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Ann Med Surg (Lond)
Ann Med Surg (Lond)
MS9
Annals of Medicine and Surgery
2049-0801
Lippincott Williams & Wilkins Hagerstown, MD

10.1097/MS9.0000000000000253
00035
3
Case Reports
Idiopathic recurrent pyoderma gangrenosum with cobalamin deficiency in a 62-year-old male: a case report
Sitaula Seema MD aseema.sitoula@gmail.com

http://orcid.org/0000-0001-9591-3168
Kharel Sanjeev MBBS bkharel_sanjeev@iom.edu.np

Sherpali Aakash MBBS baakashsherpaali2074@iom.edu.np

Shrees Vijay MBBS vijayshreesmagar@gmail.com
b
Mainali Atul MBBS batulmainali1@gmail.com

a Department of Dermatology and Venereology, Maharajgunj Medical Campus
b Department of Internal Medicine, Maharajgunj Medical Campus, Tribhuvan University Teaching Hospital, Maharajgunj, Kathmandu, Nepal
* Corresponding author. Address: Maharajgunj Medical Campus, Tribhuvan University Teaching Hospital, P.O. Box: 4240, Maharajgunj, Kathmandu, Nepal. Tel: +977-9868620160. E-mail address: kharel_sanjeev@iom.edu.np (S. Kharel).
3 2023
17 2 2023
85 3 528531
31 10 2022
25 12 2022
Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.
2023
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0/

Introduction:

Pyoderma gangrenosum (PG) is a rare, neither infectious nor gangrenous, neutrophil-mediated inflammatory dermatosis. In 50–70% of cases, systemic disease is the underlying cause, and the remaining is idiopathic.

Case Presentation:

The authors here present a case of a 62-year-old male with a history of recurrent ulcer over the dorsum of hand diagnosed with recurrent PG with cobalamin deficiency treated with intralesional steroid injection and topical antibiotics along with intramuscular vitamin B12 injections. The patient returned after a year with a history of swelling in the left hand for 1 week, which was managed with intravenous antibiotics.

Clinical Discussion:

The most common kind of PG is ulcerative, which accounts for around 85% of cases that have been found. Ulcerative PG begins as small, painful erythematous or violaceous papules and pustules that quickly develop into ulcers with an exudative, mucopurulent, hemorrhagic base or with areas of necrosis and high, well-defined, serpiginous, violet-blue, or metallic grey borders, which are its defining feature. Glucocorticoids, along with a wide range of additional systemic immunomodulatory medication as alternatives and antibiotics to prevent infection are used for treatment.

Conclusion:

PG is a rare form of neutrophilic dermatosis that can be difficult to diagnose and treat. PG has a mixed nutritional deficiency and a history of ulcers. It is crucial to have a high degree of suspicion when making a diagnosis, as well as to look for associated diseases and start treatment as soon as possible.

Keywords:

cobalamine
idiopathic
pyoderma gangrenosum
OPEN-ACCESSTRUE
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pmcHIGHLIGHTS

Pyoderma grangrenosum is a rare, neither infectious nor gangrenous, neutrophil-mediated inflammatory dermatosis.

The most common kind of pyoderma grangrenosum is ulcerative, which accounts for around 85% of cases that have been found.

Glucocorticoids, along with a wide range of additional systemic immunomodulatory medication as alternatives and antibiotics to prevent infection are used for treatment.

Introduction

Pyoderma gangrenosum (PG) is a rare, neither infectious nor gangrenous, neutrophil-mediated inflammatory dermatosis. It frequently begins as an erythematous nodule or sterile inflammatory pustule, which rapidly transforms into painful ulceration with violaceous undermined margins. Trauma frequently causes or exacerbates PG, a condition known as pathergy1. In 50–70% of cases, systemic disease is the underlying cause. Inflammatory bowel conditions, arthritis, immunoglobulin A monoclonal gammopathies, and myeloid hematological malignancies are the main underlying illnesses. PG, however, can also manifest by itself2. PG also presents with a mixed nutritional deficiency, usually cobalamin and iron deficiency, along with atrophic gastritis in some cases, for which nutritional supplements are also necessary3.

We here present a case of a 62-year-old male with a history of recurrent ulcer over the dorsum of hand diagnosed with recurrent PG with cobalamin deficiency treated with intralesional steroid injection and topical antibiotics along with intramuscular (IM) vitamin B12 injections. Our case has been reported in line with SCARE criteria4.

Case presentation

A 62-year-old male from Kathmandu presented with a history of an ulcer over the right hand. He first noticed the ulcer 2 weeks ago as a small red wound. He treated it as a minor injury and continued his daily activities. As he was a gardener, he thought he might have some injuries from the thorns or while doing his daily work of gardening. Day by day, the wound increased in size and was painful. He noticed that he could see the underlying tissue from the wound and that it was spreading very fast. For this problem, he visited a renowned surgeon in the town. He investigated the gentleman. His regular blood investigations came out to be normal. Then, the surgeon planned for debridement the next day. He explained the nature of the debridement process to the gentleman. The gentleman got scared about the nature of the process, and as the wound was over the skin, he thought of having a second opinion with the dermatologist and came to us. After examination of the nature of the wound and considering the fast spread of a painful ulcer, the first diagnosis made was PG. He also complained of an unintentional weight loss of 12 kg over a period of 3 months. He also complained of generalized weakness. He gave no history of fever, rashes, or loss of hair. He denied consuming alcohol. He used to smoke occasionally. A review of other systems was unremarkable. Family and past medical histories were insignificant. He was not a vegetarian and consumed meat regularly. There was no known food intolerance, medication allergies, or surgical history.

On examination, the patient was afebrile and normotensive with a pulse rate of 110 bpm. He had glossitis and very fragile skin. No lymph nodes were palpable. A single ulcer around 6×4 cm in size, irregular in shape, with erythematous to hyperpigmented borders, and a yellowish necrotic base was present on the right dorsum of the hand. Peripheral pulses were palpable (Fig. 1).

Figure 1 A single ulcer around 6×4 cm in size, irregular in shape, with erythematous to hyperpigmented borders, and a yellowish necrotic base was present on the right dorsum of the hand.

Laboratory investigations revealed hemoglobin of 9 g/dl (reference range: 12–18 g/dl). A peripheral blood film showed megaloblastic anemia. Workup for anemia was done with low serum iron of 2.5 μmol/l (reference range: 10.74–30.43 μmol/l), a total iron binding capacity value of 19.6 μmol/l (reference range: 45–72 μmol/l), low transferrin saturation of 13.8% (reference range: 20–50%), serum ferritin of 86 ng/ml [reference range: 15–200 ng/ml (males), 12–150 ng/ml (females)], low cobalamin level of 113 pg/ml (reference range: 200–1100 pg/ml), and normal folate level of 17.8 pg/ml (reference range: 2–20 ng/ml). Serum cortisol level was low at 82.6 nmol/l (reference range: 138–635 nmol/l) at 8 a.m. in the morning, while sodium and potassium levels were also low at 135 mmol/l (reference range: 135–146 mmol/l) and 3.0 mmol/l (reference range: 3.5–5.2 mmol/l), respectively. The liver function test showed a low albumin level at 27 g/l (reference range: 35–52 g/l) with slightly raised aspartate aminotransferase at 63 U/l (reference range: 0–50). The renal profile and coagulation screening were normal. The antinuclear antibody was negative. Erythrocyte sedimentation rate was 68 mm/h (reference range: 0–22 mm/h for men and 0–29 mm/h for women), and C-reactive protein was 188 mg/l (reference range: <3 mg/l). Serological surveys were negative. He was reevaluated, and it came out that he had a severe vitamin B12 deficiency. The vitamin B12 level was severely low. It was 113 pg/ml (reference range: 200–600 pg/ml).

Cultures and sensitivities from the ulcer were positive for Proteus mirabilis. He was treated with a 2-week course of intravenous amoxicillin and clavulanic acid. A histopathological examination was not done because of pathergy.

The case was diagnosed as pyoderma gangrenous with cobalamin deficiency. The patient was immediately started on an intralesional steroid injection and topical antibiotics. The daily dressing was done with normal saline, placentrex gel, and vaseline gauze dressing. He also consulted a hematologist, who, after further evaluation. He was kept on IM vitamin B12 injections. The patient showed instant improvement; he was very thankful and healed well in 3 weeks’ time.

The patient returned after a year with a history of swelling in the left hand for 1 week. When reviewing the history, he had small injuries that would heal on their own in the past year. The swelling had increased suddenly in the past week. It was painful and extended. He immediately came to visit us. It took no time to make a diagnosis of PG on examination. There were multiple ulcers of varying sizes over the dorsum of the hand, some with the necrotic center, exfoliation, and pus-like discharge (Fig. 2). Pus culture and sensitivity from the site showed Enterobacter and Proteus mirabilis. He was admitted and started intravenous antibiotics with amikacin and flucloxacillin. He gradually improved with daily dressing and was treated on a daily OPD basis after 3 days of hospitalization.

Figure 2 Multiple ulcers of varying sizes over the dorsum of the hand, some with the necrotic center, exfoliation, and pus-like discharge.

Clinical discussion

In 1916, Brocq initially characterized PG, referring to it as ‘phagedenisme geometrique’ because of its cutaneous manifestation. For a while, it was thought that PG was related to disseminated streptococcal bacterial cutaneous infections that could progress to gangrene4. A number of mechanisms appear to interact in the pathophysiology of PG, including abnormalities in neutrophil function, dysregulation of the adaptive immune system caused by an imbalance between T-cell subtypes (predominance of proinflammatory Th17 cells and a minority of regulatory T cells), and dysfunction of the innate immune response caused by the expression and over-activation of multiprotein complexes known as inflammasomes that promote an excessive release of proinflammatory cytokines5. In our case, no other association with systemic or autoimmune disease was identified, and we were diagnosed with idiopathic PG.

There are five recognized categories of clinical presentation, which are based on the characteristics of the lesions and their behavior and are distinct in terms of clinical presentation: classic or ulcerative, bullous or vesiculobullous, vegetative, pustular, and peristomal6. The most common kind of PG is ulcerative, which accounts for around 85% of cases that have been found. Ulcerative PG begins as small, painful erythematous or violaceous papules and pustules that quickly develop into ulcers with an exudative, mucopurulent, hemorrhagic base or with areas of necrosis and high, well-defined, serpiginous, violet-blue, or metallic grey borders, which are its defining feature. These inflammatory lesions heal with amorphous, cribriform scars that resemble ‘cigarette paper’7. Our case also had the classical type of PG that developed into recurrent ulcers.

Cobalamin and iron deficiency were both present in our patient’s case of mixed nutritional deficiency anemia. The cause behind this can be simply due to nutritional deficiency or atrophic gastritis, which are present in most of the Nepalese population. Stomach mucosa parietal cells are destroyed by chronic atrophic gastritis, which reduces gastric acid secretion. This accelerates the decline in the generation of intrinsic factors and the solubilization of iron in the diet, which causes cobalamin deficiency and iron malabsorption8.

In a recent case report also, a systemic lupus erythematosus patient had PG along with cobalamin and iron deficiency, along with atrophic gastritis treated with prednisolone, hydroxychloroquine, methotrexate, parenteral cyanocobalamin, and iron supplements3. IM injections of cyanocobalamin or oral therapy are the two most efficient treatments for cobalamin deficiency. In patients with severe deficiency or severe neurologic symptoms, fast replenishment is possible because only 10% of the usual injectable dose of 1 mg is absorbed9. Our patient was given IM cyanocobalamin injections.

The likelihood of concurrent autoimmune illnesses should be raised by the diagnosis of PG and cobalamin deficiency because if they are not, a considerable delay in treatment with potentially fatal consequences could occur3. Though our patient tested negative for tests of autoimmune disease.

PG is a rare form of neutrophilic dermatosis that can be difficult to diagnose and treat. Squamous cell carcinoma is more likely to form in a chronic PG wound, and patients with unmanaged PG are three times more likely to die. If untreated, PG can have a major adverse impact on morbidity and mortality. PG has historically been treated with a wide variety of therapies, with varying response rates described in the literature, given our poor understanding of its pathophysiology5.

Although there are no guidelines for treatment, it is generally centered on lowering inflammation, facilitating healing, and avoiding infection, and it varies depending on how severe the clinical presentation is. In mild cases, systemic steroids are the best option due to their possible analgesic effect and fast recovery, but in moderate to severe cases, wound care and topical therapy with steroid hormones or immunosuppressants like tacrolimus are advised10,11. Surgical debridement should be avoided due to the pathergy phenomenon12. In patients with PG who do not respond to glucocorticoids, a wide range of additional systemic immunomodulatory medications, such as antitumor necrosis factor alpha medicines, may be used as alternatives or supplementary therapies13. Our patient responded well with intralesional steroid injection and topical antibiotics along with daily wound care and dressing.

Conclusion

PG is a rare form of neutrophilic dermatosis that can be difficult to diagnose and treat. We presented an unusual case of idiopathic recurrent PG with cobalamin deficiency presenting with a history of ulcers. It is crucial to have a high degree of suspicion when making a diagnosis, as well as to look for associated diseases and start treatment as soon as possible. The diagnosis of this patient requires the use of a multidisciplinary approach with a variety of subspecialties, such as surgeons, rheumatologists, gastroenterologists, dermatologists, etc.

Ethical approval

This is a case report, therefore, it did not require ethical approval from the ethics committee.

Consent

Written informed consent was obtained from the patient for the publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Sources of funding

The study did not receive any grant from funding agencies in the public, commercial or not-for-profit sectors.

Author’s contribution

S.S. involved in counseling and treatment of the patient. S.K., A.S., A.M., and V.J.: collected all the required case information, images, reports; reviewed the literature and contributed in both writing and editing the manuscript. S.S. and S.K.: involved in editing the manuscript. All authors read and approved the final manuscript.

Conflicts of interest disclosure

The authors report no conflicts of interest.

Research registration unique identifying number (UIN)

None.

Guarantor

Seema Sitaula a accept full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Author agreement statement

We, the undersigned, declare that this manuscript is original, has not been published before, and is not currently being considered for publication elsewhere.

We confirm that the manuscript has been read and approved by all named authors and that there are no other persons who satisfied the criteria for authorship but are not listed. We further confirm that the order of authors listed in the manuscript has been approved by all of us.

We understand that the corresponding author is the sole contact for the editorial process. He or she is responsible for communicating with the other authors about progress, submissions of revisions, and final approval of proofs.

Acknowledgments

None.

Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article.

Published online 17 February 2023
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