The Dermatologist’s Board Review

The content of these questions and answers are taken from the Galderma Pre-Board Slide Seminar. The course was most recently held July 17-19, 2009, at the Crowne Plaza Chicago O’Hare, Rosemont, IL. Questions

1. This condition:
a) is endemic in the Southeast United States.
b) is associated with high antibody titers in self-limited infections.
c) is a highly contagious infection.
d) can occur in epidemics due to natural events.
e) is usually symptomatic with signs of pneumonia and lung nodules.

2. The most common predisposing factor to this condition in black patients is:
a) basal cell nevus syndrome.
b) scarring conditions, such as cutaneous lupus and burns.
c) ultraviolet exposure history.
d) albinism.
e) chemical carcinogens.

3. This condition:
a) is not related to the PTCh gene.
b) is an autosomal recessive disorder.
c) does not occur in black patients.
d) is related to a gene on Chromosome 9.
e) is caused by mutation in an oncogene.

Answers

1. Coccidiomycosis This condition: d) can occur in epidemics due to natural events. This condition is a non-contagious infection. Causes include dimorphic fungus and inhalation of arthroconidia in soil. Hosts are mammals, including dogs and horses. Cell-mediated immunity establishes host defense. Self-limited infections have low ab titer and high DTH. Disseminated infections have high ab titers and diminished DTH.

• Epidemiology: 100,000 cases per year. Endemic in California, Arizona, New Mexico,and Texas. Incidence affected by rainfall and season. (Arizona winter is highest.) Cities with highest rates: Bakersfield, CA; Phoenix, AZ; Tucson, AZ. Epidemics from dust storms, earthquakes, droughts, occupational and military exposure. Host risk factors: Immunocompromised patients at higher risk of contraction; Filipino and black patients at increased risk of disseminated disease. Retrospectively, 13 patients on TNF-a inhibitors had coccidioidomycosis, 2 on methotrexate and infliximab died of dissemination.
• Clinical features: 60% asymptomatic. Flu-like symptoms resolving over 2 to 3 weeks. Lungs are prime focus of infections: pneumonia, pleural effusion, hilar adenopathy and lung nodules. Dissemination (1%): skin, meninges, bones and joints.
• Cutaneous lesions: Erythema nodosum (EM) within 48 hours of symptoms, especially in white females (sign of increased cell-mediated defense), which is associated with good prognosis, particulary in pregnant patients. Exanthem, possibly oral, followed by palmar desquamation. Sweets syndrome. Interstitial granulomatous dermatitis, mixed infiltrate with eosinophils, neutrophils, leukocytoclasia. Disseminated organisms (months to years after diagnosis) systemic and localized. Primary cutaneous rare; only 20 cases, seen in agricultural workers, lab workers, embalmer, splinter injury/laceration.
• Diagnosis: Serology, including low titers (1:2) of complement fixing ab. Titer correlates with disease severity. False negatives in a few HIV patients. Culture: Rapid growth of white cottony colonies. Endospores within spherules. PCR in paraffin embedded tissue. Skin testing: DTH, limited in acute disease. Imaging: abnormal chest X-ray (75%): segmental pneumonia, nodules, lymphadenopathy, effusions, CT for disseminated
• Treatment: Itraconazole 200 mg bid for 3 to 6 months or fluconazole 400 mg per day for 3 to 6 months. Those at risk for dissemination: HIV, transplant, at-risk races; all should be treated. Pregnant patients: preferred treatment is amphotericin (azoles contraindicated). Immunocompetent with primary pulmonary: no therapy, followup chest X-ray for 2 years to confirm resolution.

2. Skin Cancer in Skin of Color The most common predisposing factor to this condition in black patients is: b) scarring conditions such as cutaneous lupus and burns.

• Epidemiology: 2% to 4% neoplasms in Asians and 1% to 2% neoplasms in blacks. Incidence 232/100,000 in Caucasians, 3.4/100,000 in black patients; mortality in black patients remains high.
• Role of UV radiation Inherent SPF of 13.4 (approximately) in blacks due to increased melanocyte activity and melanosomes in darker skin. Black epidermis transmits 7.4% of UVB and 17.5% of UVA vs. 24% and 55% in Caucasians; MED score is 6% to 30% greater in darker skin tones. Incidence of NMSC in Japanese patients in Hawaii is 40 times less than Caucasians in same area. UV may not be as important an etiologic factor in darker skin types. UVR increases NMSC in sun-exposed skin in Caucasian patients. Decreasing latitude increases MSC and NMSC in Caucasians; decreased level of skin pigmentation also increases NMSC. Asian patietns are an exception in skin of color; fairer Chinese patients had 2 times increase in skin cancer. BCC is 2 times greater in Japanese in Hawaii than those in Japan. While BCC has definite link with UV in black patients, UVR exposure and NMSC in black patients is unclear. One study showed that 60% of BCC patients had fairer skin vs. 10% in control. Another study showed increase with decreasing latitude. Study in Africa showed increased BCC in albino blacks. Melanoma and SCC are less clearly related to UVR in black Americans.
• SCC: Most common cutaneous malignancy in blacks and Asian Indians; 30% to 65% of skin cancers, respectively. Anogenital SCC: 10% to 23%; penis and vulva third most common site. Penile SCC in blacks has higher stage and greater mortality. SCC in situ is rare, onset age usually older than 60. Pigmented SCC in situ mimics melanoma. Chronic scarring processes most common predisposing factors in black patients (20% to 40% of patients with SCC). Metastatic SCC associated with the following in 20% to 40% of patients: burns, radiation sites, ulcers, discoid lesions of lupus, GA, Leprosy, osteomyelitis, hidradenitis. Other risk factors include albinism, HPV, EDV, immunosuppression, chemical carcinogens (tar and arsenic). Mortality is 25% for black patients.

3. Skin Cancer in Skin of Color This condition: d) is related to a gene on Chromosome 9. BCC is the most common skin cancer in Caucasians, Hispanics, Chinese and Japanese. It is 19 times more common in Caucasians than American blacks, whose prevalence is 1% to 2%. UVR related to BCC in all races. Rarely occurs in non-exposed sites. Nearly always occurs after 5th decade of life in all races. Radiation has mild increase in BCC incidence in blacks as opposed to Caucasians, who have greatly increased risk. Other risk factors for black patients include: scars, albinism, nevus sebaceous, arsenic, immunosuppression, xeroderma pigmentosa, trauma. Not associated with increased mortality (as opposed to SCC). BCC syndrome: Autosomal dominant, PTCH gene, Chromosome 9, tumor suppressor gene. Occurs in blacks. Multiple BCC in skin of color should prompt suspicion. Melanoma: Incidence increasing for Caucasians. Sixth most common cancer overall in the United States with 59,000 cases per year. White to black ratio in United States is 16:1; Hispanics have intermediate incidence between black and white patients. Mortality: third highest of all cancers. Risk factors include blistering and repeated intense sun exposure for Caucasians, atypical/multiple nevi, family/personal history of melanoma, skin types I and II.