Multicentric reticulohistiocytosis: a unique case with pulmonary fibrosis.
Arch Dermatol
; 148(2): 228-32, 2012 Feb.
Article
en En
| MEDLINE
| ID: mdl-22351825
ABSTRACT
BACKGROUND:
Multicentric reticulohistiocytosis (MRH) is a rare disease of uncertain etiology that most commonly presents as a papulonodular cutaneous eruption accompanied by erosive polyarthritis. Although MRH is considered a systemic disorder in that it targets skin and joints, involvement of thoracic and visceral organs is uncommon. OBSERVATIONS A woman presented with diffuse cutaneous nodules, and skin biopsy findings revealed classic features of MRH. However, she also manifested severe pulmonary symptoms. A lung biopsy specimen showed prominent histiocytic infiltrates exhibiting the same characteristic morphologic features as those seen in her skin. Furthermore, the lung biopsy findings were significant for a pattern of usual interstitial pneumonia accompanied by notable lymphoid aggregates, a pattern of interstitial lung disease typical of systemic autoimmune and inflammatory conditions.CONCLUSIONS:
These findings are notable because a histiocytic pulmonary infiltrate suggestive of direct pulmonary involvement by MRH is a rare event. In addition, presentation of MRH in the setting of usual interstitial pneumonia is unique. These observations document a new clinical and histopathologic presentation of MRH that is significant for expanding the idea of MRH as a systemic disease while supporting the notion that MRH is promoted by an inflammatory milieu.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Enfermedades de la Piel
/
Histiocitosis
/
Fibrosis Pulmonar Idiopática
Tipo de estudio:
Diagnostic_studies
Límite:
Female
/
Humans
/
Middle aged
Idioma:
En
Revista:
Arch Dermatol
Año:
2012
Tipo del documento:
Article
País de afiliación:
Estados Unidos