a rare etiology of flagellate erythema: a case report & review...• flagellate erythema is a...

• 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 DO1, Gregory Bartos OMS III 2, Francisco Kerdel MD 3,4, Stanley Skopit DO, MSE, FAOCD, FAAD5

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, 5Program 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

dermatomyositisCASE 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 dermis8

- 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

use1, dermatomyositis2, adult-onset stills disease3,

& shiitake mushroom consumption4.

• 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 EFigure 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.