Tag Archives: Dermatology

#ClinicalPearls: Dermatology for the Internist

Today Dr. Heather Wickless from the Department of Dermatology at UT Southwestern gave a great, interactive lecture on skin conditions that internists should know about and how to evaluate and treat them. Below are takeaway points from her lecture:

  • Tinea versicolor is characterized by well-demarcated, tan, salmon, or hypopigmented patches, occurring most commonly on the trunk. KOH confirms the diagnosis.
  • Tinea pedis can be interdigital, moccasin, and vesiculobullous.
  • Candida intertrigo classically has satellite macules and papules or pustules around patches of erythema
  • If it scales, scrape it! KOH examination is the easiest and most cost effective method used to evaluate for fungal and yeast infections of the skin; culture can also aid in diagnosis.
  • Topical treatment is usually appropriate as a first-line agent for tinea pedis, tinea corporis, tinea versicolor, seborrheic dermatitis and candidal intertrigo, however oral medications are called for when involvement is extensive
  • Perioral dermatitis is an analogue of rosacea, and topical steroids may perpetuate the eruption.
  • When first line treatments fail, consider alternate diagnoses

Answer to Case Challenge #4

Case challenge # 4 presented a 21 yo white male with recurrent fevers and joint pains. Symptoms started at age 10, episodes occur every 6 months. Fever episodes last about 2 weeks, also associated with periorbital edema and spreading rash on extremities. Father has similar symptoms and also developed renal failure and CHF in his 40s. On exam, he had a fever of 102.3 F, periorbital edema, patchy macular rash on extremities.

What is the most likely diagnosis in this case?

A) Familial Mediterranean Fever

B) TNF Receptor-1 associated periodic syndrome

C) Hyper IgD syndrome

D) Trichinosis

E) Cryopyrin-associated periodic syndromes

Here are the results of the voting:
Screen Shot 2015-02-20 at 9.57.49 AM
The patient has TNF Receptor-1 Associated Periodic Syndrome (TRAPS).
  • Autosomal dominant, one of periodic fever syndromes
  • First described in pt of Irish (Hibernian) origin
  • Due to genetic defect in TNF receptor gene (TNFR1)
  • Presents with fever of longer duration (5-20 days) plus abdominal pain, characteristic rash, conjunctivitis and periorbital edema
  • 25% develop amyloidosis with renal or cardiac dz.
Look forward to a more extensive review of TRAPS. There will be a new case challenge next week!

Answer: Case Challenge #3

Case Challenge #3 presented a 19 year old white male is admitted with acute onset fever and abdominal pain. He was noted to have multiple recurrences over last 10 years, with each episode resolving in 3-4 days. On exam, he had a fever of 102.2F and peritoneal signs on exam. Labs revealed WBC 17k and a normal CTAP.

 

Which test is most likely to provide the diagnosis?

A) Measurement of C4 and C1 inhibitor levels

B) ANA and ENA panel

C) Colonoscopy with random biopsies

D) 24 hour urine collection for urine porphyrins

E) MEFV gene sequencing

 

Here are the results of the voting: Screen Shot 2015-02-13 at 8.58.07 AM

(Note: images may not display in your email – click on the link above to access the post on our site).

The diagnosis is Familial Mediterranean Fever, and the MEFV gene sequencing is most likely to provide the diagnosis.

  • Autosomal recessive disorder marked by episodic febrile attacks with serositis (peritonitis, pleuritis, arthritis)
  • Mutations in MEFV gene leading to ineffective pyrin regulation of inflammation
  • Ethnic predisposition: Armenians, Sephardic and Ashkenazi Jews, Turks, Greeks, N. Africans
  • Rx: Colchicine

Look forward to a more extensive review on Familial Mediterranean Fever!

Anticonvulsant Hypersensitivity Syndrome

  • General Information
    • Drug-induced, multiorgan syndrome – potentially fatal.
    • Reported with anticonvulsants such as carbamazepine, phenytoin, phenobarbital and lamotrigine
    • Other medicines, such as sulphonamides, sulphones, allopurinol and NSAIDs
  • Clinical Diagnosis
    • Triad: fever, rash, and organ involvement (i.e. hepatitis)
      • Fever: 90-100% of cases – characteristically high, spiking fevers. May persist for weeks after the offending drug is discontinued. The fever may precede or be concurrent with the cutaneous eruption.
      • Rash: 90% of cases – typically macular erythema that becomes confluent and may generalize into erythroderma. The face, trunk and upper limbs are the first to be involved, followed by the lower limbs. The rash may spare the face. Desquamation occurs with resolution. Periorbital and facial edema may be severe and occurs in 25% of cases. Blistering may be seen over edematous areas.
      • Organ Involvement:
        • Hepatitis: 50% of cases – usually mild, but can be severe. The mortality rate is between 18 and 40% if hepatitis is present. Liver function tests may be grossly elevated and continue to rise after the drug has been discontinued. Return of liver function tests to normal may take up to a year.
        • Tender lymphadenopathy: occurs in 70% of cases and may be either local or generalized.
        • Splenomegaly: may also be seen, but is less common.
      • Hematologic abnormalities: 50% of cases – Leukocytosis with atypical lymphocytes and eosinophilia (only in 20%)  can be seen. Coagulopathy may also occur.
    • Each clinical feature may be of variable onset, leading to confusion and delay in diagnosis
    • Interval between first drug exposure and symptoms is usually 2-4 weeks, but has been reported in individuals receiving anticonvulsants for 3 months.
    • Syndrome is more severe in previously sensitized individuals.
  • Mechanism
    • Some features to suggest that it is a form of allergic hypersensitivity.
    • Phenytoin is metabolized by cytochrome P-450 to intermediate metabolites, known as arene oxides. Arene oxides can contribute to an immunological response or even cause cell death.
    • Usually detoxified by epoxide hydroxylase, but those who develop AHS may have decreased enzyme function.
  • Treatment
    • Treatment of AHS is largely symptomatic.
    • The offending medicine should be immediately discontinued.
    • Topical steroids and antihistamines are helpful in controlling symptoms associated with the rash.
    • Systemic corticosteroids are often used, although there have been no trials to assess the efficacy of this treatment.
    • Relapse of the condition is often seen; thus, patients who have experienced AHS should avoid arene oxide anticonvulsants (carbamazepine, phenytoin and phenobarbital) in the future.

Answer to Case Challenge #2

Case Challenge #2 presented a 42 yo female with fever, LAD, macular rash, and elevated LFTs after the recent intiation of phenytoin for new onset seizures.

CC2

What is the best management step?

D) Stop phenytoin – the patient has Anticonvulsant Hypersensitivity Syndrome

  • Starts within 2-4 weeks of starting anti-epileptics with aromatic benzene rings (subset of DRESS)
  • Main culprits: Carbamazepine, Phenytoin, Primidone, Phenobarbital
  • Stop drug and avoid repeat exposure to similar drugs
  • Buzz words: Rash, LAD, hepatitis, renal failure in person on offending agent
  • Eosinophilia only seen in about 20% of cases

Look forward to a more extensive review of Anticonvulsant Hypersensitivity Syndrome. There will be a new case challenge next week!

Sweet’s Syndrome

Clinical Manifestations:

Sweet’s syndrome (the eponym for acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis. Several hundreds cases of Sweet’s syndrome have been published.

Presentation:

Sweet’s syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced. Classical Sweet’s syndrome (CSS) usually presents in women between the age of 30 to 50 years, it is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. Approximately one-third of patients with CSS experience recurrence of the dermatosis.

Associated Diseases:

The malignancy-associated Sweet’s syndrome (MASS) can occur as a paraneoplastic syndrome in patients with an established cancer or individuals whose Sweet’s syndrome-related hematologic dyscrasia or solid tumor was previously undiscovered; MASS is most commonly related to acute myelogenous leukemia. The dermatosis can precede, follow, or appear concurrent with the diagnosis of the patient’s cancer. Hence, MASS can be the cutaneous harbinger of either an undiagnosed visceral malignancy in a previously cancer-free individual or an unsuspected cancer recurrence in an oncology patient.

Drug-induced Sweet’s syndrome (DISS) most commonly occurs in patients who have been treated with granulocyte-colony stimulating factor, however, other medications may also be associated with DISS.

Pathogenesis:

The pathogenesis of Sweet’s syndrome may be multifactorial and still remains to be definitively established. Clinical and laboratory evidence suggests that cytokines have an etiologic role.

Diagnosis:

Sweet Syndrome Dx Criteria

Treatment:

Systemic corticosteroids are the therapeutic gold standard for Sweet’s syndrome. After initiation of treatment with systemic corticosteroids, there is a prompt response consisting of dramatic improvement of both the dermatosis-related symptoms and skin lesions. Topical application of high potency corticosteroids or intralesional corticosteroids may be efficacious for treating localized lesions. Other first-line oral systemic agents are potassium iodide and colchicine.

Second-line oral systemic agents include indomethacin, clofazimine, cyclosporine, and dapsone. The symptoms and lesions of Sweet’s syndrome may resolve spontaneously, without any therapeutic intervention; however, recurrence may follow either spontaneous remission or therapy-induced clinical resolution.

Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. By Philip R Cohen. Orphanet Journal of Rare Diseases 2007, 2:34  doi:10.1186/1750-1172-2-34

Answer: Case Challenge #1

Case Challenge #1 presented a 32 year old male with AML s/p induction chemotherapy who presents with fever and a rash. The skin biopsy showed dense neutrophilic infiltrates without vasculitis and negative infectious stains. This patient has:

B) Sweet’s Syndrome

  • General information:
    • Classified as neutrophilic dermatosis
    • Associated with heme malignancy, autoimmune disease or inflammatory bowel disease; onset described with marrow recovery from G-CSF
  • Exam: Abrupt papulonodular lesions on face, neck, hands with antecedent fevers
  • Biopsy: Neutrophilic infiltrates without organisms or vasculitis
  • Rx: steroids

Look forward to a more extensive review of Sweet’s Syndrome. There will be a new case challenge next week – get ready for it!

HOLIDAY BOWL THIS FRIDAY!

HOLIDAY BOWL
Friday, December 19th

Housestaff vs Faculty

Starting at noon in D 1.520

The faculty took the win last year in a close battle,
but the housestaff are ready to take back the crown!

(hint: read the blog)

Holiday Bowl and Xmas Sweater

Cutaneous manifestations of vasculitis…..there are many!

Yesterday at VA intern chart conference we had some great discussion about the skin manifestations of vasculitis. The morphology of these lesions can be quite variable and correlates with the pathologic process that is happening microscopically to the skin. In general, most vasculitis skin lesions are due to inflammation and damage to the vessels that leads to extravasation of RBC, which leads to NON-blanchable skin findings. Here are a few skin manifestations of vasculitis with definitions. Learn these terms and you will sound so impressive when you consult Dermatology 🙂

  • Petechiae: < 1-2 mm, non-blanchable, non-palpable pinpoint macules that result from capillary inflammation.
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  • Palpable purpura: Raised, non-blanchable lesions due to brisk inflammation of venules and arterioles leading to infiltrated erythematous papules and plaques.
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  • Hemorrhagic bullae: Small vessel involvement throughout the dermis that leads to necrosis of overlying tissue.
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  • Subcutaneous nodules: medium vessel inflammation of vessels within the deep dermis or subcutis leading to nodular lesions.
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  • Digital ulceration/necrosis: Ischemia due to vessel inflammation. The size and depth of the ulceration correlates to small vs medium vessel vascultiis.
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  • Livedo reticularis: Localized or wide-spread, patchy, reticulated vascular network of red-blue or violacious hues. Due to medium vessel blood flow compromise (can also be due to vasospasm or blood flow obstruction in other diseases).
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  • Urticaria – Uriticaria due to vasculitis tend to persist for > 24 hours and are frequently painful or burning vs the transient, pruritic lesions associated with non-vasculitis causes. Lesions may contain purpuric areas and can lead hyperpigmented lesions as they resolve.
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