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Background
This is an uncommonn skin rash, typically presenting as erythematous plaques on the face. Rarely, extrafacial cases have been described. Because many dermatological conditions may mimic this disease, a skin biopsy is the only way to definitely confirm the diagnosis.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Granuloma faciale eosinophilicum
Granuloma faciale with eosinophiliaINCIDENCE Uncommon AGE RANGE-MEDIAN Median 45 years Males GEOGRAPHY Mainly caucasians
DISEASE ASSOCIATIONS CHARACTERIZATION EOSINOPHILIC ANGIOCENTRIC FIBROSIS
Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases.Thompson LD, Heffner DK.
Department of Endocrine and Otorhinolaryngic-Head & Neck Pathology, Armed Forces Institute of Pathology, 6825 16th St, NW, Washington, DC 20306-6000, USA.
Am J Clin Pathol 2001 Feb;115(2):243-8 Abstract quote Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum.
Histologically, the lesions demonstrated a characteristic perivascular "onion-skin" fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. Necrosis and foreign body-type giant cells were not identified.
Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.
Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale.
Burns BV, Roberts PF, De Carpentier J, Zarod AP.
Department of Otolaryngology, North Manchester General Hospital, Manchester.
J Laryngol Otol 2001 Mar;115(3):223-6 Abstract quote Eosinophilic angiocentric fibrosis (EAF) is an unusual fibrotic condition affecting the mucosa of the upper respiratory tract, of which there have been eight reported cases. The condition is thought to be associated with the rare skin disorder granuloma faciale, which is histologically identical, and was present in two cases of EAF.
We report the third case where EAF and granuloma faciale occurred together, to highlight this type of intranasal fibrosis as a distinct pathological entity.
PATHOGENESIS CHARACTERIZATION GAMMA-INTERFERON
Immunophenotypic analysis suggests that granuloma faciale is a gamma-interferon-mediated process.Smoller BR, Bortz J.
Department of Dermatology, Stanford University Medical Center, CA 94305.
J Cutan Pathol 1993 Oct;20(5):442-6 Abstract quote Granuloma faciale (GF) is an uncommon inflammatory dermatosis with characteristic clinical and histologic features. Very little is known about its pathogenesis. We used a battery of immunoperoxidase lymphocyte markers to study the population of hematopoietic cells present in a case of GF.
The majority of non-myelocytic hematopoietic cells present were T-helper lymphocytes. The cells stained strongly with antibodies against the interleukin-2 receptor and with anti-lymphocyte functional antigen (LFA 1 alpha) antibodies. Overlying keratinocytes did not stain with ICAM-1 or HLA-DR, which may account for the presence of the Grenz zone in granuloma faciale.
These findings suggest that a gamma-interferon-mediated process may play some role in the pathogenesis of this disorder.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL VARIANTS EXTRAFACIAL Granuloma faciale with lesions on the face and hand.
Okun MR, Bauman L, Minor D.
Dermatopathology Laboratory, Boston City Hospital, Department of Dermatology, Tufts University School of Medicine, USA.
Arch Dermatol 1965 Jul;92(1):78-80 Abstract quote A patient with granuloma faciale had multiple lesions on the face and one lesion on the dorsal surface of one hand.
This case adds a clinical parallel to the recognized histological parallel between granuloma faciale and erythema elevatum diutinum.
Disseminated granuloma faciale.Rusin LJ, Dubin HV, Taylor WB.
Arch Dermatol 1976 Nov;112(11):1575-7 Abstract quote Granuloma faciale is a well-defined entity that almost exclusively involves the face, although it has been reported in extrafacial locations.
The infrequent reports of extrafacial lesions may reflect the inconspicuous nature of extrafacial lesions or a failure to specifically examine the patient for such lesions.
Extrafacial granuloma faciale.Konohana A.
Department of Dermatology, Nippon Kokan Hospital, Kawasaki, Japan.
J Dermatol 1994 Sep;21(9):680-2 Abstract quote Granuloma faciale nearly always occurs on the face; extrafacial lesions are extremely rare. This is the tenth reported case.
Extrafacial granuloma faciale closely resembles erythema elevatum diutinum; however, they are distinct entities which can be differentiated from each other.
Granuloma faciale with extrafacial lesions.Roustan G, Sanchez Yus E, Salas C, Simon A.
Department of Dermatology, Clinica Universitaria Puerta de Hierro, Madrid, Espana.
Dermatology 1999;198(1):79-82 Abstract quote Extrafacial involvement in granuloma faciale (GF) is rather exceptional.
We report herein a patient with GF associated with lesions on the trunk and the forearm. Histological studies of facial and extrafacial lesions shared similar characteristics: a mixed inflammatory infiltrate with abundant eosinophils in the superficial and middle dermis with a narrow grenz zone of uninvolved dermis between the epidermis and the infiltrate. Evidence of vasculitis was clearly observed in both biopsy specimens. Treatment with dapsone did not alter the course of the disease.
We review the 12 cases of extrafacial GF that have been reported in the English and Spanish literature. In these cases a diagnosis of erythema elevatum diutinum (EED) may be suggested. Although GF and EED may share some pathogenic mechanisms, there are several clinical and histological differences between them that make us consider EED and GF as distinct entities.
Extrafacial granuloma faciale: report of a case and response to treatment.
Castellano-Howard L, Fairbee SI, Hogan DJ, Fenske NA, Messina JL.
Division of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine, 12901 Bruce B. Downs Blvd, MDC II, Tampa, FL 33612, USA.
Cutis 2001 May;67(5):413-5 Abstract quote Granuloma faciale (GF) is a rather uncommon form of chronic vasculitis that infrequently involves extrafacial sites. Treatment of this disease is extremely challenging.
We report a case of GF with extrafacial lesions and a unique response to treatment. The diseases that are clinical and histologic mimics of this disorder, as well as a review of various treatment modalities, are discussed.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL Grenz zone of uninvolved dermis beneath the epidermis
Polymorphous inflammatory infiltrate in the papillary and mid dermis
Neutrophils, lymphocytes, eosinophils, monocytes, and mast cells
Vasculitic changes with nuclear dust and vessel wall damage
Extravasated RBCs and hemosiderin depositionLater lesions show considerable fibrosis around vessels
SPECIAL STAINS/IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE ELECTRON MICROSCOPY
Granuloma faciale. Ultrastructural study of three cases.Schnitzler L, Verret JL, Schubert B.
J Cutan Pathol 1977 Aug;4(3):123-3 Abstract quote Light and electron microscopic observations were carried out in three cases of granuloma faciale. The dense granulomatous infiltrate of the corium showed: 1) more eosinophils than suspected by light microscopy, most of them with degenerative changes; 2) Charcot-Leyden chrystals; 3) numerous large histiocytes with their cytoplasms filled with lysosomal vesicles containing various inclusions (lamellar figures, myelin bodies, circular concentric organelles, digested membrane structures).
The lack of Langerhans granules in dermal cells seems a valid reason to differentiate granuloma faciale from eosinophilic granuloma of the skin, usually considered a variety of histiocytosis X. No evidence of bacterial or viral infections was noticed. Granuloma faciale, despite its uncertain etiology, seems to represent a true entity.
Granuloma faciale: an ultrastructural study.Vicente Ortega V, Sanchez-Pedreno P, Rodriguez Vicente J.
Department of Pathology, Faculty of Medicine, University of Murcia, Spain.
Ultrastruct Pathol 1998 Mar-Apr;22(2):135-40 Abstract quote Granuloma faciale is an uncommon process that can easily be confused with other skin diseases.
To avoid incorrect treatment, correct diagnosis is of primary importance. A diagnosis of granuloma faciale can be made by a microscopic study of the dense granulomatous infiltrate in the reticular dermis with abundant polynuclear eosinophils and by an ultrastructural study of the eosinophils, which show characteristic alterations in their cytoplasmatic granules. The absence of Langerhans granules differentiates granuloma faciale from histiocytosis X.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES LYMPHOMA
Cutaneous lymphoma masquerading as granuloma faciale.Noe JM, Sober AH, Fein SH, Grendell JH, Sasken H.
Ann Plast Surg 1979 Oct;3(4):361-7 Abstract quote Granuloma faciale is a presumably benign disorder of the skin--usually of the face--characterized by a dense, polymorphous, inflammatory infiltrate including numerous eosinophils, separated from the epidermis by a clear or "grenz" zone, and possessing a small vessel, leukocytoclastic vasculitis.
Primary malignant lymphoma of the skin, other than mycosis fungoides, is an unusual entity that may follow a widely variable course and is often extremely difficult to diagnose definitively. A patient is presented in whom a lesion consistent with granuloma faciale changed its histological appearance and clinical behavior into that of a malignant lymphoma.
PTERYGIUM
Pterygium with granuloma faciale-like histologic picture.Rausing A, Havelius U.
Department of Pathology, Malmo University Hospital, Sweden.
Cornea 1998 Sep;17(5):562-5 Abstract quote PURPOSE: Description of a case with an atypical conjunctival pterygium with an unusual histologic picture suggesting granuloma faciale, a vasculitis-like disease of facial skin with a chronic, indolent course. Discussion of diagnosis and treatment.
METHODS: Repeated clinical and histologic observations.
RESULTS: A pterygium with an unusual nasosuperior localization was excised and examined microscopically. There was a dense vessel-related inflammatory infiltrate, essentially identical to granuloma faciale. The lesion recurred and, as granuloma faciale is amenable to dapsone (diphenyl sulfone), treatment with 100 mg daily resulted in rapid improvement, whereas reduction of the dose to 50 mg daily resulted in relapse. Reinstitution of the original dose resulted in normalization of the histologic picture. However, a recurrent pterygium developed, which had a quite unspecific histologic picture.
CONCLUSION: A disease with a histologic picture essentially identical to granuloma faciale may manifest itself as a pterygium. Treatment with dapsone may be beneficial.
TINEA FACIALE Tinea of the face caused by Trichophyton rubrum with histologic changes of granuloma faciale.
Frankel DH, Soltani K, Medenica MM, Rippon JW.
Department of Medicine, University of Chicago Pritzker School of Medicine, IL.
J Am Acad Dermatol 1988 Feb;18(2 Pt 2):403-6 Abstract quote We report a case of tinea of the face caused by Trichophyton rubrum in which the histologic changes were consistent with granuloma faciale.
We recommend that local infection with dermatophytes be considered in all cases of histologic granuloma faciale, especially when the clinical presentation is atypical for this dermatosis.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS TREATMENT GENERAL Granuloma faciale. Comparison of different treatment modalities.
Dinehart SM, Gross DJ, Davis CM, Herzberg AJ.
Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock
Arch Otolaryngol Head Neck Surg 1990 Jul;116(7):849-51 Abstract quote Granuloma faciale is an uncommon cutaneous condition characterized by brownish-red macules, papules, nodules, and plaques. Separate areas of granuloma faciale involving the nasal skin of a 32-year-old man were treated using electrosurgery, carbon dioxide laser, and dermabrasion.
All modalities provided good cosmetic and functional outcomes. The relative advantages and disadvantages of each technique are presented.
CRYOTHERAPY
Granuloma faciale: successful treatment of nine cases with a combination of cryotherapy and intralesional corticosteroid injection.Dowlati B, Firooz A, Dowlati Y.
Center for Research and Training in Skin Diseases and Leprosy, Tehran University of Medical Sciences, Iran.
Int J Dermatol 1997 Jul;36(7):548-51 Abstract quote BACKGROUND: Granuloma faciale is a rare disorder characterized by asymptomatic papules, nodules, and plaques on the face. Although the exact pathogenesis of this disease in unclear, it is considered a variant of leukocytoclastic vasculitis confined to the skin. Several medical and surgical methods have been used to treat it with variable results.
CASE REPORTS: We report nine cases of granuloma faciale treated with a combination of cryotherapy with liquid nitrogen and intralesional injection of corticosteroids.
RESULTS: The lesions cleared completely in all of the patients without any side-effects. No recurrences have been observed.
CONCLUSIONS: Cryotherapy with liquid nitrogen, followed by intralesional injection of corticosteroids, is a safe and effective method to treat granuloma faciale.
DAPSONE On the efficacy of dapsone in granuloma faciale.
van de Kerkhof PC.
Department of Dermatology, University of Nijmegen, The Netherlands.
Acta Derm Venereol 1994 Jan;74(1):61-2 Abstract quote Granuloma faciale is a relatively rare skin disorder with a chronic course. In many cases treatment of granuloma faciale is difficult. In the present report the beneficial effect of dapsone in this condition is described.
LASER
Granuloma faciale in a child successfully treated with the pulsed dye laser.Welsh JH, Schroeder TL, Levy ML.
Department of Dermatology, Baylor College of Medicine, Houston, Texas, USA.
J Am Acad Dermatol 1999 Aug;41(2 Pt 2):351-3 Abstract quote Granuloma faciale, a rare, chronic, cutaneous disorder, is uncommon in children. Numerous treatment modalities have been used to treat granuloma faciale but none are consistently effective. In addition, many of the previously used therapies have unwanted side effects or are not well tolerated by children.
We report a case of granuloma faciale in an 11-year-old child successfully treated with the pulsed dye laser. To our knowledge, this is the first report of this treatment modality for granuloma faciale.
Treatment of granuloma faciale with the pulsed dye laser.
Elston DM.
Wilford Hall Air Force Medical Center, Lackland AFB, Texas 78236, USA.
Cutis 2000 Feb;65(2):97-8 Abstract quote Granuloma faciale is a chronic benign vasculitis that generally affects the skin of the face. The lesions are commonly refractory to therapy. A patient with long-standing granuloma faciale refractory to topical corticosteroid and dapsone therapy had an excellent response to treatment with the pulsed dye laser.
Granuloma faciale successfully treated with long-pulsed tunable dye laser.Chatrath V, Rohrer TE.
Department of Dermatology, Boston University School of Medicine, Boston, Massachusetts 02118, USA.
Dermatol Surg 2002 Jun;28(6):527-9 Abstract quote BACKGROUND: Granuloma faciale has been treated in the past with different modalities but the majority have had the risk of scarring.
OBJECTIVE: Based on the principle of selective photothermolysis, we considered using the newer long-pulsed tunable dye laser for the treatment of granuloma faciale to target the vessels and minimize scarring.
METHODS: Confirmation of the diagnosis by a punch biopsy of the lesion was followed by three treatments on separate occasions 6 weeks apart with the long-pulsed tunable dye laser.
RESULTS: There was significant flattening of the lesions after two treatments, with complete clearing after the third. No scarring was detectable and there was no recurrence in the 9-month follow-up.
CONCLUSION: We conclude that granuloma faciale may be successfully treated with the long-pulsed tunable dye laser with minimal risk of scarring, especially in cosmetically sensitive areas.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Weedon D. Weedon's Skin Pathology. Second Edition. Churchill Livingstone. 2002.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Last Updated 10/4/2002
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