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Cutaneous and visceral granulomas in common variable immunodeficiency
J C Pierson1, C Camisa, K B Lawlor
1Department of Dermatology, Cleveland Clinic Foundation, Ohio.
Cutis
|October 1, 1993
Summary
Common variable immunodeficiency can manifest with skin issues resembling dermatomyositis. This case highlights noninfectious granulomas in both skin and internal organs in such patients.
Area of Science:
- Immunology
- Dermatology
- Pathology
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low immunoglobulin levels and impaired B-cell function.
- Patients with CVID are susceptible to recurrent infections and autoimmune phenomena.
- Cutaneous manifestations in CVID are diverse, but granulomatous inflammation is less commonly reported.
Observation:
- A middle-aged woman with known CVID presented with a papular skin eruption on her dorsal hands.
- The eruption clinically mimicked Gottron's sign, a characteristic feature of dermatomyositis.
- Skin biopsy revealed noninfectious granulomatous inflammation.
Findings:
- Further investigation, including laparotomy, identified visceral granulomas in addition to the cutaneous findings.
- The histopathological findings confirmed noninfectious granulomas as the underlying pathology.
- This presentation underscores a potential, albeit uncommon, extragastrointestinal manifestation of CVID.
Implications:
- Noninfectious granulomas can occur in both the skin and viscera of patients with CVID.
- This finding expands the spectrum of potential clinical presentations in common variable immunodeficiency.
- Recognition of these granulomatous manifestations is crucial for accurate diagnosis and management of CVID patients.