Ecchymosis syndrome, painful R23.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.12.2020

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Synonym(s)

Autoerythrocyte Sensitization; autoerythrocytic purpura; bruise syndrome; Diamond-Gardner syndrome; Erythrocyte autosensitization; Gardner Diamond Syndrome; Gardner-Diamond Syndrome; Painful Bruising Syndrome; Painful Bruising Syndrome Sharp; Painful ecchymosis syndrome; Psychogenic Purpura; Purpura autoerythrocytes; Purpura autoerythrocytic Purpura; Purpura psychogenic

History
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Diamond and Gardner 1955

Definition
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Symptoms occurring almost exclusively in women, characterized by intermittent manifestation of painful skin bleeding and other multiple accompanying symptoms. The skin changes can be provoked by injection of autologous erythrocytes. Multiple interpretation of the clinical picture as conversion neurosis.

Etiopathogenesis
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Total unknown. Discussed are:

- ychogenic influences,

- .autosensitization to erythrocyte phosphoglycerides...

- bstances released by neurohormones that increase vascular permeability.

Manifestation
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The disease manifests itself in the 1st - 5th decade of life, with women affected in 90% of cases . Rarely seen in infants.

Localization
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The skin changes frequently occur in the area of the upper and lower extremities, but less frequently in the head and neck area.

Clinical features
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Initially, prodromes in the form of itching, stinging, burning pain. A few hours later, development of edematous, overheated, pressure-sensitive erythema. After 1 day transition to ecchymosis, slow extension of the lesions. Healing without scars after 1-2 weeks. Provocation by trauma.

Besides, multiple accompanying symptoms: fever, loss of weight, lassitude, muscular and headache, arthralgias, gastrointestinal symptoms (cramps, bleeding, vomiting, diarrhoea), epistaxis, haemoptysis, haematuria.

Almost in all cases neurotic personality structure: depressiveness, emotional immaturity, masochistic traits.

Histology
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Extravasation of erythrocytes in the dermis with perivascular lymphocytes and hemorrhage into the dermis and subcutis.

Diagnosis
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Triggering of the corresponding changes by injection of autologous erythrocytes (in 60% positive).

Differential diagnosis
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Artifacts.

Therapy
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Symptomatic therapy e.g. with ointments containing heparin(e.g. Vetren ointment), analgesic medication such as paracetamol (e.g. Ben-u-ron Tbl.) or, if necessary, tramadol (e.g. Tramal Kps.).

Therapeutically, the connection to a psychiatrist, a psychotherapy as well as a drug therapy with e.g. antipsychotics, tricyclic antidepressants and benzodiazepines is recommended.

Topically, glucocorticoid-containing topical preparations may be used.

Literature
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  1. Behrendt C et al (2001) Painful bruising syndrome. dermatologist 52: 634-637
  2. Edinger LK et al (2013) Gardner-Diamond syndrome associated with complex regional pain syndrome. J Dermatol Case Rep 7:10-14
  3. Frantzen E et al (1990) Gardner-Diamond Syndrome. dermatologist 41: 168-170
  4. Gardner FH, Diamond LK (1955) Autoerythrocyte sensitization. A form of purpura producing painful bruising following autosensitization to red blood cells in certain women. Blood 10: 675-690
  5. Hagemeier L et al (2011) Gardner-Diamond syndrome: a rare differential diagnosis of child abuse. Br J Dermatol 164: 672-673
  6. Jafferany M et al (2015) Psychogenic Purpura (Gardner-Diamond Syndrome). Prim Care Companion CNS Disord doi: 10.4088/PCC.14br01697
  7. Karatosun V et al (2003) Autoerythrocyte sensitization (Gardner-Diamond) syndrome mimicking compartment syndrome. Arch Orthopedic Trauma Surgery 123: 370-371
  8. Pevny I et al (1982) The erythrocytic sensitization syndrome. Dermatologist 33: 251-256
  9. Sridharan M et al (2019) The Mayo Clinic Experience With Psychogenic Purpura (Gardner-Diamond Syndrome). On J Med Sci 357:411-420.
  10. Tainwala RR et al (2013) Perplexing purpura in two females: Rare case of autoerythrocyte sensitization syndrome. Indian Dermatol Online J 4:305-308

Outgoing links (3)

Ecchymoses; Heparin; Paracetamol;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 18.12.2020