[Recurrent pneumothorax in interstitial lung disease, anogenital and abdominal wall exanthema and secondary amenorrhea in a 20-year-old patient. Langerhans-cell histiocytosis].
A 20-year-old female patient was admitted to the hospital because of a pneumothorax. A secondary amenorrhea was coexisting. On the abdominal wall and in the anogenital area a maculopapular exanthema was visible. Chest X-ray and HR-CT showed an interstitial lung disease with high probability of histiocytosis X which was confirmed by biopsies of lung and skin tissue. The cause of amenorrhea was a histiocytic infiltration of the pituitary stalk. Conventional "consensus-treatment" for adults with vinblastine, prednisone and etoposide did not prevent relapsing bilateral pneumothoraces. Treatment was successful with the protocol as proposed by the international study for children. This treatment is characterized by higher dosage and combination with 6-mercaptopurine.
Lockhart
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I
Medizinische Klinik, Kliniken der Stadt Köln, Krankenhaus Köln Holweide.
Meuthen
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I
Wolmerstett
M P
MP
D000328:Adult; D000568:Amenorrhea / Q000209:etiology*; D001706:Biopsy; D003937:Diagnosis, Differential; D005076:Exanthema / Q000209:etiology*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000473:pathology; D011030:Pneumothorax / Q000209:etiology*; D011379:Prognosis; D012008:Recurrence; D012867:Skin / Q000473:pathology