Wednesday, September 22, 2010

Palmoplantar Erythrodysesthesia Syndrome

Presented by Yoon Cohen, MS IV
University of New England College of Osteopathic Medicine


Abstract: 45 yo woman with a metastatic breast cancer and a painful hand/foot dermatitis.

HPI: This is a 45 yo woman who was diagnosed with a metastatic breast cancer a few years ago. She has developed "atopic dermatitis" like symptoms on her hands and feet since starting capecitabine. She has completed 5 cycles of Xeloda (capecitabine) and has had to reduce the dose because of this condition. The lesions started as dry edematous, erythematous areas on palms and soles with a tingling sensation.

O/E: The examination reveals general areas of desquamation and hyperlinearity with mild erythema on both palms. This is best shown on the first photograph. 

Clinical photographs:





Diagnosis: Palmoplantar erythrodysesthesia syndrome, hand-foot syndrome, chemotherapy-induced acral erythema

Discussion:
Chemotherapy-induced acral erythema or palmoplantar erythrodysesthesia syndrome is a well-defined reaction to some of the chemotherapeutic agents such as methotrexate, cytarabine, doxorubicin, fluorouracil, cytosine arabinoside, and bleomycin. This reaction is characterized by symmetric, well-demarcated, painful erythema of the palms and soles, which may progress to desquamation or blisters. It appears to be dose dependent, and is likely a direct toxic effect of the drug. Tingling on the palms and soles is followed in a few days by painful, symmetric, well-defined swelling and erythema [4].

Questions:
1. Please feel free to share your experiences with treatment options.

References:
1. Marini A, Hengge UR. Hand-foot syndrome with capecitabine therapy. Hautarzt. 2007 June; 58(6):532-6
Abstract: A 72-year-old patient with esophageal carcinoma developed a severe hand-foot syndrome during second-line therapy with the oral fluoropyrimidine capecitabine. We also summarize the current knowledge with regard to the hand-foot syndrome and distinguish it from palmoplantar erythrodysesthesia.

2. Degen A, Alter M, Satzger I, et al. The hand-foot-syndrome associated with medical tumor therapy- classification and management. J Dtsch Dermatol Ges. 2010 Sep; 8(9):652-61
Abstract: The hand-foot-syndrome (HFS, palmoplantar erythrodysesthesia, chemotherapy-associated acral erythema) is characterized by painful predominantly palmo-plantar lesions. The association with different chemotherapeutic agents has been known for over 20 years. More recently, HFS has been reported in association with regimens using targeted agents, in particular the multikinase inhibitors (MKI) sorafenib and sunitinib. The HFS associated with MKI has a different distribution and clinical appearance than the traditional disorder. In this review, similarities and differences between chemotherapy- and MKI-associated HFS are discussed and current recommendations for their prophylaxis and management are summarized.

3. Janusch M, Fischer M, Marsch WCh, et al. The hand-foot syndrome - a frequent secondary manifestation in antineoplastic chemotherapy. Eur J Dermatol. 2006 Sep-Oct; 16(5): 494-9
Abstract: The hand-foot syndrome (HFS) (palmoplantar erythrodysesthesia) designates acute, painful erythemas of the palms and soles of the feet caused by antineoplastic chemotherapies. The most frequent trigger substances are 5-fluoruracil and its derivates. At maximum severity, the HFS is bullous to erosive or ulcerous in character. The pathogenesis has not yet been clarified. Histologically, the HFS is characterized by a toxic keratinocyte reaction. Furthermore, there is sub-basal edema with a tendency to bullae, dilated blood and lymph capillaries and usually only mild perivascular lymphocytic infiltration. Early recognition and delineation from other differential diagnoses is prerequisite to targeted management of the disease. Depending on the severity, HFS requires dose reduction, interruption or switch in the antineoplastic chemotherapy. (You can access to the article at http://www.john-libbey-eurotext.fr/e-docs/00/04/26/D7/vers_alt/VersionPDF.pdf)

4. Habif TP. Clinical Dermatology. 5th edition. USA: Elsevier Science 2010