A 42-year-old Moroccan female was admitted to the hospital with fever lasting one month, night sweats and left cervical lymphadenopathy. Laboratory exams showed leukocytes 3300/mm3, erythrocytes 4.370.000/mm3, hemoglobin 12.7 g/dl, thrombocytes 156.000/mm3, LDH 843U/l (normal values: 230-460). Other laboratory findings were all within normal ranges. Serologic investigations excluded viral, bacterial and parasitic infections, including syphilis and cytomegalovirus, Epstein-Barr virus, HIV, Rickettsia coronii, Brucella spp, Salmonella typhi, Salmonella paratyphi, toxoplasma, Leishmania donovani infections. Blood and throat cultures were negative. QuantiFERON TB Gold (ESAT-6, CFP-10, TB7.7 antigens) was negative. Chest X-ray was normal. Chest computed tomography (CT) showed multiple, small-sized bilateral hilar and mediastinal lymphadenopathies. Abdominal CT revealed additional small interaortocaval, para-aortic mesentheric and iliac lymphadenopathies. Due to the presence of multiple lymphadenopathies, a bone marrow biopsy was performed to exclude a lymphoma. Bone marrow examination was considered normal. Histology showed a mild reactive T lymphocytosis, consisting of a regular number of interstitial lymphocytes which showed mainly a CD3+, CD 20-phenotype. Bronchial aspiration with microbiologic cultures for Mycobacterium tuberculosis were negative. A cervical lymph node biopsy was performed. The lymph node structure was completely effaced by diffuse necrotic areas with abundant karyorrhectic debris (Figure 1); the search for DNA of Mycobacterium tuberculosis was negative. A mediastinal lymph node biopsy and a liver biopsy showed normal histological findings. At this time a definitive diagnosis was not available. After two months new laboratory investigations showed a leukocyte count of 3830/mm3, erythrocytes 3360000/mm3, Hgb 9,2 g/dL, thrombocytes 353.000/mm3, AST 44 U/I, ALT 45 U/I, LDH 138 U/l. Since the patient developed recurrent papulo-squamous lesions on her scalp, ears, face, and trunk, she was referred to the dermatology department.
Skin examination showed papular and scaly plaques on the face, the scalp, the V-area of the neck, upper chest, back and shoulder in a photodistributed pattern. No malar rash was noted. Skin lesions were biopsied with a presumptive diagnosis of lupus.
At histology, the epidermis was thinned and displayed focal parakeratosis and vacuolar degeneration of basal keratinocytes, with occasional cytoid bodies. The dermis showed conspicuous interstitial mucin deposits, dense perivascular and perifollicular lymphocytic infiltrates with interface dermatitis (Figure 2) and superficial nuclear debris with karyorrhexis. Direct immunofluorescence failed to demonstrate immunoglobulin or complement deposits along the dermoepidermal junction or around the vessels. Immunologic serum investigations showed absence of antinuclear antibodies, antibodies (anti)-LA/SS-B, anti-ds DNA, anti-phospholipids, anti-pANCA, anti-Jo1, anti-Scl-70, anti-RNP. Antibodies anti-Ro/SS-A (E.I.A) were 59 (normal value: 0-10), ENA (E.I.A) 18.7 (normal value: 0-3). The patient's complement levels were low with C3 71 mg/dL (normal values: 88-201 mg/dL), C4 5 mg/dL (normal values: 16-47 mg/dL). On the basis of clinical, laboratory and histology findings, a diagnosis of SCLE was made.
The patient was given prednisone 25 mg per day and hydroxychloroquine. Skin lesions improved rapidly and healed without scarring. The dosage of corticosteroids was gradually tapered without relapse of skin lesions. After 38-month follow up the patient is free of skin and systemic symptoms. Now she is taking hydroxychloroquine 400 mg/day. Laboratory investigations are all within normal value, except for persistent complement deficiency in C3 and C4.