Skin and Rheumatic Disease

Eun-So Lee

doi:10.4078/jrd.2013.20.4.209

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Lee: Skin and Rheumatic Disease

Review Article

J Rheum Dis 2013;20(4):209-217.

Published online: 31 August 2013

DOI: https://doi.org/10.4078/jrd.2013.20.4.209

Skin and Rheumatic Disease

Eun-So Lee

Department of Dermatology, Ajou University School of Medicine, Suwon, Korea

Corresponding to : Eun-So Lee, Department of Dermatology, Ajou University School of Medicine, San 5, Wonchon-dong, Yeongtong-gu, Suwon 443-721, Korea. E-mail : esl@ajou.ac.kr

Received 15 June 201327 July 2013 Accepted 29 July 2013

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Many rheumatic diseases may involve any organ system of the body including the skin. Proper understanding of rheumatic skin disease is necessary for making a diagnosis. In addition, there are important relationships existing between the cutaneous and systemic manifestations of rheumatic diseases. In management of these diseases, the inter-disciplinary approach could generate better results. Among rheumatic diseases, the cutaneous manifestations of three major rheumatic diseases such as lupus erythematosus (LE), dermatomyositis (DM)/polymyositis and scleroderma/systemic sclerosis (SSc) will all be reviewed.

Keywords: Rheumatic disease, Skin manifestsions, Lupus erythematosus, Dermatomyositis, Scleroderma

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Table 1.

The modified Gilliam classification of skin lesions associated with LE (3)

LE-specific skin disease (Cutaneous LE, CLE)

LE-nonspecific skin disease

A. Acute cutaneous LE (ACLE)
1. Localized ACLE (malar rash; butterfly rash)
2. Generalized ACLE (lupus maculopapular lupus rash, SLE rash, rash, photosensitive lupus dermatitis)
B. Subacute cutaneous LE (SCLE)
1. Annular SCLE (syn. lupus marginatus, symmetric erythema centrifugum, autoimmune annular erythema, lupus erythematosus gyratus repens)
2. Papulosquamous SCLE (syn. disseminated DLE, subacute disseminated LE, superficial disseminated LE, psoriasiform LE, pityriasiform LE, and maculopapular photosensitive LE)
C. Chronic cutaneous LE (CCLE)
1. Classic discoid LE (DLE)
2. Localized DLE
3. Generalized DLE
4. Hypertrophic/verrucous DLE
5. Lupus profundus/lupus panniculitis
6. Mucosal DLE
7. Oral DLE
8. Conjunctival DLE
9. Lupus tumidus (urticarial plaque of LE)
10. Chilblain LE (chilblain lupus)
11. Lichenoid DLE (LE/lichen planus overlap, lupus planus)

A. Cutaneous vascular disease
1. Vasculitis
a. Leukocytoclastic
(1) Palpable purpura
(2) Urticarial vasculitis
b. Periarteritis nodosa-like cutaneous lesions
2. Vasculopathy
a. Degos disease-like lesions
b. Secondary atrophie blanche (syn. livedoid vasculitis, livedo vasculitis)
3. Periungual telangiectasia
4. Livedo reticularis
5. Thrombophlebitis
6. Raynaud phenomenon
7. Erythromelalgia (erythermalgia)
B. Nonscarring alopecia
1. “Lupus hair”
2. Telogen effluvium
3. Alopecia areata
C. Sclerodactyly
D. Rheumatoid nodules
E. Calcinosis cutis
F. LE-nonspecific bullous lesions
G. Urticaria
H. Papulonodular mucinosis
I. Cutis laxa/anetoderma
J. Acanthosis nigricans (type B insulin resistance)
K. Erythema multiforme
L. Leg ulcers
M. Lichen planus

Table 2.

Clinical and immunologic criteria used in the SLICC classification system (21)

Clinical criteria

1. Acute cutaneous lupus, including:

Lupus malar rash (do not count if malar discoid)

Bullous lupus

Toxic epidermal necrolysis variant of SLE

Maculopapular lupus rash

Photosensitive lupus rash

in the absence of dermatomyositis

OR subacute cutaneous lupus (nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias)

2. Chronic cutaneous lupus, including:

Classic discoid rash

Localized (above the neck)

Generalized (above and below the neck)

Hypertrophic (verrucous) lupus

Lupus panniculitis (profundus)

Mucosal lupus

Lupus erythematosus tumidus

Chillblains lupus

Discoid lupus/lichen planus overlap

3. Oral ulcers

Palate

Buccal

Tongue

OR nasal ulcers

in the absence of other causes, such as vasculitis, Behç et's disease, infection (herpesvirus), inflammatory bowel disease, reactive arthritis, and acidic foods

4. Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs) in the absence of other causes such as alopecia areata, drugs, iron deficiency, and androgenic alopecia

5. Synovitis involving 2 or more joints, characterized by swelling or effusion

OR tenderness in 2 or more joints and at least 30 minutes of morning stiffness

6. Serositis

Typical pleurisy for more than 1 day

OR pleural effusions

OR pleural rub

Typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 day

OR pericardial effusion

OR pericardial rub

OR pericarditis by electrocardiography

in the absence of other causes, such as infection, uremia, and Dressler's pericarditis

7. Renal

Urine protein– to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours

OR red blood cell casts

8. Neurologic

Seizures

Psychosis

Mononeuritis multiplex

in the absence of other known causes such as primary vasculitis

Myelitis

Peripheral or cranial neuropathy

in the absence of other known causes such as primary vasculitis, infection, and diabetes mellitus

Acute confusional state

in the absence of other causes, including toxic/metabolic, uremia, drugs

9. Hemolytic anemia

10. Leukopenia (4,000/mm³ at least once)

in the absence of other known causes such as Felty's syndrome, drugs, and portal hypertension

Lymphopenia (1,000/mm³ at least once)

in the absence of other known causes such as corticosteroids, drugs, and infection

11. Thrombocytopenia (100,000/mm³) at least once

in the absence of other known causes such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura

Immunologic criteria

1. ANA level above laboratory reference range

2. Anti-dsDNA antibody level above laboratory reference range (or 2-fold the reference range if tested by ELISA)

3. Anti-Sm: presence of antibody to Sm nuclear antigen

4. Antiphospholipid antibody positivity as determined by any of the following:

Positive test result for lupus anticoagulant

False-positive test result for rapid plasma reagin

Medium- or high-titer anticardiolipin antibody level (IgA, IgG, or IgM)

Positive test result for anti–2-glycoprotein I (IgA, IgG, or IgM)

5. Low complement

Low C3

Low C4

Low CH50

6. Direct Coombs’ test in the absence of hemolytic anemia

Criteria are cumulative and need not be present concurrently. SLICC: Systemic Lupus International Collaborating Clinics, SLE: systemic lupus erythematosus, ANA: antinuclear antibody, anti-dsDNA: anti-double-stranded DNA, ELISA: enzyme-linked immunosorbent assay.

Table 3.

Classification of cutaneous sclerosis (scleroderma) (25)

1. Systemic sclerosis (SSc)

SSc with diffuse cutaneous scleroderma (syn. Systemic scleroderma, proximal scleroderma)

SSc with limited cutaneous scleroderma (syn. CREST syndrome [calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactly, telangiectasia]; acrosclerosis)

2. Localized cutaneous sclerosis

(syn. Localized scleroderma, circumscribed scleroderma)

Morphea (localized and generalized forms)

Plaque, subcutaneous/profunda, guttate, keloidal, superficial, generalized

Linear scleroderma

Craniofacial

En coup de sabre

Facial hemiatrophy (syn. Parry– Romberg syndrome)

Extremities

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