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Dermatology & Pigmentation Disorders
Localized livedo reticularis and retiform purpura. Diagnostic considerations based on a clinical case
  • Bowers J ,

    Departments of Internal Medicine, Wake Forest School of Medicine, Winston-Salem, North Carolina, Tel: 336-716-4305; Email: jebowers@wakehealth.edu

  • Stanback C ,

    Departments of Internal Medicine, Wake Forest School of Medicine, Winston-Salem, North Carolina

  • Atkinson H ,

    Departments of Internal Medicine, Wake Forest School of Medicine, Winston-Salem, North Carolina

  • Taheri A ,

    Center for Dermatology Research, Departments of Dermatology, Wake Forest School of Medicine, Winston-Salem, North Carolina

Received: 02-11-2015

Accepted: 26-11-2015

Published: 30-11-2015

Citation: Bowers J, Stanback C, Atkinson H and Taheri A (2015) Localized livedo reticularis and retiform purpura. Diagnostic considerations based on a clinical case. J Der & Pig Diso. 1:100103

Copyrights: © 2015 Bowers J, et al.,

Introduction

Localized livedo reticularis and retiform purpura are signs of occlusion of medium-size vessels of the skin. (1,2)  Critical occlusion of medium-size vessels of the skin can result in retiform purpura and skin necrosis as well as necrosis of the fingers and toes. These medium size vessels are located in the hypodermis or deep dermis. Ischemia, thrombosis of small vessels, and hemorrhagic necrosis can occur distal to this occlusion, which can be due to vasculitis, thrombosis, emboli, or infection.(1,2) Diagnosis of the primary pathology requires a thorough history and physical exam, laboratory evaluation, and in some cases histopathology. Many internists, hospitalists, rheumatologists and dermatologists are not experts in this field, which may result in delay in diagnosis and unfavorable outcomes. In this article, we present an example of a complicated case of skin necrosis that was a diagnostic challenge and we discuss the approach to diagnostic evaluation.

 

Clinical scenario

 

An 85 year old female with history of Raynaud's syndrome, diabetes mellitus, and hypertension presented to the hospital with blue and black discoloration and cool temperature of the second through fifth digits of the left hand. Subsequent work

 

up revealed necrosis of the tip of her third and fourth fingers of right hand, a blanchable, erythematous area in a reticular pattern on the left foot, preserved radial pulses on both upper extremities, and a new splenic infarction.  

 

Previous evaluation of her Raynaud’s syndrome had not shown any etiology.  However, two weeks before presentation, the patient had worsening discoloration of the left third, fourth and fifth digits. She underwent left upper extremity angiogram that revealed proximal left subclavian artery high grade stenosis and a left subclavian artery stent was placed with resultant improvement in finger discoloration.

 

The constellation of findings including digital necrosis, localized livedo reticularis of the left foot and segmental splenic infarction led us toward the diagnosis of medium-size vessel occlusion as the underlying pathology. However, identifying the cause of this pathology was a challenge. 

 

Diagnostic challenge

 

Medium-size vessel occlusion can be caused by inflammation (vasculitis), thrombosis, emboli, or infection (Table 1). (2) Pure small vessel vasculitis can cause large areas of skin purpura and necrosis; however, the pattern of skin lesions is not retiform. Vasculitic processes that involve medium as well as small vessels can present with skin findings typical of both conditions. (3) Past medical history and other

 

Findings in history and physical exam can be clues to differentiate the potential causes. Unless the cause is obvious using history and physical exam, the patient should undergo skin biopsy as soon as possible. The skin biopsy should be large and include hypodermis. It should include an area of purpura and normal surrounding skin. The aim is finding the affected medium-size vessels that are typically located in the hypodermis and deep dermis and supply the purpuric area.

 

Histopathologic exam 

 

Histopathologic findings of medium-size vessels within the first few hours of the appearance of the rash can help with forming a diagnosis. The presence of vasculitis, an embolic particle, fungal elements, hyaline material, or pure thrombus in medium-size vessels can be clues to diagnosis. A few hours after occlusion of medium-size vessels, the area of skin distal to the occluded vessels shows evidence of ischemia including changes in eccrine glands. Small vessels develop thrombosis and red blood cell extravasation occurs. After 24 to 48 hours, there will be inflammatory infiltrate around the thrombosed vessels. This may cause confusion with a primary vasculitis. (2, 3)

 

It is important to note that thrombosis of small vessels in the affected area can be secondary to ischemia and is not a clue to the primary pathology.

 

Pathology

Potential causes

 

Medium vessel vasculitis

Polyarteritis Nodosa

Kawasaki disease

Granulomatosis with Polyangiitis

Churg–Strauss syndrome

Microscopic polyangiitis

Mixed cryoglobulinaemia

Vasculitis in rheumatoid arthritis, systemic lupus erythematosus , Sjogren's syndrome

Drugs

Infection (e.g. Human Immunodeficiency Virus)

 
 

Microvascular platelet plugs

 

Heparin necrosis

Thrombocytosis

Paroxysmal nocturnal hemoglobinuria

Thrombotic thrombocytopenic purpura

 
 

Cold-related gelling or agglutination

Type I cryoglobulinemia

Cryofibrinogenemia

Cold agglutinins

 
 

Other causes of medium-sized vessel occlusion

Hypercoagulable states (e.g. protein C or S deficiency, antiphospholipid antibody, disseminated intravascular coagulation )

Calciphylaxis

Oxalosis

Cholestrol emboli

Fat or air emboli

Sickle cell disease, malaria

Malignant atrophic papulosis

Livedoid vasculopathy

 
 

Infections including angio-invasive organisms

Fungi (mucormycosis, aspergillus, candida, fusarium, absidia and rhizopus)

Ecthyma ganrenosusm (gram negative bacilli, pseudomonas, Staph aureus)

Endocarditis

Nectrotizing fasciitis

Disseminated strongyloidiasis

Meningococcal infection

Rickettsial infection (e.g. Rocky Mountain Spotted Fever)

Viral hemorrhagic fevers

 

 

Table 1. Common pathologies and causes of retiform purpura

 

 

Laboratory evaluation

 

Laboratory work up varies depending on the clinical scenario and is directed based on differential diagnoses. A typical case of cholesterol emboli in a typical setting with cholesterol particles visible in retinal vessels on ophthalmoscopy may not need any additional labs for diagnosis. A patient with pure thrombosis in medium vessels may need work up for hypercoagulable states. (2) A vasculitic process may need an immunofluorescent exam of tissue as well as extensive work up for underlying etiologies of the vasculitis.

 

Case Discussion

 

In our case, the temporal association with the stent placement suggested cholesterol emboli as the cause, but there were some atypical features such as presence of Raynaud’s syndrome that raised suspicion for vasculitis.  Unfortunately, the patient and family decided to pursue a comfort care approach, foregoing further workup of the underlying etiology. However, follow up of the patient did not reveal progression of the skin lesions, favoring diagnosis of cholesterol emboli.

Conclusion

Localized livedo reticularis and retiform purpura can be signs of a severe and serious systemic disease.  Most cases need emergent diagnosis in order to control the disease if possible. Skin biopsy in the first few hours may help with diagnosis. Interpretation of histopathological findings needs expertise in this field. Good communication between internist, rheumatologist, dermatologist and dermatopathologist is essential for emergent diagnosis.

References

  1. LY X, Esparza EM, Anadkat MJ, Crone KG, Brasington RD (2009) Cutaneous manifestations of vasculitis. Semin Arthritis Rheum, 38: 348-60.
  2. Wysong A, Venkatesan P (2011) An approach to the patient with retiform purpura. Dermatol Ther, 24: 151-72.
  3. Pol-Rodriguez MM, Crane S, Feinberg DL, Glusac EJ, Bolognia JL (2003) Retiform purpura. Arch Dermatol, 139: 803-8.
     

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