We reported the first case of Rothmann-Makai syndrome associated with mononeuropathy in a 31-year-old woman. In June 1989, she noticed several small subcutaneous nodules in the bilateral upper arms. On July 2, she woke up because of severe pain below the knee in the right leg which lasted 15 hours. On admission, subcutaneous nodules were recognized on bilateral upper arms, abdomen, gluteal regions and thighs. None of these nodules were associated with local heat or pain. Muscle weakness and dysesthesia were observed in the right foot, but deep tendon reflexes were normal. Electrophysiological studies revealed a decrease of the amplitude of action potential and of the conduction velocity in the right tibial nerve. Microscopic examination of skin biopsy showed lipolysis and diffuse lymphocyte infiltration (panniculitis) and perivascular lymphocyte infiltration was also observed. Routine laboratory data including peripheral blood, ESR, blood chemistry, and CRP were in the normal range and antinuclear antibodies and LE cells were not detected. By those findings, she was diagnosed as Rothmann-Makai syndrome associated with mononeuropathy. Steroid treatment was effective on both skin lesions and mononeuropathy. Steroid treatment was effective on both skin lesions and mononeuropathy. We concluded that this mononeuropathy may be induced by a ischemic change which may be caused by panniculitis.
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G Ital Dermatol Venereol
August 2013
Dipartimento di Scienze Clinico-Chirurgiche Diagnostiche e Pediatriche, Sezione di Dermatologia Università di Pavia, Fondazione IRCCS Policlinico "San Matteo", Pavia, Italia -
Clin Exp Dermatol
May 2006
Department of Dermatology, Kanto Medical Center NTT EC, Shinagawa-ku, Tokyo, Japan.
Rothmann-Makai syndrome (lipogranulomatosis subcutanea) is a rare variant of Weber-Christian disease, but lacks visceral involvements and systemic manifestations. We herein report the case of a 56-year-old Chinese woman with this disorder who complained of subcutaneous tender nodules over her extremities, trunk, and face of 2 years' duration. She was firstly treated with 300 mg of oral roxythromycin for 8 weeks and subsequently treated with 400 mg of oral clarithromycin for the next 8 weeks.
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