Squamous cell carcinoma and eosinophilia in a long-term course of pansclerotic morphea

Inderjote Grewal1, Omar Khan2, William Davis2

  • 1University of Queensland, Brisbane, Queensland, Australia.

BMJ Case Reports
|October 15, 2014
PubMed

Insights

Pansclerotic morphea, a rare scleroderma subtype, presents significant challenges. This case highlights its chronic course, complications like skin cancer, and resistance to treatment.

Area of Science:

  • Dermatology
  • Rheumatology
  • Rare Diseases

Background:

  • Pansclerotic morphea is an exceptionally rare subtype of localized scleroderma, with limited documented cases.
  • Understanding its long-term clinical course and potential complications is crucial for patient management.

Observation:

  • A young male patient with a 15-year history of pansclerotic morphea is presented.
  • The patient experienced recurrent squamous cell carcinomas with high-risk features and frequent cellulitis, necessitating numerous hospital admissions.
  • Autoimmune markers were negative, but marked eosinophilia was observed later in the disease progression.

Findings:

  • Pansclerotic morphea demonstrates a challenging clinical course with severe complications, including malignancy.
  • The disease is characterized by resistance to conventional treatments.
  • Immunosuppressive therapy showed potential in slowing progression but did not achieve a cure.

Implications:

  • This case underscores the need for vigilant monitoring for complications such as squamous cell carcinoma in pansclerotic morphea patients.
  • Further research into effective treatment strategies for this rare and aggressive condition is warranted.
  • The findings suggest a complex pathophysiology, potentially involving factors beyond typical autoimmune markers.

Related Concept Videos

Chronic Obstructive Pulmonary Disease II: Emphysema01:23

Chronic Obstructive Pulmonary Disease II: Emphysema

Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.
16
Skin Cancer01:30

Skin Cancer

Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
5.2K
Skin Diseases and Disorders01:23

Skin Diseases and Disorders

Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
5.1K
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
23
Cellular Adaptation IV: Dysplasia and Metaplasia01:24

Cellular Adaptation IV: Dysplasia and Metaplasia

DysplasiaDysplasia refers to abnormal changes in the size, shape, and organization of mature cells, characterized by pleomorphism, nuclear abnormalities, and increased mitotic activity. It commonly affects epithelial tissues, including the cervix, gastrointestinal tract, respiratory mucosa, and endometrium. Although it may occur alongside hyperplasia, dysplasia is not a true adaptive response but a preneoplastic change with potential to progress to cancer.When confined above the basement...
11
Esophageal Achalasia01:27

Esophageal Achalasia

Esophageal achalasia is a chronic neurogenic disorder characterized by impaired relaxation of the lower esophageal sphincter (LES) and absent or ineffective peristalsis in the distal esophagus. This leads to a functional obstruction without a physical blockage, despite significant disruption of esophageal motility.EtiologyAchalasia is caused by degeneration of the myenteric (Auerbach's) plexus, specifically the loss of inhibitory ganglion cells that produce vasoactive intestinal peptide...
23