Pyoderma gangrenosum in a cesarean section wound in a woman with myelodysplastic syndrome: A case report.

INTRODUCTION: Pyoderma gangrenosum and myelodysplastic syndrome in pregnant women are both very rare, but can coexist. Here, we present a case of pyoderma gangrenosum in a cesarean section wound in a woman with myelodysplastic syndrome. CASE: A 34-year-old woman presented with thrombocytopenia and macrocytic anemia during pregnancy. The pregnancy was uneventful until 36 weeks of gestation, when premature rupture of membranes occurred and a cesarean section was performed for breech presentation. She presented four days later with redness and blisters at the wound site. Surgical site infection was diagnosed but did not improve with antibiotics, and multiple wound cultures were negative. Skin biopsy indicated pyoderma gangrenosum, and the redness and blisters responded to oral prednisolone. Post-partum bone marrow aspiration showed myelodysplastic syndrome. As the patient demonstrated no symptoms such as abnormal bleeding, no additional treatment was started, and she went on to receive regular follow-up for myelodysplastic disorder. DISCUSSION: This case shows the need for further assessment of hematological disorders diagnosed in pregnancy in women with pyoderma gangrenosum post-partum.

Introduction

Pyoderma gangrenosum (PG) is a noninfectious inflammatory dermatosis with neutrophilic infiltration. PG is likely to develop in patients aged between 20 and 50 years and is more common in women than in men [1]; however, it is very rare in pregnancy [2].

wSince PG in cesarean wounds mimics surgical site infection (SSI) it is often misdiagnosed [3]. Pyoderma gangrenosum, especially bullous PG, is often associated with hematologic disorders such myelodysplastic syndrome (MDS) [[4], [5], [6]].

Here, we present a case of PG after cesarean section in a woman with MDS who had been initially diagnosed as having SSI, thrombocytopenia and macrocytic anemia.

Case Presentation

A 34-year-old, nulliparous, healthy woman visited a local doctor after a naturally conceived pregnancy. Her platelet count was low, being 90 × 103/μL and 54 × 103/μL at 12 and 26 weeks of gestation, respectively. She had no medical history of abnormal bleeding or purpura or a family history of thrombocytopenia.

She was referred to an obstetric unit because of thrombocytopenia (42 × 103/μL) at 30 weeks of gestation. Blood tests revealed a white blood cell (WBC) count of 6.3 × 103/μL, hemoglobin level of 11.0 g/dL, mean corpuscular volume of 112 fL, and platelet count of 39 × 103/μL. The patient was referred for hematological assessment. Blood tests showed the following levels: serum B12 149 pg/mL, folate 5.4 ng/mL, and ferritin 36 ng/mL. The macrocytic anemia was treated with iron and vitamin B12. No treatment for thrombocytopenia was indicated because platelet levels were maintained above 20 × 103/μL in the antenatal period and the pregnancy developed uneventfully. Oral prednisolone at a dose of 10 mg/day was started at 35 weeks of gestation to increase the platelet count in time for delivery. Despite this treatment, no significant improvement in thrombocytopenia was seen.

Premature rupture of membranes occurred at 36 weeks of gestation. Breech presentation led to an emergency cesarean section under general anesthesia because of thrombocytopenia (40 × 103/μL). Platelet transfusion was performed before surgery, and the platelet count increased to 79 × 103/μL. A female neonate weighing 2118 g was delivered, with Apgar scores of 9 and 10 at 1 min and 5 min, respectively. Estimated blood loss at surgery was 150 mL. No abnormal bleeding in the mother was confirmed and prednisolone was discontinued. The neonate did not have thrombocytopenia.

Four days after surgery, the woman presented with redness, severe pain, and swelling, with pyrexia up to 38.5 °C. A blood test revealed elevated WBC and C-reactive protein (CRP) levels, with a WBC count of 15 × 103/μL, and a CRP level of 20 mg/dL. The initial diagnosis was SSI. Six days of antibiotic administration was ineffective, and her temperature was over 38 °C and CRP was over 30 mg/dL. Moreover, multiple wound cultures were negative. The wound demonstrated extended redness and blisters seven days after surgery (Fig. 1).

Fig. 1

Redness and blisters are seen on the cesarean wound seven days after surgery.

Eight days after surgery, computed tomography showed mild inflammation under the wound, without infection sites in the abdomen and pelvic cavity. Suspecting a noninfectious inflammatory condition, the patient was referred for dermatological assessment. Skin biopsy showed neutrophilic infiltration and bullous lesions (Fig. 2), leading to the diagnosis of bullous PG. Oral prednisolone at a dose of 20 mg/day was started.

Fig. 2

A skin biopsy shows neutrophilic infiltration and bullous lesions. (HE stain, ×20).

In view of the antenatal hematological abnormalities, bone marrow aspiration was performed 10 days post-partum, and MDS was strongly suspected because of blood megakaryocyte malformation (Fig. 3) and chromosome aberration (8 trisomy).

Fig. 3

A bone marrow aspiration shows blood megakaryocyte malformation. (MG stain).

The mother and neonate were discharged 18 days after surgery. The dose of prednisolone had tapered down to 10 mg/day at discharge. The dose was further gradually reduced over a week until discontinuation. The blisters disappeared, and redness was attenuated four weeks after treatment (Fig. 4). The platelet count of the mother remained low: 95 × 103/μL at three weeks after discharge. As the patient demonstrated no symptoms such as abnormal bleeding, no additional treatment was started, and she went on to receive regular follow-up for myelodysplastic disorder.

Fig. 4

The blisters disappeared and the redness is attenuated four weeks after treatment.

Discussion

PG is a non-infectious dermatosis presenting with expanding inflammatory papules, nodules, and ulcers with severe pain. There are several types of PG; the ulcerative (classical) type makes up approximately 85% of all PG, and rest fall under other types (bullous, pustular, and vegetative). Bullous and pustular types are associated with hematological disorders and inflammatory bowel disease, respectively [[4], [5], [6]]. The prevalence of PG is approximately 0.63 out of 100,000 in the general population [4], but its prevalence in pregnancy is unknown [2]. Despite its rarity, PG during pregnancy is sometimes found after surgery such as cesarean section [3,7]. PG in cesarean wounds mimics SSI, often leading to a misdiagnosis [3]. Therefore, a definitive diagnosis of PG via skin biopsy is essential because its treatment differs from that of SSI; PG is treated by either steroid or immunosuppressive therapy [3,8].

MDS is a heterogeneous group of hematologic neoplasms classically described as a clonal disorder of hematopoietic stem cells leading to dysplasia and ineffective hematopoiesis in the bone marrow. It affects 10 to 12 out of 100,000 in the general population [9]. It is usually diagnosed in patients over the age of 65 and is thus rare in pregnant women [10]. Pancytopenia due to MDS during pregnancy may be associated with maternal and fetal complications, such as preeclampsia, placental abruption, postpartum hemorrhage, miscarriage, premature delivery, and low birth weight [9]. Of these complications, the present case resulted in preterm delivery and low birth weight. This is partly because severe anemia leads to placental ischemia and fetal hypoxia; severe thrombocytopenia and leukopenia may cause abnormal bleeding and severe infection, respectively [9].

Although both PG and MDS are very rare in pregnancy, the presence of the former in women with hematological abnormalities should trigger further investigation. Approximately 50% of PG cases are associated with an underlying systemic disease, such as inflammatory bowel disease, arthritis, or hematologic disease; 5–8% of people with PG have hematologic disorders [[4], [5], [6]]. Specifically, approximately 70% of bullous PG cases are related to hematologic disorders, including MDS, although the etiological mechanism is unknown [4].

The diagnosis of MDS during pregnancy is often difficult because of the common hematological alterations at that time, such as anemia, gestational thrombocytopenia [11] and idiopathic thrombocytopenic purpura. Thus, bone marrow examination is essential. Treatments for the two conditions differ. MDS treatment includes blood transfusion, chemotherapy and allogeneic stem cell transplant; idiopathic thrombocytopenic purpura is treated with either steroid or immunosuppressive therapy [12].

To conclude, this case shows the need for bone marrow examination in women diagnosed with hematological disorders antenatally who develop pyoderma gangrenosum post-partum.

Contributors

All authors were involved in the clinical care of the patient and contributed to the conception, drafting, review, and revision of the manuscript. All authors read and approved the final version of the paper and take full responsibility for the work.

Conflict of Interest

The authors declare that they have no conflict of interest regarding the publication of this case report.

Funding

No specific grant from funding agencies in the public, commercial, or not-for-profit sectors supported the publication of this case report.

Patient Consent

Written informed consent was obtained from the patient for the publication of this work.

Provenance and Peer Review

This case report was peer reviewed.