Dermatology Online Journal

Granulomatous rosacea in infants. Report of three cases and discussion of the differential diagnosis
João Borges da Costa, Sousa Coutinho V, L Soares de Almeida, M Marques Gomes PhD
Dermatology Online Journal 14 (2): 22

Clínica Universitária de Dermatologia, Hospital de Santa Maria, Lisboa, Portugal. joaobc77@hotmail.com

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Abstract

Granulomatous rosacea is a variant of rosacea characterized by hard cutaneous papules and nodules in relatively normal-appearing skin that is rarely diagnosed in childhood. The differential diagnosis essentially includes perioral dermatitis and sarcoidosis. Despite the differences in clinical presentation and histopathology, there is similar responses to the same therapies used in rosacea. Therapeutic failure should lead to the investigation of other rare and controversial conditions, such as acne agminata.

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Clinical synopses

Figure 1	Figure 2
Figure 1. Nodules and erythematosus papules on the cheeks of a 15 months old infant Figure 2. Erythematous papules on the cheeks of a 14 months old infant

Figure 3
Figure 3. Biopsy of an erythematosus papule on the cheeks showing a perifollicular suppurative granuloma with a mixed cellular infiltrate (hematoxylin and eosin stain; x100)

We report three otherwise healthy male infants with granulomatous rosacea, aged 7, 14 and 15 months. On clinical examination, they had hard erythematous papules and nodules on the cheeks and nasolabial folds, with 2-3 months evolution (Figs. 1 and 2). The lesions were located on normal-appearing skin. Comedones were not present.

There was also an absence of other primary features of rosacea, such as flushing, non-transient erythema, and telangiectasias. The family history was negative for rosacea.

The two older infants improved with systemic erythromycin (30 mg/kg/day TID) and topical metronidazol (0.75% gel applied twice daily). The youngest patient had a lesser improvement with topical and systemic therapy and after 7 months of persistent lesions, a skin biopsy was done demonstrating the granulomatous changes (Fig. 3).

Comments

Granulomatous rosacea is a variant of rosacea characterized by hard cutaneous papules and nodules in relatively normal-appearing skin. It is differentiated from the more common forms of rosacea by its monomorphic lesions and typical appearance on the cheeks [1]. The presence of other rosacea signs, such as flushing and telangiectasia, are not needed for the diagnosis of this variant, which has been described mainly in middle-aged women with the face being the most affected area of the body [2].

The etiology and pathogenesis of this variant of rosacea are unknown. It is rarely diagnosed in childhood with the majority of the reported cases in this age group being between 9 and 11 years of age [3, 4]. Although the majority of cases have a good response to the topical or systemic therapies recommended for adults with rosacea, its management can also be a therapeutic challenge [4].

Differential diagnosis is essentially with Perioral Dermatitis and Sarcoidosis [3]. Our patients did not present pustules or papulovesicles on an erythematosus background in the perioral, perinasal and periocular areas which are preferentially affected in perioral dermatitis. They were also free of the pulmonary symptoms or systemic complaints usually associated with skin involvement by sarcoidosis in children [5].

The youngest patient had a lesser improvement with the conventional treatment for rosacea; a skin biopsy revealed perifollicular suppurative granuloma. These features could support a diagnosis of facial idiopathic granulomata with regressive evolution [6], also known as acne agminata, despite the location of the lesions in this disease being more on the periorbital area with the majority of cases occurring in young to middle-aged adults. Controversy still exists in literature and some authors consider this disease to be synonymous with granulomatous rosacea [7].

Conclusions

We presented 3 cases that were all initially diagnosed as granulomatous rosacea and treated with the same therapies commonly used for rosacea.

In one case, the absence of response to treatment lead us to make a biopsy of the lesions and put the diagnosis of Acne agmninata.

Granulomatous rosacea is variant of rosacea that is rarely diagnosed in childhood. Despite its differences in clinical presentation and histopathology, it has similar responses to the same therapies used in rosacea. Therapeutic failure should lead to the consideration of other rare conditions, such as acne agminata

References

1. Wilkin J MD, Dahl M MD, Detmar M MD, Drake L MD, Feinstein A MD, Odom R MD 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.

2. Helm K MD, Menz J MD, Gibson L MD, Dicken C MD. A clinical and histopathologic study of granulomatous rosacea. J Am Acad Dermatol, 1991, 25:1038-43.

3. Drolet B, Paller A MD. Childhood Rosacea. Pediatr Dermatol, 1992, 9:22-26.

4. Poblete-Gutiérrez P, Beermann T, Gruβenrorf-Conen E, Frank J. Treatment resistant granulomatous rosacea-like dermatitis in a 9 year old girl. Der Hautarzt, 2004, 55:997-9.

5. K Clark. Sarcoidosis in children. Pediatr Dermatol, 1987, 4:291-99.

6. Skowron F, Causeret AS, Pabion C, Viallard AM, Balme B, ThomasL. F.I.G.U.R.E : facial idiopathic granulomata with regressive evolution. Is "lupus miliaris disseminatus faciei" still an acceptable diagnosis in the third millennium? Dermatology, 2001, 201:287-289.

7. van de Scheur MR, van der Waal RI, Starink TM. Lupus miliaris disseminatus faciei: a distintictive rosacea-like syndrome and not a Granulomatous form of Rosacea. Dermatology, 2003, 206(2):120-3.

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