P2422A case of pediatric palmoplantar eccrine hidradenitis

Daniela Kroshinsky, MD, SUNY Downstate Medical Center, Brooklyn, NY, UnitedStates; Sharon Glick, MD, SUNY Downstate Medical Center, Brooklyn, NY,United States

We present a case of an otherwise healthy 8-year-old girl with a 2-day history ofpainful nodules on the soles of her feet. Her history was unremarkable except forplaying in cold water and walking the rest of the day in wet sneakers. Since she wasunable to bear weight despite acetaminophen use, the patient was brought forevaluation to the Emergency Department. The patient was admitted with a diagnosisof vasculitis and a dermatology consultation was placed. On examination, the onlysignificant physical findings were several erythematous to violaceous, firm,blanching, exquisitely painful papulonodules on the soles of her feet and underher toes. A CBC, CMP, C3, C4, CH50, ANA, and dsDNA were within normal limits andher ESR and CRP were elevated at 34 and 65 respectively. The differential for pain-ful acral lesions included vasculitis, erythema nodosum, chilblains, and arthropodbites. Given the history and clinical findings, a diagnosis of palmoplantar eccrinehidradenitis (PEH) was made. Biopsy demonstrated a suppurative dermatitis of theeccrine glands in the deep dermis, thus confirming the clinical diagnosis. Thepatient was discharged home on bed rest and one week later, the lesions hadspontaneously resolved. A repeat CRP 2 days after admission had significantlydeclined to 20. PEH is a condition of painful papulonodules arising abruptly on thesoles and sometimes the palms of healthy children. The condition can be unilateralor bilateral and the pain is usually so severe that it inhibits the affected individual’sability to bear weight. Fortunately, spontaneous resolution usually occurs within 1 to4 weeks and recurrence is rare. Histologically, a neutrophilic infiltrate surroundsthe eccrine ducts, producing vacuolar degeneration or necrosis of the glandularepithelium. We will compare the features of PEH to Neutrophilic EccrineHidradenitis (NEH), a similar process found on the trunk. The etiology of PEH isunclear-inciting factors may include cold, sweat, or intense or prolonged physicalactivity inducing of eccrine gland rupture with the subsequent release of inflam-matory cytokines leading to neutrophil recruitment and propagation of inflamma-tion to form the characteristic lesions. The diagnosis is most often made clinically,with potential histologic confirmation. Usually, laboratory and radiological evalua-tion is unnecessary and our patient’s elevated CRP represents an unusual finding.The patient remains asymptomatic.

Commercial support: None identified.

P2424Linoleic acid deficiency in a 15-year-old cystic fibrosis patient presents asa recalcitrant eczematous dermatitis

Rachel Ness, MD, Marshfield Clinic Dermatology Department, Marshfield, WI,United States; Erik Stratman, MD, Marshfield Clinic Dermatology Department,Marshfield, WI, United States

Case Presentation: A 15-year-old with cystic fibrosis (CF), presents with an 8-monthhistory of progressive acral eczematous dermatitis with over 40% of his body sur-face recalcitrant to high potency topical steroids. Biopsy was consistent with aspongiotic dermatitis. Zinc levels were normal. An essential fatty acid (EFA) profilerevealed a deficiency in linoleic (18:2n-6) acid. An intensive inpatient skin therapyregimen was initiated consisting of an 18-hour daily sauna suit with a compoundedtriamcinolone 0.1% cream/10% canola oil (high in linoleic acid) and daily applicationof a linoleic acid containing emollient cream when out of the sauna suit. Patientwas discharged home on compounded triamcinolone 0.1% cream/10% canola oil,emollient, oral sunflower oil supplementation, and oral elemental zinc. Patientfollowed-up in 2 weeks following discharge with dramatic improvement. A repeatEFA profile revealed normalized linoleic acid levels.

Discussion: EFA deficiencies, especially low levels of linoleic (18:2n-6) acid are wellestablished in CF patients. Studies have demonstrated that EFA deficiency is presentin CF subjects in the absence of protein-energy malnutrition, indicating that thedeficiency is most likely secondary to specific defects in fatty acid metabolism.A deficiency of EFAs can present as a dermatitis, impaired immune and renalfunction, or alterations in membrane function leading to membrane system and cellstructure impairments. The CF-associated dermatitis often presents as discreteerythematous scaling papules that expand to desquamating plaques with a predi-lection for orifices, perineum, and extremities. Biopsy findings are consistent withan eczematous dermatitis. The etiology of the dermatitis in CF patients may includedeficiencies in EFAs, zinc, and proteins, and could be related to complex interac-tions of a combination of nutritional deficiencies. There is evidence that nutritionalsupplementation with oral linoleic acid preparations has demonstrated improve-ment in linoleic acid levels, although additional studies on precise guidelines areneeded. Topical supplementation is another area of potential research. This patientis much older than typical previously reported CF patients with linoleic acid-deficient dermatitis. Topical and oral linoleic supplementation was associated in thispatient with reversal of his recalcitrant dermatitis.

Commercial support: None identified.

P2425Midline, intragluteal cleft hemangioma associated with tethered spinalcord

Lilly-Rose Paraskevas, MD, State University of New York, Downstate MedicalCenter, Brooklyn, NY, United States; Sharon Glick, MD, State University of NewYork Downstate Medical Center, Brooklyn, NY, United States

We describe a 7-month-old boy with a midline hemangioma in the intragluteal cleftwho, after an MRI, was found to have a tethered spinal cord. Although midlinelumbosacral hemangiomas have been reported to be markers for occult spinaldysraphism, there have been few case reports documenting an association betweenoccult spinal dysraphism and lesions inferior to the sacrum. To our knowledge, thiscase represents a previously unreported association and we propose that intra-gluteal cleft hemangiomas be included along with lumbosacral hemangiomas as anindication for imaging studies in order to exclude the possibility of occult spinaldysraphism.

Commercial support: None identified.

P2423Focal epithelial hyperplasia: A case report and review of a theory fordisease susceptibility

Lindsey Bennett, MD, University of Wisconsin Madison, Madison, WI, UnitedStates; Molly Hinshaw, MD, University of Wisconsin, Madison, WI, United States

Focal epithelial hyperplasia (FEH) or Heck’s disease is an uncommon asymptomaticproliferation of oral mucosa that was first introduced into dermatology literature in1965. It usually presents in young Native Americans, from Central or South America,as multiple asymptomatic and occasionally painful exophytic papules or nodules onthe oral mucosa, gingiva, tongue, and lips. The disorder is due to human papillomavirus (HPV) infection and in over 90% of the cases HPV 13 or 32 is detected. Factorsthat determine disease susceptibility are unclear, but genetics, and having the HLA-DR4 (DRB1*0404) allele in particular, are thought to play a major role in thevulnerability to HPV 13 or 32 viruses. We report a case of FEH with positive in situhybridization results for HPV 13 in an otherwise healthy 9-year-old Hispanic femaleand review the current understanding and treatment of this uncommon disorder.

Commercial support: None identified.

FEBRUARY 2007 J AM ACAD DERMATOL AB159