Granulomatous rosacea : is it a variant of lupus miliaris disseminatus faciei?

Introduction

Granulomatous rosacea (GR) is a distinct variant of rosacea, characterized by an eruption of yellowish brown papules over the face, which appear indurated and reveal non-caseating epithelioid cell granulomas on histopathology.[1] GR can often be difficult to differentiate from other facial granulomatous dermatoses of unknown etilology, especially lupus miliaris disseminatus faciei (LMDF).[1],[2] We herein report a patient of GR closely resembling LMDF having concurrent ocular involvement, which responded to dapsone.

Case Report

A 38-year-old male presented with raised erythematous facial lesions associated with a burning sensation on sun exposure of two months′ duration. There had been no response to topical corticosteroids prescribed elsewhere. He also gave a one month history of redness of eyes along with dryness, foreign body sensation and photophobia. There was no family history of similar and marked erythema over forehead, eyelids and the butterfly area of face. There were scattered, reddish brown, 1 to 2 mm, firm, smooth, shiny, discrete papules distributed over the face and neck, predominantly on the forehead and eyelids [Figure - 1]. There were minimal telangiectases without pustules, comedones or scars. Ophthalmologic examination revealed visual acuity of 6/36 in the right eye and 6/12 in the left eye with bilateral swelling of both upper and lower lids, meibomianitis, blepharitis and deep conjunctival congestion. Mild blepharospasm was observed due to photophobia. Keratoconjunctivitis sicca was confirmed by a positive Schirmer′s type 1 test having less than 4 mm wetting of the Wattman paper strip in both the eyes. Slit lamp examination revealed bilateral superficial punctate keratitis staining positively with sodium fluorescein dye. There was no evidence of uveitis with normal fundus examination. General physical and systemic examination revealed no abnormality.

Complete blood counts, renal and hepatic function tests, urine analysis, blood sugar, serum electrolytes including calcium and chest X-ray were normal. Serology for syphilis and HIV-1, anti-nuclear antibodies, and an intradermal tuberculin test were negative. A skin biopsy revealed multiple non-caseating epithelioid cell granulomas with Langhans′ type giant cells in the dermis [Figure - 2]. Demodex folliculorum was not demonstrated and polaroscopic examination gave negative results. Use of periodic acid-Schiff, Ziehl-Neelsen and Giemsa stain revealed no organism. Slit skin smears, tissue smears and culture for acid-fast bacilli was negative. A diagnosis of granulomatous rosacea with ophthalmic rosacea was considered and the patient was treated with doxycycline 100 mg twice daily. A topical ocular corticosteroid and preservative free lubricating eye drops, protective sunglasses and a broad-spectrum sunscreen were prescribed. There was resolution of the symptoms of dryness and keratitis with improvement in visual acuity; however, the cutaneous lesions persisted even after 4 weeks of doxycycline therapy. Dapsone 100 mg twice daily was initiated and doxycycline was stopped. The facial erythema and papules resolved slowly in the next 3 weeks and dapsone was continued for six weeks. No recurrences was observed over a six months follow up.

Discussion

Granulomatous rosacea, clinically characterized by yellowish re discrete facial papules and non-caseating epithelioid cell granulomas on histology, constitutes about 10% of all cases of rosacea.[1] The presence of granulomas, resembling tuberculosis led to the earlier designations of this condition as ′Lewandowsky′s rosacea like tuberculid′ and ′micropapular tuberculid′. Currently GR is considered as a type of rosacea, unrelated to tuberculosis.[1],[2] Although the exact etiopathogenesis of GR is not known, a role of delayed hypersensitivity reaction against keratinized cells, pilosebaceous structures and Demodex folliculorum mite has been suggested.[2],[3]

Granulomatous rosacea can mimic various facial granulomatous conditions both clinically and histologically such as lupus miliaris disseminatus faciei (LMDF), micropapular sarcoidosis, and tuberculosis.[2],[3] Of these, its relationship with LMDF is most intriguing. A literature review reveals that LMDF has frequently been regarded as a variant of granulomatous rosacea.[3],[4] Helm et al have even included 2 patients of LMDF in their series of 53 patients of GR regarding them to be the same.[3] Both these diseases present clinically with symmetrically distributed, discrete, firm, yellowish red papules over the face, predominantly involving the forehead, eyelids, and cheeks.[1],[2] However, there are some subtle differences among the two [Table:1]. Eye involvement can be seen in 3 to 54% of cases of rosacea.[6] while it is not reported with LMDF. On histopathology, although dermal epithelioid cell granulomas are observed in both, caseation necrosis is rarely observed in GR.[3],[4] The lesions of LMDF in their natural course regress spontaneously in 12-18 months followed by scarring occassionaly.[4],[5] On the contrary, GR has no tendency towards spontaneous involution.[7]

Treatment of GR is not different from classical rosacea and tetracycline, doxycycline or minocycline are usually effective,12 while LMDF is often resistant to these standard therapies of rosacea.4 Conticosteroids, quite effective in LMDF, generally aggravate the lesions of GR.[3],[7] While dapsone has been reported to be effective in both the conditions.[7],[8] In view of these striking differences, GR should be regarded as distinct form from LMDF as proposed recently by Skowron et al.[7] An eye examination should be carried out in all patients of facial granulomatous disorders since it may help to clinch the diagnosis as in this patient.