Page 777 - Clinical Immunology_ Principles and Practice ( PDFDrive )

748 Part SIX Systemic Immune Diseases

TABLE 55.5 Classification of
Localized Scleroderma
• Plaque types of morphea
• Circumscribed plaques
• Guttate
• Keloid/nodular
• Bullous
• Generalized morphea
• Three or more locations
• Pansclerotic morphea
• Linear scleroderma
• Frontoparietal linear morphea (en coup de sabre)
• Parry-Romberg syndrome (progressive hemifacial atrophy)
• Linear streaks on limbs or trunk
• Deep morphea

FIG 55.3 Skin Fibrosis in Systemic Sclerosis. Severe fibrosis
of the skin of the hands and forearms causing joint contractures
head leaving severe facial deformity. When this occurs, it is called and skin ulcerations in a woman with diffuse cutaneous systemic
en coupe de sabre. This should be distinguished from Perry- sclerosis.
Rhomberg syndrome, or facial hemiatrophy, which also affects
the face, usually unilaterally, with atrophy of skin rather than
fibrosis. Both en coupe de sabre and Perry-Rhomberg syndrome
can be associated with central nervous system (CNS) involvement,
presenting as headaches, visual disturbance, or a seizure disorder.
Uncommon forms of localized scleroderma include pansclerotic
morphea, keloid morphea, deep morphea presenting as a fasciitis,
and bullous morphea with blistering. Morphea lesions can be
seen in patients with SSc, and often lichen sclerosus is seen in
association with morphea.
Symptoms
Characteristically, the earliest symptoms of SSc are nonspecific
and include fatigue, musculoskeletal distress (stiffness or pain),
and feeling ill. Cold sensitivity and Raynaud phenomenon occur
early in the disease and may be the only clinical clue to the
presence of active disease. Symptoms of esophageal dysfunc-
tion with dysphagia, heartburn, and periodic GI reflux are FIG 55.4 Pigmentation Changes in the Skin. Vitiligo (salt-and-
common problems, which, along with Raynaud phenomenon, pepper) appearance of the involved skin in a patient of African
can precede other manifestations of SSc by years. Visible capillary descent with diffuse cutaneous systemic sclerosis.
abnormalities easily seen at the nailfold (dilatation and loss or
dropout of capillaries) are almost a universal finding in SSc.
These nailfold capillary changes provide a clinical clue to presence Soft tissue swelling, intense pruritus and burning, and nonpitting
of the underlying vascular disease in that changes occur early edema are signs of the early inflammatory or “edematous” phase
in the disease course and progress in association with other of the diffuse cutaneous form of the disease. The skin of the
systemic involvement. An individual presenting with Raynaud fingers, hands, distal limbs, and face are usually affected first
phenomenon, abnormal nailfold capillaries, and the presence and more severely compared with other body areas (Fig. 55.3).
of an SSc-specific autoantibody can be suspected of having SSc Carpal tunnel syndrome can be present as a consequence of soft
even before other more obvious manifestations are noted. 13 tissue inflammation around hands and wrists. Patients may note
skin hyperpigmentation (patches or generalized tanning). Other
CLINICaL PEarLS early skin changes include vitiligo-like hypopigmentation (“salt
Clinical Features of Early Systemic Sclerosis and pepper” appearance), often on the chest or the back (Fig.
55.4). Escalating musculoskeletal symptoms are common and
• Definite Raynaud phenomenon are associated with muscle weakness and decreased joint mobility.
• Gastroesophageal reflux with heartburn Significant finger and hand/wrist disease limits the patient from
• Swelling of the fingers and hands doing simple chores or performing self-care.
• Musculoskeletal pain and stiffness The early edematous phase of dcSSc has prominent inflam-
• Dilated nailfold capillaries
• Hyper- or hypopigmentary changes of skin matory features, with significant skin edema and erythema, and
is associated with inflammatory cell infiltration in the dermis.
After a period of weeks to months, the inflammatory phase evolves
Diffuse SSc into the “fibrotic” phase as increased collagen and extracellular
In general, patients with dcSSc have a short interval between material is deposited in skin, causing thickening and loss of
the onset of Raynaud phenomenon and other signs and symptoms. flexibility. The fibrotic process starts in the dermis and is associated