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Updated: Jun 12, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Porphyria cutanea tarda presenting as scleroderma.

Raquel Zemtsov¹, Alexander Zemtsov

¹University Dermatology Center, PC, Muncie, IN 47303, USA. azemtsov@iupui.edu

|May 22, 2010

Summary

Porphyria cutanea tarda (PCT) can mimic progressive systemic sclerosis (PSS), leading to misdiagnosis. Early phlebotomy treatment resolved sclerodactyly and fingertip ulcers in a PCT patient initially diagnosed with PSS.

Area of Science:

Dermatology
Internal Medicine
Genetics

Background:

Porphyria cutanea tarda (PCT) is a metabolic disorder.
It is characterized by photosensitivity and skin fragility.
PCT can present with atypical skin findings, mimicking other conditions.

Observation:

A patient presented with sclerodermatous skin changes, initially diagnosed as progressive systemic sclerosis (PSS).
The patient exhibited sclerodactyly, fingertip ulcerations, facial features, and skin tightness typical of PSS.
Typical PCT findings like blisters and scarring were absent.

Findings:

The patient's sclerodermatous changes, including sclerodactyly and fingertip ulcerations, resolved after therapeutic phlebotomy.
Phlebotomy led to significant improvement in facial and forearm sclerodermatous skin changes.

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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Published on: June 16, 2020

This suggests a potential misdiagnosis of PCT as PSS.

Implications:

PCT should be considered in the differential diagnosis of sclerodermatous skin conditions, even without classic PCT signs.
Prompt diagnosis and treatment of PCT, such as phlebotomy, can reverse severe cutaneous manifestations.
This case highlights the importance of recognizing overlapping clinical features between PCT and PSS.