dermatitis with disrupted dermo-epidermal junction

Differential Diagnosis

  • Discoid lupus: 28 % with generalized rash can develop SLE
  • Discoid mimics:
    • Actinic keratosis
    • Cutaneous tuberculosis
    • Subcutaneous sarcoidosis

Recommended Workup

  • ANA
  • Skin biopsy may be required.
Photo showing annular polycyclic plaques with a raised red scaly border and central clearing

Clinical Description

  • Annular polycyclic plaques with a raised red scaly border and central clearing
  • Can lead to post-inflammatory dyspigmentation
  • Non-scarring
  • Distribution usually photosensitive areas – shoulders, arms, upper torso, upper back

Brief Histopathologic Description

  • Interface dermatitis with disrupted dermo-epidermal junction

Differential Diagnosis

  • SCLE occurs in 10-15% with SLE; 50% of SCLE progresses to SLE
  • Drug associations include but not limited to: tamoxifen, lansoprazole, statins, ACEi, TNFi, anti-epileptics, PPIs, naproxen, etc.
  • Tinea corporis
  • Erythema annulare centrifugum
  • Psoriasis
  • Lichen planus
  • Eczema

Recommend Workup

  • ANA, SSA autoantibodies
Left photo shows a patient with a heliotrope, facial rash. Top right photo shows periungual erythema. Bottom right photo shows Gottron’s papules on a patient's hands.

Photo of child with the characteristic facial rash of dermatomyositis involving forehead, eyelids (heliotrope rash) with periorbital edema, and malar region, and an erythematous rash over the extensor surfaces of the elbows.

  • Heliotrope, facial rash, and Gottron’s papules
  • Gottron sign asymmetrical violaceous macules can also be observed

Clinical Description

  • Flat violaceous scaly rash on the face
  • Involves nasolabial folds and eyelids
  • Symmetrical erythematosus papular lesions over the metacarpophalangeal and interphalangeal joints
  • Periungueal erythema

Brief Histopathologic Description

  • Interface dermatitis – unable to differentiate from skin histopathology of lupus

Differential Diagnosis

A skin biopsy cannot help distinguish between SLE and DM.

  • Creatinine kinase and aldolase levels, ANA, myositis panel
  • Classic presentation may not need a skin biopsy.
  • Screen for an underlying malignancy!

Left-most photo shows a patient with telangectasias on the cheeks, nose, and lips. Top right photo shows “salt-pepper lesions (hyperpigmentation and hypopigmentation). Bottom right photo shows finger “sclerodactyly“

Clinical Description

  • Limited or diffuse skin thickening and tightness (face, torso, extremities, finger “sclerodactyly“)
  • Coexistence of hyperpigmentation and hypopigmentation (“salt-pepper lesions”)
  • Lesions sparing the perifollicular area
  • Telangiectasias on the cheeks, nose, and lips

Brief Histopathologic Description

  • Thickened collagen in the dermis and dermal fibrosis

Differential Diagnosis

  • Scleroderma mimics (scleromyxedema, scleredema, eosinophilic fascitis)
  • Autoantibody profile (Scl-70 [or anti-topoisomerase I], anti-centromere, RNA polymerase III)
  • Screen for lung or cardiac involvement

Photo of a patient's arm and left torso showing salmon colored edematous plaques and rash

Shows the rash of systemic-onset juvenile rheumatoid arthritis, which is classically salmon-colored, pink, or erythematosus. The rash may be macular or papular with areas of confluence. The rash varies markedly in its distribution over the trunk and extremities. It is usually evanescent and often accompanies episodes of fever.

Clinical Description

  • Salmon colored edematous plaques or salmon colored edematous rash
  • Evanescent rash coinciding with fever spikes
  • Non-pruriginous

Brief Histopathologic Description

  • Neutrophilic inflammation in the dermis

Differential Diagnosis

  • Urticaria: pruriginous transient lesions
  • Urticarial vasculitis:
    • Hives usually persist for longer than 48 hours.
    • Can be associated with low complement levels

Recommended Workup

  • C-reactive protein, sedimentation rate, ferritin levels

 

Photo showing numerous clusters of small and large red-purple non-blanchable discoloration on a patient's legs

Many conditions can be associated with finding of LCV on histopathology.

  • Different infections including but not limited to: streptococcal upper respiratory tract infection, mycobacterium, staphylococcus aureus, chlamydia, neisseria, and HIV, hepatitis B, hepatitis C, and syphilis.
  • Systemic diseases including: any small vessel vasculitis, ANCA-associated vasculitis, cryoglobulinemic vasculitis, various connective tissue diseases, erythema elevatum diutinum
  • Malignancies including but not limited to: lymphomas, leukemias, lung cancer
  • Various drug associations including but not limited to: beta-lactams, erythromycin, clindamycin, vancomycin, sulfonamides, furosemide, allopurinol, NSAIDs, amiodarone, gold, thiazides, phenytoin, beta-blockers, TNF-alpha inhibitors, selective serotonin reuptake inhibitors, metformin, warfarin, valproic acid

Clinical Description

  • Clusters of small and large red-purple non-blanchable discoloration in the lower extremities

Brief Histopathologic Description

  • Neutrophilic inflammation around the blood vessels leads to vessel wall inflammation. This causes vascular injury and extravasation of red blood cells into the dermis, hence the purpuric appearance.

Differential Diagnosis

  • Leukocytoclastic vasculitis: petechia and palpable purpura (dependent areas)
  • Small vessel vasculitis
  • ANCA-associated vasculitis
  • Cryoglobulinemic vasculitis
  • Associated with connective tissue diseases
  • Urticarial vasculitis syndrome
  • Hypersensitivity vasculitis
  • Scurvy (view images)
  • Meningococcemia
  • IgA vasculitis (Henoch-Schonlein purpura) (view image)
  • Disseminated intravascular coagulation
  • Thrombotic thrombocytopenic purpura
  • Emboli: cardiac myxoma, cholesterol emboli, septic and infectious emboli
  • Pigmented purpuric dermatosis
  • Schamberg disease
  • Gardener Diamond syndrome

Recommended Workup

  • ANA, ANCA with PR3/MPO specificity, cryoglobulin, complement level, IgA
  • Urinalysis with sediment
  • Skin biopsy is required.

Photo showing several tender red subcutaneous nodules on the legs

Associated Systemic Conditions

Of the rheumatic diseases, sarcoidosis and Behcet’s are the most commonly associated

  • Sarcoidosis
  • Behcet’s syndrome
  • Inflammatory bowel disease
  • Strep-related illness
  • TB

Clinical Description

  • Several tender red subcutaneous nodules on the legs

Brief Histopathologic Description

  • Inflammation in the subcutaneous fat (“septal panniculitis”) with giant cells

Differential Diagnosis

  • In contrast to lupus panniculitis (lobular), E. nodosum does not cause scarring or involve the face
  • Eosinophilic fasciitis
  • Insect bites

Recommended Workup

  • ANA, extractable nuclear antigens, rheumatoid factor, vitamin D 1,25, angiotensin converting enzyme level
  • Chest x-ray
  • Pathergy test, if history of recurrent oral or genital ulcerations

We Want Your Input

Find what you needed? We would love your feedback so that we can ensure the site meets the needs of primary care providers.