Miescher's granulomatosis (granulomatosis disciformis chronica et progressiva) in a non-diabetic patient – case report

We present case of granulomatosis disciformis chronica et progressiva Miescher with good response to systemic corticosteroids therapy.Patient 45 years old woman, with primary yellow-brown areas skin lesions, with foci well separated from surroundings on both lower legs, that occurred 5 years ago. In laboratory tests there was no abnormalities. Because of advance suggestion (after last admit in dermatological ward) of observation according to xantogranuloma necrobioticum tests for paraproteinemia were made. Immunoelectrophoresis, IgG, IgM, IgA levels, kappa light chain, lambda heavy chain; were correct, Bence-Johns protein-negative. During hospitalization in Clinic methylprednisolone in dose of 32 mg od, vascular drugs and local steroidotherapy was applied with good therapeutic response.We described case of typical clinical and histological characters of necrobiosis lipoidica. without diabetes-granulomatosis disciformis chronica et progressiva Miescher that despite of suspicion of proper diagnosis for a long time was not treat effective.Necrobiosis lipoidica diabeticorum is a rare disease of unclear etiology that occurs in about 1% of diabetic patients [1]. The disease is characterized by a chronic inflammatory granulomatous process accompanied by vasculitis with perivascular deposits of complement C3 and immunoglobulins IgG, IgM and IgA. Typically found on the lower legs, focal skin lesions comprise well-separated irregular areas of discolored (yellow-brown) tissue. Tissue damage in the centre of foci includes atrophy, sclerosis and teleangiectasia [1-3].Miescher's granulomatosis (also known as granulomatosis disciformis chronica et progressiva) was first reported by Miescher and Leder in 1948. In this condition skin lesions are typically localized bilaterally and symmetrically on the lower legs, and in the absence of diabetes [1,4]. Treatment of all types of necrobiosis lipoidica is difficult, and the condition can be unresponsive to systemic corticosteroids, cyclo