|Year : 2020 | Volume
| Issue : 2 | Page : 121-123
Mononeuritis multiplex with multiple skin ulcers
Mansoor C Abdulla MD
Department of Internal Medicine, M.E.S. Medical College, Perinthalmanna, Kerala, India
|Date of Submission||12-May-2019|
|Date of Acceptance||06-Feb-2020|
|Date of Web Publication||09-Jun-2020|
Mansoor C Abdulla
Department of General Medicine, M.E.S. Medical College, Perinthalmanna, Kerala
Source of Support: None, Conflict of Interest: None
A heterogeneous group of diseases lead to mononeuritis multiplex and multiple skin ulcers. A 45-year-old man presented with multiple painful subcutaneous nodules with ulcerations, distal predominant weakness of both upper and lower limbs, reduced sensations over ulnar distribution of the right hand and high-grade intermittent fever for 2 months. The patient was diagnosed to have cutaneous polyarteritis nodosa based on clinical and histopathological evidence after ruling out other conditions. The patient was treated with prednisolone 1 mg/kg, which was tapered over months and azathioprine was added. When reviewed after 6 months he was afebrile, skin lesions were healed, and the power was improving. Mononeuritis multiplex and multiple skin ulcers have various etiologies. A detailed workup is essential to rule out other systemic diseases before making a diagnosis of cutaneous polyarteritis nodosa which is a benign disorder with a favorable outcome.
Keywords: cutaneous polyarteritis nodosa, mononeuritis multiplex, multiple skin ulcers
|How to cite this article:|
Abdulla MC. Mononeuritis multiplex with multiple skin ulcers. Egypt J Dermatol Venerol 2020;40:121-3
| Introduction|| |
Cutaneous polyarteritis nodosa (CPAN) is a rare form of cutaneous vasculitis involving small- and medium-sized arteries of the dermis and subcutaneous tissue . The lesions are limited to the skin, adjacent muscles, nerves, and joints. Skin manifestations include tender subcutaneous nodules, livedo reticularis, and ulceration, mostly localized to the lower extremity . Extracutaneous manifestations such as arthralgia, myalgia, and peripheral neuropathy [mononeuropathy and mononeuritis multiplex (MNM)] may be associated with the disease . The clinical course is chronic with remissions, relapses, and a favorable prognosis .
| Case report|| |
A 45-year-old man presented with multiple painful subcutaneous nodules with ulcerations, distal predominant weakness of both upper and lower limbs, reduced sensations over ulnar distribution of the right hand, and high-grade intermittent fever for 2 months. The study was approved by the Ethical Committee of the M E S Academy of Medical Sciences. Informed consent was obtained from the patient. There was no history of purpura, Raynaud’s phenomenon, recurrent oral ulcers, hair loss, or malar rash. His medical history was unremarkable. He was a smoker for 25 years (pack year: 75) with occasional alcohol consumption. He denied any history of weight loss, drug abuse, high-risk behavior, and had no sick contacts. He had multiple ulcers of varying sizes with central pallor, crusting, and sloping edges over the medial aspect of the left leg, sacrum, and right trochanteric region ([Figure 1]). All peripheral pulsations were palpable equally and blood pressure was 110/80 mmHg. He had weakness in the distribution of bilateral ulnar, right median, right tibial, and right femoral nerves with reduced pain, temperature, and touch over ulnar distribution of the right hand.
|Figure 1 Multiple ulcers with central pallor, crusting, and sloping edges over the medial aspect of the left leg.|
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He had mild normocytic normochromic anemia, high erythrocyte sedimentation rate (75 mm in 1 h), and C-reactive protein (22.9 mg/dl). Biochemical parameters showed hypoalbuminemia and hyperglobulinemia with reversed albumin/globulin ratio (0.5). Arterial doppler of the left lower limb was normal. Ultrasonography of the abdomen was normal. Nerve conduction studies showed sensory with motor axonal multifocal neuropathy of the upper and lower limbs (MNM). Transthoracic echocardiogram showed no vegetations. Pus culture grew pseudomonas aeruginosa and blood cultures were negative. Differential diagnosis for a man with fever, MNM and multiple skin ulcers include vasculitis, plasma cell dyscrasias, primary systemic amyloidosis (AL), infections, and connective tissue disorders. HIV, venereal disease research laboratory test, and hepatitis B and hepatitis C serologies were negative. Autoantibody profile including antinuclear antibody, rheumatoid factor, C-antineutrophil cytoplasmic antibody, p-antineutrophil cytoplasmic antibody, and antiphospholipid antibodies were all negative. Serum complement levels were normal. Radiograph of the skull and serum protein electrophoresis were normal. Urinary Bence Jones protein measures were negative. Cryoglobulins were not detected. Bone marrow aspirate and trephine showed plasmacytosis (20%) but there was no κ/λ restriction. Serum light chain assay showed elevated light chains with normal κ/λ ratio: free κ 123 mg/l (normal range: 3.3–19.4 mg/l), free λ 96.2 mg/l (normal range: 5.7–26.3 mg/l), and free κ/λ ratio=1.28). Deep incisional skin biopsy from the ulcer showed ulceration and vasculitis ([Figure 2]a and b). Sural nerve biopsy showed lymphocytic vasculitis ([Figure 2]c). Abdominal fat pad biopsy did not show any evidence of AL ([Figure 2]d). He was diagnosed to have CPAN based on clinical and histopathological evidence after ruling out other conditions. The patient was treated with piperacillin tazobactam for the pseudomonal skin infection and later prednisolone 1 mg/kg, which was tapered over months and azathioprine was added. When reviewed after 6 months he was afebrile, skin lesions were healed, and the power was improving.
|Figure 2 (a) Low power view of skin biopsy showing ulceration (H&E, ×100), (b) high power showing focus of vasculitis (H&E, ×400), (c) nerve biopsy showing lymphocytic vasculitis (H&E, ×200), (d) abdominal fat pad biopsy histologically unremarkable (H&E, ×100). H&E, hematoxylin and eosin.|
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| Discussion|| |
CPAN is a rare variant with symptomatic vasculitis limited to the skin, adjacent muscles, nerves, and joints . It involves small- and medium-sized arteries of the dermis and subcutaneous tissue without systemic involvement . Usual clinical manifestations are tender subcutaneous nodules, livedo reticularis, and subcutaneous ulcerations mostly confined to the lower extremity . Extracutaneous manifestations include arthralgia, myalgia, constitutional symptoms, and MNM. The diagnosis is based on clinical features of isolated skin involvement with histopathological evidence of leukocytoclastic vasculitis in the small- to medium-sized arterioles of the deep dermis or the hypodermis with or without associated fibrinoid necrosis .Corticosteroids are a major group of drugs used for treating CPAN . The exact duration of treatment is uncertain. No specific guidelines for the management of CPAN have been developed. Immunosuppressive agents such as cyclophosphamide, azathioprine, or methotrexate are used for treating steroid-resistant cases ,. Although most cases of CPAN has a chronic and benign course, rare instances of evolving into systemic PAN were reported .
Differential diagnosis for a man with fever, MNM, and multiple skin ulcers include vasculitis, plasma cell dyscrasias, primary systemic AL, infections, and connective tissue disorders. Our patient had a negative autoimmune antibody and antiphospholipid antibody profile, negative cryoglobulin, and normal arterial Doppler ultrasonography. The presence of hypoalbuminemia, hyperglobulinemia with reversed albumin/globulin ratio, and significant bone marrow plasmacytosis raised the possibility of plasma cell disorder. The absence of κ/λ restriction in bone marrow immunohistochemistry with normal κ/λ ratio in serum light chain assay ruled out the possibility of a plasma cell disorder. Abdominal fat pad biopsy did not show any evidence of AL. Polyclonal bone marrow plasmacytosis in our patient was probably reactive.
To conclude, MNM and multiple skin ulcers have various etiologies. A detailed workup is essential to rule out other systemic diseases before making a diagnosis of CPAN, which is a benign disorder with a favorable outcome.
On behalf of co-authors the author submit the enclosed manuscript for consideration by the journal. The author confirm that it has not been published in this or a substantially similar form (in print or electronically, including on a website), nor accepted for publication elsewhere, nor is it under consideration by another publication at this time.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]