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Autumn 2018, Vol. 26 No. 3

Hong Kong J. Dermatol. Venereol. (2018) 26, 122-126


Views and Practice

A case of rosacea fulminans in a pregnant woman

JE Seol 薛禎恩, SH Park 朴蘇熙, JU Kim 金鐘旭, GJ Cho 趙敬濟, SH Moon 文勝鉉, H Kim 金孝鎮

Introduction

Rosacea fulminans (RF) is a rare type of inflammatory dermatosis that predominantly affects healthy young women.1 It typically manifests as a sudden onset of prominent erythema, pustules, cysts, and sinuses involving the face.2 Systemic symptoms are often absent.2 The aetiology of RF is unclear, but hormonal factors, such as pregnancy and oral contraceptive use, as well as immunological, vascular, and emotional factors, are considered possible causes.3

Case report

A 32-year-old primiparous woman with a pregnancy of 18 weeks presented with a painful erythematous patch, as well as numerous pustules and cystic nodules, on her face. The skin lesions started in week 6 of pregnancy. The pustules had been extracted and she had been using topical mupirocin ointment for the previous three months, but the lesions had gradually worsened. She had no constitutional symptoms and no previous history of dermatological disease, such as acne vulgaris, rosacea, or seborrhoeic dermatitis

Physical examination revealed tender erythematous patches and confluent cystic nodules on her face (Figure 1). Other regions of the body were not affected. Laboratory test showed a slightly increased C-reactive protein level of 2.49 mg/dL, and an increased total immunoglobulin E level of 410.4 IU/ml. The values of all other tests were within normal limits, including liver and renal function tests. Gram staining of the pustules revealed the presence of gram-positive cocci and gram-negative rods, but cultures were negative for all organisms. Histopathological examination showed spongiotic neutrophilic aggregation in the epidermis and diffuse perivascular and interstitial infiltration of neutrophils and a few eosinophils in the dermis (Figure 2). Based on the clinical, laboratory, and histopathological findings, RF was diagnosed. Since the patient refused systemic treatment during her pregnancy, she was treated only with topical corticosteroid (mometasone furoate) during week 18 to week 34 of her pregnancy. At week 35, the skin lesions were still uncontrolled and low-dose systemic corticosteroid (methylprednisolone 2 mg/day) was added to the treatment regimen. Thereafter, the skin lesions remained stable. Moreover, they improved rapidly after delivery. Two months after delivery, she was started on systemic isotretinoin to treat the residual lesions, most of which improved within three months. During 18 months of follow-up, there was no sign of recurrence.

Figure 1 (A) Marked erythema with coalescing nodules and pustules on the face at first visit. (B) Mild aggravation of lesions despite 6 weeks of treatment with topical corticosteroids and emollients. (C&D) Close-up view of erythema, nodules, and pustules on the right cheek (C) and the chin (D).

Figure 2 (A) Histopathological examination shows a spongiotic neutrophilic aggregation in the epidermis and diffuse perivascular and interstitial infiltration of inflammatory cells in the dermis (H&E, x40). (B) High-power view. Infiltration of neutrophils in the epidermis (H&E, x400). (C) Dense perivascular and interstitial infiltration of neutrophils and a few eosinophils in the reticular dermis (H&E, x400).

Discussion

Rosacea fulminans is considered to be a rare and extreme form of rosacea.2 It predominantly affects healthy young women and is characterised by the sudden onset of prominent erythema, pustules, cysts, and sinuses on the face.1 Although the exact cause is unclear, hormonal changes are probably a factor, since a relationship between RF and pregnancy or contraceptive use has been demonstrated.3 In 17 cases of RF in pregnant woman reported in the English-language medical literature,1-9 RF occurred during pregnancy and improved significantly after delivery, as in our patient. It is therefore likely that the hormonal changes occurring during pregnancy affect the incidence or exacerbation of RF; however, the exact mechanism linking hormonal changes to RF has yet to be clarified.

RF is usually very stressful to patients because of the severe facial lesions. It may cause physical and psychological stress to the pregnant patient and thus contribute to a poor delivery outcome.7 Early diagnosis and proper management of RF is important, but the therapeutic choices available to pregnant women are limited. While systemic retinoids, tetracycline antibiotics, anti-androgenic contraceptives, and dapsone are generally used to treat RF,8 all of these drugs are contraindicated during pregnancy.9 In previously reported cases of RF in pregnancy, patients were treated surgically, including pustule extraction and incision and drainage. Other forms of treatment include topical antibiotics, topical corticosteroids, and systemic antibiotics, all of which are permitted during pregnancy, as are low-dose and short-term systemic corticosteroids (Table 1). In most pregnant patients with RF, these drugs result in a stable state of skin disease during pregnancy. Thus, until an optimal regimen for RF in a pregnant woman is determined, topical and systemic antibiotics with proper surgical drainage and low-dose systemic corticosteroids are effective choices for preventing an acute exacerbation during pregnancy. Above all, it is important that RF during pregnancy be diagnosed quickly, and that appropriate treatment and support are provided through cooperation with the patient's obstetrician.

RF is a rare and extreme form of rosacea that can occur in association with pregnancy. While hormonal changes, such as those occurring during pregnancy, seem to be the trigger for RF, further research is needed to determine the exact mechanism. Early diagnosis and proper surgical treatment, together with medications deemed safe for use during pregnancy, are important in the management of RF skin lesions, and to decrease the stress caused by the disease.

Table 1 Reported cases of rosacea fulminans (RF) associated with pregnancy
No. Author Patient age Development of RF Treatment during pregnancy Outcome
1-5 (5 cases) Massa and Su4 N/A 4 cases: 3rd trimester
1 case: post-partum
N/A N/A
6 Marks Briggaman5 25 2nd trimester Incision and drainage, triamcinolone intralesional injection
Topical corticosteroid (1% hydrocortisone)
Oral antibiotics (erythromycin)
Oral corticosteroid (prednisolone)
Healthy full-term delivery
7-10 (4 cases) Plewig et al.2 N/A 2 cases: 1st trimester
1 case: 3rd trimester
1 case: post-partum
Topical antibiotics (erythromycin, clindamycin) N/A
11 Haugstvedt and Bjerke6 35 N/A N/A N/A
12 Lewis et al.1 28 1st trimester Oral antibiotics erythromycin,
Oral corticosteroid (prednisolone)
Intra-uterine death
13 Fehrabas et al.3 31 1st trimester Incision and drainage, wet compression
Topical antibiotics (fusidic acid, metronidazole)
Oral corticosteroid (methylprednisolone)
Healthy full-term delivery
14-16 (3 cases) Jarrett et al.7 N/A 3 cases: 1st trimester
  1. Oral corticosteroid (prednisolone)
  2. Oral antibiotics (erythromycin), Oral corticosteroid (prednisolone)
  3. Oral antibiotics (erythromycin)
  1. Intra-uterine death
  2. Termination due to anxiety
  3. Healthy full-term delivery
17 de Morais el al.9 26 2nd trimester Oral antibiotics (erythromycin)
Oral corticosteroid (prednisolone)
Healthy full-term delivery
18 This case 32 1st trimester Topical corticosteroid (mometasone furoate)
Oral corticosteroid (methylprednisolone)
Healthy full-term delivery
N/A, not available

References

1. Lewis VJ, Holme SA, Wright A, Anstey AV. Rosacea fulminans in pregnancy. Br J Dermatol 2004;151:917-9.

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

3. Ferahbas A, Utas S, Mistik S et al. Rosacea fulminans in pregnancy: case report and review of the literature. Am J Clin Dermatol 2006;7:141-4.

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

5. Marks VJ, Briggaman RA. Pyoderma faciale: successful treatment with isotretinoin. J Am Acad Dermatol 1987;17:1062-3.

6. Haugstvedt A, Bjerke JR. Rosacea fulminans with extrafacial lesions. Acta Derm Venereol 1998;78:70-1.

7. Jarrett R, Gonsalves R, Anstey AV. Differing obstetric outcomes of rosacea fulminans in pregnancy: report of three cases with review of pathogenesis and management. Clin Exp Dermatol 2010;35:888-91.

8. Fuentelsaz V, Ara M, Corredera C, Lezcano V, Juberias P, Carapeto FJ. Rosacea fulminans in pregnancy: successful treatment with azithromycin. Clin Exp Dermatol 2011;36:674-6.

9. de Morais e Silva FA, Bonassi M, Steiner D, da Cunha TV. Rosacea fulminans in pregnancy with ocular perforation. J Dtsch Dermatol Ges 2011;9:542-3.