1/7. Non-Hodgkin's lymphoma and auricular hypoplasia: associated with juvenile colloid milium or ligneous conjunctivitis?The exact nature of amyloid-like hyaline material deposits in the skin is not well understood in some disorders. Three of those--ligneous conjunctivitis, ligneous periodontitis and colloid milium--have been rarely reported in a same patient. We report a case of mucosal and skin deposits of an amyloid-like homogeneous material associated with non-Hodgkin's lymphoma and congenital auricular hypoplasia. We discussed and reviewed the literature on these unique associations to determine whether these are the same pathological process. We also noted whether this case represents a new syndrome or a coincidental association.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/7. Unilateral refractory (erosive) conjunctivitis: a peculiar manifestation of pemphigus vulgaris.A woman aged 57 years had conjunctivitis of the right eye since February 2003. It had started with a pinhead-sized blister at the margin of the upper eyelid and was progressive in nature. Intense redness of the right eye, lacrimation, and severe pain confined to the right forehead were the major complaints. She had been under medical care ever since, without any tangible outcome. Its refractory nature impelled the ophthalmologist to seek dermatologic consultation, for apparently the diagnosis seemed to have eluded the consultant. Accordingly, she reported on March 2, 2004 for the opinion of severe incessant itching, profuse lacrimation, and pain that was confined only to the right eye and forehead. The very fact that she had reported with continuous rubbing of the right eye re-enforced the suspicion of the episode being an exclusive expression of pemphigus vulgaris of the eye that probably was the reason for ineffectiveness of the drugs given thus far to her. Examination of the right eye was marked by intense inflammation of the palpebral and bulbar conjunctiva, apparent in the form of severe redness (Figure 1). The conjunctiva was studded with several scattered minute erosions. corneal opacity and cataract were its accompaniment, whereas the left eye was normal. mucous membrane of the oral cavity was thoroughly scanned for blister and/or erosions but was normal. The rest of the skin surface was also unaffected. Nikolsky's sign was elicited by asking the patient to rub the eye. In fact, this was responsible for periodic recurrence of the episode. Tzanck test was performed by preparing, fixing, and Giemsa staining the smear from one of the erosions over the conjunctiva. The stained slides were examined under oil-immersion, which revealed plentiful acantholytic cells characterized by large nucleus containing nucleoli and occupying almost the entire eosinophilic cytoplasm and basophilic cell wall. The two biopsies from the conjunctiva were also taken with the help of a 3-mm punch. One of the biopsies was subjected to serial sections. The sections were stained with hematoxylin and eosin stain and examined by light microscopy. The presence of split/cleavage in the epidermis (intraepidermal) above the basal (suprabasal) was cardinal. The cleavage was filled with acantholytic cells of characteristic morphology (vide supra) (Figure 2). The other biopsy was subjected to direct immunofluorescence and was found complementary to the preceding microscopic pathology. Routine investigations comprising total and differential leukocyte count, liver and kidney function tests, chest skiagram, and electrocardiogram were normal. The preceding findings were fairly conducive to form the diagnosis of erosive conjunctivitis emanating from pemphigus vulgaris. Accordingly, pulse therapy (intermittent high-dose) containing 100 mg dexamethasone in 5% glucose daily by slow IV infusion on 3 consecutive days, along with 500 mg of cyclophosphamide on Day 1 only followed by continuous low-dose 50 mg cyclophosphamide, was administered. The patient has since recovered completely and is now under surveillance.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
3/7. Ligneous inflammation of the cervix: a case report.BACKGROUND: Ligneous change of the genital tract is a very rare condition and may result in infertility. CASE: A 27-year-old woman presented with primary infertility and ligneous changes of the genital tract. Cervical and endometrial samples revealed subepithelial deposition of dense, amorphous, eosinophilic material associated with intensive inflammatory cell infiltration. plasminogen functional activity was 16%. CONCLUSION: The clinician must be aware of the systemic nature of this disorder and the multisystem complications.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/7. Ligneous conjunctivitis with ear involvement.We report the association of ligneous conjunctivitis with middle-ear and tympanic membrane involvement in two children. eye and ear specimens revealed histopathologic and ultrastructural findings consistent with ligneous conjunctivitis, characterized by an amorphous, eosinophilic material with acute and chronic nongranulomatous inflammation. ear specimens demonstrated granulation tissue along with the above features. Conjunctival and ear lesions from these patients were studied histochemically, immunohistochemically, and ultrastructurally. Our studies demonstrate fibrin and albumin deposition as a common feature in all lesions. Deposition of other components, such as mucopolysaccharides, amyloid, immunoglobulins, and mast cells, were, however, variable. The diversified nature of these lesions, together with the association of eye and ear disease, substantiates the existence of an underlying systemic disorder of unknown cause.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/7. The masquerade syndrome.Malignant lesions of the lid and ocular adnexae may present with striking inflammatory signs and this has most often been described with meibomian gland carcinoma and given the name of the masquerade syndrome. This paper presents examples of lid malignancies which have not only masqueraded as predominantly inflammatory lesions but whose origin was further obscured by the nature of the spread of the tumour in lid and conjunctiva. Recognition of the true nature of the malignancy only follows biopsy of the centre of the lesion which is fully justified in an older patient presenting with unilateral chronic, unresponsive inflammation especially involving the canthus and bulbar conjunctiva as well as the lid. Histological confirmation of the nature of the peripheral spread of the tumour is also of importance since pagetoid spread has been shown to be associated with more aggressive neoplasia and increased mortality.- - - - - - - - - - ranking = 3keywords = nature (Clic here for more details about this article) |
6/7. Focal giant papillary conjunctivitis from retained contact lenses.Three cases of retained contact lenses leading to focal papillary conjunctivitis of the upper lids reminiscent of the more diffuse changes of lens or suture-induced giant papillary conjunctivitis are reported. One silicone and two cabufocon lenses were responsible. Corneal changes occurred in two of the three cases. Secondary infection with staphylococcus aureus was found in one patient. In two, conjunctival cytology suggested a hypersensitivity mechanism. In all three patients the simple removal of the retained lens was sufficient to reverse pathology. The focal nature of the papillary changes observed in these patients as well as in those cases occurring post-operatively from exposed suture edges would point towards an important direct mechanical etiologic factor in addition to the proposed hypersensitivity basis for the similar diffuse upper lid papillary hypertrophy characteristic of giant papillary conjunctivitis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/7. Juvenile Behcet's disease in Arab children.Behcet's disease is a chronic, relapsing, multisystem disease characterized by the clinical triad of genital ulcers, oral ulcers and ocular involvement. Twelve Saudi children are presented, all of whom satisfied the international criteria for the classification of Behcet's disease and whose initial manifestations appeared at or before the age of 16 years. The male-to-female ratio was 1.4:1. The mean age at onset was 11.5 years (range 7-16 years) and the mean duration of disease was 6.5 years (range 3-13 years). Oral ulcers were present in all patients (100%), genital ulcers in 11 patients (91%), ocular involvement in the form of anterior and/or posterior uveitis in 6 patients (50%), skin manifestations in 10 patients (83%), musculoskeletal symptoms in 9 patients (75%), and central nervous system involvement in 6 patients (50%). One patient had thrombophlebitis and another had pulmonary artery aneurysm. No renal, cardiovascular or gastrointestinal abnormalities were detected. The pathergy test was positive in 3/7 patients. HLA B5 (W51) typing was positive in 5/10 patients. This report of juvenile Behcet's disease in Saudi children suggests that this multisystem disease has an aggressive nature and should be considered in the differential diagnosis of childhood vasculitis in endemic areas.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |