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CASE #1
A nurse, age 54 years, arrived for evaluation of what she and her fellow hospital workers presumed to be lupus. The rash had been present for approximately one month. Such systemic symptoms as joint pain, fever and malaise were not reported. The rash burned and itched and had persisted despite the application of a number of OTC creams and ointments. An antinuclear antibody (ANA) test ordered by the patient’s primary-care provider was nonreactive. A complete blood count and comprehensive metabolic profile showed no abnormal results.
CASE #2
A 26-year-old woman first noticed a facial rash early in the summer but had to wait several weeks before she could be seen in the dermatology clinic. Feeling the need to try something in the meantime, she used OTC moisturizers and 1% hydrocortisone cream to obtain relief from mild itching and burning. Neither of the OTC treatments was helpful, and the rash grew more erythematous. The woman’s symptoms were particularly exacerbated after a day spent in the sun working as a landscaper.
What is the diagnosis?
For CASE #1, click “NEXT.” For CASE #2, click “3.”
CASE #1: Seborrheic dermatitis
Every medical student hears about the butterfly rash of lupus. However, the classic bimalar rash of lupus is not at all common, even in confirmed lupus cases. Additionally, as with virtually all medical diagnoses, there is a differential for florid facial rashes that includes a number of far more common conditions, including contact vs. irritant dermatitis, psoriasis, and seborrheic dermatitis.1
This patient’s rash was reddish-orange and florid, affecting sharply demarcated convex areas of the cheeks, chin and forehead, where the lesions took on more of an annular morphology. The nasal bridge was spared. Little if any edema was appreciated, and there was no increase in surface warmth in the areas. A KOH examination of scrapings from the surfaces of the lesions showed no fungal elements or signs of Demodex, a mite that is part of normal flora inhabiting follicles and sebaceous glands, especially in periocular areas, and occasionally implicated in rosacealike facial eruptions.2
Approximately 95% of systemic lupus cases are ANA-positive. The negative ANA in this case served only to rule out systemic lupus erythematosus, since seronegative cutaneous lupus can still present in a wide variety of ways, including facial involvement. A 3-mm biopsy was performed and showed none of the features consistent with lupus (i.e., vacuolar interface dermatitis, a universal finding in active lupus lesions). Such other findings consistent with lupus as parakeratosis and mild hyperkeratosis were also missing in this case.1
Instead, spongiotic dermatitis with mild follicular accentuation was seen in this patient. While not diagnostic, these changes made lupus less likely, as did the lack of such other findings as atrophy, dilated follicles or alopecia.2
Clinicians were also able to rule out sarcoidosis, polymorphous light eruption, lichen planus, tertiary syphilis and lymphocytic infiltration of Jessner, all of which would have shown findings suggestive — if not diagnostic — of those entities.
Unfortunately, a diagnosis of spongiotic dermatitis, however useful, only gains the clinician admission to a rather large ballpark and not to a specific seat. Commonly seen in irritant and contact dermatoses, spongiosis simply implies intercellular edema of the epidermis triggered by inflammation.3
“Clinical correlation” comes into play at this point, which involves a more in-depth discussion with the patient to determine exactly when and how this condition started and whether there is any history of skin problems or other significant medical problems or family history of such skin diseases as psoriasis. It is also helpful to ask which products had been used to treat the rash.
The woman described in this case was desperate to obtain relief and had used a number of products on her face over the first few weeks of her rash. She was advised to stop all topical products except those prescribed by a clinician. It is extremely common for a patient’s self-prescribed “treatment” to cause far more problems than the original condition he or she set out to remedy. Neither stopping OTC treatment nor ceasing b.i.d. application of topical desonide (DesOwen, Tridesilon) lotion was successful in controlling the woman’s rash.
There was no family history of skin disease, but the patient did recall having a milder version of her current outbreak on a number of occasions over the years. In the past, these eruptions had always involved the same areas of her face as well as the scalp and bilateral postauricular skin. These episodes always were preceded by periods of exceptional emotional stress. The patient did confirm that similar conditions had preceded this most recent eruption, and examination confirmed the involvement of the skin behind and in both ears with a shiny and scaly rash similar to the one on her face.
A diagnosis of seborrheic dermatitis — possibly worsened by irritation brought on by use of OTC products — was eventually made. Seborrheic dermatitis is an extremely common papulosquamous condition that affects up to 20% of the population. Its most common form is that of dandruff, but seborrheic dermatitis also manifests as a minimally scaly and pinkish-red rash on nasolabial folds, beard area, glabella, eyebrows, and in and behind the ears. Thought to represent an abnormal response to the commensal yeast Malassezia furfur, seborrheic dermatitis can affect a wide array of other anatomic areas as well, including the trunk, chest, axillae, and genitals.4
The patient was successfully treated with b.i.d. application of diflorasone (Apexicon, Maxiflor, Psorcon) ointment, a relatively powerful (Class 2) topical steroid that is seldom used on the face. The diflorasone was tapered down over a period of two weeks and was gradually replaced by tacrolimus (Hecoria, Protopic) ointment, a topical macrolide immunosuppressant.5 She was also advised to wash her face twice daily with OTC ketoconazole (Nizoral) shampoo to reduce the numbers of M. furfur yeast. Within three weeks, the patient was able to stop all medications.
Since a permanent cure is not available, treatment of seborrheic dermatitis usually focuses on control with a combination of topical steroids and such antifungal measures as imidazole creams or shampoos used as face wash. Seborrheic dermatitis is usually obvious clinically, but as this case illustrates, it can be complicated by patient and provider misdiagnosis.
CASE #2: Chronic cutaneous lupus
Facial rashes, an extremely common complaint, are typically diagnosed and treated with relative ease. But there is a bewildering and extensive range of possible explanations for facial complaints that falls outside the usual and customary. For obvious reasons, patients are often very concerned about their appearance, especially early on when a clear diagnosis and effective treatment are elusive.
By the time this patient was examined by dermatology, both sides of her face were quite red. Discrete and confluent blanchable papules, nodules and plaques (many of which were polycyclic) with clearing and slightly atrophic centers were noted. Little if any scale was seen, although the patient reported scrubbing fine scale away each morning and night. Her lesions were confined to sun-exposed skin and stopped abruptly at her neckline.
The patient reported no fever, joint pain or malaise and denied any personal or family history of similar problems. Strongly suspecting lupus, the provider obtained a 3 mm punch biopsy specimen from the peripheral face and submitted it to pathology with a working differential of lupus vs. polymorphous light eruption. At the same time, an additional specimen was obtained for separate processing and sent to pathology in Michel’s transport medium for direct immunofluorescence studies.
A dermatopathologist was able to verify the diagnosis of probable subacute cutaneous lupus erythematosus (SCLE), a clinically distinct form of lupus first described in 1979.6,7 Pathology showed vacuolar interface dermatitis but no follicular plugging, basement membrane thickening, or heavy lymphoid aggregates, which might have suggested discoid lupus (DLE).
Differential item functioning studies were positive for a dustlike particulate deposition of immunoglobulin G in epidermal nuclei, also consistent with what is seen in approximately one third of individuals with SCLE.8
SCLE is mostly found in white women aged 15 to 40 years. Lesions can be scaly and evolve as polycyclic annular lesions with clearing centers.6
Unlike DLE, SCLE lesions seldom involve the follicle, do not scar, and tend to be transitory. However, patients with SCLE can have concomitant DLE in other locations. As seen in this case, photosensitivity is a prominent feature in the majority of individuals with SCLE.
Bloodwork from this patient demonstrated a positive ANA, as is the case approximately 80% of the time with SCLE.9 The rest of this patient’s lupus profile is pending, but a positive Ro/SSA antigen is expected.
Clinically, most cases of SCLE run a mild course and respond to sun protection and the use of antimalarials. Topical steroids are usually not necessary but can be useful for local control.10
Drugs that can trigger an eruption similar to that of SCLE include hydrochlorothiazide, ACE inhibitors and calcium channel blockers, among others.
Besides the differential mentioned above, other items to be considered include erythema multiforme, psoriasis, tertiary syphilis and dermatomyositis.
The woman in this case will be managed by dermatology and rheumatology, given her potential for systemic involvement, which can include such overlap syndromes as Sjögren’s. It is possible that she will have to be treated with multiple systemic medications in the event of nonresponse to conservative treatment. Because of the role sun exposure played in the genesis of the patient’s disease, she was advised to consider changing careers.
Joe R. Monroe, MPAS, PA, is a physician assistant specializing in dermatology at Dawkins Dermatology in Oklahoma City.
References
1. Medscape Reference. Seborrheic dermatitis.
2. James WD, Berger TG, Elston DM. Seborrheic dermatitis. In: Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, Pa.: Saunders-Elsevier; 2006:191-193
3. Belew PW, Rosenberg EW, Jennings BR. Activation of the alternative pathway of complement by Malassezia ovalis (Pityrosporum ovale). Mycopathologia. 1980;70:187-191.
4. Green CA, Farr PM, Shuster S. Treatment of seborrhoeic dermatitis with ketoconazole: II. Response of seborrhoeic dermatitis of the face, scalp and trunk to topical ketoconazole. Br J Dermatol. 1987;116:217-221.
5. Meshkinpour A, Sun J, Weinstein G. An open pilot study using tacrolimus ointment in the treatment of seborrheic dermatitis. J Am Acad Dermatol. 2003;49:145-147.
6. James WD, Berger TG, Elston DM. Connective tissue diseases. In: Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, Pa.: Saunders-Elsevier; 2006:157-166.
7. Sontheimer RD. Subacute cutaneous lupus erythematosus: 25-year evolution of a prototypic subset (subphenotype) of lupus erythematosus defined by characteristic cutaneous, pathological, immunological, and genetic findings. Autoimmun Rev. 2005;4:253-263.
8. Medscape Reference. Subacute cutaneous lupus erythematosus (SCLE).
9. Fabbri P, Cardinali C, Giomi B, Caproni M. Cutaneous lupus erythematosus: diagnosis and management. Am J Clin Dermatol. 2003;4:449-465.
10. Wozniacka A, McCauliffe DP. Optimal use of antimalarials in treating cutaneous lupus erythematosus. Am J Clin Dermatol. 2005;6:1-11.
All electronic documents accessed May 9, 2012.
