Autoerythrocyte Sensitization Syndrome Presenting with General Neurodermatitis: Factitious Purpura or Psychophysiological Entity?

Abstract

Autoerythrocyte sensitization syndrome (AES) is a disorder characterized by recurrent, spontaneous, painful bruising in patients with underlying psychosis and neurosis. Its pathogenesis is uncertain. Purpuric lesions reproduced by injections of washed autologous erythrocytes suggest an autoimmune etiology. The authors described a 50-year-old woman who presented with recurrent episodes of painful bruising for 8 months, along with a 2-year history of general chronic neurodermatitis. A diagnosis of AES was made on the basis of the clinical symptoms and laboratory examinations results: the positive autoerythrocyte sensitization test and psychophysiologic derangement. However, the noticeable symptoms, simultaneously identical distribution of the ecchymosis and neurodermatitis lesions, as well as concomitant symptom of itching over the ecchymosis, created doubts regarding the real etiopathogenesis of the ecchymosis and whether the ecchymosis in the present patient were just factitious purpura. The authors discuss the rarity of this case, which to the authors’ knowledge, had not been previously reported in the literature.

Investigating patients for bleeding disorders when most of the “usual” ones have been ruled out

“…These conditions include hematological disorders such as amyloidosis or hereditary hemorrhagic telangiectasia, congenital disorders such as connective tissue disorders, as well as endocrinological disorders such as hypothyroidism, among others [ 8 , 9 ]. A comprehensive overview of clinical conditions that may resemble a bleeding disorder is provided in Table 1 [ [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] ].…”

“…[ 9 ]. Disorder Clinical manifestation Achenbach’s syndrome [ 10 ] Paroxysmal bruising of the fingers Amyloidosis [ 11 ] Cerebral, cutaneous, intracranial and gastrointestinal bleeding Angina bullosa hemorrhagica Acute painful blisters in the mount Autoerythrocyte syndrome (also known as psychogenic purpura and Gardner–Diamond syndrome) [ 12 , 13 ] Recurrent, spontaneous, painful ecchymosis, frequently preceded by a prodrome of pain or itching of the skin Exercise induced purpura [ 14 ] Purpuric patches on the lower limbs after exercise Hereditary hemorrhagic telangiectasia [ 15 ] Recurrent epistaxis, gastrointestinal bleedings, hemorrhage from visceral arteriovenous malformation (eg, cerebral and pulmonary), telangiectasia on skin and mucosal surfaces Hypothyroidism [ 16 ] Often mild mucocutaneous bleeding tendency and rarely severe (eg, hemorrhages after trauma or surgery) Medications Bruising and bleeding from skin and the gastrointestinal tract Noonan syndrome [ 17 ] Postsurgical bleeding without any coagulation or platelet function abnormality Osteogenesis imperfecta [ 18 ] Variety of bleeding symptoms including bruising, bleeding after interventions and epistaxis Scurvy [ 19 ] Malaise, lethargy, purpura, intracerebral hemorrhage, subperiosteal hemorrhage, perifollicular hemorrhage, corkscrew hairs, poor wound healing and scorbutic gums Senile purpura [ 20 ] Scattered purpuric patches and white pseudoscars with skin atrophy Skin fragility and connective tissue disorders (eg, Ehlers–Danlos syndrome) [ 21 ] Excessive bruising but systemic manifestations of the specific condition Uremia [ 22 ] Bruising and hemorrhage Vasculitis (eg, Henoch–Schönlein purpura) [ 23 ] Purpuric rash and pulmonary hemorrhage …”

Role of autoerythrocyte sensitization test in the diagnosis of recurrent spontaneous bruising

“…Its pathogenesis has been proposed as a synergism between the psycho-immunology and mechanical injury of the skin in which preexisting psychosomatic disturbances may affect the skin’s immunological function and weaken the dermal capillaries so that even a mild injury to the skin can damage the capillary walls and eventually result in the permeation of RBCs. [ 4 ] It has been observed in association with emotional stress, hysteria, masochism, egoistic characters, emotional liabilities, self-mutilating behavior, aggressive behavior, depression, dissociative disorders, convulsions, hallucinations, and syncope. [ 5 ]…”

Clinical spectrum of autoerythrocyte sensitization syndrome: A series of five cases

“…[ 10 ] Such psychosomatic instabilities include emotional stress, hysteria, masochism, egoistic characters, emotional liabilities, self-injuries, difficulty in suppression of aggression, depression, aphonia, paralysis, paresthesia, dissociative disorders with convulsions, hallucinations, and syncope. [ 4 11 ] All three of our patients with psychiatric conditions were managed exclusively with cognitive behavioral therapy. Numerous medications have been proposed according to the type and severity of psychiatric symptoms, however, better responses have been observed with psychotherapy.…”