Skin involvement in rheumatic diseases

Dr. Enida Xhaferi1, Prof.asc Teuta Backa Cico2, Dr Fatbardha Lamaj3, Dr. Shkelzen Recica4, Dr.
Robert Qirko5, Dr. Sabri Hoxha6.
1
University of Medicine/Faculty of Medical Technical Sciences, Tirana, Albania
2
University Hospital Center “Mother Theresa”, Rheumatology Clinic, Tirana, Albania
3
Intermedica Laboratory, Tirana, Albania
4
Pathologic anatomy institute, University Clinical Center of Kosovo, Pristina, Kosovo
5
University Hospital "Queen Geraldine", Tirana, Albania
6
Albanian University Tirana, Albania
Skin lesions are observed in a variety of rheumatologic conditions and constitute the primary features in lupus
erythematosus, dermatomyositis and systemic sclerosis. Cutaneous involvement is also observed in systemic
vasculitides, rheumatoid arthritis, Sjögren syndrome, psoriatic arthritis, relapsing polychondritis and systemic-
onset juvenile rheumatoid arthritis.

The clinician must be able to recognize and discern the most common cutaneous lesions and patterns encountered
in patients with rheumatic diseases (like macule, papule, nodule, plaque, purpura, petechia, pustul, squam, erosion,
erythema, onychodystrophy, onycholysis, urticaria, butterfly rash, Gottron papules and sign), because they
provide clues about the systemic involvement of the pathology, diagnosis, therapeutic approach and prognosis.
Skin biopsies are usually useful in determining the precise nature of the skin manifestation.

Below are presented briefly the major skin manifestations observed in lupus erythematosus, dermatomyositis,
scleroderma and rheumatoid arthritis.

Skin involvement occurs in 2/3 of patients with lupus erythematosus and the SLICC Classification 2012, contains
four dermatological criteria. Cutaneous manifestations are commonly divided into lupus lesions and non lupus
lesions and vascular ones. Patients with SLE and no signs of skin involvement, comprise the “lupus sine lupo”
condition. LE-specific skin manifestations are categorized into three major clinical subtypes according to their
disease acuity: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE),
and chronic lupus erythematosus (CLE).

ACLE lesions include - lupus malar rash (a slightly edematous erythema above the malar region), maculo -papular
rashes mainly on photo exposed areas, bullous lupus and toxic epidermal necrolysis (TEN). In SCLE the maculo-
papular lesions have annular or psoriasis like configuration with erythema squamous borders and presence of
vesiculae and crusts. CLE includes discoid lupus erythematosus (DLE), lupus timidus, hypertrophic DLE,
chilblain lupus and lupus panniculitis. Patients with ACLE will develop lupus in more than 90% of cases.

Vascular lesions comprise livedo reticularis, erythromelalgia, leg ulcers, urticaria, angioedema, splinter
hemorrhages, skin necrosis, while non vascular lesions include non- scarring alopecia, papular mucinosis,
calcinosis, aseptic pustulosis, anetoderma.

Skin lesions precede muscular manifestations in half dermatomyositis patients. Characteristic findings include the
heliotrope rash (a pinkish erythema of the periorbital area), Gottron sign (erythematous squamous plaques located
in the dorsum and lateral part of interphalangeal and metacarpophalangeal joints), Gottron papules; rashes can
also involve the malar area of the face, the posterior side of the neck and shoulders (the “shawl sign”), and the
scalp. Patients with anti- synthetase syndrome have “mechanic hands”. Other lesions that could be observed in
patients with dermatomyositis include moderate Raynaud phenomenon, thrombosis and vasculitis.

The two major scleroderma subtypes, limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc), are
distinguished partially by the severity and extent of cutaneous involvement. Some of the most common skin
disorders observed in patients with SSc are symmetric cutaneous sclerosis, Raynaud phenomenon, finger swelling,
sclerodactyly, digital pits and ulcers, calcinosis cutis, dilated or atrophic nail-fold capillaries, and
hyperpigmentation. Skin sclerosis is due to the excessive deposition of interstitial collagen and subsequent tissue
fibrosis

Dermatologic manifestations in rheumatoid arthritis include palisading granulomas (rheumatoid nodules,

palisaded neutrophilic granulomatous dermatitis, pseudorheumatoid nodules), rheumatoid vasculitis and
neutrophilic dermatoses (pyoderma gangrenosum, rheumatoid neutrophilic dermatitis). Some patients with
rheumatoid arthritis develop cutaneous drug toxicity during treatment

Conclusion. Skin manifestations are observed frequently among patients with rheumatic disorders. Their correct
identification will help diagnose the specific pathology. Consultation with a dermatologist, may be warranted for
particular conditions that require further management.

Key words: Rheumatic diseases, lupus erythematous, dermatology.