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Rheumatologic diseases, such as systemic lupus erythematosus (SLE, shown), have a wide variety of cutaneous manifestations that can be easily confused. Careful attention to characteristic physical examination findings can help determine the diagnosis even before a lengthy laboratory panel has been ordered. This patient presented with the classic malar rash for SLE, also known as a butterfly rash, with distribution over the cheeks and nasal bridge. Note that the fixed erythema, sometimes with mild induration as seen here, characteristically spares the nasolabial folds. An appropriate initial workup would include an ANA, urinalysis, and double-stranded DNA antibody screen to evaluate the risk for future renal disease.[1]
Image courtesy of Wikimedia Commons.
The rash of subacute cutaneous lupus erythematosus (shown) has a similar hue to SLE but preferentially affects single covered areas with psoriasiform or polycyclic lesions. The rash tends to be persistent but does not result in scarring. It generally responds very well to antimalarial therapy. Subacute cutaneous lupus erythematosus is seen in about 10% of patients with lupus, however, 50% of patients with this condition will have it in isolation without systemic lupus.[2] Patients are typically positive for Sjögren precipitins, especially SSA. SSA (Ro) is associated with congenital heart block in children born to women with the antibody.
Image courtesy of Dirk M. Elston, MD.
Acute and subacute cutaneous lupus erythematosus are characterized by variable hyperkeratosis (thickening of the cornified layer) and vacuolar alteration of basal layer keratinocytes, as shown histologically. The presence of these findings in a photosensitive rash suggests a diagnosis of lupus erythematosus or dermatomyositis. The distribution of the rash and degree of pruritus will distinguish the two. Lupus preferentially affects the phalanges and sun-exposed areas, while dermatomyositis affects the scalp, knuckles, extensors, hips (holster sign), and upper back (shawl sign).[3]
Image courtesy of Dirk M. Elston, MD.
Chronic cutaneous (discoid) lupus erythematosus, shown, may be purely cutaneous or may be associated with systemic disease. Direct immunofluorescence typically demonstrates a "full house" of continuous granular immunoglobulins and complement. Sun avoidance and sunscreen use are critical. Treatment with topical or intralesional corticosteroids, systemic antimalarial agents, and immunosuppressive medication such as mycophenolate mofetil may be needed to prevent disfiguring scarring and dyspigmentation. Patients with systemic disease may require antimalarial agents, cytotoxic agents or targeted biological agents such as belimumab, depending on their systemic manifestations.
Image courtesy of Dirk M. Elston, MD.
Scarring alopecia (shown) can be one of the most devastating effects of chronic cutaneous lupus, with an impact on quality of life equal to severe systemic manifestations of the disease (eg, lupus nephritis). The alopecia is slow but progressive unless treated aggressively. Topical and intralesional corticosteroids, antimalarial therapy, and immunosuppressive agents such as mycophenolate mofetil may be needed to control the disease.
Image courtesy of Dirk M. Elston, MD.
Key histologic features of chronic cutaneous (discoid) lupus include vacuolar interface dermatitis, pigment incontinence (pigment from the damaged basal layer depositing into the upper dermis), hyperkeratosis (shown as red-colored keratin with routine hematoxylin and eosin staining), follicular plugging (blue-colored keratin with hematoxylin and eosin staining), and basement membrane thickening (demonstrated by a PAS [periodic acid-Schiff] stain in the right panel).
Image courtesy of Dirk M. Elston, MD.
Dermal mucin (shown) is characteristically increased between collagen bundles (the light blue stringy material between the red collagen bundles). This is characteristic of both lupus erythematosus and dermatomyositis, but is more pronounced in lupus, helping to distinguish the two.
Image courtesy of Dirk M. Elston, MD.
The inflammatory infiltrate in advanced cases of chronic cutaneous lupus erythematosus has a characteristic pattern, with dense superficial and deep perivascular dermal infiltrates present. These infiltrates also involve the secretory coil of the eccrine sweat duct and the fibrous tract remnant marking the position of involuted hair follicles. This results in characteristic vertical columns of lymphocytes within the mid-dermis.
Image courtesy of Dirk M. Elston, MD.
Lupus panniculitis presents with areas of induration (shown). When the overlying skin breaks down, oil may drain from the lesions. About half of all patients have overlying discoid lesions; associated systemic lupus erythematosus is common. Lupus panniculitis often responds to antimalarial drugs such as hydroxychloroquine. Control is important, as it may lead to scarring. A small proportion of patients will develop subcutaneous lymphoma in the involved areas.
Image courtesy of Dirk M. Elston, MD.
The characteristic histologic features of lupus panniculitis (shown) are lobular necrosis, resulting in lipid vacuoles of varying sizes, nodular lymphoplasmacytic infiltrates, and fibrin deposition (noted as deep pink staining at the periphery of the necrotic lobules).
Image courtesy of Dirk M. Elston, MD.
Tumid lupus presents with indurated plaque-like lesions of the trunk (shown). This condition typically responds readily to antimalarial agents. Some dispute whether this disorder belongs in the classification of lupus, as patients lack systemic manifestations. However, the histologic features (except for the lack of epidermal change) and the lack of systemic findings are typical of other forms of lupus as well, including most cases of discoid lupus. Biopsy demonstrates superficial and deep perivascular and periadnexal lymphoid infiltrates with dermal mucin.
Image courtesy of Dirk M. Elston, MD.
Hypertrophic lupus erythematosus presents with polygonal violaceous plaques that preferentially involve the lips, scalp, and acral portions of the body (shown). The lesions may ulcerate or become thickly hyperkeratotic. They resemble lichen planus both clinically and histologically but differ by the presence of continuous granular IgG, IgM, IgA, and C3 deposition noted by direct immunofluorescent microscopy. These lesions of hypertrophic lupus appear hyperkeratotic clinically and lichenoid histologically.
Image courtesy of Dirk M. Elston, MD.
Hypertrophic lupus erythematosus is the form of lupus erythematosus most likely to produce aggressive squamous cell carcinomas if left untreated, as with the patient shown here who lost his ears and much of his nose to squamous cell carcinoma. Squamous cell carcinomas arise as a result of poorly controlled hypertrophic lupus.
Image courtesy of Dirk M. Elston, MD.
Dermatomyositis often presents with polygonal flat-topped papules (Gottron papules) over the knuckles, as shown. These patients often have muscle disease and may have an associated systemic malignancy. Age-appropriate cancer screening is recommended. Skin lesions of dermatomyositis tend to involve the knuckles, proximal nail folds, scalp, upper back, hips, and extensor surfaces. Pruritus is often the most prominent symptom.[4]
Image courtesy of Wikimedia Commons / Elizabeth M. Dugan, Adam M. Huber, Frederick W. Miller, Lisa G. Rider.
The patient shown presented with hundreds of small Gottron papules (flat-topped polygonal papules). The characteristic skin atrophy results in a shiny surface that reflects light. The color is characteristically pink with a faint violaceous hue. Gottron papules may clinically resemble lesions of lupus erythematosus; lichen planus; or, particularly in the event of psoriasiform scaling, psoriasis.
Image courtesy of Dirk M. Elston, MD.
The violaceous heliotrope rash on the eyelids (shown) is closely associated with underlying muscle disease in dermatomyositis, especially when the overlying skin lacks scale and lichenification. A heliotrope is a flower with a characteristic violaceous color that gives the rash its name.
Image courtesy of Wikimedia Commons / Elizabeth M. Dugan, Adam M. Huber, Frederick W. Miller, Lisa G. Rider.
This patient presented with a poikilodermatous (mottled hyper- and hypopigmentation, atrophy, and telangiectasia) shawl, which is diagnostic for dermatomyositis. Similar changes on the hips are referred to as the holster sign. These signs are virtually pathognomonic for a diagnosis of dermatomyositis. The skin color is typically pink to violaceous, but the scaly nature of the rash, especially on the hips, may suggest eczema rather than connective tissue disease unless one is familiar with its appearance.
Image courtesy of Dirk M. Elston, MD.
The proximal nail fold in dermatomyositis typically demonstrates dilatation and dropout (segmental scarring) of vessels (arrow). Scleroderma may produce a similar vascular pattern but typically has associated sclerodactyly. Lupus produces wandering glomeruloid loops, and Osler-Weber-Rendu syndrome has loops with one half normal and the other half dilated. A hand lens, dermatoscope, or ophthalmoscope can be used to demonstrate these characteristic patterns.[5]
Image courtesy of Dirk M. Elston, MD.
Extensor surface involvement in dermatomyositis (shown) may resemble psoriasis but differs in that it has the characteristic pink to violaceous hue, less scale, and intense pruritus. The distinction is important, as dermatomyositis may be the presenting sign of an underlying malignancy. Phototherapy for psoriasis would cause a flare of dermatomyositis.
Image courtesy of Dirk M. Elston, MD.
Progressive systemic sclerosis commonly presents with sclerosis of the dorsal hands (shown). Proximal sclerosis, bibasilar lung fibrosis, and renal disease are typical features. The condition is associated with a nucleolar ANA pattern. The sclerotic plaques may have a coppery color and abrupt transition between sclerotic and adjacent normal skin.
Image courtesy of Dirk M. Elston, MD.
Raynaud phenomenon (shown) is a recurrent vasospasm of the fingers and toes that occurs in response to stress or cold exposure. Raynaud phenomenon is most commonly associated with scleroderma (90%), mixed connective-tissue disease (85%), and, to a lesser extent, lupus erythematosus and other non-autoimmune disorders, such as frostbite, vibration injury, polyvinyl chloride exposure, and cryoglobulinemia. Raynaud phenomenon is a prominent component of CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia) syndrome. Treatment may require calcium channel blockers or phosphodiesterase-5 (PDE5) inhibitors. Treatment of calcinosis cutis includes simple curettage, as well as a wide variety of systemic interventions with variable effect.[7]
Image courtesy of Medscape.
Morphea, also known as localized scleroderma, presents with thickening of the dermis and/or subcutaneous tissues in a linear or blaschkoid distribution (corresponding to fetal developmental strip-like segments), as shown in this patient. A lilac border may be noted in the acute phase. Progression may be halted in some patients with the use of corticosteroids and psoriatic doses of methotrexate. Unlike systemic sclerosis, morphea lacks features such as sclerodactyly, Raynaud phenomenon, nailfold capillary changes, telangiectasias, and progressive internal organ involvement.[8]
Image courtesy of Dirk M. Elston, MD.
Morphea profunda (shown) overlaps histologically with eosinophilic fasciitis. The former demonstrates full-thickness sclerosis so that the overlying skin cannot be pinched. Morphea profunda can cause great disability and typically requires treatment with agents such as methotrexate. Morbidity is significant, but systemic involvement is unusual.
Image courtesy of Dirk M. Elston, MD.
Patients with eosinophilic fasciitis often present with the "dry riverbed sign" (shown). When the arm is raised, the vessels retract, leaving the appearance of a dry riverbed. The overlying skin is easily pinched, as the sclerosis is not full thickness. This condition tends to occur after acute exertion, is associated with peripheral eosinophilia, and responds to corticosteroid therapy.
Image courtesy of Dirk M. Elston, MD.
Psoriasis (shown) is a chronic, relapsing, inflammatory skin disorder with a strong genetic basis. Plaque psoriasis is the most common type, characterized by circular to oval red plaques distributed over the extensor body surfaces and scalp. Epidermal hyperproliferation and dermal inflammation lead to dry, thin, silvery-white scaling. Ten percent to 20% of patients with plaque psoriasis will develop psoriatic arthritis, which can be destructive and asymmetrical or may mimic rheumatoid arthritis.
Image courtesy of Dreamstime / Christine Langer-püschel.
Psoriatic nail changes (shown) may be the only cutaneous finding in patients with psoriatic arthritis. Typical findings include nail pitting, transverse depressions, and subungual hyperkeratosis. There is a direct correlation between the number of pits and the severity of disease. Fungal infection is the major differential diagnosis to consider.
Image courtesy of Dirk M. Elston, MD.
A reticular (net-like) pattern of purpura correlates with vasculitis involving medium-sized vessels, producing necrosis of the endothelium (shown). Common causes include Churg-Strauss syndrome, Wegener granulomatosis, microscopic polyangiitis, rheumatoid vasculitis, and septic vasculitis. Nonvasculitic causes of retiform purpura include thrombotic or embolic vascular diseases.[9]
Image courtesy of Dirk M. Elston, MD.
Histologically, leukocytoclastic vasculitis demonstrates deeply staining red fibrin within the vessel wall together with fragmented neutrophils (leukocytoclasis), as shown. Leukocytoclasis refers to vascular damage caused by nuclear debris from infiltrating neutrophils. This process is dynamic, and biopsy of a lesion performed too early or too late in its evolution may not reveal these findings. The presence of eosinophils has been correlated with drug-associated disease.
Image courtesy of Dirk M. Elston, MD.
Polyarteritis nodosa is a systemic vasculitis characterized by necrotizing inflammatory lesions affecting medium and small muscular arteries. This results in microaneurysm formation, which leads to hemorrhage, thrombosis, and tissue ischemia. The classic skin manifestations include livedo reticularis (shown), lower-extremity ulcerations, digital ischemia, and firm, tender subcutaneous nodules. Common systemic symptoms include fever, malaise, fatigue, weight loss, myalgias, and arthralgias.
Image courtesy of Medscape.
Churg-Strauss syndrome presents with necrotizing leukocytoclastic vasculitis and nodules on the extensor surfaces (shown). The nodules represent Churg-Strauss granulomas. Systemic features include asthma, cardiac vasculitis, and rapidly progressive glomerulonephritis. Peripheral antineutrophil cytoplasmic antibodies (p-ANCAs) are characteristically present.
Image courtesy of Dirk M. Elston, MD.
Churg-Strauss granuloma presents with histiocytes surrounding a core of collagen and degranulated eosinophils, as shown. The granulomatous material is helpful in distinguishing one form of granulomatous vasculitis from another. Churg-Strauss disease is associated with "red" collagenolytic granulomas.
Image courtesy of Dirk M. Elston, MD.
With GPA, granulomas (shown) are characterized by a palisaded arrangement of histiocytes and multinucleated giant cells surrounding a necrotic vessel with many neutrophils. The presence of neutrophils, multinucleated giant cells, c-ANCA, and upper airway necrosis without asthmatic symptoms distinguishes GPA from Churg-Strauss syndrome.
Image courtesy of Dirk M. Elston, MD.
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