Chapter 11 Rash and Arthritis
Manual of Rheumatology and Outpatient Orthopedic Disorders
Chapter 11 Rash and Arthritis
Michael I. Jacobs
Beh §et's syndrome
Dermatomyositis/polymyositis
Erythema nodosum (EN)
Juvenile rheumatoid arthritis (JRA)
Lupus erythematosus
Lyme arthritis
Psoriasis
Pyoderma gangrenosum
Reiter's syndrome
Rheumatic fever
Rheumatoid arthritis
Sarcoidosis
Scleroderma and variants
Vasculitis, cutaneous
The differential diagnosis of rash and arthritis is complex. Knowledge of the plethora of cutaneous manifestations of the arthritic diseases is important in making a correct diagnosis. A complete examination of the body surface is essential, because it may reveal lesions of psoriasis or discoid lupus erythematosus (DLE) that are hidden in the scalp, psoriatic pitting of the nails , or infiltration of old scars secondary to sarcoidosis. The purpose of this chapter is to provide the physician with diagnostic information about specific cutaneous findings in each disorder . Where applicable , immunofluorescent techniques, which may help confirm the diagnosis, are described.
I. Beh §et's syndrome. Originally described as the triad of iritis and recurrent oral and genital ulcerations, Beh §et's syndrome is a multisystem disease involving the eyes, mucous membranes, skin, blood vessels, joints, bowel, kidneys, nervous system, lungs, and heart.
- The oral lesions resemble those of aphthous stomatitis. They begin as small areas of macular erythema that then develop into superficial gray ulcers. Larger, deeper ulcerations occasionally occur.
- Genital ulcerations are most frequently located on the scrotum and labia but may also be found on the penis or in the vagina.
- Other cutaneous manifestations of Beh §et's syndrome are erythema nodosum-like lesions on the lower extremities, folliculitis-like lesions, pustules, furuncles, and superficial phlebitis. A positive pathergy test can be elicited at 24 or 48 hours (i.e., the development of a pustule at the site of skin trauma such as a needle stick).
II. Dermatomyositis/polymyositis. The cutaneous manifestations specific for dermatomyositis are heliotrope rash and Gottron's papules. Additional cutaneous findings common to other connective tissue diseases are seen.
- Heliotrope rash is a violaceous discoloration of the upper eyelids accompanied by edema. It develops early in the course of the disease and often coincides with a similar violaceous eruption on the butterfly area of the face, neck, anterior chest, and other sun-exposed regions . On the extremities, the rash characteristically involves the extensor surfaces of both large and small joints, in contrast to the rash of lupus erythematosus, which involves the skin between the joints on the dorsum of the hand.
- Poikiloderma, which consists of speckled hyperpigmentation and hypopigmentation, telangiectasis, and cutaneous atrophy, appears later in the course of the disease.
- Gottron's papules, which are violaceous and flat-topped and appear on the extensor aspect of the interphalangeal joints, are a late manifestation.
- Calcification of skin, fascia, and muscle occurs in those patients , especially children, with severe muscle involvement.
- Other findings seen in dermatomyositis are Raynaud's phenomenon , erythema, papules, and ulcerations or leukoplakia of the mucous membranes. There is generally no correlation between the extent of cutaneous involvement and the severity of the myositis. Correlation exists between the degree of nailfold capillary abnormality, as viewed with a wide-field microscope, and the number of organ systems involved by the disease process.
III. Erythema nodosum (EN) appears as crops of discrete, tender subcutaneous nodules that are erythematous and whose centers are slightly raised. Lesions are usually 2 cm or more in size and are characteristically located on the shins and ankles, but they may occur symmetrically on the extensor aspect of the extremities and, infrequently, on the face. Prodromal symptoms may include fever, chills, malaise, and polyarthralgia. Lesions heal without scarring or ulcerations and undergo color changes similar to those of bruises.
- Etiology. EN is a hypersensitivity reaction involving the vasculature of the subcutaneous tissue and should alert the physician to search for an underlying disease process. Specific etiologies follow.
- Infection
- b -Hemolytic streptococci. EN may appear within 3 weeks of an upper respiratory infection.
- Tuberculosis was once a common cause of EN. Skin lesions appear 3 to 8 weeks after the primary infection.
- Deep fungal infection. Coccidioidomycosis, histoplasmosis, and North American blastomycosis can produce EN lesions.
- Lepromatous leprosy. When accompanied by EN, iritis, orchitis, lymphadenopathy, and polyneuritis, the disease is referred to as erythema nodosum leprosum.
- Sarcoidosis. Lofgren syndrome, consisting of bilateral hilar adenopathy and EN, is a benign form of sarcoidosis.
- Drug allergy. Allergy to sulfonamides, bromides, iodides, oral contraceptives, and other drugs has been associated with EN.
- Inflammatory bowel disease. Both ulcerative colitis and Crohn's disease produce EN in about 10% of cases.
- Beh §et's syndrome. EN is seen in association with other cutaneous manifestations of this disease.
- Differential diagnosis. Diseases to be considered in the differential diagnosis of EN are erythema induratum, Weber-Christian disease, subcutaneous nodular fat necrosis in association with pancreatic disease, recurrent thrombophlebitis, cutaneous arteritis, and lupus profundus.
IV. Juvenile rheumatoid arthritis (JRA). The characteristic rash of JRA is seen in approximately 30% of patients, mostly those with systemic-onset disease. It is more common in patients under the age of 2 years . Lesions are erythematous and flat or very slightly raised; they vary in diameter from 3 to 10 mm and have diffuse borders. Lesions occur on the trunk, extremities, and face and may become confluent. The rash is evanescent, is often associated with fever spikes, and occurs most frequently in the early evening. It may be pruritic. Koebner's phenomenon is common (i.e., rash is triggered by skin trauma).
The cutaneous eruption does not correlate with presence of rheumatoid factor (RF) in the serum, although the rash is often present during periods of active systemic disease. Skin nodules are rarely found in younger patients.
Teens with RF may have subcutaneous nodules similar to those in patients with adult RA.
V. Lupus erythematosus
- The lupus band test (LBT) demonstrates deposits of immunoglobulin and complement at the dermal-epidermal junction by direct immunofluorescent staining of the skin. Biopsy of cutaneous lesions in DLE or systemic lupus erythematosus (SLE) yields a positive LBT in about 90% of patients.
Clinically normal skin of patients with DLE demonstrates a negative LBT. The LBT in clinically normal skin of patients with SLE varies with sun exposure. Approximately 50% of patients with SLE have a positive LBT in clinically normal, sun-protected skin, whereas 80% have a positive LBT in clinically normal, sun-exposed areas.
- Discoid lupus erythematosus. The characteristic lesion of DLE is a scaly plaque that ranges in color from red to violaceous, with sharply-defined borders, central atrophy, telangiectasia, and areas of hypopigmentation or hyperpigmentation. Keratinous plugging of the hair follicles can sometimes be detected as tiny rough projections across the lesion. Lesions may be multiple and asymmetric and are most commonly found on the head and neck, particularly in the malar areas, ears, and scalp. When the scalp is involved, hair loss with scarring at the site of the lesion results. Lesions on the oral and nasal mucosae may ulcerate. Lesions of DLE produce scarring; those that result in severe scarring may, infrequently, produce skin cancer.
When discoid lesions are present below the neck, the term generalized DLE is used. SLE develops in fewer than 10% of patients who present with lesions of DLE.
- Systemic lupus erythematosus. Cutaneous manifestations of SLE comprise several of the criteria for the classification of SLE. These criteria are facial erythema, DLE lesions, photosensitivity, and oral or nasopharyngeal ulceration.
- Facial erythema. The classic butterfly rash of SLE occurs in up to 40% of patients. It begins as a transient erythematous, edematous eruption across the bridge of the nose and malar areas. It is often exacerbated by sun exposure and may accompany a flare of systemic disease. If the rash is persistent, atrophy, telangiectasia, and scaling will develop.
- Discoid lesions seen in SLE are identical to those of DLE and occur in approximately 20% to 30% of patients. Patients with DLE only above the neck are at less risk for development of SLE.
- Photosensitivity will often precipitate butterfly-pattern erythema, the rash of subacute lupus erythematosus, and lesions of DLE. The active spectrum is ultraviolet light of 280 to 320 nm (ultraviolet B), which normally produces sunburn erythema. Ultraviolet A (320 to 400 nm) has been implicated in lesions of subacute cutaneous lupus erythematosus (SCLE). Sunlight may also cause a flare of systemic disease.
- Oral or nasopharyngeal ulcerations are shallow and have gray bases with red borders. They are often painful and usually seen in patients with severe cutaneous disease.
- Raynaud's phenomenon is seen in up to 30% of cases.
- Alopecia can be of two types in SLE.
- Scarring alopecia is produced when discoid lesions affect the scalp; these lesions are easily recognized.
- More subtle is the reversible (diffuse or patchy) alopecia that may arise. Diffuse alopecia is more common than patchy alopecia. It may accompany a clinical flare of SLE and is sometimes elicited only by specifically questioning the patient about increased hair loss. The frontal hairline may consist of short, broken hairs, as in traction alopecia.
- Subacute cutaneous lupus erythematosus is a nonscarring photosensitive eruption with papulosquamous and annular-polycyclic patterns. The distribution is over sun-exposed areas. Patients have a high frequency of anti-Ro auto-antibodies and generally have a milder systemic disease.
- The papulosquamous eruption is composed of confluent and discrete scaly erythematous papules and plaques.
- In the annular-polycyclic type, scaly erythematous borders surround central areas of subtle hypopigmentation and telangiectasia.
- Vasculitis
- Arteritis may result in focal areas of gangrene on the fingers or toes.
- Livedo reticularis is a purple, netlike, deep vascular discoloration that is most common on the lower extremities.
- All manifestations of leukocytoclastic vasculitis (see section XIV.A.2 ) may be observed .
- Painful ulcers may develop over the forearms, hands, and fingers, and near the malleoli.
- Periungual telangiectasia develops on the fingers. This disorder commonly occurs in scleroderma, dermatomyositis, and, less frequently, RA.
- Urticaria may be the presenting cutaneous manifestation of SLE.
- Lupus profundus is characterized by firm vasculitic nodules in the subcutaneous fat. Erythema of the overlying skin sometimes occurs. The nodules may be found on the forehead, cheeks, buttocks, and upper arms.
VI. Lyme arthritis. This multisystem inflammatory disorder is caused by the spirochete Borrelia burgdorferi, which is transmitted by the bite of an Ixodes tick. Erythema migrans, present in up to 75% of patients, is a rapidly expanding annular erythematous lesion that begins at the site of the tick bite. The central portion may remain erythematous, clear, or rarely become necrotic. The rash appears within days to 4 weeks after the tick bite. Patients at this stage will often have fever, fatigue, headache , and arthralgias. A secondary rash consisting of multiple annular lesions resembling those of secondary syphilis may follow. The nervous system, heart, eyes, and joints can become involved weeks to years later. Borrelia has also been implicated in some cases of acrodermatitits chronica atrophicans, morphea, and progressive facial hemiatrophy of Parry-Romberg.
VII. Psoriasis. This chronic disease involves the skin and joints and may present at any age. There is a family history of the disease in approximately 30% of cases. Psoriasis has been estimated to affect about 2% of the population in the United States.
- Characteristics. Psoriasis is characterized by increased epidermal proliferation . The typical skin lesion is a well-delineated, raised, erythematous plaque covered by a loosely adherent silvery scale. As the scale accumulates, the lesion may even appear white. Lesions vary in size from small papules to extensive plaques and occasionally assume an annular or gyrate configuration. Lesions of psoriasis heal without scarring.
- Distribution
- Although psoriasis usually has a symmetric distribution, a solitary lesion is sometimes seen.
- Sites of predilection include the elbows, knees, scalp, and lumbosacral region.
- Any clinical pattern of psoriasis may be associated with psoriatic arthritis. However, nail involvement is seen in 80% of patients with arthritis and in only 30% of patients without joint involvement.
- Medications that may cause psoriasis to flare include chloroquine and lithium; withdrawal of systemic corticosteroids is also a possible cause.
- Koebner's phenomenon is the appearance of new lesions at sites of trauma, such as scratching, sunburn, or physical injury . Bluntly scraping off the scale to reveal punctate areas of bleeding underneath produces the Auspitz sign.
- The chronic plaque type is seen on the sites of predilection noted in B and on the trunk and extremities. Lesions may become confluent.
- Inverse psoriasis is localized to intertriginous areas.
- Guttate psoriasis is characterized by teardrop-shaped lesions on the trunk and proximal extremities; it is often precipitated by a b -hemolytic streptococcal infection.
- Palmar psoriasis is characterized by scaly, erythematous patches on the palms and fingers. This form may be mistaken for a dermatophyte infection.
- Pustular psoriasis may present as localized sterile pustules of the palms, soles, or paronychial skin or as severe generalized pustules accompanied by fever, arthralgia, and leukocytosis.
- Exfoliative erythroderma , in which the skin of the entire body is thickened, erythematous, and scaly, may be precipitated by an infection, drug allergy, sunburn, or severe contact dermatitis.
- Nail changes include surface pits, yellow discoloration of the nail plate, lifting of the nail bed, subungual keratotic accumulation, thickening, crumbling, grooving, and splitting.
VIII. Pyoderma gangrenosum is frequently associated with ulcerative colitis, Crohn's disease, RA, myeloproliferative disorders, and leukemia. Rarely, it is associated with chronic active hepatitis, myeloma, sarcoidosis, and diabetes mellitus. It begins as a tender pustule that rapidly expands to become a large ulcer many centimeters in diameter with a bluish, undermined border and a necrotic, purulent center. Lesions most frequently occur on the legs and trunk and heal with scar formation. Cutaneous trauma may exacerbate existing ulcers or cause the formation of new lesions.
Skin biopsy of pyoderma gangrenosum is not diagnostic. The diagnosis can be made only when other causes of cutaneous ulcerations, such as vasculitis, syphilis, tuberculosis, and bacterial, fungal, and protozoal infection, are excluded.
IX. Reiter's syndrome consists of the tetrad of arthritis, urethritis, conjunctivitis, and mucocutaneous lesions. The mucocutaneous lesions, present in about 80% of cases, may be divided into the following categories:
- Mucosal lesions. The penis is commonly affected by superficial ulcerations around the urethral meatus. Eroded red papules involve the corona and glans and become confluent ( balanitis circinata ). Erosion, erythema, and purpura are seen frequently in the mouth and pharynx.
- Cutaneous lesions
- Red macules may develop on the palms and soles, then form pustules and progress to thick hyperkeratotic plaques ( keratoderma blennorrhagica ).
- Psoriasiform plaques, which may form pustules, are sometimes found scattered on the scalp, trunk, extremities, and scrotum.
- Nail changes include subungual hyperkeratosis and thickening of the nail plate.
- Generalized exfoliative erythroderma may be seen in severely ill patients.
X. Rheumatic fever. Three type of skin lesions are observed in rheumatic fever. They are listed in decreasing order of frequency.
- Subcutaneous nodules are usually less than 0.5 cm in diameter and are found over bony prominences of the elbows, knuckles, ankles, and occiput. Nodules may persist up to a month or recur over several months and are frequently associated with carditis.
- Erythema marginatum is most commonly seen on the trunk, extremities, and the axillary vault. These flat or slightly raised polycyclic and annular lesions begin as small erythematous macules or papules that rapidly spread peripherally. The outlines of the lesions may become irregular. Usually associated with carditis, the lesions often erupt soon after the onset of arthritis and may recur for months.
- Erythema papulatum, an extremely rare manifestation of rheumatic fever, is characterized by indolent papules that are found over the flexor and extensor surfaces of large joints.
XI. Rheumatoid arthritis. Rheumatoid nodules and manifestations of vasculitis are the major cutaneous findings in RA and are observed primarily in patients with RF.
- Rheumatoid nodules are found in approximately 20% of patients. They are firm, up to several centimeters in diameter, and most frequently located subcutaneously, although they can involve tendon sheaths and periosteum. They are found in skin subjected to repeated minor trauma, such as the juxtaarticular region of the elbows, Achilles tendons, ischial tuberosities, scapular areas, and hands and feet. If they occur on the sclera, scleromalacia with possible globe perforation may result. Rheumatoid nodules may rarely soften and ulcerate.
- Vasculitic lesions are commonly found on the digits. They present as tiny nail infarcts or red or purpuric macules or papules that can progress to painful subcutaneous nodules or ulcers, 2 to 3 mm in diameter. In severe cases of digital arteritis, gangrene of the finger pulp can ensue. Vasculitis frequently affects the dependent lower extremities, manifesting as purpuric macules and papules, urticarial lesions, hemorrhagic bullae, painful ulcers, and livedo reticularis.
- Other rare cutaneous features of RA are palmar erythema and skin atrophy of the hands.
XII. Sarcoidosis. All cutaneous manifestations of sarcoidosis, except EN and transient maculopapular eruptions, show histologic evidence of sarcoid granulomas on skin biopsy.
Circulating immune complexes may be responsible for both EN and transient maculopapular eruptions, either of which may be a presenting sign of the disease.
- Erythema nodosum is a septal panniculitis that appears as painful, erythematous, slightly raised, rounded lesions symmetrically distributed on the extensor aspect of the extremities, often localized to the shins. Fever and polyarthralgias may occur. When healing, the lesions assume the color of a bruise. Recurrent lesions appear in crops. EN is not specific for sarcoidosis, but the condition is termed Lofgren syndrome when accompanied by bilateral hilar adenopathy.
- Transient maculopapular eruptions occur on the trunk, face, or extremities and may be accompanied by acute uveitis, peripheral lymphadenopathy, and parotid enlargement .
- Granulomatous cutaneous lesions of sarcoidosis include the following:
- Papules. Translucent, reddish brown; they are found on the periorbital area, ala nasi, and upper torso.
- Annular lesions. Formed from papules coalescing in rings.
- Nodules. Rarely found on the extremities or trunk.
- Plaques. Purple to reddish brown; they are symmetrically located on the extremities and buttocks. Plaques on the face, ears, fingers, or toes are referred to as lupus pernio.
- Ichthyosis-like lesions. Large scales , usually located on the lower extremities.
- Generalized erythroderma.
- Infiltration of old scars. A phenomenon peculiar to sarcoidosis; scars become purple and raised.
XIII. Scleroderma and variants. Scleroderma may present as a disease localized to the skin or as a systemic process.
- Localized scleroderma
- Morphea is a discrete, well-defined plaque of scleroderma that is smooth, indurated, and yellowish white in color. It has a violaceous halo when the disease is active.
- Generalized morphea. Numerous large plaques may involve almost the entire body, usually sparing the face. Underlying muscle atrophy is often prominent on the extremities.
- Guttate morphea consists of small, white, sclerotic lesions usually located on the chest and shoulders.
- Linear scleroderma generally begins during childhood, is unilateral, and may result in joint contractures secondary to involvement of muscle and bone.
- Facial hemiatrophy may be associated with facial linear scleroderma.
- Coup de sabre refers to linear scleroderma that involves the face and scalp.
- Systemic scleroderma (progressive systemic sclerosis, or PSS). Skin involvement in PSS may be classified by type of onset and presence or absence of Raynaud's phenomenon.
- Acrosclerosis is the predominant type, accounting for 90% of cases. It is characterized at an early stage by edema of the hands, fingers, feet, and legs and by Raynaud's phenomenon. Later, the skin of the hands and feet becomes indurated, thick, taut, smooth, and bound down. The process may extend onto the extremities. The face, neck, and trunk are commonly involved. Cutaneous manifestations include the following:
- Sclerodactyly. Smooth, shiny, tapered fingers with taut, bound-down skin from the MCPs to the fingertips.
- Joint contractures occur principally over small joints but may involve large joints as well. Hand contractures result in claw hand deformity.
- Ulcerations usually develop over the fingers, toes, malleoli, and knuckles and may result in infection.
- Facial involvement may result in either a waxy, expressionless facies or in a pinched facies with thin lips, radial furrows about the mouth, sunken cheeks, and a beaklike nose.
- Pigment alterations appear as three types.
- The most common type is post-inflammatory hyperpigmentation or hypopigmentation in sclerotic areas.
- In areas of normal skin, complete pigment loss, dotted centrally by perifollicular pigment resembling vitiligo, may develop.
- Rarely, generalized hyperpigmentation similar to that seen in Addison's disease occurs.
- Matlike telangiectases may occur on the face, lips, hands, oral mucosa, and upper trunk. There is a high correlation between the degree of nail fold capillary abnormality, as viewed with a wide-field microscope, and the extent of internal organ involvement in scleroderma.
- Bullae may rarely occur in areas of sclerosis.
- Calcinosis cutis generally occurs late in the course of scleroderma and is usually limited to the skin over joints. The CREST syndrome consists of c alcinosis, Raynaud's phenomenon, e sophageal dysfunction, s clerodactyly, and t elangiectasia. It identifies a group of PSS patients with a more favorable prognosis .
- Diffuse systemic sclerosis often begins on the trunk and rapidly spreads to involve the extremities and face.
- The differential diagnosis of fibrotic skin includes the following:
- Eosinophilic fasciitis.
- Tryptophan-induced eosinophilia myalgia syndrome.
- Borreliosis.
- Graft-versus-host disease.
- Porphyria cutanea tarda.
- Scleroderma.
- Carcinoid syndrome.
- Scleromyxedema.
- Lichen sclerosis et atrophicus.
- Bleomycin-induced sclerosis.
- Chlorinated hydrocarbon-induced sclerosis (polyvinyl chloride).
- Occupational trauma.
- Primary amyloidosis.
- Melorheostosis with linear scleroderma.
- Progeria.
- Werner syndrome.
- Phenylketonuria.
- Toxic oil syndrome (caused by adulterated rapeseed oil).
- Eosinophilic fasciitis can usually be distinguished from systemic scleroderma by examination of the skin. On the trunk and proximal extremities, localized areas of induration develop that are firmly bound to underlying tissues. A cobblestone or puckered surface is formed secondary to involvement of the subcutaneous fibrous tissue and is most obvious during overhead extension of the upper extremities. Raynaud's phenomenon and internal organ involvement do not occur. A biopsy specimen that includes skin, fascia, and muscle is necessary for diagnosis. Fibrotic thickening and a cellular infiltrate, often including eosinophils, are found in the deep fascia and may also be present in the lower dermis, fat, and muscle. Laboratory abnormalities include blood eosinophilia in 30% of patients, an increased erythrocyte sedimentation rate, and hypergammaglobulinemia. A history of tryptophan ingestion should be elicited.
- Tryptophan-induced eosinophilia myalgia syndrome. In 1989, a disease characterized by peripheral eosinophilia and myalgia with features of both eosinophilic fasciitis and systemic scleroderma was seen following tryptophan ingestion. Clinical features include fever, fatigue, weakness, muscle cramps, arthralgia, cutaneous edema, rashes, pruritus, and thickening of the skin. The degree of both eosinophilia and myalgia is variable. Scleroderma-like skin changes can involve the extremities as in eosinophilic fasciitis, or become more generalized as in PSS.
- Undifferentiated connective tissue syndrome, also known as mixed connective tissue disease, denotes combined clinical features of SLE, scleroderma, and polymyositis. High titers of antibodies to U1RNP occur in many of these patients. Some authorities feel that undifferentiated connective tissue syndrome represents a prodrome of SLE or scleroderma in most patients (see Chapter 31). Raynaud's phenomenon and swollen hands are the most frequent skin manifestations.
Direct immunofluorescent studies of clinically normal skin in patients with undifferentiated connective tissue syndrome reveal subepidermal immunoglobulin deposits in approximately one-third of cases.
XIV. Vasculitis, cutaneous (See also Chapter 32.)
- Classification. Cutaneous vasculitis includes septic and leukocytoclastic categories.
- Septic vasculitis. Skin manifestations of the gonococcal arthritis-dermatitis syndrome occur during the initial bacteremic phase and are accompanied by fever, rigor, tenosynovitis, and migratory polyarthritis. The skin lesions are usually tender, few in number, and located on the distal extremities. They may present as petechiae, small ecchymoses, hemorrhagic papules, vesiculopustules on a hemorrhagic base, or hemorrhagic bullae. It is difficult to culture gonococci from skin lesions.
- Leukocytoclastic vasculitis. Lesions of palpable purpura on the lower extremities may be easily recognized. The morphologic expression of the immune complex vasculitis that involves the postcapillary venules of the skin is manifold . Lesions are usually concentrated on the legs and distributed symmetrically. They may begin as erythematous macules or urticarial papules. These lesions then become purpuric. The vasculitis may rapidly progress to form hemorrhagic vesicles and bullae, nodules, or superficial ulcers covered by eschars. Such lesions are painful and may appear in recurrent crops that last for weeks. Three important syndromes manifested by leukocytoclastic vasculitis follow.
- Henoch-Sch nlein purpura occurs primarily in children and young adults following an upper respiratory tract infection. Purpuric lesions develop over the extensor surfaces and buttocks. Edema of the lower legs is common; edema of the hands, scalp, and periorbital areas occurs in young children. Arthritis, abdominal pain, gastrointestinal bleeding, and renal involvement presenting as proteinuria and hematuria are other features. Serum complement levels are usually normal. Immunofluorescent staining of skin biopsy specimens of early lesions reveals mainly immunoglobulin A and complement deposition in the walls of affected vessels.
- Hypocomplementemic vasculitis is characterized by recurrent attacks of urticarial skin lesions accompanied by arthritis and hypocomplementemia. The urticarial lesions may last for days, and small purpuric lesions may occasionally be present. Abdominal pain, edema of the face and larynx, and mild renal disease may occur. Biopsy and immunofluorescent staining of early skin lesions reveal immunoglobulin and complement deposition in vessel walls.
- Mixed cryoglobulinemia demonstrates the spectrum of cutaneous manifestations of leukocytoclastic vasculitis and is accompanied by immune complex renal disease (often severe), hepatosplenomegaly, and lymphadenopathy. The presence of mixed cryoglobulinemia, positive RF, and hypocomplementemia help to define this disease. A majority of patients have hepatitis C infection. Immunofluorescent staining of early skin lesions reveals immunoglobulins and complement in the walls of affected vessels.
- Differential diagnosis of cutaneous vasculitis
- Infectious agents . Gonococcus, Meningococcus, Staphylococcus aureus, b -hemolytic streptococci, hepatitis B virus, hepatitic C virus, human immunodeficiency virus, Mycobacterium leprae, endocarditis-producing bacteria.
- Drugs. Penicillin, sulfonamides, thiazides, phenothiazines, aspirin, allopurinol.
- Rheumatic disease. SLE, RA, sicca syndrome, Henoch-Sch nlein syndrome, hypocomplementemic vasculitis, mixed cryoglobulinemia, hyperglobulinemic purpura, paraproteinemia, Wegener's granulomatosis, polyarteritis nodosa, polyangiitis syndrome.
- Malignancies. Lymphoproliferative disorder, Hodgkin's disease, carcinoma .
- Systemic diseases. Serum sickness, ulcerative colitis, chronic active hepatitis, primary biliary cirrhosis, bowel bypass syndrome, Goodpasture syndrome, retroperitoneal fibrosis.
- Genetic disorders. C2 deficiency.
Books@Ovid
Copyright 2000 by Lippincott Williams & Wilkins
Stephen A. Paget, M.D., Allan Gibofsky, M.D., J.D. and John F. Beary, III, M.D.
Manual of Rheumatology and Outpatient Orthopedic Disorders
