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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
- Eosinophilic angiocentric fibrosis and granuloma faciale: analysis of cellular infiltrate and review of literature.
Narayan J, Douglas-Jones AG.
Department of ENT, University Hospital of Wales, Cardiff, Wales.
Ann Otol Rhinol Laryngol. 2005 Jan;114(1 Pt 1):35-42. Abstract quote
Eosinophilic angiocentric fibrosis (EAF) is a rare specific inflammatory condition that affects the nose and upper airways and is characterized by distinctive histopathologic features.
We report the clinical, histopathologic, and radiologic features of EAF in an elderly woman who presented to us with complete stenosis of the left nostril, nasal swelling, and granuloma faciale (GF) of the overlying nasal skin.
The histopathologic spectrum of the early, intermediate, and late stages of EAF in the deeper nasal tissues is illustrated along with the adjacent changes of GF in the overlying nasal skin. Quantification of immunohistochemically stained lymphocyte cell subsets in the perivascular inflammatory component was performed by using software to compare the proportions of lymphocyte subsets in the two conditions. The results showed similar proportions of perivascular CD4 T lymphocytes in EAF and GF.
We believe that both EAF and GF may represent unusual tissue responses. Similar analysis in other cases will help confirm these findings. A review of the literature suggests that the condition, if localized, is amenable to surgical treatment. In progressive late stages, EAF causes fibrotic stenosis of the affected site with localized extension.
Eosinophilic angiocentric fibrosis: an unusual entity of the sinonasal tract.
Onder S, Sungur A.
Department of Pathology, Hacettepe University, Sihhiye, Ankara, Turkey.
Arch Pathol Lab Med. 2004 Jan;128(1):90-1 Abstract quote.
We present the case of a 45-year-old man who presented with medication-resistant chronic bilateral nasal obstruction. The patient had no known history of allergy or any other disease.
Nasal septoplasty was performed to relieve his symptoms. Histologically, thick collagen bundles were seen, characteristically whorling around vessels in a fibrotic stroma. Inflammatory cells, rich in eosinophils, were scattered throughout the lesion. No granulomas, necrosis, or vessel destruction were present. A diagnosis of eosinophilic angiocentric fibrosis was made.
This neoplasm, which has very characteristic histologic features, is a rare entity of the sinonasal tract.
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
- Granuloma faciale: a clinicopathologic study of 66 patients.
Ortonne N, Wechsler J, Bagot M, Grosshans E, Cribier B.
Department of Pathology, Hopital Henri Mondor, Creteil, France.
J Am Acad Dermatol. 2005 Dec;53(6):1002-9. Abstract quote
BACKGROUND: Only case reports or studies of small series of patients have focused on granuloma faciale (GF).
OBJECTIVE: We sought to describe the clinicopathologic characteristics of GF in a large series of patients.
METHODS: We conducted a retrospective analysis of 66 patients and 73 skin specimens.
RESULTS: GF mostly presented as reddish plaques or nodules in middle-aged adults. One third of patients had multiple sites involved and 5 patients had extrafacial lesions. A clinical diagnosis of GF was made in only 10 cases; sarcoidosis, lymphoma, lupus, and basal cell carcinoma were the main differential diagnoses. The most frequent histopathologic features were the presence of a grenz zone, neutrophils, and telangiectases. Vascular changes were frequent, although necrotizing vasculitis appeared to be rare. There was often an association of acute and chronic inflammatory patterns, suggesting that GF follows a chronic history with recurrent acute phases, rather than distinct successive acute and chronic stages.
LIMITATIONS: In this retrospective study, a reliable analysis of the outcome of patients could not be performed. Results of direct immunofluorescence tests and laboratory investigations were lacking in many cases.
CONCLUSION: GF is often clinically misdiagnosed; its morphologic spectrum is broader than usually described and includes a lack of eosinophils, the presence of vascular changes with rare vessel wall necrosis, and associated acute and chronic inflammatory patterns.VARIANTS DISSEMINATED Disseminated granuloma faciale.
Zargari O.
Department of Dermatology, Razi Hospital, Guilan University of Medical Sciences, Booali Building, Hafez Street, Rasht, Iran 41378.
Int J Dermatol. 2004 Mar;43(3):210-2 Abstract quote
A 40-year-old man presented in January 2001 with multiple purple plaques and nodules, which had been present on the back for approximately 3 years. The lesions had gradually extended over the face, trunk and proximal extremities. He had no symptoms except occasional mild pruritus. The patient was in good health and was on no medications.
Physical examination revealed multiple violaceous to brown, indurated, 5-50-mm, round to oval plaques on the face, arms, shoulders, and back (Fig. 1), as well as a solitary lesion on the right thigh. Surface telangiectases were noted, especially on the shoulder lesions. There was no scaling or ulceration. Routine laboratory tests were unremarkable. In April 1999, another medical center performed a biopsy of what they thought was sarcoidosis. The results were reported as "possible angiolymphoid hyperplasia with eosinophilia."
With the possibility of granuoma faciale (GF) in mind, another skin biopsy was obtained from a facial lesion. This revealed a diffuse, relatively dense infiltrate of neutrophils, eosinophils and mononuclear inflammatory cells in dermis with an obvious Grenz zone (Fig. 2). Pilar units were intact, and endothelial cell swelling was present (Fig. 3).
Retrospective evaluation of the initial biopsy, taken from the back, revealed the same changes, and helped confirm the diagnosis of GF. The patient was treated with liquid nitrogen for 20 s followed immediately by intralesional triamcinolone acetonide (5 mg/ml). This treatment was repeated every 4 weeks for three courses, resulting in partial resolution of the lesions.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
- Granuloma faciale: a clinicopathological study of 11 cases.
Marcoval J, Moreno A, Peyr J.
Department of Dermatology, Hospital de Bellvitge, University of Barcelona, Spain.
J Am Acad Dermatol. 2004 Aug;51(2):269-73. Abstract quote
BACKGROUND: Granuloma faciale is an uncommon disease of unknown etiology that is often misdiagnosed clinically and by general pathologists.
OBJECTIVE: To describe the clinicopathological features of a series of patients with granuloma faciale.
METHODS: Eleven patients diagnosed with granuloma faciale between 1990 and 2002 were included in the study.
RESULTS: Granuloma faciale was diagnosed in 11 patients (9 male and 2 female, mean age 53.45 years). All of them presented facial cutaneous lesions and two of them also developed extrafacial lesions. Histologically, in 8 cases the infiltrate was limited to the upper half of the dermis. Two specimens showed fibrinoid necrosis. Concentric fibrosis around small blood vessels was demonstrated in 5 patients.
CONCLUSION: The presence of abundant fibrosis in 5 of our patients similar to that observed in erythema elevatum diutinum suggests that granuloma faciale and erythema elevatum diutinum may be produced by similar or the same pathogenic mechanism.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE DIRECT IMMUNO-FLUORESCENCE
Department of Dermatology, Hospital de la Sta. Creu i St. Pau, Barcelona, Spain.
A 49-year-old male presented at our department with erythematous brownish plaques in the malar areas and left cheek of 9 years' evolution.
Histopathological study revealed a dense, predominantly perivascular, inflammatory infiltrate in the reticular dermis. The infiltrate was composed of abundant neutrophils, lymphocytes, histiocytes, and eosinophils. Leukocytoclasia and fibrin in some vessel walls were also observed. The patient was diagnosed with granuloma faciale (GF).
Direct immunofluorescence (DIF) study showed heavy immunoglobulin G (IgG) and less intense deposits of IgA, IgM, C(3) and C(1q) surrounding superficial and deep vessels of the reticular dermis. Perivascular and diffuse fibrinogen deposits were also present. No deposits were detected at the dermoepidermal junction.
Our findings lend support to the hypothesis that classical pathway activation of complement may be involved in the development of vasculitis in GF.
Immunofluorescence findings in granuloma faciale: report of two cases.Zirwas MJ, Abell E, Ruben A, Silverman AR, Wolff J, Deng JS.
University of Pittsburgh School of Medicine, Department of Dermatology, Pittsburgh, PA; The Dermatopathology Laboratory, Pittsburgh, PA; Wheeling, West Virginia; Westmorland Dermatology Associates, Greensburg, PA; VA Pittsburgh Healthcare System, Dermatology Service, University Drive, Pittsburgh, PA 15240, USA.
J Cutan Pathol. 2003 May;30(5):314-317. Abstract quote BACKGROUND: Immunofluorescence findings in granuloma faciale have been infrequently described. Reported findings include granular IgA, IgG, IgM, and C3 deposits in the dermoepidermal junction, in blood vessel walls, and on connective tissue fibers; IgG in the basement membrane zone, and IgG around blood vessels.
OBSERVATIONS: We report two cases of granuloma faciale in which biopsy specimens were sent for routine pathology as well as immunofluorescence. Additional work-up included serologic testing for markers of lupus erythematosus (LE). Immunofluorescence in both cases revealed granular IgA, IgG, IgM, C3, and C5-9 deposits along the dermoepidermal junction only. Blood vessel walls and connective tissue fibers were spared. Serologic studies for markers of LE were negative.
CONCLUSION: Immunofluorescence findings were essentially identical to those which would be expected in cutaneous LE; however, the combination of negative serologic studies for LE, histopathologic findings typical of granuloma faciale, and clinical findings most consistent with granuloma faciale allowed the diagnosis of granuloma faciale to be rendered with certainty. Interpretation of results of direct immunofluorescence of skin should be correlated with clinical presentation, histopathological findings and other laboratory results in order to render final diagnosis of a given patient.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.
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Last Updated August 28, 2006
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