CHAPTER 10

SKIN MANIFESTATIONS OF THE COLLAGEN DISEASE

（膠原病の皮膚病変）

 

I. SLE（全身性紅斑性狼瘡）

A.   Epidemiology

Age: 20 to 40 years

Sex: Male << female

B.   Physical Examination

Skin Lesions

Erythematous, confluent, macular butterfly plaque (malar area) with scaling, erosions, crusts

Discoid papules or plaques as in DLE (face, scalp, hands)

Palpable purpura (vasculitis), urticarial vasculitis, atrophie blanche skin ulcer

Hair

Diffuse alopecia is common

Patchy alopecia is associated with DLE lesions

Mucous Membranes

Ulcers on palate, buccal mucosa and gum

Systemic Multisystem Involvement

Arthralgia or arthritis, renal disease, pericarditis, pneumonitis, gastrointestinal disease, hepatic involvement, lymphadenopathy, peripheral neuropathy, CNS disease

C.   Laboratory Examination

Positive ANA (>95%)

Peripheral pattern of nuclear fluorescence and anti-ds-DNA are specific for SLE.

Reduced level of complement

Leukopenia, anemia, elevated ESR

 

II. Chronic Discoid Lupus Erythematosus (DLE)（円板状狼瘡）

A.   Epidemiology

Age: 20 to 45 years

Sex Females > males

B.   Skin Lesions

Type: Sharply marginated scaly papules and plaques.  Atrophic, depressed plaques with slightly raised border, scaling and follicular plugging.  Heals leaving scars.

Color: red, pink or hypopigmented

Shape: Round, oval, polycyclic

C.   Sites of Predilection

Face, scalp (scarring alopecia), dorsa of hands

D.   Dermatopathology

Hyperkeratosis, epidermal atrophy, follicular plugging, liquefaction degeneration of the basal cell layer. 

In the dermis, there is perivascular and periappendegeal lympho-histiocytic cell infiltration.  Strong PAS reaction on the thick basement membrane zone.

E.   Immunofluorescence

Granular deposits of IgG (or IgM) at the dermo-epidermal junction in the active lesions.

F.    Laboratory Examination

Usually no autoantibody

G.   Course and Prognosis

Rarely ( < 5%) develop SLE

H.   Treatment

Topical sunscreen, topical corticosteroids, intralesional steroids

 

III. Subacute Cutaneous Lupus Erythematosus(SCLE)（亜急性皮膚型狼瘡）

A.   Epidemiology

Age: Young and middle aged

Sex: Females > males

B.   Skin Lesions

Psoriasiform papulosquamous eruptions

Annular, arciform, iris-shaped (erythema multiforme-like) plaques

No scarring, atrophy, or follicular pluggings

C.   Dermatopathology

Liquefaction degeneration of the basal layer and edema of upper dermis

IMMUNOFLUORESCENCE

Positive IgG in the dermo-epidermal junction

D.   Laboratory Examination

Minimal abnormality

E.   Treatment

Sunscreen and topical corticosteroid

Low dose of systemic corticosteroid

 

IV. Dermatomyositis（皮膚筋炎）

A.   Epidemiology

Age: Juvenile and over 40 years

Cases over 55 years often associated with malignancy

B.   Skin Lesions

Periorbital heliotrope (reddish-purple) flush with edema

Dermatitis with erythema and scaling on the face, chest, upper back and shoulders

Flat-topped violaceous papules (Gottron's sign) on dorsa of knuckles

Periungual erythema, telangiectasia, thrombosis of capillary loops

Calcification of subcutaneous tissues common in juvenile DM

C.   Muscle Involvement

Muscle weakness affecting proximal girdle muscles

Difficulty to rise from sitting or supine position

Occasional involvement of facial and pharyngeal muscles may result in dysphagia

D.   Laboratory Examination

Blood Chemistry: Elevation of CPK, aldolase, GOT, LDH

Urine Chemistry: Elevated 24-hour creatine excretion

Electromyography: Increased irritability, spontaneous fibrillations, pseudomyotonic discharges, positive sharp waves

EKG: Signs of myocarditis, atrial and ventricular irritability, A-V block

Chest X-ray: Interstitial pneumonitis

E.   Dermatopathology

Flattening of epidermis, hydropic degeneration of basal cell layer, edema of upper dermis

PAS positive fibrinoid deposits at dermo-epidermal junction

F.    Muscle Biopsy

Degeneration, loss of striation, lymphocytic infiltration

G.   Treatment

Systemic glucocorticoids

Immunosuppressant

 

V. Systemic sclerosis（全身性硬化症）

A.   Epidemiology

Age: 30 to 50 years

Sex: Females>males

B.   Skin Lesions

Hands and Feet: Raynaud's phenomenon, ulceration of fingertips, sclerodactyly with tapering fingers, flexion contractures, calcification

Face: masklike face, thin lips, microostia,radial perioral furrow, thin sharp nose

Hair: Loss of hair and sweat glands on distal extremities

Nail: Periungual telangiectasia, claw-like nails

C.   Systemic Involvement

Lung: Interstitial pneumonitis, pulmonary fibrosis

GI Tract: Sclerosis, esophageal dilatation

Kidney: Nephrosclerosis

D.   Dermatopathology

Disappearance of rete ridges and melanin hyperpigmentation in the basal layer

Eosinophilic, homogeneous collagen bundles

Atrophic dermal appendages and sweat glands in the upper dermis

Thickening and hyalinization of vessel wall, and narrow lumen

E.   Laboratory Examination

Positive ANA, Scl-70, and anti-centromere antibody

F.    Treatment

Symptomatic treatment with vasodilators (prostaglandins etc)

 

 

 

VI. Morphea（斑状強皮症）

A.     Epidemiology

Usually occurs in children

B.     Skin Lesions

1.     Plaque type: round or oval shape, smooth surface, ivory color, lilac ring

2.     Linear type: on anterior scalp and extremities, induration of skin, atrophy of subcutaneous fat

3.     Segmental type: occurs in one side of the face

4.     Guttate type: small and superficial lesions

5.     Subcutaneous type: skin feels thickened and bound to the underlying fascia and muscle

6.     Generalized type: extensive involvement

C.     Dermatopathology

1.     Dermis is initially edematous

2.     Later dermal collagen becomes eosinophilic and homogeneous

3.     Perivascular lymphocytic infiltration

D.     Treatment

1.     Symptomatic treatment