Download MBBS Dermatology PPT 3 Cutaneous Manifestations of Connective Tissue Disease II Lecture Notes

Download MBBS (Bachelor of Medicine, Bachelor of Surgery) Dermatology PPT 3 Cutaneous Manifestations of Connective Tissue Disease II Lecture Notes

Cutaneous Manifestations of

Connective Tissue Disease - II

Connective tissue diseases

? SLE
? Dermatomyositis
? Systemic sclerosis (scleroderma)
? Rheumatoid arthritis
? Mixed CT disease
? others
? Spectrum ranging from benign cutaneous variants to severe, often

fatal, multisystem diseases

? Inflammation of the connective tissue - resulting in changes in skin,

joints, vasculature and other Organs

? Antibodies formed against cell components - Serological markers

Dermatomyositis

? Dermatomyositis (DM) - systemic autoimmune disease characterized

by inflammation and damage to the skin and muscle

? Interstitial lung disease (ILD) - 20%
? In adults - DM - heralds the diagnosis of a coexisting internal

malignancy in 10% to 20% of cases


? Characteristic autoantibodies
?Antisynthetase
?Anti?Mi-2
?Anti?transcriptional intermediary factor [TIF1]-gamma
?Anti?melanoma differentiation?associated gene 5 [MDA5]
?Anti?nuclear matrix protein 2 [NXP2]
?Anti?small ubiquitin-like modifier activating enzyme [SAE]
? May be useful in identifying clinical subsets


Violaceous patches and plaques

? Characteristic cutaneous feature
? violaceous patches and plaques, varying from a bright pink to a deep

violet color


The heliotrope sign

? Exemplies the pink to purple violet hue of the eruption
? color of the flower petals after which the sign named
? The eyelid eruption can be associated with periorbital edema


The V-neck sign

? Confluent violaceous erythema on the sun-exposed areas of the

lower anterior neck and anterior chest


? The skin changes - often distributed to typical regions on the body
? Trunk involvement is often seen on the posterior neck, upper back,

and shoulders, known as the shawl sign


Gottron papules

? The violaceous to pink papules over the IP and MCP joints


Gottron sign

? Symmetric macular violaceous erythema over the IP joints, olecranon

processes, patellae, and medial malleoli

? May be atrophy and poikiloderma in classic areas of Gottron sign


The Holster sign

? The violaceous erythema and poikiloderma on the lateral hips and

lateral thighs

? Often patterned as folliculocentric macules or subtle papules


"Mechanic's" hands

? Hyperkeratosis and fissuring along the medial thumb and lateral

second and third digits

? A cutaneous clue to the possible presence of ILD
Systemic sclerosis (Scleroderma)

? Multisystemic autoimmune disease - by vasculopathy, inflammation,

and fibrosis of the skin and many other organs due to extensive

activation of fibroblasts

? Raynaud phenomenon, circulating autoantibodies, and skin sclerosis

are almost always present

? Important for the early diagnosis

? An overlap syndrome, including mixed connective tissue disease ?

characterized by additional clinical features of other RHDs

? The highest case-specific mortality of any of the autoimmune

rheumatic diseases
? SSc usually starts with a Raynaud phenomenon - precede the disease

for many years

? The clinical manifestations - diverse with severe fibrosis of the skin

and all additional cutaneous manifestations

? E.g. hardening of the skin, development of contractures, digital

ulcerations and calcifications

? Multiple patterns of internal organ involvement

Raynaud's phenomenon

? Earliest symptom to appear in more than 90% of SSc patients
? Characterized by painful pallor/ischemia of single or several digits

followed by reactive hyperemia after reheating at the end of a RP

attack f/b in some cases cyanosis (triphasic RP)

? Worse in winter/by emotional stress



? Eventually leads to:
?Finger tip ulcers
?Loss of finger pulp
?Gangrene


? An increasing induration and skin thickening (sclerodactyly)
? Depending on the localization of skin thickening restricted mobility of

joints (dermatogenous contractures)



? Facial appearance - characterized by a radial furrowing around the

mouth, no expression, a stiff and mask-like facial appearance, and

sclerosis of the frenulum

? Besides cosmetic/aesthetic problems, this causes considerable

difficulties regarding eating and oral hygiene



Rheumatoid Arthritis

? Affects roughly 1% of the world population
? Skin findings quite varied, including papules, plaques, and nodules

with multiple histologic types, vasculitis/Bywaters lesions, pyoderma

gangrenosum/Felty ulcers

Rheumatoid Nodules and Nodulosis

? The usual location is over pressure points such as the olecranon, the

extensor surface of the forearms, and the Achilles tendon

? Benign, they can lead to complications, including ulceration, infection,

joint effusion (rheumatoid chyliform bursitis), and fistulas (fistulous

rheumatism)


? Accelerated nodulosis- Low-dose methotrexate, often used for the

treatment of RA, may precipitate erythema in and enlargement of

preexisting rheumatoid nodules


Rheumatoid neutrophilic dermatosis

? Rare cutaneous manifestation
? Lesions are usually chronic, erythematous, and urticaria-like plaques

and papules; sharply marginated