A computed axial tomography of the chest, abdomen, and pelvis did not furnish any significant data

A computed axial tomography of the chest, abdomen, and pelvis did not furnish any significant data. 2.?OBSERVATION A 85\year\old Tunisian woman, with a history of osteoporosis, was admitted in our department because of swelling reddened and indurated skin on the face, ears, neck, upper limbs, and back. These skin abnormalities started 2?months before and were progressively worsening. No improvement was noted under antihistamine therapy BML-284 (Wnt agonist 1) (Desloratadine 5?mg daily). On examination, we Rabbit polyclonal to LIN41 found a generalized eruption of 2C3?mm lichenoid papules. Lesions were most common on upper trunk, neck, and face with leonine facies (Figures ?(Figures1,1, ?,2).2). These lichenoid lesions coalesced, leading to the induration of the underlying tissue. There is no Raynaud phenomenon or dysphagia. The remainder of the physical examination was normal; notably, no tumoral syndrome was found. Complementary tests revealed a microcytic hypochromic anemia to 11.1?g/dl due to iron deficiency and an inflammatory biologic syndrome. Calcemia, renal, and liver functions were all normal. Serum protein electrophoresis showed a narrow spike in the gamma region suggesting monoclonal gammapathy. Immunoelectrophoresis revealed monoclonal spikes IgG Lambda () in both blood and urine samples. The bone marrow examination did not find any abnormalities especially plasma cells of aberrant morphology. Thus, the diagnosis of monoclonal gammapathy of the IgG was made. All other tests, including antinuclear antibodies, rheumatoid factors, anti\DNA antibodies, anti\neutrophil cytoplasmic antibodies, and cryoglobulin results, were normal or negative. Serology tests for hepatitis B and C virus and human immunodeficiency virus were negative. Upper and lower gastrointestinal endoscopy were normal. A computed axial tomography of the chest, abdomen, and pelvis did not furnish any significant data. A skin biopsy showed a thinned epidermis with irregularly arranged fibroblasts, fibrosis and diffuses mucin deposition within the upper and mid reticular dermis. A perivascular, lymphocytic, and plasmacytic infiltrate was also present. Hair follicles were atrophic. No signs of malignancy were detected. On that account, the diagnosis of Arndt\Gottron scleromyxedema was retained. The patient was treated with double antihistamine therapy (Hydroxyzine hydrochloride 25?mg was associated with Desloratadine because of severe pruritus with sleep disturbance), prednisone (orally, 1?mg/Kg/day the first month and then gradually tapered), and 9 courses of polyvalent immunoglobulins (intravenously, 2?g/kg for 4?days monthly). Within the next three months, the lesions had almost completely disappeared and antihistamine therapy was stopped. During 9\year follow\up period, the patient remained stable. She did not present any systemic manifestation of (S\AG). Open in a separate window FIGURE 1 Reddened and indurated skin on the neck and on the upper trunk with eruption of lichenoid papules Open in a separate window FIGURE 2 Reddened and indurated BML-284 (Wnt agonist 1) skin on the BML-284 (Wnt agonist 1) neck with eruption of lichenoid papules 3.?DISCUSSION Herein, we report an exceptional case of scleromyxedema in Tunisia. First of all, Arndt\Gottron (S\AG) syndrome is a very rare condition occurring usually in middle\aged adults. 1 , 2 In our patient, the onset of S\AG symptoms began at about 85?years old (elderly onset), which is not frequently described. Moreover, the etiopathogenesis of S\AG still enigmatic to date and its optimal therapeutic strategy remains challenging. 1 , 2 Actually, as noted in our patient, there is hyperproliferation of dermal fibroblasts, with dermal mucin deposition and increased collagen deposition on histology. 3 Clinically, S\AG is a condition revealed, like our patient’s case, by diffused and symmetric lichenoid papules often arranged linearly and causing extensive thickening of the skin. These features usually involve the face, neck, distal forearms, and hands, and set the palms and mucous membranes apart. Even if extracutaneous manifestations of S\AG were absent in our case, it is crucial to constantly seek them out as they can be life\threatening in some cases by involving cardiovascular, gastrointestinal, pulmonary, and central nervous systems. Rheumatologic manifestations can on the one hand.