TY - JOUR
T1 - Novel Cutaneous Manifestations of Pleuroparenchymal Fibroelastosis
AU - Lowther, Christopher M.
AU - Morrison, Annie O.
AU - Candelario, Nicole M.
AU - Khalafbeigi, Sheva
AU - Cockerell, Clay J.
PY - 2016/10/1
Y1 - 2016/10/1
N2 - Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive disease that manifests as parenchymal fibrosis of the upper lobe and pleura. Approximately 100 cases have been reported. Cutaneous manifestations of PPFE have not previously been described. Diagnosis is dependent on histologic identification of fibrosis with atypical elastic fibers, necessitating an invasive peripheral lung wedge biopsy.A 68-year-old male with a history of pleuroparenchymal fibroelastosis presented with an asymptomatic, telangiectatic erythematous eruption on bilateral lower extremities. Biopsies demonstrated a subtle perivascular infiltrate with marked increase in atypical elastic fibers, similar to the elastosis in the patient's lungs.This is the first documented case of cutaneous manifestations in PPFE. Clinicians need to be aware that cutaneous eruptions clinically simulating telangiectasia macularis eruptiva perstans but lacking a mast cell infiltrate histologically, may have increased abnormal elastic fibers. Thus, early recognition of these lesions in patients with an undefined restrictive lung disorder, may facilitate the diagnosis of PPFE in some patients.
AB - Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive disease that manifests as parenchymal fibrosis of the upper lobe and pleura. Approximately 100 cases have been reported. Cutaneous manifestations of PPFE have not previously been described. Diagnosis is dependent on histologic identification of fibrosis with atypical elastic fibers, necessitating an invasive peripheral lung wedge biopsy.A 68-year-old male with a history of pleuroparenchymal fibroelastosis presented with an asymptomatic, telangiectatic erythematous eruption on bilateral lower extremities. Biopsies demonstrated a subtle perivascular infiltrate with marked increase in atypical elastic fibers, similar to the elastosis in the patient's lungs.This is the first documented case of cutaneous manifestations in PPFE. Clinicians need to be aware that cutaneous eruptions clinically simulating telangiectasia macularis eruptiva perstans but lacking a mast cell infiltrate histologically, may have increased abnormal elastic fibers. Thus, early recognition of these lesions in patients with an undefined restrictive lung disorder, may facilitate the diagnosis of PPFE in some patients.
KW - atypical elastosis
KW - cutaneous disease manifestations
KW - cutaneous fibroelastosis
KW - fibroelastosis
KW - pleuroparenchymal fibroelastosis
KW - PPFE
KW - pulmonary fibrosis
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U2 - 10.1097/DAD.0000000000000664
DO - 10.1097/DAD.0000000000000664
M3 - Article
C2 - 27643829
AN - SCOPUS:84988365935
SN - 0193-1091
VL - 38
SP - e140-e143
JO - American Journal of Dermatopathology
JF - American Journal of Dermatopathology
IS - 10
ER -