Circumscribed Palmar Hypokeratosis With Superimposed Actinic Keratosis.

A man in his late 70s with a history of psoriasis and non-melanoma skin cancer presented with a progressive rash on his right thenar eminence. He first noticed it about one year ago. He denied any pruritus in the affected region but did note some overlying skin breakdown. He had used topical betamethasone and calcipotriene cream in the past with minimal improvement. Physical examination revealed a pink atrophic plaque with linear hyperkeratotic borders and central fissuring on the right thenar eminence extending into the first webspace. A shave biopsy revealed hypokeratosis with a rim of surrounding hyperkeratosis and associated parakeratosis, basal keratinocyte atypia, and lichenoid inflammation. These histopathological features were consistent with circumscribed palmar hypokeratosis and central actinic keratosis. Circumscribed palmar hypokeratosis is often considered a benign entity, but there have been reports suggesting an association with premalignancy. The decision was made to treat with 5-fluorouracil and calcipotriene cream twice daily for six weeks. At his two-month follow-up, he endorsed a robust reaction, which was further suggestive of premalignant change. He had a near-complete resolution of the rash. This case features circumscribed palmar hypokeratosis and suggests a novel treatment option for patients who develop concomitant actinic keratosis.

Severity of multisystem inflammatory syndrome in children (MIS-C) in patients with and without mucocutaneous findings.

BACKGROUND: There are few studies comparing severity of MIS-C disease with mucocutaneous symptoms, age, race, and ethnicity. OBJECTIVE: To describe the mucocutaneous symptoms present on admission and evaluate whether these symptoms are correlated with a more severe MIS-C disease course. METHODS: Retrospective cohort study of hospitalized patients with suspected MIS-C between May 13, 2020 to April 21, 2021. RESULTS: Of the 66 patients who met the inclusion criteria, 84.8% (56/66) exhibited mucocutaneous findings. The most common mucocutaneous symptoms were rash, conjunctivitis, cracked lips, and sore throat. Children with mucocutaneous symptoms were younger (median 9.8 years) compared to those without (11.4 years), p = .39. The groups had similar proportions of pediatric intensive care unit admission, abnormal cardiology studies, and necessity of pressors. The presence of mucocutaneous findings on admission was associated with a lower troponin level on admission (median 0.08 ng/ml vs. 0.52, p = .003). Black children had higher odds of severe MIS-C compared to White children (odds ratio [95% CI]: 3.30 [1.02, 10.72], p = .047). Children ≥5 years of age had greater odds of severe MIS-C compared to children <5 years of age (odds ratio [95% CI]: 5.43 [1.39, 21.23], p = .02). LIMITATIONS: The sample size was relatively small, there was no dermatologist present on admission, initial diagnostic testing and management varied if patients presented at outside hospitals, and the CDC case definition for MIS-C was highly sensitive. CONCLUSION: The presence of mucocutaneous symptoms negatively correlated with troponin levels, but there was no significant association between these symptoms and other markers of cardiac involvement (echocardiogram, ejection fraction, electrocardiogram).

Hyperphosphatemic Tumoral Calcinosis With Pemigatinib Use.

Background/Objective: Pemigatinib, a fibroblast growth factor receptor (FGFR) 1-3 inhibitor, is a novel therapeutic approach for treating cholangiocarcinoma when an FGFR fusion or gene rearrangement is identified. Although the most reported side effect of pemigatinib is hyperphosphatemia, tumoral calcinosis with soft tissue calcifications is not widely recognized as a complication. We report a case of patient with hyperphosphatemic tumoral calcinosis on pemigatinib. Case Report: A 59-year-old woman with progressive metastatic cholangiocarcinoma, despite receiving treatment with cisplatin and gemcitabine for 7 months, was found to have an FGFR2-BICC1 fusion in the tumor on next-generation sequencing. Pemigatinib was, therefore, initiated. Four months into the therapy, multiple subcutaneous nodules developed over the lower portion of her back, hips, and legs. Punch biopsies revealed deep dermal and subcutaneous calcifications. Investigations revealed elevated serum phosphorus (7.5 mg/dL), normal serum calcium (8.7 mg/dL), and elevated intact fibroblast growth factor-23 (FGF23, 1216 pg/mL; normal value <59 pg/mL) levels. Serum phosphorus levels improved with a low-phosphorus diet and sevelamer. Calcifications regressed with pemigatinib discontinuation. Discussion: Inhibition or deficiency of FGF-23 results in hyperphosphatemia and can lead to ectopic calcification. Pemigatinib, a potent inhibitor of FGFR-1-3, blocks the effect of FGF-23 leading to hyperphosphatemia and tumoral calcinosis as observed in our case. Treatment is aimed primarily at lowering serum phosphate levels through dietary restriction or phosphate binders; however, the regression of tumoral calcinosis can occur with pemigatinib cessation, as seen in this case. Conclusion: As the use of FGFR 1-3 inhibitors becomes more prevalent, we aim to raise attention to the potential side effects of tumoral calcinosis.

A rare presentation of herpes simplex virus encephalitis occurring in a pediatric patient on dupilumab for atopic dermatitis.

A 4-year-old female with a history of atopic dermatitis developed herpes simplex virus (HSV) encephalitis while being treated with dupilumab and concomitant topical steroids. There was no prior history of HSV infections or immunodeficiency. To our knowledge, this is the first case of HSV encephalitis in a patient receiving dupilumab.