Necrobiotic Xanthogranuloma with paraproteinemia

Upper and lower eyelid reconstruction for severe disfiguring necrobiotic xanthogranuloma.Orbit. 2004 Mar;23(1):65-76.

BACKGROUND: Necrobiotic xanthogranuloma is a rare disease featuring generalized xanthomatous inflammatory skin lesions associated with paraproteinemia and possible lymphoproliferative diseases. Eyelid involvement can be unilateral or bilateral and ranges from minor xanthelasma-like lesions to severe ulcerative disease with consecutive keratitis and scleritis. CASE REPORT: The authors report the case of a 67-year-old woman with extensive necrobiotic xanthogranuloma involving the eyelids, head and neck, anterior chest, and both upper and lower extremities. Periorbital involvement caused severe upper and lower lid ectropium with chronic conjunctival inflammation and unilateral exposure keratitis. During a persistent period of low disease activity, granulomatous lesions and scars were widely excised, lids partially shortened and large full-thickness skin grafts applied. Uninvolved parts of the upper arms had to serve as donor sites, as other possible donor sites were not available. After successful reconstruction of the left side and no local recurrence of the disease, the right side was corrected in the same way. Full eyelid closure was achieved and skin grafts healed without complications. No recurrence of the disease appeared at the sites of operation, despite continuous new lesions elsewhere. CONCLUSION: Severe cicatricial eyelid deformation caused by necrobiotic xanthogranuloma can be treated with success by excision and free skin grafting. The mechanisms of recurrence at excision sites described by others remain unclear, but at least during phases of low activity, the described treatment is safe and recurrence is not to be expected.

Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: Case report and review of literature
Klin Monatsbl Augenheilkd. 2002 Jan-Feb;219(1-2):55-8.

BACKGROUND: Necrobiotic xanthogranuloma is a rare disorder which predominantly affects the periorbital region and is frequently associated with paraproteinemia and lymphoproliferative diseases. PATIENT AND METHODS: A 49-year old woman presented with bilateral, yellowish, subcutaneous lesions involving all four eyelids. These lesions have developed over the last year. Additionally, similar skin lesions developed on the trunk and the lower extremities. Two years ago, the patient had a bilateral episcleritis. An incisional biopsy was taken form the lesion. In addition, we reviewed the literature concerning similar cases. RESULTS: Histologic examination revealed a granulomatous process consisting of histiocytes, foamy cells, multinucleated giant cells (touton- and foreign body-type) and associated with necrobiotic collagen and cholesterol clefts. General examination revealed the presence of a paraproteinemia of the IgG-typ associated with a hyperlipidemia. The patient was treated with a low dose chemotherapy which resulted in improvement of signs and symptoms. We could identify in the literature 75 similar cases. The mean age of those patients was 53 years. CONCLUSION: The clinical and histologic findings in our patient were typical for a necrobiotic xanthogranuloma with associated paraproteinemia. The most favorable treatment response has been reported with low doses of systemic administered chemotherapeutic agents in combination with radiation therapy; however, the mortality due to underlying diseases is high.

Paracoccidioidomycosis

Eyelid involvement in paracoccidioidomycosis. Ophthal Plast Reconstr Surg. 2004 May;20(3):212-6

PURPOSE: To describe a series of patients with eyelid lesions caused by paracoccidioidomycosis and to estimate the prevalence of eyelid involvement in this disease METHODS: The medical records of 439 patients with paracoccidioidomycosis admitted to our hospital from 1992 to 2002 were reviewed. Age, sex, and clinical forms of the disease were recorded. All patients with eyelid involvement had a skin biopsy positive for paracoccidioidomycosis and were examined by an ophthalmologist with oculoplastic training. RESULTS: Of 439 patients with acute, subacute, or chronic paracoccidioidomycosis, 11 (2.5%) had eyelid involvement. Active lesions ranged from erythematous patches of madarosis to frank destructive ulcers indistinguishable from malignancies. Healed lesions were characterized by a high degree of fibrosis. Cicatricial changes induced eyelid malpositions (entropion or ectropion) and fusion of eyelid tissues to the globe. Madarosis was a constant finding in the inactive lesions. CONCLUSIONS: The prevalence of eyelid involvement in paracoccidioidomycosis is low. Isolated active lesions are usually diagnosed as malignant tumors. Cicatricial changes are characterized by a high degree of fibrosis. If not treated, the mycosis can destroy the eyelid.

Palpebral paracoccidioidomycosis.Mycopathologia. 1997;140(1):29-33

Paracoccidioidomycosis: study of six cases with ocular involvement.
Mycopathologia. 1988 May;102(2):87-96.

Molluscum Contagiosum

Ocular manifestations of molluscum contagiosum. Clin Exp Optom. 2006 Nov;89(6):390-3.

PURPOSE: Two cases of molluscum contagiosum (MC) are presented to illustrate the range of potential anterior segment complications of this condition. METHODS: Clinical records for two patients diagnosed with MC are retrospectively reviewed. Diagnosis and management of both cases are presented. RESULTS: The first patient demonstrates a classic presentation of ocular MC. The patient was young and had several dermal lid lesions at the time of presentation. The second case represents a less common presentation. The patient was an adult and had a single lid lesion that was not apparent at the initial examination. Both patients had follicular conjunctivitis that resolved with excision of the concomitant eyelid lesions. CONCLUSION: MC is a treatable cause of chronic conjunctivitis or keratoconjunctivitis. Eye-care providers should be mindful that MC could present as a follicular keratoconjunctivitis with or without obvious dermal lid lesions. The possibility of immuno-compromise must be considered in patients with multiple large lesions, cutaneous lesions that do not respond to standard therapy, or recurrent lesions.

Molluscum contagiosum eyelid lesions as the initial manifestation of acquired immunodeficiency syndrome.Am J Ophthalmol. 1997 Aug;124(2):240-1.

PURPOSE: To report molluscum contagiosum as the initial manifestation in acquired immunodeficiency syndrome (AIDS). METHOD: Case report. A 34-year-old man was examined with atypical, extensive molluscum contagiosum of the eyelids. RESULTS: Biopsy of the lesions confirmed molluscum contagiosum, and a previously normal fundus now disclosed bilateral cotton wool spots and classic signs of cytomegalovirus retinitis in the left eye. Human immunodeficiency virus (HIV) antibody testing was positive. CONCLUSIONS: Manifestation of atypical and extensive eyelid molluscum contagiosum may warrant additional history taking, comprehensive ophthalmic examination, including dilated ophthalmoscopic examination, and HIV testing.

Molluscum contagiosum of the eyelid.J Fr Ophtalmol. 2004 Mar;27(3):301-3.

Multiple bilateral eyelid molluscum contagiosum lesions associated with TNFalpha-antibody and methotrexate therapy. Am J Ophthalmol. 2002 Aug;134(2):270-1

Eyelid molluscum contagiosum in patients with human immunodeficiency virus infection.Orbit. 1999 Jun;18(2):75-81.

Molluscum contagiosum, involving the upper eyelids, in a child infected with HIV-1.Pediatr AIDS HIV Infect. 1996 Feb;7(1):43-6.

Glossary   

Gross examination of the skin specimen

Reporting of biopsies taken for Inflammatory Skin Diseases

Lichenoid (Interface)Tissue Reaction Pattern

Psoriasiform Reaction Pattern

Granulomatous Reaction Pattern

Vesiculobullous Reaction Pattern

Spongiform Reaction Pattern

Vasculopathic Reaction Pattern

Lichen planus-like lesions

Lichen Nitidus

Bullous Pemphigoid

Hailey-Hailey Disease 

Granuloma Annulare

Necrobiosis Lipoidica

Necrobiotic Xanthogranuloma   

Rheumatoid Nodule

Cutaneous Sarcoidosis

Foreign body granuloma

Rosacea

Erythema Nodosum

Folliculitis

Impetigo

Furuncle(Boil)

Carbuncle

Role of immunohistochemistry in Dermatopathology 

Cutaneous Pseudolymphoma

Drug related cutaneous lesions

Cutaneous lesion in graft-
versus host disease

Cutaneous infection and infestations

Histopathological patterns in cutaneous infections

Cutaneous Infection and Infestation 1: Bacterial, Rickettsial and Chlamydial infection

Cutaneous Infection and infestation 2: Spirochetal Infection

Cutaneous Infection and infestations 3 : Mycoses and algal infections

Cutaneous infection and infestations 4 : Protozoal Infections

Cutaneous Infections and infestations 5 : Helminth Infections

Cutaneous Infection and infestation 6: Viral Infections

Cutaneous lesion associated
with AIDS

Myc. Kansasii Inf.

Myc.Leprae Inf.

Myc.Marinum Inf.

Myc.Ulcerans Inf.

Onchocerciasis

Paragonimiasis

Paramyxovirus Infection

Protothecosis