Pregnancy-Related Morphea: A Case Report

Abstract

Background:

Morphea is a skin condition marked by erythematous and hardened inflammatory lesions that can progress to atrophic and sclerotic plaques. In this case report, we present a case of a pregnant woman who showed morphea presentation.

Case presentation:

A 37-year-old G₂P₁L₁ woman with a gestational age of 32 weeks and 2 days was referred to the hospital with complaints of swelling, pain, and erythema in both legs for the past week, without any obstetric complaints. Upon initial examination at the time of admission, swelling, erythema, warmth in both legs, ecchymotic lesions, and non-pitting edema were observed. Biometry and color Doppler ultrasound for evaluation of fetus growth was normal. Skin examination revealed mild erythema, warmth, and purpura and petechiae in some areas, with an orange peel appearance and firm texture on palpation. Leg lesion skin punch biopsy reported a sclerodermoid reaction pattern and findings more compatible with morphea. The patient was managed for morphea during pregnancy. However, at 38 weeks and 6 days of gestation, due to worsening symptoms and spreading stiffness to the groin area, and the need for initiating pulse corticosteroids and mycophenolate mofetil (CellCept), the patient was re-admitted for cesarean section. In the follow-up assessment, ultrasound findings were normal, IGRA, HIV antibody, HCV antibody, HBC antigen, and HBS antigen were negative, and no edema or inflammation was seen in the patient.

Conclusions:

In conclusion, although our patient recovered without specific treatment, predicting the recurrence or worsening of morphea in future pregnancies is challenging.

Introduction

Morphea, also recognized as localized scleroderma, is a rare autoimmune condition characterized by inflammation and sclerosis of the skin and underlying soft tissue.^1-3 The disease progresses through phases of activity, marked by inflammation and fibrosis, leading to damage and atrophy. Histologically, active morphea presents with an inflammatory dermal and subcutaneous lymphocytic infiltrate, clinically evident as erythema, edema, and lesion extension, often accompanied by symptoms such as pain and itching.^4-6

Dense collagen deposition occurs during the fibrotic phase, which initially overlaps with inflammation, resulting in hardened yellow to white plaques with an erythematous or violaceous border. These mixed inflammatory and sclerotic lesions eventually transition into an inactive phase, characterized by resolved inflammation and sclerosis progressing to dermal and sometimes underlying soft tissue atrophy. Morphea’s pathological changes can affect the dermis, subcutis, soft tissue, and occasionally bone, leading to significant deformities and functional impairments, such as contractures, limb length discrepancy, or restricted range of motion.^7-9 Hence, early diagnosis and intervention are crucial to minimize damage. ¹⁰

Morphea encompasses several subtypes, each presenting varied clinical manifestations and degrees of involvement of subcutaneous soft tissues. ¹¹ Although the exact pathogenesis of morphea remains incompletely understood, ongoing research suggests a multifactorial etiology involving dysregulated immune and fibrotic pathways, alongside genetic predisposition, traumatic or environmental factors, and vascular dysregulation.^12-14

Morphea impacts individuals of all ages, including children and adults. Pregnancy and other hormonal changes, such as those induced by hormonal treatments, appear to increase the risk of developing this skin condition.^15,16

Although the exact mechanism behind hormonally related morphea remains unclear, it is believed that hormonal and immunological changes may play a role. ¹⁷

In this case report, we present a case of a 37-year-old pregnant woman who showed morphea presentation.

Case Presentation

A 37-year-old G₂P₁L₁ (previous NVD 2 years before the current pregnancy) woman with a gestational age of 32 weeks and 2 days and one prior childbirth presented to the hospital’s emergency department with complaints of swelling, pain, and erythema in both legs for the past week, without any obstetric complaints. The patient’s medical history included an appendectomy with a McBurney incision, vitiligo since childhood affecting the hands, feet, and knees, and a history of hypothyroidism with irregular medication use. Before hospital admission, the patient took no specific medications but iron tablets, vitamin D, and multivitamins.

The findings of the first and second trimester screenings were checked, and no abnormalities were seen.

Physical examination revealed a blood pressure of 120/80 mmHg, heart rate of 80 beats/min, respiratory rate of 18 breaths/min, temperature of 37°C, and oxygen saturation of 98% in room air.

Upon initial examination at the time of admission, swelling, erythema, warmth in both legs, ecchymotic lesions, and non-pitting edema were observed (Figure 1). Therefore, biometry and color Doppler ultrasound for evaluation of fetus growth was performed which was normal, and diagnostic tests were requested for the patient to evaluate the possibility of deep vein thrombosis (DVT), thrombophlebitis of the lower extremities, Eosinophilic fasciitis (EF), or preeclampsia.

Figure 1.

The patient’s leg appearance in the first examination.

A color Doppler ultrasound and superficial probe of the lower extremities were performed for limb evaluation. Deep vein thrombosis was ruled out. Increased subcutaneous fat echogenicity was seen but no evidence of collection was observed.

With the possible diagnosis of thrombophlebitis, an infectious disease consultation was requested for the patient and she was prescribed enoxaparin 40 mg daily, and cefazolin 2 g every 8 hours until 72 hours after admission when the possibility of cellulite and DVT was ruled out.

For the possible diagnosis of preeclampsia, we asked for a blood pressure chart and preeclampsia pathological tests. 24-hour urine protein was requested for the patient and it was 276 mg/dl and normal.

Then, skin examination revealed mild erythema, warmth, and purpura and petechiae in some areas, with an orange peel appearance and firm texture on palpation. Differential diagnoses included morphea, lipo scleroderma, scleroderma, vasculitis, and collagen vascular diseases. Therefore, a biopsy and rheumatology consultation were requested.

The rheumatologist, after examining the patient, suggested that vasculitis was less likely due to the type of skin involvement and the absence of distal limb involvement. Additionally, the prolonged duration of over a week since symptom onset made systemic sclerosis less likely. Paraneoplastic issues should also be considered.

Further tests were requested. At this time, the lab results were as follows:

Hb: 9.7 (normal range < 11 in pregnant women), MCV: 91% (normal range: 80-96), plt: 161,000 (normal range: 150,000-450,000), WBC: 6,900 (normal range: 4500-11,000), ESR: 56 (normal range < 20 mm/hour), CRP: 3 (normal range < 10 md/dl), PCT < 0.5 (normal range: 0-0.5 microgram/l), ANA, and dsDNA Abs were normal, LDH: 452 (normal range: 135-214 U/L), UREA: 13 (normal range: 15-45 mg/dl), Cr: 0.5 (normal range: 0.6-1.1 mg/dl), liver function tests: normal, Ferritin: 6 (normal range: 10-150 ng/dl), Fe: 92 (normal range: 60-160 mcg/dl), TIBC: 343 (250-450 mcg/dl), Alb: 2.5 (3.5-5 g/dl).

Cardiology consultation and echocardiography showed left ventricular ejection fraction (LVEF): of 55%, mild left ventricle enlargement, elevated pulmonary artery pressure (PAP): 25, and no evidence of atrial stenosis, mitral stenosis, or tricuspid stenosis. The right ventricle size was reported as normal.

Leg lesion skin punch biopsy reported a sclerodermoid reaction pattern and findings more compatible with morphea (Figure 2).

Figure 2.

Pathological finding of skin biopsy.

The patient was managed for morphea during pregnancy. However, at 38 weeks and 6 days of gestation, due to worsening symptoms and spreading stiffness to the groin area, and the need for initiating pulse corticosteroids and mycophenolate mofetil (CellCept), the patient was re-admitted for delivery upon the dermatologist’s request. Given the extent of skin sclerosis reaching the perineum, a cesarean section was indicated.

Post-cesarean, the patient was re-evaluated for skin issues, including:

• (1) Soft tissue ultrasound of the lower extremities for cellulitis and morphea depth assessment.

• (2) IGRA, HIV antibody, HCV antibody, HBcAg, and HBsAg tests.

• (3) Follow-up at the dermatology clinic with ultrasound and lab results after discharge.

We did not administer any specific treatment for the patient, and the symptoms of the patient’s morphea spontaneously improved after childbirth. In the follow-up assessment, ultrasound findings were normal, IGRA, HIV antibody, HCV antibody, HBC antigen, and HBS antigen were negative, and no edema or inflammation was seen in the patient.

Discussion

Morphea is a skin condition marked by erythematous and hardened inflammatory lesions that can progress to atrophic and sclerotic plaques. The complex pathogenesis of morphea involves immune-driven fibrosis. ¹⁸ This condition mainly affects the dermis and can extend to the subcutaneous fat and fascia, causing skin thickening and hardening due to fibrosis. About half of the patients experience spontaneous remission or skin softening approximately 2.7 years after onset. ¹⁹ Morphea can affect both children and adults, with pregnancy and hormonal changes, such as those from hormonal treatments, being potential predisposing factors. ²⁰

We report a 37-year-old pregnant woman with a gestational age of 32 weeks and 2 days who was admitted to the hospital with clinical presentations of morphea. At the three-month follow-up after the cesarean section, the symptoms of morphea in our patient resolved on their own, and the patient experienced no pain, inflammation, or edema.

Several cases of morphea that either developed or worsened during pregnancy were previously reported.^15-17,21 In the reported cases, the proposed mechanism was microchimeric, a process where cells transferred from the fetus to the mother, promoting the immune system’s activation.

For instance, in a case by Saravia et al ¹⁷ morphea appeared after fertility treatment and worsened with subsequent treatments. Although fertility drugs have not been directly linked to morphea, it was hypothesized that elevated 17 b-estradiol levels from fertility treatments could promote cutaneous fibrosis, highlighting the potential role of sex hormones and the need to consider fertility drugs as a possible trigger for morphea.

Walker et al ²² studied 10 pregnant patients, finding that three (30%) experienced reactivations of morphea during pregnancy, with only one needing immunosuppressive therapy. Most patients had cesarean deliveries, and there was one adverse fetal outcome, but no adverse pregnancy outcomes were directly related to morphea. Similar to our case, most patients maintained stable morphea during pregnancy.

Pham et al ¹⁶ emphasized the importance of recognizing morphea during pregnancy and noted that it might improve or resolve postpartum. Their patient, like ours, recovered without specific treatment. However, the potential for recurrence or worsening in subsequent pregnancies remains unclear, necessitating further studies.

Conclusions

In conclusion, although our patient recovered without specific treatment and had no pain, edema, or inflammation, predicting the recurrence or worsening of morphea in future pregnancies is challenging, and additional research is needed for definitive conclusions.