Granuloma faciale: exclusively extrafacial.

We report a case of granuloma faciale over distal extremities in a 60-year-old man without facial lesions who did not show any response to dapsone.

What was known?

Granuloma Faciale is a chronic disorder known to occur predominantly over the face.

Introduction

Granuloma faciale (GF) is a rare skin disorder of chronic nature. It is characterized by single or multiple, sometimes erythematous, papules, plaques or nodules, usually involving the face.[1] Involvement of extrafacial sites may rarely be seen, e.g., the trunk, extremities, scalp, but facial lesions are almost always present as well.[2] The lesions may be asymptomatic or be associated with mild pruritus. Extrafacial or disseminated GF has been reported but is very rare.[3-6] We report a case of an adult with granuloma faciale over distal extremities without facial lesions for its rarity.

Case Report

A 60-year-old man patient presented with multiple persistent asymptomatic erythematous nodules and plaques over dorsum of fingers and toes of both the extremities since last 8 months without any surface changes [Figure 1]. There was no history of pain and redness on cold exposure, arthralgia, or features suggestive of systemic involvement, without any surface changes. The lesions did not show spontaneous regression.

Figure 1

Adult with lesions of GF over distal extremities

On cutaneous examination, there was no tenderness and the lesions were free from the underlying structures. No other site was involved including the face and mucosa. General and systemic examination was unremarkable. A provisional diagnosis of erythema elevatum diutinum was made. Patient was put on dapsone 100 mg/d but did not show any significant response even after 2 months.

Histopathological examination of one of the lesions revealed hyperkeratosis, hypergranulosis with prominent Grenz zone and a dense polymorphous infiltrate of neutrophils, lymphocytes, plasma cells and very few eosinophils in the dermis [Figure 2]. A diagnosis of GF was thus made.

Figure 2

Photomicrograph of H and E, × 400 showing Grenz zone and dense polymorphous infiltrate in the dermis

All routine investigations were within normal limits.

Discussion

It was Pinkus who first coined the term GF in 1952.[7] The lesions of GF usually involve the nose, forehead, or cheeks.[7] Extrafacial involvement is rare and has been reported to involve the back, arms, chest, shoulders, and thighs.[3-6] Our case shows bilateral symmetrical involvement of the acral parts without involving face.

There are both clinical and histological similarities between extrafacial GF and erythema elevatum diutinum (EED), and some authors suggest an association between the two conditions.[8] However, the symmetrical and acral involvement clinically, as well as the absence of Grenz zone with notable fibrosis histologically, helps distinguish EED from GF. In our case, similar presentation like EED was seen, but absence of arthralgia or tenderness of the lesions clinically and presence of Grenz zone and polymorphous infiltrate histologically excluded the possibility of EED.

GF is notoriously resistant to treatment. Many different medical therapies, including topical or intralesional corticosteroids, dapsone, anti-malarials, isoniazid, clofazimine, and topical nitrogen mustard, have been tried with variable results. A variety of surgical procedures, like surgical excision, dermabrasion, argon laser, pulsed dye laser,[9] have been used for the management of GF. Our patient received 2 months of dapsone 100 mg/day with minimal response. He was advised to apply very potent topical corticosteroids and showed substantial response after 1 month.

Thus, the presence of lesions of GF over hands and feet without lesions over face was unique in our case and should be considered in the differential diagnosis of persistent asymptomatic erythematous nodules and plaques over distal extremities.

What is new?

Granuloma Faciale may very rarely involve extrafacial sites without any facial involvement as in our case.