Skin disorders are observed in a variety of rheumatologic conditions and constitute the primary features in lupus erythematosus, dermatomyositis and systemic sclerosis. Skin involvement is also observed in systemic vasculitides, rheumatoid arthritis, Sjögren syndrome, psoriatic arthritis, systemic-onset juvenile rheumatoid arthritis, and relapsing polychondritis. It is important for the clinician to recognize and discern the most common cutaneal lesions and patterns encountered in patients with rheumatic diseases (like makule, papule, nodul, plaque, purpura, petechia, pustul, squam, erosion, erythema, onychodystrophy, onycholysis, urticaria, butterfly rash, Gottron papules and sign etc) because they provide clues regarding the systemic involvement of the pathology, diagnosis, therapeutic approach and prognosis. Skin biopsies are usually useful in determining the precise nature of the skin disorder. Below are presented briefly the major skin manifestations observed in lupus erythematosus, dermatomiositis, scleroderma and rheumatoid arthritis

1. Dr. Enida Xhaferi
University of Medicine,Tirana
National Conference of Medical Sciences 5,Tirana

2.  Skin is the largest organ of the human body. It serves as an
external protective barrier and continues with the mucous
membranes of the mouth, nose, eyes, genital organs and anal
area. Surface varies between 1,5-2 m2 and the organ contains
approximately 1500 sensitive receptors and more than 3 million
cells.
 Skin derivatives/appendages include:
 Sweat glands (eccrine/apocrine)
 Sebaceous glands (part of the pilosebaceous units and
independent ones)
 Mammary glands
 Hair follicles
 Hair
 Nails
Dr. Enida Xhaferi

3. .Dr. Enida Xhaferi
Dr. Gunther von Hagens skin anatomy

4.  Sensory function. Receptors (mechanoreceptors, nociceptors, thermoreceptors,
chemoreceptors) activated by different stimuli provide information about heat, cold,
pressure, touch, pain; data is carried to the central nervous system through afferent
fibers.
 Protective function. Skin offers support and helps protect the body from mechanical
injury (dermis and especially hypodermis serve as shock absorbers for pressure and
blows), toxic/chemical and thermal threats; ultraviolet rays and radiation (melanocytes
secrete melanin). Skin also helps defend against microorganisms/pathogens (through
acid ph, different antimicrobial substances present in the skin; the fingers skin has
distinctive bactericidal properties).
 Thermoregulatory function. Plays an important role in the process of heat transfer
(through convection, conduction, radiation, evaporation). In cold weather skin blood
vessels contract and the skin surface shrinks; shivering creates heat from the
involuntary movement of muscle. In warm weather skin capillaries dilate and skin
surface enlarges, increasing thus the quantity of the transferred heat. Sweating increases
body heat loss through evaporation.
 Participates in the metabolism of water, proteins, mineral salts, carbohydrates.
Vitamin D, keratin, melanin, some enzymes and the secretion of the oil and sweat glands
are formed in the skin.
.Dr. Enida Xhaferi

5.  Penetrability. Alcohol, iodine, sexual hormones, some
vitamins (A, E, D, K), suprarenal hormones and
liposoluble substances can penetrate through the skin
together with oxygen, carbon dioxide and toxic
/poisonous gases.
 Regeneration. Skin has good regenerative properties
and can be transplanted easily from one area to
another of the same person or in different individuals.
.Dr. Enida Xhaferi

6. • Epidermis – is the keratinized, avascular, stratified, squamous
epithelium which serves as the outer layer of the skin.
Keratinocytes are the predominant cells of epidermis, whose
thickness is not the same in thin and thick skin. Epidermis largely
represents the barrier function of the organ, it regenerates every
25-45 days and undergoes changes in thickness in response to
different factors such as trauma or pressure.
• Dermis - lies under the epidermis, is 0,3-4 mm thick and contains
collagen, elastic and reticular fibers, matrix, connective tissue and
immune cells. It has nerves, blood/lymphatic vessels, nerve endings
and smooth muscle cells. It consists of the papillary and reticular
layer.
• Hypodermis - helps anchor skin to underlying organs and contains
mainly adipose tissue. It protects the organism from chemical and
thermal insults and serves as an important source of food reserve.
.Dr. Enida Xhaferi

7. .Dr. Enida Xhaferi

8. .Dr. Enida Xhaferi
 Consists of the following layers - stratum basale (the functional layer of the skin
where cells are continuously proliferating), stratum spinosum (8-10 rows of
keratinocytes with lots of desmosomes) stratum granulosum (cells here start to
lose their nuclei and cytoplasmic organelles, and turn into the keratinized squames
of the next layer), stratum lucidum (is present in the palm of the hand and sole of
the feet), stratum corneum (contains keratinized, dead cells reduced to flattened
scales or squames).
 Keratinocytes are the principal cells of the layer and the keratin they produce
hardens the epidermis, protecting the skin and tissues that lie beneath from
microbes, heat, chemicals.
 Other cells:
 Melanocytes (secrete melanin)
 Sensory cells (Merkel cells)
 Immune cells ( Langerhans cells)
 Thick epidermis is found only on the palm of the hands and
feet soles. Arrangements made from the papillary ridges on the
ends of the fingers and thumbs form fingerprints which are used to reveal an
individual’s true identity because of their singularity. Fingerprints, footprints, and
handprints reflect patterns of dermal papillae; their identification is known as
dactyloscopy, while the study of fingerprints is called dermatoglyphics.

9. • Consists of dense irregular connective tissue; its thickness differs
and is consists of the papillary and reticular layer.
• The papillary layer contains capillaries which provide nutrients to
the avascular epidermis through diffusion; sensory receptors
(tactile/Meissner corpuscles, pain/free nerve endings).
• Reticular layer has the blood vessels that help maintain body’s
homeostasis, sweat glands, oil glands, connective tissue fibers –
collagen and elastin, deep pressure receptors.
.Dr. Enida Xhaferi

10.  The organ reflects the health status of the individual and
diseases of other systems manifest themselves in the skin and
its derivative structures.
 Skin lesions are observed in a variety of diseases, like – immune,
autoimmune, autoinflammatory, rheumatic (LES,
dermatomyositis, vasculitis, scleroderma etc) disorders,
endocrine pathologies (morbus Cushing, diabetes mellitus,
morbus Graves and hyperthyroidism, morbus Addison,
hypothyroidism and myxedema), metabolic, nutritional
(marasmus, pellagra), renal, neoplastic, hematological, psychiatric,
systemic infectious diseases and sexually transmitted ones.
Dr. Enida Xhaferi

11.  Skin disorders are found in many rheumatologic
conditions. They constitute the primary features in
lupus erythematosus, dermatomyositis and systemic
sclerosis and are also present in systemic vasculitides,
rheumatoid arthritis, Sjögren syndrome, psoriatic
arthritis, systemic-onset juvenile rheumatoid arthritis,
and relapsing polychondritis.
.Dr. Enida Xhaferi

12.  Macula (circumscribed area of skin color change, flat and
unpalpable; with well or ill defined margins, could be of any color
and may be referred to as a patch, if it is bigger than 1 cm); papule
(a solid raised lesion; superficial, usually elevated above not within
and < 1 cm) nodule, plaque (solid, circumscribed elevation, whose
surface area is bigger than the height, usually with well defined
borders) purpura, petechia, pustul, squam, erosion, erythema,
onychodystrophy, onycholysis, urticaria, ulcer, butterfly rash,
Gottron papules and sign.
 Color – the lesion could be red, pink, violet, purple (purpuric
lesions do not blanch with vitro pressure), brown, blue, gray, black,
yellow.
 Margins – borders could be ill or well defined.
 Shape of the lesion – oval, polygonal, polycyclic, annular,
serpiginous etc.
 Number – solitary or multiple. .Dr. Enida Xhaferi

13.  Pattern – lesion arrangements vary and they may be
grouped in herpetiform, arciform, annular structures or
may be disseminated.
 Distribution – localized, regional, symmetrical, generalized,
localized in pressure points etc.
 Palpation should help define consistency, mobility,
presence of tenderness, changes in temperature and depth
of the lesion (dermal, subcutan).
 Dermatologic manifestations provide clues regarding the
systemic involvement of the pathology, diagnosis,
therapeutic approach and prognosis. Skin biopsies are
usually useful in determining the precise nature of the skin
disorder.
.Dr. Enida Xhaferi

14.  A typical autoimmune disease which affects a variety of
organs and systems like the skin, joints, kidneys, central
nervous system, hematological system, muscles and heart.
 SLE occurs more frequently in women of child bearing
age and higher disease rates are reported in Hispanics
and blacks.
 Tissue damage is caused by immune complexes,
autoantibodies and lymphocytes. Like in other
autoimmune disorder immunological alteration are due
to interaction of predisposing genes (and inadequacy of
protective genes) with external factors.
.Dr. Enida Xhaferi

15.  Cutaneal manifestations in this disease are commonly divided into
lupus lesions, vascular lesions, non vascular, non lupus ones.
 Patients with SLE and no signs of skin involvement, comprise the
condition “lupus sine lupo”.
 Lupus erythematosus specific skin manifestations are categorized into
three major clinical subtypes according to disease acuity - acute
cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus
erythematosus (SCLE), and chronic cutaneous lupus erythematosus
(CCLE).There is also drug induced lupus and neonatal lupus.
 There are histological differences and similarities between lupus
subtypes.
 The SLICC classification 2012 contains 4 dermatological criteria. Skin
involvement is observed in 2/3 of patients with lupus erythematosus.
.Dr. Enida Xhaferi

16.  Lupus malar rash (slightly edematous erythematous rash over
the malar region), maculo-papular rash on photo exposed areas
(lesions are located especially above the waistline, but can also
be found in the dorsum of the hand), bullous lupus and toxic
epidermal necrolysis (TEN).
 Butterfly rash located over the bridge of the nose and cheeks is
the most common lesion; diffuse maculopapular pruritic
eruptions especially above the waistline can occur. These lesion
may be similar to drug reactions. Bullous lesions are not that
common.
 Patients with ACLE develop lupus in more than 90% of cases.
 Differential diagnosis include rosacea, dermatomyositis,
contact/seborrheic dermatitis, drug reactions.
.Dr. Enida Xhaferi

17.  In subacute cutaneous lupus erythematosus, the maculo-papular
lezions have annular or psoriasis like configuration; their margins are
erythemato-squamous with vesiculae and crusts.
 Lesions are usually superficial without hyperkeratosis or follicular
plugging; occur mainly in sun exposed areas, spare the mid face and
leave frequently hipo or hiper pigmented patches.
 50% of patients with SCLE have SLE
 Differential diagnosis includes dermatomyositis, eczema, psoriasis,
pityriasis rosea, eritythema multiforme, drug eruption.
.Dr. Enida Xhaferi

18.  Chronic cutaneous lupus erythematosus includes discoid lupus
erythematosus (DLE), lupus timidus, hypertrofic discoid lupus,
chilblain lupus and lupus panniculitis
 Discoid lupus erythematosus lesions usually have well defined
borders and the following morphologic characteristics: congestive
erythema and central teleangiectasis; follicular plugging and adherent
scales; hypopigmented scarring and central atrophy. DLE could be
localized or disseminated and sun exposed areas are mainly affected.
Scalp patches are observed in 60% of patients.
 Lupus timidus is characterized by the presence of well defined
purplish edematous, plaques in the face and upper body without
follicular plugging or scales.
 In lupus chilblain bluish purple lesions are present in fingers and toes.
Lesions can also appear in the ears, elbows, knees, nose.
.Dr. Enida Xhaferi

19.  Vascular lesions comprise - livedo reticularis,
erythromelalgia, leg ulcers, urticaria, angioedema,
splinter haemorrhages, skin necrosis, palmar erythema,
erosive oral lesions.
 Non vascular, non lupus lesions include - non scarring
alopecia, papular mucinosis, calcinosis, aseptic
pustulosis, antederma.
.Dr. Enida Xhaferi

20.  Clinical signs, biochemical and immunologic tests results help
diagnose the disease (patients have autoantibodies – ANA,
dsDNA, anti Sm, anti RNP). ACR and SLICC criteria are used
for the classification of patients.
 Glucocorticosteroids are the mainstay of therapy for severe
SLE. They are combined with cyclophosphamide for
management of lupus nephritis.
 Antimalarials(hydroxychloroquine) methotrexate, mykofenolat
mofetil, dapsone and biologics are also used.
 Patients should avoid sun exposure, utilize sunscreens and hats,
follow an healthy diet and lead active lives.
.Dr. Enida Xhaferi

21.  Inflammatory myopathies are a heterogeneous group
of systemic autoimmune rheumatic disorders
characterized by chronic muscle weakness, fatigue,
organ and skin involvement. Disease etiopathogenetic
is unknown and women are affected more than men.
 Disorder is characterized by progressive weakness of
the major limb and trunk muscles. Proximal weakness
predominates and patients find it difficult to rise from
chairs, climb stairs, raise hands and move in the bed.
 Skin lesions are common and precede muscular
manifestations in 50% of patients with dermatomyositis.
.Dr. Enida Xhaferi

22.  Heliotrope rash (a pinkish erythema that involves the periorbital
area), Gottron sign (erythematous squamous plaques located in
the dorsum and lateral part of interfalangeal and
metacarpophalangeal joints), Gottron papules are characteristic
skin manifestations in dermatomyositis.
 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 may have diffuse erythematosquamous scalp lesions,
poikiloderma (hypo or hyper pigmentation, atrophy, telangiectasia)
and moderate alopecia.
 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.
.Dr. Enida Xhaferi

23.  Increased levels of creatine kinase, aldolase, LDH, ALT
AST, ERS, PCR.
 Autoantibodies present(ANA, anti-sintetase, anti Mi2,
anti-SRP,
 Changes in muscle biopsy – interstitial inflammation
and vasculopathy, segmentary muscle fibrosis.
 Changes in skin biopsy.
.Dr. Enida Xhaferi

24.  Chronic autoimmune disease with complex and poorly understood
pathogenesis, characterized by prominent vascular alterations with
endothelial cell damage and proliferation of subendothelial connective
tissue; immunological alterations and fibrosis. Skin, lungs, gastrointestinal
tract, heart, kidneys and central nervous system are commonly affected.
 Scleroderma occurs more frequently in women than men. There are two
major forms of the disease : localized scleroderma and systemic sclerosis
(SSc) which is divided in turn into two major categories - limited
cutaneous SSc (lcSSc) that usually associated with mild to moderate,
delayed organ fibrosis and - diffuse cutaneous SSc (dcSSc).
 Raynaud phenomenon, finger and hands edema and migratory arthritis
are the initial diseases manifestations. Skin fibrosis in lcSSc is not very
progressive and involves mainly the fingers (sclerodactyly) and distal
extremities (distally to elbows and knees), while diffuse cutaneous SSc is
characterized by progressive skin fibrosis, starting in the fingers and
moving up from distal to proximal extremities (proximal to knees and
elbows), the face, and the trunk.
 Autoantibodies commonly found in SSc include: ANA, anti Scl-70,
anticentromere/ACA, anti-U3-RNP.
.Dr. Enida Xhaferi

25.  The most common skin disorders affecting patients with SSc
include - ssymmetric cutaneous sclerosis, Raynaud
phenomenon, mask-like appearance of the face, 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. Dermis is
thickened with dense collagen bundles and elastic tissue is
reduced.
.Dr. Enida Xhaferi

26.  Autoimmune disease with unknown etiopathogenesis,
which affects women more than men.
 Symmetric arthritis involving the interphalangeal
proximal, metacarpophalangeal joints, wrists, knees,
ankles and cervical spine.
 Invalidating disease – patients have musculoskeletal and
extraarticular manifestations.
.Dr. Enida Xhaferi

27.  Dermatologic manifestations in rheumatoid arthritis include palisading
granulomas (rheumatoid nodules involving usually mechanically stressed
areas; palisaded neutrophilic granulomatous dermatitis, pseudorheumatoid
nodules), rheumatoid vasculitis (symptoms vary from presence of purpuric,
papule, petechiae to ulcers and peripheral gangrene) and neutrophilic
dermatoses (pyoderma gangrenosum, rheumatoid neutrophilic dermatitis).
Some patients with rheumatoid arthritis develop cutaneous drug toxicity
during treatment
.Dr. Enida Xhaferi

28.  Skin manifestations are observed frequently among
patients with rheumatic disorder and their correct
identification will help diagnose the specific
pathology. Consultation with a dermatologist, may be
warranted for particular conditions that require
further specialized management.
.Dr. Enida Xhaferi

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 .
.Dr. Enida Xhaferi

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.Dr. Enida Xhaferi

31. .Dr. Enida Xhaferi