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Live Blog: What are the Basics of Connective Tissue Disease in The Skin? – Richard D. Sontheimer, MD

In this live blog from the 12th Annual SDPA conference in Indianapolis, Dr. Richard Sontheimer lectured on “What are the Basics of Connective Tissue Disease in The Skin?” Here are some of the highlights. 

Richard D. Sontheimer, MD, started his lecture by laying out some of the learning objectives. His goal was to review the presenting visible manifestations of lupus erythematosus (LE), dermatomyositis (DM), and scleroderma, with an emphasis on the keys of early recognition of the underlying systemic manifestations of these complex autoimmune disorders. 

Auto-immune connective tissue diseases are characterized by multi-system clinical abnormalities resulting from disordered immune regulation and have variable clinical expressions, including polygenetic predisposition and environmental precipitating factors such as infection or UV light exposure.

Skin involvement is second most prevalent clinical manifestation of systemic lupus erythematosus (SLE) and second most common presenting clinical manifestation.

Dr. Sontheimer described a comprehensive list of skin lesions associated with LE. Some LE specific indicators include: localized ACLE, generalized ACLE, annular papulosquamous, hypertrophic DLE, LE profundus, Mucosal DLE, and chilblains.

While some LE-non-specific indicators include: cutaneous vascular disease, vasculitis, alopecia (nonscarring), alopecia aerata, bullous pemphigoid, urticaria, nail changes (red lunulae, dyschromasia), leg ulcers, and lichen planus.

Dr. Sontheimer stressed that board-certified dermatopathologists are typically the providers who are best prepared to accurately interpret inflammatory skin disease biopsies. And he offered the following differential Dx for times a patient presents a red butterfly pattern on the face:

• ACLE/SCLE
• Acne rosacea
• Contact dermatitis (photo, airborne)
• Photo-sensitive drug eruptions
• Seborrheic dermatitis

“When in doubt, biopsy!” said Sontheimer.

Skin disorders displaying the lichenoid tissue reaction or interface dermatitis include: dermatomyositis, erythema multiforme, fixed drug eruptions, Graft-versus-host disease, lichen planus, lichenoid drug eruptions, lupus erythematosus, and viral exanthems.

Dr. Sontheimer provided the following snapshot of subacute cutaneous lupus erythematosus (SCLE):
• Non-scarring, highly photosensitive
• ANA (+); anti-Ro/SS-A & anti-La/SS-B
• Genetic associations
• Associations: SSj, neonatal LE
• Can be drug-induced
• Approximate 10% – severe SLE
• ~75% respond to anti-malarials

Dr. Sontheimer offered the following definition of dermatomyositis (DM): A member of the idiopathic inflammatory myopathies that produces unique patterns of inflammatory injury to skin and/or proximal skeletal muscles.

Finally, Dr. Sontheimer covered some basics of management of DM, both adult-onset DM and juvenile-onset DM.

Adult-onset DM 
Screen for internal malignancy (greatest risk [25%] beyond 50 years of age)
      – Sex- and age-specific approach

Screen for interstitial lung disease
      – Baseline – pulmonary function tests with diffusion capacity (PFT) (25% abnormalities)
      – Persistent dry cough, dyspnea – repeat PFT, consider referral to pulmonary medicine,            rheumatology management of DM

 

Juvenile-onset DM
Monitor for vasculopathic tissue damage
      – Eye, GI tract
Monitor for calcinosis cutis
No significant risk for interstitial lung disease or internal malignancy

 

Image: Andreas Nilsson