ABSTRACT
CASE REPORT: A 54-year-old female presented with a progressive swelling of the upper eyelid since 6 months. Magnetic resonance imaging (MRI) showed an extraconal supratemporal orbital lesion. After resection the histopathological diagnosis confirmed a Merkel cell carcinoma in the lacrimal gland. CLINICAL COURSE: Due to an incomplete resection the patient underwent adjuvant radiochemotherapy and is relapse-free for 1.5 years. CONCLUSION: Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor, usually occurring on sun-exposed skin and in 10% in the region of the eyelids. The occurrence of MCC in the lacrimal gland is even less common with only two published cases.
Subject(s)
Carcinoma, Merkel Cell/diagnosis , Carcinoma, Merkel Cell/therapy , Eyelid Neoplasms/diagnosis , Eyelid Neoplasms/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/therapy , Chemoradiotherapy, Adjuvant , Diagnosis, Differential , Female , Humans , Magnetic Resonance Imaging , Middle Aged , Rare Diseases/diagnosis , Rare Diseases/pathology , Treatment OutcomeABSTRACT
The ocular cicatricial pemphigoid (OCP) belongs to a family of chronically progressive autoimmune disorders, predominantly affecting mucous membranes (mucous membrane pemphigoids). It is an immunopathologically heterogeneous group of disorders with variable phenotypes that share the unique feature of a subepidermal blistering, through disruption of the adhesion between epidermis and dermis. A key feature is the chronically active inflammation with consecutive fibrosis, leading to a partial or complete loss of function of the affected organ. The ocular disease as a chronic cicatrising conjunctivitis is a common manifestation of the mucous membrane pemphigoid. The identification of the subtle pathology and the prompt initiation of an appropriate therapy are of pivotal importance. One purpose is to prevent further vision loss due to extensive corneal scarring and life-threatening systemic complications, such as the formation of oesophageal or tracheal strictures. So far there are no prospective, randomised studies, regarding the therapy guidelines with an evidence level more than III. The autoimmune nature of the disease implies that systemic immunosuppression is the only effective treatment option, most notably in extended stages. The aim of our study is to give a guideline for a stage adjusted therapy with conventional immunosuppressants and to give a perspective for alternative therapies, especially for recalcitrant disease.
