1. POSTERIOR SQUAMOUS-LIKE MULTIFOCAL PIGMENTED EPITHELIOPATHY (PSMPE) AS THE FIRST CLINICAL MANIFESTATION OF A 23 YEAR OLD FEMALE PATIENT WITH UNCLASSIFIED CONNECTIVE TISSUE DISEASE Panagiotis Heras (1), Ioannis Tsiverdis (1), Theodosis Andrianopoulos (1), Ilias Georgopoulos (1), Antonios Hatzopoulos (1) (1) Clinic of Internal Medicine, General Hospital of Nafplio, GREECE

2. Introduction: PSMPE is generally of unknown pathogenesis. It is reported in cases of thyroiditis, CNS systemic vasculitis, TBC infection, sarcoidosis after hepatitis B vaccination. Case description: A 23 year old female patient admitted to our clinic exhibiting myalgias, fatigue, fever, erythema nodosum. Two days later during hospitalization a loss of sight 9/10 bilaterally was detected. Clinical examination revealed flushing, reticular peliosis and erythema nodosum. The laboratory findings were: Hct: 44%, Hb: 15,2g/dl, ESR: 13, urea: 31, creatinine: 0.74, ANA (+) 1/640 dotted, positive mitotic figures, antidsDNA: 3.0, antiENA (- ), anti-TG (-), ANCA (-), anticardiolipin IgG antibodies: 10.4, IgM: 8.7, anti v2GP1 IgG: 0.8, SACE: 43.3. Antigens and antibodies for hepatitis (A, B, C): negative, antibodies against viruses (EBV, Coxsackie, CMV, Parvo): negative. HLA: A2.26, B44 (W4) 51 (W4) (W2) DR14 (52) DQ 5.3. A Skin-muscle biopsy showed lymphocytic vasculitis of small vessels (non-necrotic). Cerebral MRI-MRA and CSF tests were conducted revealing on specific findings. Fluorescein angiography revealed PSMPE. She started treatment with impulses of corticosteroids and then a 0.5mg/kg of body weight dose with a drastic improvement. Three months later she is submitted to a middle corticoid dose, with her vision fully restored.

3. Discussion-Conclusions: Gass described PSMPE in 1968. It is of unknown etiology, probably due to microangiopathy of the choroid. It may be associated with systemic vasculitis or microscopic vasculitis in other tissues. The patient exhibited symptoms compatible with systemic vasculitis (Raynaud, reticular peliosis, ANA, lymphocytic micro-vasculitis). In this case, PSMPE may be the first manifestation of a yet undifferentiated connective tissue disease.