Granuloma Faciale With Extrafacial Lesions

Abstract

A 35-year-old man presented with a 7-year history of gradually enlarging plaques on his face and trunk. The first lesions had developed on both sides of the forehead and the left cheekbone (Figure 1). Four years later similar lesions appeared on his neck and back. He presented a histologic report of a biopsy specimen from a facial plaque performed 5 years earlier that was diagnostic for granuloma faciale. He had different treatments such as topical steroids and cryotherapy without improvement. The appearance of new lesions on his trunk and the gradual enlarging of the old lesions convinced the patient to seek further treatment. Physical examination revealed dusky, violaceous plaques and papules, 0.5 to 2 cm, well-circumscribed, slightly elevated, and located on the face and trunk, with mild pruritus (Figure 1 and Figure 2). Laboratory investigations, including complete blood cell count, VDRL test, antinuclear antibody test, biochemical parameters, and chest x-ray, did not reveal any abnormalities. A skin biopsy taken from the upper part of the back showed similar features to the facial lesion, detected 5 years before, revealing a dense, polymorphous infiltrate involving mid and deep dermis and displaying a diffuse and perivascular pattern (Figure 3A). A narrow grenz zone of normal collagen was consistently observed between dermal infiltrate and epidermis as well as around the pilosebaceous follicles (Figure 3A). The infiltrate mainly consisted of eosinophils and lymphocytes, but neutrophils (often displaying leukocytoclasis), macrophages, and plasma cells were also present (Figures 3B, 3C). Some mast cells were also identified by staining with toluidine blue (Figure 3D). Perivascular infiltrates were often seen, sometimes penetrating vessel walls and in association with leukocytoclasis. Hyalinization of vessel walls, extravasation of red blood cells around capillaries, and nuclear dust were also noted. The epidermis did not show any remarkable change except for slight acanthosis. A diagnosis of granuloma faciale with extrafacial lesions was made, and a systemic therapy with hydroxychloroquine (200 mg twice daily for 6 weeks) was recommended.(1,2).

Extrafacial Granuloma Faciale: A Case Report and Brief Review

“…To our knowledge there have been 32 previously reported cases of extrafacial GF (Table 1) [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25]. In these cases the average age of the patients was 51 years, with 68.5% being male.…”

Pathology Quiz Case 2: Diagnosis

“…1,2 The mean age at diagnosis is 53 years (age range, 20-85 years). Ninety-two percent of GF cases are confined to the face, but, rarely, extrafacial lesions have been reported.…”

“…The cause of GF has not been determined, but several factors have been suggested, including UV exposure, trauma, allergy, radiation history, and a localized Arthus-like response. 1,2 Distinctive histopathologic features of GF include a dense, diffuse infiltrate of neutrophils, lymphocytes, and variable numbers of eosinophils in the upper dermis, sparing the epidermis. The lesions are relatively asymptomatic, with an indolent course.…”

“…Pathologically, GF has features that are similar to those of eosinophilic angiocentric fibrosis (EAF), a rare fibroinflammatory disorder that affects upper respiratory mucosa. 2 Because these entities all share histologic similarities, the clinical location of the lesion becomes very important in establishing the final diagnosis. These 2 entities have been found to occur concurrently in patients, so suspicion of EAF should prompt the clinician to perform a thorough examination of the upper respiratory tract.…”

Drei Kasuistiken eines Granuloma faciale – Stand der aktuellen Therapie

“…Angesichts der Remission ohne Narbenbildung auch nach wiederholter Behandlung mit dem gepulsten Farbstoff-Laser ist dieser einer medikamentösen Langzeittherapie vorzuziehen. Medikamentös unterschiedlich gut wirksam sind topische Kortikosteroide oder eine Kryotherapie [6,7], besser eine "first-line" intraläsionale Glukokortikosteroidinjektion mit Triamcinolonacetonid-Kristallsuspension, ferner intraläsionale Goldinjektionen, Einnahme von Antimalariamitteln, Calciferol, Isoniazid, Paraaminobenzoesäure und Bismuth, zudem lokale PUVA-Therapie, Röntgenweichstrahltherapie und topische Anwendung von Tacrolimus [8]. Zusammenfassend sollte der Patient aufgrund des chronisch persistierenden und häufig therapieresistenten Verlaufs mit zumeist atrophisch narbiger Abheilung frühzeitig über das Krankheitsbild und das hohe Rezidivrisiko bei alleiniger ablativer Therapie informiert werden.…”