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This interesting disease is characterized by single or multiple painful, erythematous to purplish swellings which may evolve to ulceration and necrosis. The lesions characteristically occur on the distal extremities, especially involving fingertips and toes. Other sites include the heels, ears, nose, calves, and thighs. They are more common in the elderly and usually resolve within 1-3 weeks. There are several recent reports which suggest an association with connective tissue disorders such as systemic lupus erythematosus. Some investigators had used the term idiopathic perniosis to designate patients who have no underlying systemic disease.


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Humid climates of northern Europe


Systemic lupus erythematosus  
Antiphopholipid antibody syndrome  
Viral hepatitis  
Rheumatoid arthritis  
Crohn's disease  
Raynaud's phenomenon  
Small joint arthralgias  
Positive ANA  



J Am Acad Dermatol 1990;23:257-262

Cold may induce vasospasm leading to a lymphocytic vascular reaction and lymphocytic infiltration

In cases of chilblains Lupus erythematosus, autoantibodies directed against the endothelium, in the form of anti-Ro antibodies or a positive rheumatoid factor, may predispose the vessels to attack and vasculitis


Chilblains lupus erythematosus
Br J Dermatol 1978;98:497



Clin Exp Dermatol 1981;6:263

Epidermal necrosis may be present

Extensive papillary dermal edema with a superficial and deep perivascular and periadnexal lymphocytic infiltrate

Lymphocytic vasculitis in advanced cases
May have frequent fibrin vascular deposition

Perniosis: clinical and histopathological analysis.

Boada A, Bielsa I, Fernández-Figueras MT, Ferrándiz C.

Department of Dermatology, Hospital Universitari Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, Spain.

Am J Dermatopathol. 2010 Feb;32(1):19-23. Abstract quote

Perniosis are inflammatory cutaneous lesions, located on acral skin, which present in association with cold exposure. They can appear as an idiopathic dermatosis or with an underlying autoimmune disease. The use of cutaneous biopsy to distinguish between both types is controversial.

We analyze the histological findings in 9 cases of idiopathic perniosis (IP) and compare them with those obtained from 11 cases of perniosis associated with an autoimmune disease (autoimmune perniosis). The most frequent histopathological features observed in cases of IP were a lymphocytic infiltrate with perivascular (8 cases, 89%) and perieccrine distribution (6 cases, 66%), dermal edema (5 cases, 55%), and necrotic keratinocytes (5 cases, 55%), whereas those found in perniosis associated with an autoimmune disease were lymphocytic infiltrate with perivascular distribution (11 cases, 100%) but without perieccrine distribution (3 cases, 27%), vacuolation of the basal layer (7 cases, 63%), and necrotic keratinocytes (5 cases, 45%).

The only significant difference between both groups was the perieccrine distribution of the lymphocytic infiltrate in cases of IP, which, as mentioned in previous studies, could be considered a histopathological clue to differentiate both types of perniosis.

Idiopathic perniosis and its mimics: a clinical and histological study of 38 cases.

Crowson AN, Magro CM.

Department of Laboratories, Misericordia General Hospital, Winnipeg, Manitoba, Canada.

Hum Pathol 1997 Apr;28(4):478-84 Abstract quote

Perniosis is a term applied to cold-induced painful or pruritic erythematous or violaceous acral papular or nodular lesions.

We examined 39 skin biopsies from 38 patients who presented with acral purpuric lesions, suggesting a diagnosis of perniosis clinically or pathologically. The presence of a systemic or extracutaneous disease was established in 17 patients, including 5 with systemic lupus erythematosus (SLE), 3 with antiphospholipid antibodies, in 1 in whom there was underlying HIV disease, 2 with viral hepatitis, 2 with rheumatoid arthritis (RA), 2 with cryofibrinogenemia, 1 with hypergammaglobulinemia, 1 with iritis, and 1 with Crohn's disease. In the other 21 patients, the clinical presentations prompted further studies in 12, which showed a positive antinuclear antibody (ANA) in 10. A diagnosis of idiopathic perniosis (IP) was rendered in all 21 of these patients including those in whom a positive ANA was discovered, based on the absence of any other serological markers, signs, or symptoms indicative of a specific systemic disease complex; many had Raynaud's phenomenon, small joint arthralgias, atopy, or a family history of either connective tissue disease or Raynaud's disease.

The histopathology of IP comprised a superficial and deep angiocentric lymphocytic infiltrate with papillary dermal edema and lymphocytic exocytosis directed to retia and acrosyringia. A few cases showed a mild vacuolopathic or lichenoid interface dermatitis, adventitial dermal mucinosis, lymphocytic eccrine hidradenitis, vascular ectasia, and thrombosis confined to dermal papillae capillaries.

The biopsies from patients with iritis, RA, and Crohn's disease showed a granulomatous vasculitis and a granuloma annulare-like tissue reaction. The biopsies from the patients with SLE, cryofibrinogenemia, primary antiphospholipid antibody syndrome, and hypergammaglobulinemia shared a similar histopathology comprising an interface dermatitis, superficial and deep angiocentric and eccrinotropic lymphocytic infiltrates, vascular ectasia, and dermal mucinosis with prominent involvement of the eccrine coil.

Many cases did not show features of IP, namely papillary dermal edema, thrombosis of dermal papillary capillaries, and lymphocytic exocytosis into the retia and acrosyringia. There was frequent vascular fibrin deposition involving reticular dermal vessels. The latter two variables were statistically significant discriminators between IP and in perniotic lesions observed in the setting of underlying systemic disease.

With respect to the latter, some cases occurred in the setting of cold exposure and were designated by us as "secondary perniosis" (SP), whereas others showed no specific association with cold exposure and were designated as perniotic mimics (PMs) based exclusively on the gross and microscopic morphology of the lesions.

Granulomatous pernio

Clin Exp Dermatol 1981;6:263-271

Histiocytic infiltrate may become pronounced
These lesions correlate with clinical presence of erythema induratum and clear with antituberculous therapy-suggesting these reactions may be an id reaction to occult mycobacterial infection




A histologic and immunohistochemical study of chilblains

Bernard Cribier, MD, PhD
Nadia Djeridi, MD
Bernard Peltre
Edouard Grosshans, MD

Strasbourg, France

J Am Acad Dermatol 2001;45:924-9. Abstract quote

Background: The histopathologic diagnosis of chilblains is controversial and the histologic changes are often considered nonspecific, mainly because they are poorly documented. Although a dermal inflammation in chilblains has been noticed, the infiltrate has not yet been characterized.

Objective: Our purpose was to analyze microscopic and immunohistochemical findings in a large series of chilblains and to compare the results with those of lupus erythematosus (LE).

Methods: We included 36 cases of clinically typical chilblains of the hands, of which 17 were thoroughly investigated to rule out cryopathy or LE. Ten biopsy specimens of hand lesions from patients with proven LE were included as controls. All slides were analyzed by conventional microscopy and by immunohistochemistry with anti-CD3, anti-CD20, and anti-CD68 antibodies.

Results: The most characteristic finding in chilblains (47% of cases) was the association of edema and reticular dermis infiltrate that showed a perieccrine reinforcement. Such a combination of changes was not observed in LE. Epidermal changes in chilblains consisted mainly in necrotic keratinocytes in 52% of cases. The comparison of 17 idiopathic chilblains with LE showed significant differences in spongiosis (58% vs 0% respectively), vacuolation of basal layer (6% vs 60%), edema of the dermis (70% vs 20%), and deep perieccrine inflammation (76% vs 0%). Immunohistochemistry showed that the infiltrate was composed of a majority of T cells associated with macrophages and a few B lymphocytes. The same pattern was observed in both chilblains and LE.

Conclusion: Our results show that a predominantly T-cell papillary and deep infiltrate with a perieccrine reinforcement, associated with dermal edema and necrotic keratinocytes, are the hallmarks of chilblains of the hands. These changes can help differentiate idiopathic perniosis from LE; immunohistochemistry is of no use in differentiation.



Hum Pathol 1997;28:478-484

This study suggests that as a baseline investigation, any patient presenting with chilblains-like lesions should have the following studies performed:
Antibodies to Ro, ANA, rheumatoid factor, cryofibrinogens, and antiphospholipid antibodies
Hepatitis screen
Serum protein electrophoresis


Prophylactic avoidance of cold

Br Med J 1986;293:923

Acta Derm Venereol 1989;69:320

Ultraviolet light

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Last Updated February 8, 2010

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