Facial granuloma L92.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 15.01.2021

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Synonym(s)

Eosinophilic granuloma of the face; Facial eosinophilic granuloma; facial granuloma eosinophilicum; Granuloma eosinophiles of the face; Granuloma eosinophilicum faciei; Granuloma faciale with eosinophilia; Granuloma of the face eosinophil

History
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Colcott-Fox 1895; Wigley 1945; Pedace and Perry 1966

Definition
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Mostly harmless, chronically persistent, inflammatory skin disease of unknown aetiology with a distinct histological substrate characterized by (inconstant), but mostly formative histoeosinophilia as well as signs of leukocytoclastic vasculitis which is superimposed by fibrosis over time.

Occurrence/Epidemiology
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Rarely, in large dermatological centres this diagnosis is made 1-2 times a year.

Preferably in whites.

Men are significantly more frequently affected than women. In a "medium-sized" study (n=11) the ratio was 9:2.

Etiopathogenesis
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Ultimately unknown. In the past, the disease was (erroneously) classified as a granulomatous disease. However, the vasculitic phenomena with leukocytoclasia, which can be detected in the early stage of the disease, indicate a vasculitic genesis.

Associated infections (streptococcal infections, syphilis, viral infections (hepatitis virus (K73.9), HIV(Z21) tumor diseases (prostate carcinoma, lymphoma, multiple myeloma and others) as well as autoimmune diseases (rheumatoid arthritis (M06.99), celiac disease (K90.0), Crohn's disease (K50.9), uveitis (H20.1) were observed.

The concordant occurrence with sinonasally localized eosinophilic angiocentric fibrosis suggests a close pathogenetic relationship (see below).

Manifestation
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Possible at any age; adults between 40 and 60 years of age are preferred; children may also be affected less frequently.

Localization
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Especially face, preferably on the nose, chin, forehead, temples and cheeks, also on the capillitium.

About 10% of cases are extrafacially localized: upper extremity, trunk.

Rare is an involvement of the oral mucosa (see also eosinophilic angiocentric fibrosis.

Clinical features
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Roundish to oval, 0.5-2.0 cm large, mostly solitary but also several or numerous, slightly raised, firm, symptomless, red or brown-red, scale-free plaques with dilated follicle orifices. This results in an "orange peel-like" surface aspect in an early stage of the disease. This is no longer detectable in later, long-term plaques or nodules. In these cases, the surface is smooth and free of follicles.

Rare are large, 5.0-8.0 cm large, bizarrely configured, solid plaques that prove to be extremely resistant to therapy.

Diascopically, a yellow-brownish infiltrate is characteristic.

Concordant occurrence with other diseases:

  • Eosinophilic angiocentric fibrosis: Inflammatory pseudotumorous mucosal affection which is interpreted as a monotopic sinonasal variant of an IgG4-associated autoimmune disease. Since it may occur concordantly with the facial granuloma, a close pathogenetic connection between the two entities can be assumed, especially since their histological substrate shows a high degree of communities.
  • A concordant occurrence of the facial granuloma with retroperitoneal fibrosis is rare.
  • In very rare cases the facial granuloma has been described as a precursor of severe vasculitides like Wegener's granulomatosis(granulomatosis with polyangiitis) or Churg-Strauss syndrome(eosinophilic granulomatosis with polyangiitis) (individual case reports - confirmations are missing).

Histology
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  • In the early stage, signs of leukocytoclastic vasculitis can be detected with perivascularly oriented neutrophil granulocytes and nuclear dust and fibrin in the vessel walls. Fibrinoid vessel occlusions are also observed in early stages.
  • In clinically "fully developed" lesions, a dense, diffuse infiltrate is found in the upper and middle dermis. Vascular orientation is usually not (no longer) detectable. Epidermis and skin appendages remain unaffected. The infiltrate-free border strip is typical. The infiltrate consists of lymphocytes, neutrophilic leukocytes and nuclear dust, eosinophilic leukocytes, histiocytes and plasma cells. In addition, proliferation of fibrocytes.
  • In the late stage, onion-skin fibrosis can be impressive, with centrally located remnants of leukocytoclastic vasculitis. Storiform patterns are also possible. Frequently accentuated plasmacellular component. Occasionally, granulomatous tissue reaction with histiocyte- and granulocyte-rich infiltrates are observed.

The following algorithm can be used as a schematic diagram:

Histopathological algorithm of the facial granuloma (lowest common denominator: italics, leading symptoms: bold) varies according to Ratzinger et al. 2105
Accentuated around post-capillary venules
Capillaries left out of the less strongly involved
perivascular leukocytoclasia
Damage to endothelial cells
Fibrin in/around vessel walls
Perivascular extravasation of erythrocytes
Edema in the papillary dermis
Collagen degeneration
Variable number of eosinophils
Plasma cells and fibrosclerosis

Direct Immunofluorescence
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No groundbreaking fluorescence pattern. Deposits of immunoglobulins (especially IgG), complement (C3/C4) and fibrinogen were detected at the dermo-epidermal junction zone, but in the vessel walls and around vessels of the middle dermis.

Differential diagnosis
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Clinical differential diagnosis:

  • Drug reaction, fixed: Rarely occurring in the facial region; short anamnesis; succulent foci without follicular emphasis; tendency to blistering.
  • Lymphadenosis cutis benigna: Clinically and anamnestically very similar; almost always solitary; follicular accentuation is possible; the colour is not red but brown; diascopic: self-infiltrate.
  • Lupus erythematosus (CDLE): Mostly a long disease career, atrophic surface epithelium; in older herds scarring; painful when spread with fingernail. Histology and IF are diagnostic.
  • Sarcoidosis: Brown plaques with smooth surface; no follicular accentuation; usually multiple. Histology safely excludes the granuloma eosinophilicum faciei.
  • Tuberculosis cutis luposa: Rarely on the capillitium; acral localisation is typical; atrophic surface epithelium, no follicular accentuation; brown-red colour, typical yellow-brown intrinsic filtrate.
  • Erythema elevatum diutinum: Rare; mostly on the extensor side of the extremities; more rarely on the face; polycyclic, nodular, blue-red or red-brown, succulent, large plaques. Possible stabbing pain, burning and itching.

Histological differential diagnoses:

  • Leucocytoclastic vasculitis of other genesis: Mostly strong erythrocyte extravasations; no marked eosinophilia; the infiltrate is less pronounced overall.
  • Erythema elevatum diutinum: Considered by some authors as a variant of granuloma (eosinophilicum) faciei. Histologically not to be distinguished.
  • Eosinophilic cellulitis (Wells syndrome): Density, perivascular and interstitial infiltrates; almost exclusively eosinophilic and (few) neutrophil granulocytes. Polygonally limited eosinophilic flame figures in the dermis. No signs of vasculitis.
  • Lymphadenosis cutis benigna (cutaneous pseudolymphoma): dense mature cell dermal infiltrate of lymphocytes, plasma cells, reticulum cells; only occasionally eosinophilic leukocytes. Formation of lymph follicles with cells of the germinal centre. No signs of vasculitis.

Therapy
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Due to benignity of the disease, the therapeutic risk should be weighed. Therapy attempt with DADPS (e.g. Dapson Fatol): 100-200 mg/day over 4 months (therapy success only moderate!). Cave! Determination of glucose-6-phosphate dehydrogenase before starting therapy. If necessary also intralesional glucocorticoid injection with triamcinolone acetonide crystal suspension(Volon A 10 mg, 1:3-1:5 diluted with local anaesthetics, e.g. scandicain).

In case of resistance to therapy, or in case of solitary lesions only, excision in toto, dermabrasion in flat foci, CO 2 laser, argon laser, cryosurgery or cauterization.

Positive therapy effects (individual case reports) were reported by locally applied tacrolimus (temporarily occlusive).

The rare large-area or large-nodular variants of the facial granuloma can persist for years, slowly growing. They prove to be extremely resistant to therapy. In these cases, irradiation with fast electrons is indicated.

Progression/forecast
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Chronic course, occasionally spontaneous healing under scarred atrophy.

Note(s)
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There is a close etiopathogenetic relationship to the erythema elevatum diutinum. In both cases the processes are vasculitic. The histological substrate (factor: eosinophilia) is decisive for differential diagnosis. The clinical aspect of the punched surface (orange peel-like) is caused by the pressure of the dermal infiltrate. This leads to an interfollicular protrusion of the surface. If the follicle is preserved at the same time, it is pulled inwards like a wick, resulting in these "follicular impressions". Important differential-diagnostic feature to differentiate between malignant (follicle-destroying) processes.

However, tissue eosinophilia is not a constant feature of the facial granuloma. It fluctuates between predominantly strong and absent. In addition and thus misleadingly, the term "eosinophilic granuloma" is occupied by a variant of Langerhans cell histiocytosis. To avoid this misunderstandable situation the term "granuloma faciale" is increasingly used, alternatively granuloma faciale with eosinophilia.

Literature
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  1. Barnadas MA et al (2006) Direct immunofluorescence in granuloma faciale: a case report and review of literature. J Cutan pathogen33:508-511
  2. Bonet J et al (2001) Eosinophilic granuloma of the jaws: a report of three cases. Med Oral 6: 218-224
  3. Lazarov A et al (2003) Contact orofacial granulomatosis caused by delayed hypersensitivity to gold and mercury.J Am Acad Dermatol 49: 1117-1120
  4. Ludwig E et al (2003) New treatment modalities for granuloma faciale. Br J Dermatol 149: 634-637
  5. Madan V (2011) Recurrent granuloma faciale successfully treated with the carbon dioxide laser. J Cutan Aesthet Surg 4:156-157
  6. Müller CSL et al (2016) Diagnostic and histological features of cutaneous vasculitis/vasculopathies. Act Dermatol 42: 286-301
  7. Nayak NV et al (2014) Postirradiation periocular granuloma faciale associated with uveitis. Ophthal Plast Reconstr Surg 30:e92-95.
  8. Nogueira A et al (2011) Granuloma faciale with subglottic eosinophilic angiocentric fibrosis: case report and review of the literature. Cutis 88:77-82
  9. Ortonne N et al (2005) Granuloma faciale: a clinicopathologic study of 66 patients. J Am Acad Dermatol 53:1002-1009.
  10. Ratzinger G et al (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitis. JDDG 1092-1118
  11. Rieker J et al (2006) Multifocal Granuloma eosinophilicum faciei. Successful treatment with topical tacrolimus. dermatologist 57: 324-326
  12. Rütten A et al (2007) Extrafacial Granuloma eosinophilicum. dermatologist 58: 435-439
  13. Taylor G et al (1994) Granuloma faciale successfully treated with the argon laser - a case report. Eur J Dermatol 4: 623-624
  14. Tojo G et al (2012) Successful treatment of granuloma faciale with topical tacrolimus: a case report and immunohistochemical study. Case Rep Dermatol 4:158-162
  15. Vassallo C et al (2015) Chronic localized leukocytoclastic vasculitis: clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement. G Ital Dermatol Venereol 150:87-94
  16. Wigley JEM (1945) Sarcoid of Boeck? Eosinophilic granuloma. Br J Dermatol 57: 68-69

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Last updated on: 15.01.2021