Skyscape Insights: Dermatology
Specialty-Focused News for Skyscape Users
Clinical Case Study: Diagnosing and Treating Rare Skin Diseases
The Scenario
You are a dermatologist based in the Midwest. Today, you are seeing Monica, a 32-year-old white woman referred by her Internist for evaluation of painful red lesions on her forearm for the past 12 days.
You take a detailed history. She developed fever, headache, cough and a cold 2 weeks back. She self-administered some OTC "cold and cough medication" for these symptoms. Within a day, she noticed multiple reddish "bumps" on her forearm. She says that the bumps "came together" to form the present lesions. She has had similar episodes, about 2-3 times over the past year. In the past, she reports the lesions were milder and gradually disappeared over several months. However, the current episode has more widespread and painful lesions which has now led to her seeking your opinion.
On examination, her vitals are as follows: HR:120/min, BP 120/70 mmHg, Temp 39.3°C (102.7°F) , RR 25/min. CV/Resp examination is normal, except for tachycardia. Abdomen and CNS exams are unremarkable.
Dermatological examination reveals the following:
- Multiple, somewhat symmetrical erythematous papules and nodules over both the forearms
- Several plaques formed by coalescing of nodules. A central area of necrosis is obvious in the plaques, giving it a targetoid appearance
- Few lesions, which appear to be vesicles, are actually nodules on palpation
There is no peripheral nerve thickening or hypopigmentation. The rest of the skin elsewhere, including palms and soles, are normal. Scalp, hair, nail and mucous membrane are normal.
From the history and presentation, you suspect erythema multiforme, erythema nodosum or drug eruption. However, the examination doesn't truly fit with any of these possibilities.
You decide to send baseline blood work, including CBC, peripheral smear, erythrocyte sedimentation rate (ESR), comprehensive panel, and C-reactive protein (CRP). The significant results are:
- WBC's: 10,000 cells/mm3 with 78% neutrophils
- ESR: 50 mm/hr
- Comprehensive panel and CRP are completely normal
Given the lack of clear diagnosis, you have biopsied a lesion. You receive the report from dermatopathology of "neutrophilic infiltration of the dermis and mild vasodilatation."
To get further assistance in diagnosing the condition, you decide to refer to your Skyscape resources installed on your Android smartphone.
You begin with Fitzpatrick’s Atlas of Dermatology and as you search for images for your differential diagnoses, you come across an image of a patient with Sweet’s Syndrome. You notice that the lesions are similar to what Monica has. You decide to read the section on clinical manifestations of Sweet’s syndrome and note that bright red, smooth painful papules which coalesce to form irregular plaques along with pseudovesicle formation are characteristic of this condition. Acute onset and central clearing of plaques further support the diagnosis.
Since Sweet’s syndrome is a rare condition, you only vaguely recall the diagnostic criteria. You look through
Diagnostic Criteria in Dermatology and on searching for Sweet’s syndrome; you confirm that there are 2 sets of diagnostic criteria. The more recent one, known as the von den Driesch Criteria (1994) requires the following:
Both major criteria
PLUS >=2 minor criteria
Major Criteria:
- Abrupt onset of painful or tender erythematous plaques or nodules (occasionally with vesicles, pustules, or bullae)
- Predominance of neutrophilic infiltration in the dermis in the absence of leukocytoclastic vasculitis
Minor Criteria:
- Preceded by non-specific respiratory or gastrointestinal tract infection or vaccination or associated with:
Inflammatory disease (e.g. chronic autoimmune disorders, infections)
or
Hematoproliferative disorders or solid malignant tumors,
or
Pregnancy
- Accompanied by fever (>38°C) and generalized malaise
- >=3 out of 4 abnormal laboratory values during the onset:
- ESR >20 mm
- +ve C reactive protein
- >70% segmented nuclear neutrophils and stabs on peripheral blood smear
- Leukocytosis (>8,000/mm3)
- Excellent response to treatment with systemic steroids or potassium iodide
From the above criteria, biopsy, and clinical presentation, it is your opinion that Monica probably has Sweet’s syndrome. You treat her with 30 mg prednisone per day tapered to 10 mg over 2 weeks. During her follow-up visit two weeks later, you see that the lesions have nearly disappeared. The excellent response makes you to taper the drug further to 5 mg and withdraw it completely after another 1 week.
Your Skyscape resources have once again helped you in correctly identifying and managing a rare skin disorder.
Drug Topic: Vaccine for Advanced Melanoma Yields Promising Results
A phase 3 trial compared those given a vaccine and interleukin-2 or just interleukin-2 alone. The vaccine group had better outcomes. In the combination group, a percentage of people (16%) had 50% shrinkage in tumors. The progression-free survival was also longer in the vaccine group, 2.2 months versus 1.6 months.
http://www.nejm.org/doi/full/10.1056/NEJMoa1012863
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Product Spotlight: Diagnostic Criteria in Dermatology
Diagnostic Criteria in Dermatology provides dermatologists and other physicians with the most up-to-date and evidence-based diagnostic criteria for dermatologic diseases and syndromes. For a number of conditions in dermatology, evidence-based diagnostic criteria have been developed to improve the diagnostic acuity. An accurate diagnosis is essential for proper patient management.
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