Novel Cutaneous Manifestations of Pleuroparenchymal Fibroelastosis.

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.

Id-like reaction to BCG therapy for bladder cancer.

Id reaction, also known as autoeczematization, is the development of dermatitis that is distant to an initial site of infection or sensitization. Clinical findings typically include an acute, intensely pruritic maculopapular or papulovesicular eruption that most frequently involves the extremities. Histology typically reveals spongiotic dermatitis that often is vesicular, and eosinophils may be present in the infiltrate. Id reactions can result from inflammatory skin conditions such as stasis dermatitis as well as infectious entities including mycobacterial infections. BCG live therapy consists of an attenuated strain of Mycobacterium bovis that is utilized as a first-line treatment of superficial transitional cell carcinomas. We report the case of an id-like reaction in a 90-year-old man who developed an intensely pruritic, scaly, erythematous eruption on all 4 extremities 2 weeks after starting weekly intravesical use of BCG therapy for superficial transitional cell carcinoma. A representative biopsy demonstrated spongiotic dermatitis with overlying scaling and an eosinophilic infiltrate. The eruption resolved after discontinuation of BCG therapy and treatment with topical corticosteroids.