RRosacea fulminansosacea fulminans · co-amoxiclav and ciprofloxacin. The patient was then...

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Letters to the Editor Indian Journal of Dermatology, Venereology and Leprology | May-June 2014 | Vol 80 | Issue 3 272 the dermis and distortion of fat architecture, dermal edema and ectatic lymphatics. [2] The pathogenesis remains unknown although mechanical factors like prolonged use of tight head gear and hormonal factors have been implicated. [4] Incidental associations include diabetes mellitus, renal failure, joint and skin hyperelasticity, Sjogren’s syndrome, hyperlipidemia and ovarian cysts. [2,5] There is no definitive option for treatment or to halt progression of the condition. Our patients were initiated on antihistamines. In conclusion, lipedematous scalp is a possible cause of dysesthetic syndrome of the scalp. In patients with no obvious cause for dysesthesia of the scalp, it is advisable that the scalp be palpated to identify this rare presentation that may otherwise be missed. C.V. Dincy Peter, Anne Jennifer C.V. Dincy Peter, Anne Jennifer 1 , , Tanumay Raychaudhury, Tanumay Raychaudhury, Laxmisha Chandrashekhar, Sophia Merilyn Laxmisha Chandrashekhar, Sophia Merilyn 1 , Sadashiv Gowda Sadashiv Gowda 2 , Aparna Shyam Aparna Shyam 2 Departments of Dermatology, Venereology and Leprosy, 1 Pathology, and 2 Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India Address for correspondence: Address for correspondence: Dr. C.V. Dincy Peter, Department of Dermatology, Venereology and Leprosy, Christian Medical College, Vellore, India, Tamil Nadu, India. E-mail: [email protected]. REFERENCES REFERENCES 1. Cornbleet T. Cutis verticis gyrata? Lipoma? Arch Derm Syphilol 1935;32:688. 2. Martín JM, Monteagudo C, Montesinos E, Guijarro J, Llombart B, Jordá E. Lipedematous scalp and lipedematous alopecia: A clinical and histologic analysis of 3 cases. J Am Acad Dermatol 2005;52:152-6. 3. Müller CS, Niclou M, Vogt T, Pföhler C. Lipedematous diseases of the scalp are not separate entities but part of a spectrum of lipomatous lesions. J Dtsch Dermatol Ges 2012;10:501-7. 4. El Darouti MA, Marzouk SA, Mashaly HM, El Nabarawi EA, Abdel-Halim MR, El Komy MM, et al. Lipedema and lipedematous alopecia: Report of 10 new cases. Eur J Dermatol 2007;17:351-2. 5. Kavak A, Yuceer D, Yildirim U, Baykal C, Sarisoy HT. Lipedematous scalp: A rare entity. J Dermatol 2008;35:102-5. Access this article online Quick Response Code: Website: www.ijdvl.com DOI: 10.4103/0378-6323.132266 Rosacea fulminans Rosacea fulminans Sir, Rosacea fulminans is an intensely inflammatory facial eruption that affects young women. It can be mistaken for cutaneous infections and inappropriately treated with surgery or short courses of antibiotics. Accurate diagnosis and treatment reduces the potential for scarring. A 32-year-old woman presented with a painful facial eruption which started 2 years ago as a carbuncle. The first culture from pus grew Enterobacter but the rash responded only partially to incision and drainage and various antibiotics including cephalexin, cloxacillin, co-amoxiclav and ciprofloxacin. The patient was then suspected to have nodulocystic acne vulgaris and isotretinoin at 15 mg daily initially led to resolution, but she relapsed after 7 months of treatment. She was afebrile and had no relevant medical or drug history. Physical examination showed erythema, edema and multiple pustules over most of the face [Figure 1]. The total white cell count was 10.9 × 10 9 /L with neutrophilia (81.3%). Erythrocyte sedimentation rate was 59 mm/h. Repeat cultures for bacteria, fungi and mycobacteria were negative and serum antinuclear antibody was non-reactive. Skin biopsy from a pustule on an erythematous, edematous plaque on the cheek showed a folliculo-centric infiltrate of neutrophils within the upper to mid-dermis with a ruptured hair follicle, upper dermal edema and superficial and mid-dermal perivascular lymphocytic infiltrate [Figure 2]. Rosacea fulminans was diagnosed and she was treated with doxycycline 100 mg twice a day, topical metronidazole gel and 2 weeks of indomethacin. The facial erythema and edema improved with cessation of new pustule formation. After 5 months of doxycycline and topical metronidazole, she achieved complete resolution [Figure 3]. Rosacea fulminans (synonym: pyoderma faciale) is a rare, inflammatory dermatosis that predominantly afflicts women in the 2 nd to 3 rd decades of life. A recent review found about 60 published articles since 1978. [1] It presents as an acute facial eruption of coalescing nodules, pustules and draining sinuses with pronounced erythema and edema. Men are uncommonly affected and extra-facial involvement is rare. [1,2] A history of seborrhea or sudden increase in oiliness prior to eruption may be elicited. Notwithstanding the fulminant nature of the outbreak, systemic symptoms are uncommon. The etiopathogenesis of rosacea fulminans remains unknown. Plewig et al . in their study classified it

Transcript of RRosacea fulminansosacea fulminans · co-amoxiclav and ciprofloxacin. The patient was then...

Page 1: RRosacea fulminansosacea fulminans · co-amoxiclav and ciprofloxacin. The patient was then suspected to have nodulocystic acne vulgaris and isotretinoin at 15 mg daily initially led

Letters to the Editor

Indian Journal of Dermatology, Venereology and Leprology | May-June 2014 | Vol 80 | Issue 3272

the dermis and distortion of fat architecture, dermal edema and ectatic lymphatics.[2] The pathogenesis remains unknown although mechanical factors like prolonged use of tight head gear and hormonal factors have been implicated.[4] Incidental associations include diabetes mellitus, renal failure, joint and skin hyperelasticity, Sjogren’s syndrome, hyperlipidemia and ovarian cysts.[2,5] There is no definitive option for treatment or to halt progression of the condition. Our patients were initiated on antihistamines.

In conclusion, lipedematous scalp is a possible cause of dysesthetic syndrome of the scalp. In patients with no obvious cause for dysesthesia of the scalp, it is advisable that the scalp be palpated to identify this rare presentation that may otherwise be missed.

C.V. Dincy Peter, Anne JenniferC.V. Dincy Peter, Anne Jennifer1, , Tanumay Raychaudhury,Tanumay Raychaudhury,

Laxmisha Chandrashekhar, Sophia MerilynLaxmisha Chandrashekhar, Sophia Merilyn1,Sadashiv GowdaSadashiv Gowda2, Aparna ShyamAparna Shyam2

Departments of Dermatology, Venereology and Leprosy,1Pathology, and 2Radiodiagnosis, Christian Medical College,

Vellore, Tamil Nadu, India

Address for correspondence:Address for correspondence: Dr. C.V. Dincy Peter,Department of Dermatology, Venereology and Leprosy,

Christian Medical College, Vellore, India, Tamil Nadu, India. E-mail: [email protected].

REFERENCESREFERENCES

1. Cornbleet T. Cutis verticis gyrata? Lipoma? Arch Derm Syphilol 1935;32:688.

2. Martín JM, Monteagudo C, Montesinos E, Guijarro J, Llombart B, Jordá E. Lipedematous scalp and lipedematous alopecia: A clinical and histologic analysis of 3 cases. J Am Acad Dermatol 2005;52:152-6.

3. Müller CS, Niclou M, Vogt T, Pföhler C. Lipedematous diseases of the scalp are not separate entities but part of a spectrum of lipomatous lesions. J Dtsch Dermatol Ges 2012;10:501-7.

4. El Darouti MA, Marzouk SA, Mashaly HM, El Nabarawi EA, Abdel-Halim MR, El Komy MM, et al. Lipedema and lipedematous alopecia: Report of 10 new cases. Eur J Dermatol 2007;17:351-2.

5. Kavak A, Yuceer D, Yildirim U, Baykal C, Sarisoy HT. Lipedematous scalp: A rare entity. J Dermatol 2008;35:102-5.

Access this article online

Quick Response Code: Website: www.ijdvl.com

DOI: 10.4103/0378-6323.132266

PMID:*****

Rosacea fulminansRosacea fulminans

Sir,Rosacea fulminans is an intensely inflammatory facial eruption that affects young women. It can be mistaken for cutaneous infections and inappropriately treated with surgery or short courses of antibiotics. Accurate diagnosis and treatment reduces the potential for scarring.

A 32-year-old woman presented with a painful facial eruption which started 2 years ago as a carbuncle. The first culture from pus grew Enterobacter but the rash responded only partially to incision and drainage and various antibiotics including cephalexin, cloxacillin, co-amoxiclav and ciprofloxacin. The patient was then suspected to have nodulocystic acne vulgaris and isotretinoin at 15 mg daily initially led to resolution, but she relapsed a fter 7 months of treatment. She was afebrile and had no relevant medical or drug history. Physical examination showed erythema, edema and multiple pustules over most of the face [Figure 1]. The total white cell count was 10.9 × 109/L with neutrophilia (81.3%). Erythrocyte sedimentation rate was 59 mm/h. Repeat cultures for bacteria, fungi and mycobacteria were negative and serum antinuclear antibody was non-reactive. Skin biopsy from a pustule on an erythematous, edematous plaque on the cheek showed a folliculo-centric infiltrate of neutrophils within the upper to mid-dermis with a ruptured hair follicle, upper dermal edema and superficial and mid-dermal perivascular lymphocytic infiltrate [Figure 2]. Rosacea fulminans was diagnosed and she was treated with doxycycline 100 mg twice a day, topical metronidazole gel and 2 weeks of indomethacin. The facial erythema and edema improved with cessation of new pustule formation. After 5 months of doxycycline and topical metronidazole, she achieved complete resolution [Figure 3].

Rosacea fulminans (synonym: pyoderma faciale) is a rare, inflammatory dermatosis that predominantly afflicts women in the 2nd to 3rd decades of life. A recent review found about 60 published articles since 1978.[1] It presents as an acute facial eruption of coalescing nodules, pustules and draining sinuses with pronounced erythema and edema. Men are uncommonly affected and extra-facial involvement is rare.[1,2] A history of seborrhea or sudden increase in oiliness prior to eruption may be elicited. Notwithstanding the fulminant nature of the outbreak, systemic symptoms are uncommon. The etiopathogenesis of rosacea fulminans remains unknown. Plewig et al. in their study classified it

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Page 2: RRosacea fulminansosacea fulminans · co-amoxiclav and ciprofloxacin. The patient was then suspected to have nodulocystic acne vulgaris and isotretinoin at 15 mg daily initially led

Letters to the Editor

273Indian Journal of Dermatology, Venereology and Leprology | May-June 2014 | Vol 80 | Issue 3

Figure 1: Erythematous, edematous plaques with pustules and crusts over the whole face

Figure 2: Folliculocentric infi ltrate of neutrophils in the upper to mid-dermis with ruptured hair follicle, upper dermal edema and superfi cial and mid-dermal perivascular lymphocytic infi ltrate

as a severe form of rosacea on morphologic and histologic grounds and a history of flushing in their patients, though this classification is not universally accepted.[3,4] Recently, Kim et al. reported a case of male rosacea fulminans associated with ocular rosacea and a history of papulo-pustular rosacea.[2] Most patients, however, have no prior history of rosacea and the disease usually does not recur after resolution. Significant associations have been reported with inflammatory bowel disease and pregnancy. Less common associations include rheumatoid arthritis, thyroid and liver diseases, oral contraceptive pills, pegylated interferon alpha-2b and ribavirin therapy and high dose vitamins B6 and B12.[3,5] Some patients recall major emotional experiences before onset of disease.[5]

Histological examination shows extensive dermal and perifollicular inflammatory infiltrates of neutrophils, lymphocytes, histiocytes and occasional eosinophils, giant cells and granulomas. Cultures are either sterile or grow skin commensals, e.g., Staphylococcus epidermidis, Staphylococcus aureus, Corynebacterium or Propionibacterium species. Gram-negative organisms are rarely isolated.

The differential diagnosis includes acne fulminans which generally afflicts adolescent men with a history of acne vulgaris. Acne fulminans has more comedones, hemorrhagic ulcerations, a more extensive distribution on neck and trunk and prominent systemic symptoms including fever, arthralgia, myalgia and osteolytic bone changes. Gram-negative folliculitis usually presents as a flare of follicular pustules after prolonged antibiotic therapy for acne vulgaris. Other considerations include Sweet’s syndrome, halogenoderma, androluteoma and granulomatous infections.

Systemic prednisolone (0.5-1.0 mg/kg/day) is rapidly effective for rosacea fulminans though relapses are common when it is tapered. Isotretinoin may be started concurrently at low dose, or later after inflammation has abated, to maintain remission. A dose of 0.5-1.0 mg/kg/day over 6-12 months is generally required. Oral tetracycline, erythromycin, dapsone and azithromycin have also been used successfully, the latter often employed in pregnancy where alternatives are limited. Topical therapies include compresses, topical steroids and topical antibiotics. While most reports cite isotretinoin as the most effective treatment, our patient relapsed while on the drug and responded instead to a combination of

Figure 3: Complete resolution with mild scarring after 5 months of treatment with doxycycline, non-steroidal anti-infl ammatory drugs and topical metronidazole

Page 3: RRosacea fulminansosacea fulminans · co-amoxiclav and ciprofloxacin. The patient was then suspected to have nodulocystic acne vulgaris and isotretinoin at 15 mg daily initially led

Letters to the Editor

Indian Journal of Dermatology, Venereology and Leprology | May-June 2014 | Vol 80 | Issue 3274

doxycycline, non-steroidal anti-inflammatory agents and topical metronidazole. Most patients achieve remission within 1 year and relapses are uncommon. Scarring is a significant sequelae.[5]

Hong Yi Koh, See Ket Ng, Wee Ping TanHong Yi Koh, See Ket Ng, Wee Ping TanDepartment of Dermatology, National Skin Centre, Singapore

Address for correspondence:Address for correspondence: Dr. Hong Yi Koh,National Skin Centre(s) Pte Ltd., 1 Mandalay Road,

Singapore 308205, Republic of Singapore. E-mail: [email protected]

REFERENCESREFERENCES

1. Ribeiro LB, Ramos-e-Silva M. Rosacea fulminans. Cutis 2013;92:29-32.

2. Kim TG, Noh SM, Do JE, Lee MG, Oh SH. Rosacea fulminans with ocular involvement. Br J Dermatol 2010;163:877-9.

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Quick Response Code: Website: www.ijdvl.com

DOI: 10.4103/0378-6323.132267

PMID:*****

3. Plewig G, Jansen T, Kligman AM. Pyoderma faciale. A review and report of 20 additional cases: Is it rosacea? Arch Dermatol 1992;128:1611-7.

4. Wilkin J, Dahl M, Detmar M, Drake L, Feinstein A, Odom R, et al. Standard classification of rosacea: Report of the National Rosacea Society Expert Committee on the Classification and Staging of Rosacea. J Am Acad Dermatol 2002;46:584-7.

5. Massa MC, Su WP. Pyoderma faciale: A clinical study of twenty-nine patients. J Am Acad Dermatol 1982;6:84-91.

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