Marco Matucci-Cerinic - Skin Manifestations in Rheumatic Disease
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Rheumatologic diseases are highly associated with clinical symptoms of the skin, nails, and mucosal membranes. This book provides the practicing rheumatologist with a rapid, easy to consult reference to help interpret the nature of these cutaneous lesions and then quickly decide upon the appropriate follow-up tests. Utilizing an abundance of informative photographs, it contains all of the essential information needed for early diagnosis and treatment. This comprehensive guide properly orients the practitioner within the challenging world of rheumatologic disorders of the skin and gives insight into when consultation with a dermatologist is advisable.
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Part 1
Presentation of Skin Manifestation
Presentation of Skin Manifestation
Marco Matucci-Cerinic , Daniel Furst and David Fiorentino (eds.) Skin Manifestations in Rheumatic Disease 2014 10.1007/978-1-4614-7849-2_1 Springer Science+Business Media, LLC 2014
1. Approach to Patients with Skin Manifestations
Richard D. Sontheimer 1
(1)
Department of Dermatology, University of Utah School of Medicine, 30 North 1900 East #4A 330, Salt Lake City, UT 84132, USA
Richard D. Sontheimer
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Abstract
A hypothetical case of subacute cutaneous lupus erythematosus (SCLE) will be presented and discussed to illustrate the authors approach to diagnosis and management of complex medical dermatology problems involving rheumatologic diseases. The case discussion will involve three different time points in the patients disease course to illustrate key elements of the initial evaluation and diagnosis of such patients, the recognition and management of adverse effects of treatment, and the management of disease complications resulting from the development of overlapping autoimmune disorders during a patients disease course.
In this presentation, I will discuss a hypothetical case of subacute cutaneous lupus erythematosus (SCLE) representing a mosaic of several real-life patients for whom I have personally cared over the past three decades. The case will be discussed at three different time points in the patients disease course to illustrate my approach to the initial evaluation and diagnosis of such patients, recognition and management of adverse effects of treatment, and management of complications resulting from the failure to recognize clinical issues related to the development of overlapping autoimmune disorders over a patients disease course.
My Initial Interaction with Patient
When I first see the patient, I want to know what part of the body on which the skin change or rash first appeared. Some skin conditions reveal their identities by the regional skin anatomy that they prefer or tend to avoid. For example, the early inflammatory manifestations of cutaneous dermatomyositis prefer the stretch areas over the knuckles of the hands and fingers, while early cutaneous LE inflammation prefers the hair-bearing areas of skin overlying the dorsal aspects of the fingers between the knuckles. I want to know whether the skin change has been present continuously throughout the present illness or whether it waxes and wanes and whether environmental stimuli are associated with such cycles.
I then question the patient about self-treatments with over-the-counter products that may have been used for the skin problem as well as prescription treatments that have been given by physicians prior to the patients seeing me. Adverse reactions to prior treatments can sometimes mask the underlying primary skin problem. As an example, patients typically have used several over-the-counter products for their skin problem before seeing a dermatologist. When a topical sensitizing chemical (such as topical diphenhydramine, or Benadryl) touches the skin, a poison ivy-like allergic contact dermatitis reaction will develop several days after contact. Such superimposed, self-treatment-elicited skin changes can mask the underlying primary dermatologic process.
When managing chronic multisystem autoimmune disorders such as SLE, one must always keep in mind Greenwalds Law of Lupus. In 1992, Bob Greenwald, a rheumatologist, published his Law of Lupus. That law states that if a patient is diagnosed with SLE, there is a tendency to attribute (rightly or wrongly) everything that subsequently happens to the patient to SLE []. Banal skin changes such as rosacea are often confused with cutaneous LE by failure to apply this law. This is likely true for many of the connective tissue diseases.
Case Presentation
History of Present Illness . The patient is a 50-year-old white female who presented with a 6-month history of a persistent, non-pruritic rash that started initially on her arms and then spread to her upper chest, upper back, and neck. By history her central face had never been involved and she had never experienced similar skin changes below her waist. She had noticed that the rash worsened by sunlight exposure but indicated that some skin areas that were affected such as her shoulders and upper back were never exposed to sunlight. The patient had tried a nonprescription topical corticosteroid without benefit. Her primary care physician prescribed a topical cream containing both clotrimazole and betamethasone with only mild improvement of the rash. However, the rash returned quickly to its original appearance after this topical combination treatment was stopped.
Personal analysis of history of present illness . A chronic eruption presenting in an anatomical distribution such as this raises the question of a photosensitive cutaneous process (Table ). The absence of pruritus argues against photosensitive disorders that are characterized by pruritus including cutaneous dermatomyositis, solar urticaria, a photosensitive drug eruption, and polymorphous light eruption. Cutaneous lupus is a photosensitive disorder that characteristically does not cause significant itching, but as always in medicine there are exceptions.
Table 1.1
Photosensitive skin disordersa
Those not associated with a systemic illness |
Photosensitive drug eruptions |
Photoallergic contact dermatitis |
Polymorphous light eruption and its variants |
Solar urticaria |
Those that can be associated with a systemic illness |
Cutaneous LE |
Cutaneous dermatomyositis |
Porphyria/pseudoporphyria |
aExtremely rare causes of photosensitivity not relevant to this discussion were not included in this table (e.g., Blooms syndrome, xeroderma pigmentosum)
Some photosensitive disorders can display skin changes in areas not directly exposed to natural (sunlight) or artificial forms of ultraviolet light (e.g., cutaneous dermatomyositis, cutaneous LE, eczematous or lichenoid photosensitive drug eruptions) as well as in photoexposed areas. Typically, the rash starts in the areas of skin directly exposed to ultraviolet light and then spreads to contiguous nonexposed areas. Other photosensitive disorders characteristically produce skin involvement limited to areas directly exposed to ultraviolet light (e.g., polymorphous light eruption, solar urticaria, photoallergic contact dermatitis).
The patient denied using any over-the-counter topical products likely to contain contact-sensitizing chemicals (neomycin, bacitracin, diphenhydramine). Therefore, it is likely that the observed skin changes are the expression of the primary disease process rather than secondary changes produced by allergic contact dermatitis.
Clinical Context . The patients Past Medical History includes mild hypertension over the past 5 years currently controlled with medical therapy. For the past 10 years, the patient had been under medical care for gastroesophageal reflux disease. The patient has a 20-year history of hypothyroidism. Review of Systems The patient admitted to mild joint pains predominantly in her wrists and fingers over the past 3 months. She had also recently noticed the onset of malaise and easy fatigue upon exertion. Social History The patient has smoked one-half pack of cigarettes daily for the past 30 years. Family History The patients mother had a history of alopecia areata and her younger sister developed vitiligo as a youth. Current Medications Hydrochlorothiazide, lisinopril, omeprazole, and levothyroxine. Medication Allergies None known.
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