• 45 yo female with a hx of migraines & excessive sun exposure • Presents with complaints of a very pruritic rash on her abdomen, buttocks, & lower extremities. • 2 days prior, took acetaminophen-butalbital- caffeine & consumed 2 new cooking ingredients: Malanda (Xanthosoma sagittifolium) & boniato (Ipomoea batatas) • Pt denies any prior occurrences or any other associated symptoms. • On exam, patient presented with multiple erythematous, hyperpigmented linear streaks scattered on bilateral legs, buttocks, & inferior abdomen consistent with flagellate erythema. Excoriations were diffusely present. • Histology: a dense, perivascular lymphocytic infiltrate with very few eosinophils & marked dermal edema. Melanin diffusely scattered within epidermal basal layer but not within the dermis. No iron dermal deposition. • Treatment: Stop all recent medications & cooking ingredients, 40mg of IM triamcinolone acetonide, triamcinolone acetonide 0.1% topical cream BID x 2 wks, & fexofenadine 180mg PO QD • 2 wks after visit, the patient cooked & consumed food containing malanga & boniato again. She experienced diffuse pruritus, but denied any rash. Pruritus was relieved with diphenhydramine. • At 3 wk follow-up, pt showed improvement of rash & pruritus, & was instructed to continue her fexofenadine. INTRODUCTION CLINICAL & HISTOPATHOLOGICAL IMAGES TREATMENT REFERENCES 1. Moulin, G., B. Fiere, and A. Beyvin, [Cutaneous pigmentation caused by bleomycin]. Bull Soc Fr Dermatol Syphiligr, 1970. 77(2): p. 293-6. 2. Nousari, H.C., et al., "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis. J Rheumatol, 1999. 26(3): p. 692-5. 3. Bolognia, J., Jorizzo, J. L., & Schaffer, J. V., Dermatology. 2012, Philadelphia: Elsevier Saunders. 4. James W, B.T., Elston D, Andrews Diseases of the Skin Clinical Dermatology. 11th ed. 2011: Saunders Elsevier. 5. Callen, J.P. and R.L. Wortmann, Dermatomyositis. Clin Dermatol, 2006. 24(5): p. 363-73. 6. Suzuki, K., et al., Persistent plaques and linear pigmentation in adult-onset Still's disease. Dermatology, 2001. 202(4): p. 333-5. 7. Hanada, K. and I. Hashimoto, Flagellate mushroom (Shiitake) dermatitis and photosensitivity. Dermatology, 1998. 197(3): p. 255-7. 8. Yamamoto, T. and K. Nishioka, Flagellate erythema. Int J Dermatol, 2006. 45(5): p. 627-31. 9. Fernandez-Obregon, A.C., K.P. Hogan, and M.K. Bibro, Flagellate pigmentation from intrapleural bleomycin. A light microscopy and electron microscopy study. J Am Acad Dermatol, 1985. 13(3): p. 464-8. 10. Wright, A.L., S.S. Bleehen, and A.E. Champion, Reticulate pigmentation due to bleomycin: light- and electron-microscopic studies. Dermatologica, 1990. 180(4): p. 255-7. 11. Eungdamrong, J. and B. McLellan, Flagellate erythema. Dermatol Online J, 2013. 19(12): p. 20716. 12. Scheiba, N., M. Andrulis, and P. Helmbold, Treatment of shiitake dermatitis by balneo PUVA therapy. J Am Acad Dermatol, 2011. 65(2): p. 453-5. Ryan Schuering DO 1 , Gregory Bartos OMS III 2 , Francisco Kerdel MD 3,4 , Stanley Skopit DO, MSE, FAOCD, FAAD 5 1 PGY-3 Dermatology Residency Training Program, LCH/LECOM, South Miami, FL, 2 OMS III Nova Southeastern University (NSU) College of Medicine, 3 Department of Dermatology, Florida International University, Miami, FL, 4 Florida Academic Dermatology Center, LCH, South Miami, Fl, 5 Program Director, Dermatology Residency Training Program, LCH/LECOM A Rare Etiology of Flagellate Erythema: A Case Report & Review • Discontinuation of offending agent • Treatment for flagellate erythema is mostly symptomatic: pruritus may be targeted with topical corticosteroids & oral antihistamines while hyperpigmentation usually resolves spontaneously within 1-8 weeks. 3 • Areas of lasting hyperpigmentation have been treated with intense pulse light therapy & Erbium 1540nm non-ablative laser. 11 • Erythematous papules from shiitake consumption have been targeted with short-term balneo-PUVA therapy showing complete clearance of itch & healing of lesions. 12 • Evaluation for systemic etiology such as dermatomyositis CASE PRESENTATION • Flagellate erythema is a rare cutaneous phenomenon described as linear erythematous streaks with pruritus & hyperpigmentation. Known etiologies are bleomycin, dermatomyositis, adult-onset stills disease, & shiitake dermatitis. Our patient did not fall into any common etiological category & historically was newly exposed to Butalbital-acetominophen- caffeine, malanga, & boniato prior to onset. A thorough literature search on these three compounds showed no evidence of flagellate erythema as an adverse reaction. • Bleomycin, an antitumor medication, is used as treatment with certain malignancies. Flagellate erythema has been reported as an adverse effect of bleomycin with an incidence rate of 10-20%. 3 The precise mechanism remains unknown although some speculate that bleomycin induces generalized pruritus leading to scratching. The scratching allows for the drug to exit blood vessels & reacts toxically with the skin. • Dermatomyositis is an inflammatory myositis with cutaneous manifestations. Well characterized cutaneous manifestations are heliotrope rash, Gottron’s papules, periungal telangiectasia, & shawl sign. Flagellate erythema has been reported in association with disease activity & may precede muscle symptoms. 4 Dermatomyositis has a 15-25% increased risk for malignancy. 5 • Adult-onset Still’s disease is an inflammatory disease comprised of high spiking fevers, arthralgia, hyperferritinemia, hepatosplenomegaly & rash. The characteristic rash is a salmon maculopapular erythema that appears during high fevers. Persistent erythematous plaques suggesting flagellate erythema have been reported in few cases. 6 • Shiitake dermatitis, AKA toxicoderma, is caused by the consumption of undercooked shiitake mushrooms. Incidence is highest in China & Japan where the mushroom is commonly grown & consumed. Flagellate erythema originates from significant pruritus & the Koebner phenomenon leading to linear grouping of non-pigmented papules. The rash improves on its own within two weeks. 7 DISCUSSION CONCLUSION Clinical Finding Histology Pearls Bleomycin Linear streaks located on trunk and/or shoulders. It is unique that these linear streaks are hyperpigmented, & devoid of inflammation. 8 - Epidermis shows increased melanin pigment, hyperkeratosis with focal parakeratosis, irregular acanthosis, spongiosis, & exocytosis of lymphocytes. - Dermis shows edema, vasodilation & perivascular lymphocytic infiltration. 9,10 - Patient will have started chemotherapy regimen within last 6 months. - Flagellate erythema is not a sufficient cause to stop cancer therapy Dermatomyositis Reddish, Linear streaks reflecting strong inflammation commonly on back, lack brown hyperpigmentation seen with Bleomycin. 2 - Epidermis shows mild atrophy with vacuolization of the basal layer. - Dermis shows lymphocytic infiltration in upper dermis & moderate edema in papillary dermis 8 - Elevated creatine kinase & ESR/CRP - Look for heliotrope rash, Gottron’s papules, muscle weakness - Screen for malignancies Adult-Onset Still’s Disease Persistent plaques with linear pigmentation with or without coalescent erythematous plaques - Mild perivascular infiltration of mononuclear cells & neutrophil, dyskeratotic cells in the epidermis - Monitor blood count - Monitor cardia function - Serial LFT’s & lipids Shiitake Mushrooms Widespread, disseminated, very small erythematous papules, no pigmentation, truncal involvement. 7 - Epidermis shows elongation of rete ridges, spongiosis & spongiotic bullae, with infiltration of inflammatory cells. The dermis shows edema, & superficial & intermediate perivascular infiltrates of mononuclear cells - Recent preparation of mushrooms or visit to Japanese restaurant - Avoid sun exposure due to photosensitive lesions • Flagellate erythema is a dermatosis comprised of hyperpigmented, pruritic, linear, & erythematous streaks. • It has been described in association with bleomycin use 1 , dermatomyositis 2 , adult-onset stills disease 3 , & shiitake mushroom consumption 4 . • The patient presented here did not encounter or meet the criteria for any of the known etiologies. • The recognition of this rare diagnostic clue is paramount in discovering its underlying condition as it may have significant health implications for the patient. • Flagellate erythema has been reported in association with several systemic diseases & chemical agents. • A thorough history & evaluation is important in determining the underlying cause. • Our patient did not appear to have the history or clinical features to indicate any of the known causes for flagellate erythema. • Thus, this case possibly demonstrates a novel cause of flagellate erythemadue to consumption of malanga (Xanthosoma sagittifolium) and boniato (Ipomoea batatas). A B C D E Figure A-D Erythematous, hyperpigmented linear streaks on the anterior, lateral, & posterior aspects of the lower extremities & anterior aspect of the lower abdomen. Figure D Left lateral lower extremity, punch biopsy site. Figure E A dense, perivascular lymphocytic infiltrate with very few eosinophils & marked dermal edema.