Papulosis atrophicans maligna (Köhlmeier-Degos disease): a disseminated occlusive vasculopathy.

[malignant atrophic papulosis]

Malignant atrophic papulosis usually presents as pathognomonic skin lesions followed by acute abdominal pain , bowel perforation , peritonitis , and death . Rare patients who may lack gastrointestinal symptoms present with central nervous system manifestations , including headache , paresthesias , weakness , and rapid deterioration to death . The patient reported here was a 47 -year -old man whose neurological symptoms apparently preceded his cutaneous lesions . His course consisted of a disseminated neurological disease and exacerbated following a herpes zoster infection . His condition rapidly deteriorated despite corticotropin , glucocorticoids , and low -molecular-weight dextran . Necropsy revealed a disseminated occlusive vasculopathy and diffuse encephalomyelomalacia of the brain and spinal cord . A review of autopsied patients with central nervous system involvement is provided .

Diseases
Validation

Diseases presenting "weakness" symptom

achondroplasia

adrenomyeloneuropathy

alexander disease

aniridia

aromatase deficiency

coats disease

congenital toxoplasmosis

epidermolysis bullosa simplex

esophageal adenocarcinoma

familial hypocalciuric hypercalcemia

familial mediterranean fever

focal myositis

gm1 gangliosidosis

hydrocephalus with stenosis of the aqueduct of sylvius

inclusion body myositis

locked-in syndrome

lymphangioleiomyomatosis

malignant atrophic papulosis

neuralgic amyotrophy

pendred syndrome

pleomorphic liposarcoma

pyomyositis

pyruvate dehydrogenase deficiency

sneddon syndrome

systemic capillary leak syndrome

thoracic outlet syndrome

triple a syndrome

typhoid

von hippel-lindau disease

waldenström macroglobulinemia

werner syndrome

x-linked adrenoleukodystrophy

This symptom has already been validated