Rosacea Fulminans With Extrafacial Lesions in an Elderly Man: Successful Treatment With Subantimicrobial-Dose Doxycycline
June 2014 | Volume 13 | Issue 6 | Case Report | 763 | Copyright © 2014
Lauren A. Smith MD, Shane A. Meehan MD, and David E. Cohen MD MPH
Department of Dermatology, New York University Langone Medical Center, New York, NY
Rosacea fulminans, previously known as pyoderma faciale,
is a rare disease occurring almost exclusively in young women
characterized by the sudden eruption of coalescing papules and pustules,
and large cystic nodules limited to the face. Patients generally respond
well to standard therapy consisting of systemic isotretinoin in combination
with topical and systemic corticosteroids. Lesions usually resolve with
minimal scarring with appropriate management. We describe an elderly male
patient with extrafacial rosacea fulminans successfully treated with daily
subantimicrobial (40mg) dose doxycycline (SDD). To our knowledge, this is
the first report of rosacea fulminans with extrafacial lesions in an elderly male.
We suggest that SDD may be a safe and effective alternative, particularly for those unable to tolerate standard therapy.
J Drugs Dermatol. 2014;13(6):763-765.
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Rosacea fulminans (RF) is a rare and severe form of rosacea characterized by the sudden eruption of coalescing papules and pustules, and large cystic nodules limited almost exclusively to the face of young women.1,2 Patients typically respond well to systemic isotretinoin in combination with topical and systemic corticosteroids, though use of systemic antibiotics has also been described.1,3,4 With appropriate management, lesions may resolve with minimal scarring, and recurrence has not been reported.1,2
We describe an unusual case of an elderly male with extrafacial RF successfully treated with daily subantimicrobial (40mg) dose doxycycline (SDD), a therapy, to our knowledge, not yet reported for this condition.
An 80-year-old male with a history of erythemotelangiectatic and papulopustular rosacea, emphysema, and cardiovascular disease presented with a 3- to 4-year progressive, pruritic eruption on his chest, abdomen, and arms. At the time of presentation he was in mid-course of 100mg doxycycline twice daily. He was previously treated with topical betamethasone, benzoyl peroxide, 2% Erythromycin solution, and pramasone with no improvement.
His current medications included metoprolol, dabigatran, tiotropium bromide inhaler, and amiodarone. Other than Penicillin, he reported no history of drug, food, or pet allergies; and he denied prior chemical or environmental exposures. A complete review of systems was otherwise negative.
Examination revealed erythematous, slightly scaly, confluent plaques punctated with pustules and hyperkeratotic papules over the chest, abdomen, arms, and face (Figure 1). Our differential diagnosis included infectious, allergic, and autoimmune dermatoses. Brief trials of oral fluconazole (200 mg/day) followed by doxycycline (100mg twice daily) were empirically administered for a presumed fungal or bacterial folliculitis; however, minimal clinical improvement was noted on short-term follow-up.
Patch testing, laboratory tests, and wound cultures were negative. A punch biopsy of the upper left shoulder revealed a dense infiltrate of neutrophils within the papillary and upper reticular dermis with occasional granulomas comprised of epithelioid and multinucleated histiocytes (Figure 2), consistent with a diagnosis of RF.
Given the lack of a demonstrable infectious etiology, subantimicrobial-dose (40mg daily) Doxycycline (SDD) was initiated pending biopsy reports. Substantial clinical improvement was noted at one-month follow-up with a complete response after 7 months of SDD therapy (Figure 3).
Rosacea fulminans (RF), also known as pyoderma faciale, is a rare disease first reported in 1940 as the acute onset of clinical features localized to the face of young women.5 Scarce exceptions with respect to gender and anatomic propensity have since been reported.6-8
Characterized by the sudden eruption of coalescing papules and pustules, and large cystic nodules limited to the face, immunological, hormonal, and vascular factors have all been suggested as causative factors; however, the etiology of RF remains unknown.9-13 Reported cases have also suggested its association with various vitamins and medications, and its in-