Eyelid tumour and juvenile hyaline fibromatosis
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1 The British Association of Plastic Surgeons (2005) 58, CASE REPORT Eyelid tumour and juvenile hyaline fibromatosis J. De Wan*, F. Evens, A. De Mey Department of Plastic and Recontructive Surgery, Brugmann University Hospital, Brussels, Belgium Received 20 August 2003; accepted 20 April 2004 KEYWORDS Juvenile hyaline fibromatosis; Eyelids; Neoplasms; Blepharoptosis; Gingival fibromatosis Summary We report the case of a 2-year-old boy suffering from juvenile hyaline fibromatosis. Our patient had all the main clinical features of the disease, i.e. multiple cutaneous facial nodules, gingival fibromatosis and osteolytic lesions in the proximal metaphysis of the tibia and humerus symmetrically. However, he also presented an eyelid tumour scalloping the superior orbital osseous rim, causing blepharoptosis. The histopathology confirmed this very rare condition. After 6 months, our patient has improved functionally and cosmetically following surgical resection of the eyelid tumours without recurrence. Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. *Corresponding author. address: jeandewan@hotmail.com Juvenile hyaline fibromatosis (JHF) is a very rare condition, with only 65 cases described world-wide but none in the plastic surgery literature. This hereditary condition has an autosomal recessive transmission pattern, as suggested by investigation of family cases. 1,2 Consanguinity is not rare and observed in 30% of the cases. 3 JHF may be a metabolic disease of collagen type VI, 4,5 but it is more likely the result of an aberrant synthesis of proteoglycans and glycosaminoglycans by fibroblasts. 6 8 Murray described molluscum fibrosum in and in 1962 Puretic et al. reported several cases. 10 Ishikawa and Mori referred to this condition as systemic hyalinosis in 1964, 11 and the name fibromatosis hyalinica multiplex juvenilis was introduced by Drescher et al. in Gingival fibromatosis with multiple dermal hyaline tumours is also known as Murray-Puretic- Drescher syndrome. 12 The current term JHF was coined by Kitano et al. in Clinically, there are multiple subcutaneous lesions mostly on the face and the scalp, gingival enlargement covering the teeth and large painful joint contractures. Radiologically osteolytic and scalloping lesions are present mainly at the proximal metaphysis of long bones, usually bilaterally. The age of onset ranges from birth to 4 years. 2 The mental development is normal. 2,3,13 There is an almost equal sex distribution, 3 and no ethnic differences. 14 Some clinical reports with long follow-up 8,15 show that the life expectancy is good. Surgery substantially improves these patients condition both functionally and cosmetically. Recurrence of the soft tissue masses have frequently been reported in the few long-term follow-up studies. 8,16 The present paper deals with a child affected by this condition associated with a palpebral tumour and progressive blepharoptosis. S /$ - see front matter Q 2004 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. doi: /j.bjps
2 Eyelid tumour and juvenile hyaline fibromatosis 107 Case report The male patient was first seen at 20 months for bilateral superior palpebral swelling with mild blepharoptosis. Blood and urinary exams were normal, and ultrasound showed a soft tissue subcutaneous mass with cartilaginous consistency. The father (38 years) and the mother (27 years) had no personal medical history but were cousins. The pregnancy was uneventful. A few months later, the child presented with enlargement and hyperplasia of the mandibulary and maxillary gums, which hampered feeding, a mild anaemia, and a perianal tumour. A perianal polypectomy revealed a benign verrucous papilloma. At 28-months-old he was seen again for bilateral palpebral soft tumours and moderate blepharoptosis (Fig. 1). Neck extension was necessary to allow him forward vision. He exhibited a generalised gingival hypertrophy (Fig. 2) and subcutaneous nodules of up to 1 cm, around the lips, in the nasojugal and alarjugal grooves, on the chin (Fig. 1) and around the ears. He developed a recurrence of his perianal polyp. No café-au-lait spots were seen, and his general health and intellectual development were normal. An MRI revealed subcutaneous bilateral cartilaginous palpebral masses, in close connection to the superior orbital rim (Fig. 3). A biopsy of the gums showed non-specific chronic inflammatory response. A skeletal survey showed scalloping symmetrical lesions of the proximal metaphysis of both the humerus and tibia, with no joint deformation (Fig. 4). The skin karyotype was normal and cytogenetic evaluation of the tumour showed no anomaly. The eyelid tumours were removed, under general anaesthesia via blepharoplasty incision. The mass was removed preserving the levator aponeurosis. The crescent-shaped tumour was adherent to the orbital superior rim (Fig. 5), and had to be removed on the right side along with a marginal rim orbitectomy. The skin lesions around the nose, the mouth and ears were removed by elliptical excision. A perianal polypectomy was performed, as well as an osseous biopsy of the proximal tibia. Histology revealed that all the facial lesions had similar well-defined nodules with fusiform fibroblastic cells, in a fascicular arrangement. A ground dermal fibrohyaline substance was present, preserving the epidermis, with close connection to the underlying periosteum and osseous structure, Figure 1 (A, B): Bilateral superior palpebral tumours, blepharoptosis, and facial skin lesions. especially in the palpebral lesions. The cell cytoplasm was lightly stained and abundant, with rounded or ovoid nuclei containing finely dispersed chromatin. The eosinophilic hyaline-like ground substance stained positively with periodic acid- Schiff stain (Fig. 6). No inflammatory reaction or nucleocytoplasmic atypical abnormalities were observed. The overall process was consistent with JHF. At 6 months follow-up, the blepharoptosis was corrected with a good function of the levator aponeurosis and no sign of recurrence was observed on the facial scars (Fig. 7).
3 108 J. De Wan et al. Figure 2 Generalised gingival hypertrophy. Discussion There are two types of JHF. 2,16,17 In the first, osseous lesions and joint contracture appear with skin lesions early in life, associated with visceral involvement (gastrointestinal tract, heart, lung), deterioration of the general condition, intractable diarrhoea and a recurrent pulmonary infection. This type may represent the more aggressive form of the disease and is known as infantile systemic hyalinosis (ISH). 5,18 20 ISH confers a poor life expectancy. In the second type, skin tumours appear first, followed later by osseous and joint abnormalities. This is the JHF form, which is milder and with a later onset. Kan 13 and Glover 5 suggest that the two conditions represent parts of a spectrum. These two entities may give rise to confusion in incomplete presentation cases, which are common. 1,8,14 Our patient showed the main diagnostic criteria for JHF: 3 facial skin tumours and gingival hypertrophy. He also showed minor diagnostic criteria: scalloping osseous lesions of the proximal metaphysis of the humerus and tibia. No signs of contractures in these joints or a family history of Figure 3 masses. MRI: bilateral superior subcutaneous palpebral Figure 4 lesion. Proximal tibial metaphysis scalloped by the the disease (other minor diagnostic criteria 3 ) were found. Skin lesions, which are the constant features, 3 are polymorphous and can be divided into three types: 3,4,8 small pearly papules on the face and neck; small nodules with a translucent appearance on the ears and around the nose, and firm, large, subcutaneous tumours on the scalp, 14 trunk and limbs. These are slow-growing 8,15,16 and painless. The number of cutaneous lesions varies from case to case. Occasionally perianal papillomatous lesions may also be found. 3,4,6,7 Eyelids are rarely affected by the disease. The only other case with eyelid involvement was published by Miyake 16 where there was a bilateral medial canthal nodule, but without any bony involvement.
4 Eyelid tumour and juvenile hyaline fibromatosis 109 In our patient, the bilateral eyelid subcutaneous mass, responsible for ptosis, was associated with a scalloping effect on the superior orbital rim. This scalloping may be caused, as is the case in long bones, by the tumoural infiltration of these dermal nodules. 3,21 Ishikawa and Hori found that one of these bony defects revealed infiltration of hyaline material similar to that seen in the skin of our patient. 11 The gingival tissue enlargement is rarely present at birth and usually begins with the eruption of the permanent dentition. 22 But may develop with the primary dentition. It is responsible for bleeding leasing to mild hypochromic anaemia, and for feeding problems as it covers the teeth. 7 The differential diagnosis should include: Figure 5 (A): Surgical specimen scalloping superior orbital rim (B) stretching the levator aponeurosis. Figure 6 Ground fibrohyaline substance staining positively for PAS (periodic acid-schiff). 1. Neurofibromatosis where the cutaneous lesions appear in older children, with café au lait spots. 2,4 2. The Winchester syndrome where small joints are contractured, stiffened and the skin thickened without any nodules. There is hypertrichosis, hyperpigmentation and corneal opacities. 2,3,14,21 Radiologically generalised osteoporosis and progressive resorption of the carpal bones is observed Lipoid proteinosis should also be excluded and is characterised by amorphous matrix accumulation, which is acellular. 4,24 4. The more aggressive form (ISH) must be differentiated from infantile myofibromatosis (IMF), 3,4,16 also known as congenital generalised fibromatosis (CGF). The clinical and histopathologic findings are similar, but there is never a gingival or joint involvement. 4,25 In addition, nodules can be found in muscles. The myofibroblastic nature of the constituent cells is histopathologically characteristic. 4 The specific histologic features of JHF play an important role in the final diagnosis Finally, when gingival enlargement is present alone, it is named hereditary or idiopathic gingival fibromatosis disorder. An association exists with hypertrichosis and epilepsy 12,22 and can also be caused by drugs such as phenytoin, nifedipine or cyclosporine A. 22 Gingival hyperplasia is rarely part of a syndrome. 12,22 In the Zimmerman-Laband syndrome, ears and nose are thickened and enlarged, nails are dysplastic with hypoplastic terminal phalanges. Moreover, there is a joint hyperextensibility, a hepatosplenomegaly, and often mental retardation. Other syndromes with gingival fibromatosis include Cowden (multiple hamartomas), Rutherfurd
5 110 J. De Wan et al. Figure 7 Six months follow-up with correction of the blepharoptosis and no sign of recurrence. (corneal dystrophy), Cross (hypopigmentation with athetosis), Jones (progressive deafness) and prunebelly syndromes. 12 Treatment The tumours in JFH continue to increase in size and number with age. 3,6 8,10,11,16 Intralesional steroid injections 27 and proteolytic enzymes have been tried without permanent results. 6,28 A surgical remover of symptomatic skin lesions can be useful, especially in cases (like ours) with functional impairment. 2,15 Excision of the nodules has been considered more mutilating than the primary disease, 8,29 whereas some believe that the lesions will regress spontaneously with age. 6,30,31 Woyke et al., 15 who followed two cases with a 19- year follow-up, reported the appearance of small new tumours even after puberty, but these are relatively few in number compared to those which arise in childhood and adolescence. The early surgical excision of all significant tumours when they are small is the most effective way to treat these patients. After surgical treatment, recurrences are frequent, 1,6,8,10,13 despite microscopically clear surgical margins. 15 This is the case for long-term follow-up 8,16 although others hold that these recurrences are quite rare. 15 References 1. Drescher E, Woyke S, Markiewicz C, et al. Juvenile fibromatosis in siblings (fibromatosis hyalinica multiples juvenilis). J Pediatr Surg 1967;2: Fayad MN, Yacoub A, Salman S, et al. Juvenile hyaline fibromatosis: two new patients and review of the literature. Am J Med Genet 1987;26: Gilaberte Y, Gonzalez-Mediero I, Lopez Barrantes V, et al. Juvenile hyaline fibromatosis with skull-encephalic anomalies: a case report and review of the literature. Dermatology 1993;187: Enzinger FM, Weiss SW. In: Enzinger FM, Weiss SW, editors. Fibrous tumours of Infancy and childhood, 3rd ed. Soft tissue tumours, Vol. 5. New York: Mosby; p Glover MT, Lake BD, Atherton DJ. Infantile systemic hyalinosis: newly recognised disorder of collagen. Pediatrics 1991;87(2): Kitano Y, Horiki M, Aoki T, et al. Two cases ofjuvenile hyaline fibromatosis: some histological, EM and tissue culture observations. Arch Dermatol 1972;106: Keser G, Karabulut B, Oksel F, et al. Two siblings with juvenile hyaline fibromatosis: case reports and review of the literature. Clin Rheumatol 1999;18: Quintal D, Jackson R. Juvenile hyaline fibromatosis: a 15- year follow-up. Arch Dermatol 1985;121: Murray J. On three peculiar cases of molluscum fibrosum in children. Med Chir Trans 1873;38: Puretic S, Puretic B, Fiser-Herman M, et al. A unique form of mesenchymal dysplasia. Br J Dermatol 1962;74: Ishikawa H, Hori Y. Systematisierte Hyalinose in Zusammenhang mit Epidermolysis Bullosa Polydystrophica und Hyalinosis Cutis et Mucosae. Arch Klin Exp Dermatol 1964;218: Bakaeen G, Scully C. Hereditary gingival fibromatosis in a family with the Zimmerman Laband syndrome. J Oral Pathol Med 1991;20:457 9.
6 Eyelid tumour and juvenile hyaline fibromatosis Kan AE, Rogers M. Juvenile hyaline fibromatosis: an expanded clinicopathologic spectrum. Pediatr Dermatol 1989;6(2): acyk WK, Wentzel LF. Juvenile hyaline fibromatosis in two South African black children. Int J Dermatol 1996;35(10): Woyke S, Domagala W, Markiewicz C. A 19-year follow-up of multiple juvenile hyaline fibromatosis. J Pediatr Surg 1984; 9: Miyake I, Tokumaru H, Sugino H, et al. Juvenile hyaline fibromatosis case report with five years follow-up. Am J Dermatopathol 1995;17(6): Shehab ZP, Raafat F, Proops DW. Juvenile hyaline fibromatosis. Int J Ped Otorhinolaryngol 1995;33: Landing BH, Nadorra R. Infantile systemic hyalinosis: report of 4 cases of a disease, fatal in infancy, apparently different from juvenile systemic hyalionosis. Pediatr Pathol 1986;6: Hutchinson I. Juvenile systemic hyalinosis a rare cause of gingival hypertrophy: a case report. Int J Paediatr Dent 1996;6: Glover MT, Lake BD, Atherton DJ. Clinical, histological and ultrastructural findings in two cases of infantile systemic hyalinosis. Paediatr Dermatol 1992;9(3): Larralde M, Santos-Munoz A, Calb I, et al. Juvenile hyaline fibromatosis. Pediatric Dermatol 2001;18(5): Bittencourt LP, Campos V, Moliterno LFM, et al. Hereditary gingival fibromatosis: review of the literature and a case report. Oral Med 2000;31: Dunger DB, Dicks-Mireaux C, O Driscoll P. Two cases of Winchester syndrome with increased urinary oligosaccharide excretion. Eur J Pediatr 1987;146: Moy LS, Moy RL, Matsuoka LY, et al. Lipoid proteinosis: ultrastructural and biochemical studies. J Am Acad Dermatol 1987;16: Piattelli I, Scarano A, Di Bellucci A, et al. Juvenile hyaline fibromatosis of gingiva: a case report. J Periodontol 1996; 67: Visona A, Ronconi G, Montaguti A, et al. Juvenile hyaline fibromatosis case report and review of the literature. Pathologica 1987;79: Finlay AY, Ferguson SD, Holt PJA. Juvenile hyaline fibromatosis. Br J Dermatol 1983;108: Nunziata Rega A, de Sanctis N, Caracciolo G, et al. J Pediatr Orthop 1998;7: Bedford CD, Sills JA, Sommolet-Olive D, et al. Juvenile hyaline fibromatosis: a report of two severe cases. J Pediatr 1991;119: Neill DB, Kasser JR. Juvenile hyaline fibromatosis. A case report and review of muskuloskeletal manifestations. J Bone Joint Surg 1989;71: Woyke S, Domagala W, Olszewski W. Ultrastructure of a fibromatosis hyalinica multiplex juvenilis. Cancer 1970;26:
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