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 Table of Contents  
Year : 2016  |  Volume : 36  |  Issue : 2  |  Page : 60-62

A resistant case of pyoderma gangrenosum with monoclonal gammopathy of unknown significance showing excellent response to combination therapy of methyl prednisolone pulse and oral cyclosporine

1 N.R.S. Medical College and Hospital, Kolkata, India
2 Kolkata Medical College and Hospital, Kolkata, India
3 Department of Dermatology, Venereology and Leprosy, N.R.S. Medical College and Hospital, Kolkata, India

Date of Submission24-Sep-2016
Date of Acceptance17-Nov-2016
Date of Web Publication21-Mar-2017

Correspondence Address:
Najmus S Jamadar
Kolkata Medical College and Hospital, A 4.1 N.B.C.C, Vibgyor Tower, Newtown, Rajarhat, Kolkata 700156, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-6530.202641

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Pyoderma gangrenosum (PG) is a rare and painful skin condition of uncertain etiology characterized by one or more areas of chronic ulceration with well-demarcated and undermined borders. PG often occurs in patients who have other diseases (arthritis, inflammatory bowel disease, hematologic dyscrasias, monoclonal gammopathy of unknown significance, etc). Immunosuppressive agents have been used for its management. Among them, although corticosteroid is known as the most effective agent, other immunosuppressants including cyclosporine have been selected for patients with PG who were refractory to systemic steroids. Here, we report a case of PG with monoclonal gammopathy of unknown significance, resistant to systemic steroids and cyclosporine, that was successfully treated with a combination of cyclosporine and methyl prednisolone pulse.

Keywords: cyclosporine, methyl prednisolone pulse, pyoderma gangrenosum

How to cite this article:
Das S, Jamadar NS, Roy AK. A resistant case of pyoderma gangrenosum with monoclonal gammopathy of unknown significance showing excellent response to combination therapy of methyl prednisolone pulse and oral cyclosporine. Egypt J Dermatol Venerol 2016;36:60-2

How to cite this URL:
Das S, Jamadar NS, Roy AK. A resistant case of pyoderma gangrenosum with monoclonal gammopathy of unknown significance showing excellent response to combination therapy of methyl prednisolone pulse and oral cyclosporine. Egypt J Dermatol Venerol [serial online] 2016 [cited 2022 Oct 1];36:60-2. Available from: http://www.ejdv.eg.net/text.asp?2016/36/2/60/202641

  Introduction Top

The pathogenesis of pyoderma gangrenosum (PG) remains unknown, and because there are no pathognomonic features, the diagnosis is based on clinical grounds. The disease presents as painful, violaceous, boggy, undermined ulcers, most commonly located on the lower extremities. The wounds usually begin as papulovesicles or pustules occurring either spontaneously or after minor trauma. These areas necrose and progress to a confluent ulcer [1]. In the majority of cases, PG has been reported in association with a number of systemic diseases, including inflammatory bowel disease, rheumatoid arthritis, hepatitis, myeloproliferative disorders, and monoclonal gammopathies [2],[3],[4]. Traditionally, treatment is directed at the associated systemic disease, immune suppression, and local wound care. The treatment remains empiric. Although success has been reported with various therapies, it has not generally been satisfactory.

Here, we report a successful treatment of a patient with resistant PG with a combination therapy of methyl prednisolone pulse and oral cyclosporine.

  Case report Top

A 58-year-old, diabetic patient was suffering from leg ulcer since 2008, which started on the left leg. Skin biopsy was performed and PG was reported. Since then, he was on oral hypoglycemic agent, and the first episode of leg ulcer healed within one month of steroid therapy.

He was suffering from pyrexia after 3 months of healing of the ulcer. He was treated for fever in an institution where biopsy was taken from his right leg. Again PG was confirmed on histopathology. It shows central necrotizing suppurrative inflammation with ulceration and a peripheral vascular reaction comprising perivascular and intramural lymphocytic infiltrates. However, the cause of the fever could not be ascertained. After 2 months, antitubercular drug was started on the basis of one sputum sample showing the presence of acid-fast bacillus. After 15 days of starting of antitubercular drugs, he developed ulceration on his right leg. Thereafter, there was progressive worsening of ulcer with the formation of new ones involving both the anterior and the posterior aspect of legs. He was then treated with oral prednisolone and low-dose oral cyclosporine (100 mg/day). There was no further worsening of the ulcer, with minimal improvement.

After proper renal function monitoring, he was put on cyclosporine at a dose of 100 mg twice a day in combination with oral prednisolone. Treatment was given for 15 days with minimal improvement.

In the process of workup, he was detected as a case of monoclonal gammopathy of undetermined significance, having raised IgA level with M band present in the γ-globulin region. Serum immunoelectrophoresis showed the presence of monoclonal IgA (λ chain) paraprotein. Serum calcium level was normal. Bone marrow examination showed clonal plasma cells less than 10%. One percent per year of patients with monoclonal gammopathy of undetermined significance proceed to develop myeloma. The patient was referred to a hematologist and managed subsequently. Other investigations such as antinuclear antibody and rheumatoid factor remained negative. Colonoscopy was also performed but no abnormality was detected.

Thereafter, we started methyl prednisolone pulse in conjunction with oral cyclosporine. Methyl prednisolone in 5% dextrose was started at a dose of 1 g/day run over in 4 h for consecutive 3 days in a month of each cycle. Cyclosporine was given at a dose of 100 mg twice a day. It was continued throughout the therapy, with regular renal function monitoring. In each cycle the duration of therapy was for three consecutive days. He showed excellent results with this combination therapy. After second pulse, the ulcer almost completely healed and we planned for a total of nine pulses for this patient ([Figure 1] and [Figure 2]).
Figure 1 At the initial stage of treatment.

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Figure 2 Completely healed lesion after pulse therapy.

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  Discussion Top

PG, first described by Brunsting et al. [1], is a rare noninfectious destructive neutrophilic dermatosis affecting the skin and occasionally the subcutaneous fat [5],[6]. It is characterized by single or multiple, chronic and recurrent painful cutaneous ulcerations with mucopurulent or hemorrhagic exudates [5]. PG occurs most commonly on the lower legs with preference for the pretibial area, and in many cases is associated with inflammatory bowel disease, rheumatic or hematological diseases, and malignancies [5],[6]. Diagnosis of PG is based on a history of an underlying disease, typical clinical presentation, histopathology (nonspecific), and exclusion of other diseases that would lead to a similar appearance [5]. Although etiology has not been clearly determined yet, the current accepted theory is that PG is an immunologic-based phenomenon [5],[6]. Several therapies have been used to control PG, with limited success, including systemic therapy, topical therapy, and surgical therapy [5],[6],[7].

The records of eight patients with PG and monoclonal gammopathy showed that all patients except one had an IgA paraproteinemia. To date, seven patients have had a benign course and multiple myeloma has developed in one. In seven patients, the onset of the PG preceded the detection of the monoclonal gammopathy. The monoclonal gammopathy did not seem to influence the morphologic findings, course, or therapy of the PG [8]. As regards hemopathies, the associations most frequently encountered are with myeloid malignancies and monoclonal dysglobulinemia. Evolution toward a true myeloma seems to be exceptional. In a recent publication, 17 cases of association between PG and myeloma were mentioned, the IgA type being most common. Protein electrophoresis is indispensable in patients with PG [9].

In a study from Penysylvania, an article summarizes the management of 22 cases of PG over the past 4 years at the hospital of the University of Pennsylvania; 18 patients with PG were studied using the most sensitive routine laboratory method for the detection of monoclonal immunoglobulins, immunofixation electrophoresis. Four cases of IgA gammopathy were detected, confirming previous reports of the incidence of monoclonal gammopathy in PG. High-dose glucocorticoid therapy (pulse therapy) is an effective treatment for some severe, refractory cases of PG. Eight patients were treated with pulse therapy. Six responded favorably, and none had serious complications [3].

Immunosuppressive agents have been used for its management. Among them, corticosteroid is known as the most effective. However, other immunosuppressants, including cyclosporine A, have been selected for patients with PG who were refractory to systemic steroids [10].

  Conclusion Top

Our patient was refractory to conventional therapy, but ultimately after few trials with immunosuppressive agents he showed excellent response with combination therapy of methyl prednisolone pulse and cyclosporine. Pulse is a well-accepted modality for the treatment of various skin diseases such as pemphigus vulgaris, bullous pemphigoid, systemic lupus erythematosus, and other diseases and few reports suggest pulse for the treatment of PG.We extended pulse therapy in this case as this patient was not responding to other modalities of therapy in conjunction with cyclosporine when one drug was insufficient to control disease process.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Brunsting LA, Goeckerman WH, O’Leary PA. Pyoderma (echthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol 1930; 22:655–680.  Back to cited text no. 1
Powell FC, Schroeter AL, Su WPD, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Quart J Med 1985; 55:173–186.  Back to cited text no. 2
Prystowsky JH, Kahn SN, Lazarus GS. Present status of pyoderma gangrenosum. Review of 21 cases. Arch Dermatol 1989; 125:57–64.  Back to cited text no. 3
Dresch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol 1997; 137:1000–1005.  Back to cited text no. 4
Wollina U. Pyoderma gangrenosum – a review. Orphanet J Rare Dis 2007; 2:19.  Back to cited text no. 5
Tutrone WD, Green K, Weinberg JM, Caglar S, Clarke D. Pyoderma gangrenosum: dermatologic application of hyperbaric oxygen therapy. J Drugs Dermatol 2007; 6:1214–1219.  Back to cited text no. 6
Cohen PR. Neutrophilic dermatoses: a review of current treatment options. Am J Clin Dermatol 2009; 10:301–312.  Back to cited text no. 7
Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum and monoclonal gammopathy. Arch Dermatol 1983; 119:468–472.  Back to cited text no. 8
Carsuzaa F, Pierre C, Dubegny M. Pyoderma gangrenosum and IgA gammopathy. Association with atrophic gastritis. Ann Dermatol Venereol 1989; 116:707–713.  Back to cited text no. 9
Chiba T, Isomura I, Suzuki A, Morita A. Topical tacrolimus therapy for pyoderma gangrenosum. J Dermatol 2006; 33:232.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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