Extrafacial Rosacea—A Diagnostic Challenge

Dear Editor,

A 40-year-old male, who is a driver, presented to our department with erythematous skin lesions over both the arms for the past 10 months. The lesions were occasionally pruritic and exacerbated during summer. There were no co-morbidities. Personal history was unremarkable. He had taken multiple treatments, both topical and oral, without appreciable improvement. The nature of drug therapy was not known.

On examination, there was multiple skin colored to reddish follicular and non-follicular papules with erythematous background distributed symmetrically over the outer aspect of both arms [Figure 1a and b]. No lesions were observed on the face or elsewhere on the body. Clinically, miliaria rubra, irritant contact dermatitis, and acneform eruptions were considered as the differential diagnoses. Dermoscopy revealed linear and arborizing vessels along with the opening of Demodex tails [Figure 2].

Figure 1.

(a and b) Clinical image showing multiple skin colored to reddish follicular and non-follicular papules with erythematous background over the outer aspect of both arms

Figure 2.

Dermoscopy shows linear and arborizing vessels (black arrow) and Demodex tails (Black circle) (Dermalite 4, 10x magnification)

The squeezed follicular content under the microscope showed numerous Demodex folliculorum [Figure 3]. Biopsy of the lesion revealed perifollicular and peri eccrine lymphoplasmacytic infiltration along with non-caseating granulomas composed of histiocytes and occasional multinucleated giant cells in the upper dermis [Figure 4a and b]. Based on clinicopathological correlation, a final diagnosis of extrafacial rosacea was entertained. The patient was started on doxycycline 100 mg twice daily with topical metronidazole gel application and ivermectin 12 mg once a week for 2 weeks. The lesions resolved completely within a short period of 3 weeks. Follow-up at 2 months did not show any recurrence.

Figure 3.

Numerous Demodex folliculorum seen under 10 X magnification

Figure 4.

(a) Perifollicular and peri eccrine lymphoplasmacytic infiltration along with non-caseating granuloma (H & E, 10x) (b) Histiocytes and occasional multinucleated giant cells in the upper dermis (H & E, 40x)

Rosacea is a common skin disorder which commonly affects young women and primarily involves the facial convexity affecting the nose, cheeks, chin, forehead, and glabella, with typical sparing of the periocular and perioral regions. The four subtypes of rosacea are (i) erythematotelangiectatic rosacea, (ii) papulopustular rosacea, (iii) phymatous rosacea, and (iv) ocular rosacea.

The rare variants are granulomatous rosacea, which manifests as erythematous and monomorphic papules with periorificial location, and rosacea fulminans, characterized by sudden eruption of cystic nodules, papules, and pustules over the chin, cheeks, and forehead.

The term “extrafacial rosacea” was described in the year 1967 by Fountain and Sarkany, in a patient with findings of typical rosacea affecting the face and neck along with papules on the arm and the wrist.[1]

Later, similar cases of 14 patients were reported by Marks and Jones in 1969.[2]

The extra facial lesions of rosacea clinically present with multiple, discrete, papules, or nodules similar to facial lesions. Papulonecrotic lesions have also been described in the literature.[3] These lesions are often accompanied by facial rosacea and occur at scalp, ear, neck, axilla, upper extremities, pre-sternal area, and abdominal region. The clinical diagnosis will be more difficult, and often misdiagnosed in the scenario of absent facial lesions. Majority of such lesions have granulomatous pathology in the histopathology.

Extra facial rosacea predominantly affects men.[4] The exact pathogenesis is unknown, although disseminated infestation by Demodex folliculorum plays a vital role that has been suggested as a possible cause.[5]

Ramelet and Perroulaz postulated that UV light could be the precipitating factor and produce dermal elastosis at the sun-exposed areas. However, there were no elastotic changes present in tissue specimens, and recent studies failed to show that exposure to the sun was a cause or an exacerbating agent.[6]

Histopathology depends on the disease stage, with no difference between facial and extra facial lesions. In the early stage, there is edema along with vascular ectasia. As the disease progresses, perivascular and perifollicular lympho-histiocytic infiltration is seen, with non-caseating epithelioid granulomas surrounded by lymphocytes. Necrobiotic changes have also been reported.[2] The end stage is characterized by sebaceous hyperplasia.

Various topical and systemic therapies are available to treat extrafacial rosacea. Topically, metronidazole can be tried along with oral tetracycline (doxycycline 100 mg and minocycline 100 mg once daily for 3 weeks). Low-dose isotretinoin (0.1–0.2 mg/kg/day) has been found to be effective and safe. Other oral antibiotics such as azithromycin and erythromycin have achieved good results. In addition to medical management, avoidance of exacerbating factors such as oral or topical steroids, sunlight, hot beverages, and contactants must be emphasized.

This case is quite interesting because the patient presented without facial lesions, which made early diagnosis difficult.

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Conflicts of interest

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