Non-cirrhotic Portal Hypertension as the Initial Presentation of Limited Cutaneous Scleroderma: A Case Report

Abstract

Systemic sclerosis (SSc) is an autoimmune disease characterized by progressive skin fibrosis. It has 2 main clinical subtypes-diffuse cutaneous scleroderma and limited cutaneous scleroderma. Non-cirrhotic portal hypertension (NCPH) is defined as presence of elevated portal vein pressures without cirrhosis. It is often a manifestation of an underlying systemic disease. On histopathology, NCPH may be found to be secondary to multiple abnormalities such as nodular regenerative hyperplasia (NRH) and obliterative portal venopathy. There have been reports of NCPH in patients with both subtypes of SSc secondary to NRH. However, simultaneous presence of obliterative portal venopathy has not been reported. We present a case of NCPH due to NRH and obliterative portal venopathy as a presenting sign of limited cutaneous scleroderma. The patient was initially found to have pancytopenia and splenomegaly and was erroneously labeled as cirrhosis. She underwent workup to rule out leukemia, which was negative. She was referred to our clinic and diagnosed with NCPH. Due to pancytopenia, she could not be started on immunosuppressive therapy for her SSc. Our case describes the presence of these unique pathological findings in the liver and highlights the importance of an aggressive search for an underlying condition in all patients diagnosed with NCPH.

Keywords: Raynaud phenomenon; elastography; idiopathic non-cirrhotic portal hypertension; systemic sclerosis.

Figure 1.

Physical examination findings: (A) telangiectasias on mucosal aspect of lower lip; (B) sclerotic hyperpigmented plaque on the abdomen; (C) edematous swelling of hands; and (D) multiple sclerotic plaques on the bilateral lower extremities.

Figure 2.

Changes of obliterative portal venopathy and nodular regenerative hyperplasia. (A) Portal area with small portal veins displaced out of the portal area (H&E, 400×). (B) Sinusoidal dilation (H&E, 200×). (C) Delicate perisinusoidal fibrosis (Masson trichrome, 400×). (D) Vague nodularity with zone of narrowed liver cell plates (arrows; Reticulin, 200×). Abbreviation: H&E, hematoxylin and eosin.