The chilblain-like eruption as a diagnostic clue to the blast crisis of chronic myelocytic leukemia

Yazawa, Hitoshi; Saga, Kenji; Omori, Fusayuki; Jimbow, Kowichi; Sasagawa, Yutaka

doi:10.1016/s0190-9622(03)02114-5

Cited by 29 publications

(19 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A wide range of diseases causing an increase in serum viscosity, coagulation abnormalities or pathological cell circulation has been reported in association with perniosis, such as proliferative blood cell lines disorders, viral infections, cyoproteinaemia, connective tissue disease and diseases causing weight reduction (12)(13)(14)(15)(16)(17).…”

Section: Discussionmentioning

confidence: 99%

Acute Perniosis in Elderly People: A Predictive Sign of Systemic Disease?

Guadagni¹,

Nazzari²

2010

Acta Derm Venerol

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Acute Perniosis in Elderly People: A Predictive Sign of Systemic Disease?

Guadagni¹,

Nazzari²

2010

Acta Derm Venerol

View full text Add to dashboard Cite

“…Among adolescents, pernio has been seen in association with anorexia nervosa. 15 Among adults, pernio has been reported in association with systemic lupus erythematosus, 7 lupus anticoagulant, anticardiolipin, and antiphospholipid antibodies, 14 chronic myelocytic leukemia, 16 metastases from carcinoma of the breast, 17 and reaction to medication. 18 It is important, therefore, to consider these conditions when evaluating a patient with pernio, both to exclude an underlying diagnosis and to determine whether additional testing is necessary.…”

Section: Discussionmentioning

confidence: 99%

Pernio in Pediatrics

2005

View full text Add to dashboard Cite

ABSTRACT. Pernio, or chilblains, is a localized inflammatory lesion of the skin resulting from an abnormal response to cold. Five cases were seen among adolescent female patients who presented to our rheumatology service in a pediatric tertiary care center in the winter of 2003 to 2004. All 5 patients were thin (BMI of <25th percentile) and had either toes or fingers that were affected. For each, laboratory evaluation results were unremarkable, including negative antinuclear antibody profile results. Symptomatic treatment, with or without medication, was recommended. Pernio most commonly occurs among young women but may occur among older individuals or among children. Because pernio develops among susceptible individuals who are exposed to nonfreezing cold, the lesions usually begin in the fall or winter and disappear in the spring or early summer. Acute pernio may develop 12 to 24 hours after exposure to the cold. Single or multiple erythematous, purplish, edematous lesions appear, accompanied by intense pain, itching, or burning. Chronic pernio occurs with repeated exposure to the cold and the persistence of lesions. In an acute exacerbation, the major differential diagnosis alternative would be Raynaud's phenomenon, which consists of sharply demarcated cutaneous pallor and cyanosis, followed by erythema, of far shorter duration (hours rather than days). Frostbite is freezing of tissue, with resultant tissue necrosis. Several conditions have been described as predisposing subjects to pernio, including the presence of cryoproteins, excessive cold exposure, and anorexia nervosa among children and systemic lupus erythematosus and antiphospholipid antibodies among adults. It is important, therefore, when evaluating a patient with pernio, both to exclude an underlying diagnosis and to determine whether additional testing is necessary. The lesions of acute pernio are usually self-limited but may lead to recurrent disease. The involved limb should be cleaned and dried, and rewarming should occur. Prevention is the best form of therapy, and cold exposure should be minimized after an initial insult. The prognosis for properly treated pernio is excellent. Nifedipine, which produces vasodilation, has been demonstrated to be effective in reducing pain, facilitating healing, and preventing new lesions of pernio. We think that the 5 cases seen in our rheumatology clinic represent an increase, compared with prior years; the dermatology clinic at the University of Colorado reported a series of 8 children treated during a 10-year period. The reasons for the possible increase are likely multifactorial, with cold climate, a vulnerable population with thin body habitus, and cold exposure all being contributing causes. Of note, the quality of cold in Colorado is quite dry; however, the winter of 2003 to 2004 was not particularly colder or drier than prior years. All patients were very thin, and thin body habitus may be associated with increased cutaneous vasoreactivity. It is also unclear how these cases of pernio may reflect that...

show abstract

“…[55] Pernio is usually a clinical diagnosis, but skin biopsy may be necessary to differentiate pernio from acral lesions of lupus erythematosus. [58] In any refractory case of pernio, the non-disease-specific infiltrates of chronic myelogenous leukemia (CML) [59,60] and the specific skin eruption of a CML blast crisis [61] must be in the differential diagnosis. [56] The only prospectively studied medication with proven efficacy is nifedipine, which has been shown to reduce perivascular lymphocytic infiltrates and prohibit formation of new lesions while promoting clearance of existing pernio.…”

Section: 2 Non-freezing Injurymentioning

confidence: 99%

“…Histologic hallmarks of pernio include a papillary and deep T-cell infiltrate with perieccrine reinforcement, dermal edema, and necrotic keratinocytes. [61] As discussed in section 2.1, acral lesions of systemic and cutaneous lupus erythematosus may affect the digits in various ways. [57] The natural history of untreated pernio is resolution in 1-3 weeks in healthy adults.…”

Section: 2 Non-freezing Injurymentioning

confidence: 99%

The Purple Digit

Brown

Zirwas

English

2010

American Journal of Clinical Dermatology

View full text Add to dashboard Cite

The acute onset of purple digits is a concerning manifestation and may represent underlying, potentially life-threatening disease. Correctly identifying the etiology of purple digits is essential to proper management, and can aid in the diagnosis of systemic disease. Multiple causes of purple digits and significant overlap in clinical presentation can make diagnosis difficult. Despite the various causes of acute purple digits in the published literature, an algorithmic approach to the evaluation and management of the most common and alarming etiologies has yet to be established. The initial step in evaluating a patient with purple digits is to determine if the cause is associated with hypoxemia or trauma. If the patient is in a stable condition, the dermatologist needs to determine if the process could be related to cold exposure such as Raynaud phenomenon, acrocyanosis, pernio, cryoglobulinemia or frostbite. If the disease occurs independent of temperature, physical examination and histological evaluation of the skin is recommended. The lack of peripheral pulses are concerning for acute arterial thrombosis from peripheral vascular disease or arterial embolism. Non-blanching skin changes on the digit that lack inflammation and microthrombosis most likely represent a bleeding or platelet abnormality; however, if microthrombi are identified a more life-threatening processes such as purpura fulminans or embolic phenomenon may be occurring. Evidence of blood vessel inflammation suggests a leukocytoclastic vasculitis. The patient with a purple blanching digit and normal pulses requires an extensive historical review to help determine the cause. This review presents an algorithmic approach to assist in the evaluation and management of the purple digit.

show abstract

The chilblain-like eruption as a diagnostic clue to the blast crisis of chronic myelocytic leukemia

Cited by 29 publications

References 12 publications

Acute Perniosis in Elderly People: A Predictive Sign of Systemic Disease?

Acute Perniosis in Elderly People: A Predictive Sign of Systemic Disease?

Pernio in Pediatrics

The Purple Digit

Contact Info

Product

Resources

About