Background

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.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
SEX (M:F)	Females
GEOGRAPHY	Humid climates of northern Europe

 

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

 

PATHOGENESIS	CHARACTERIZATION
Vasospasm	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

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General
VARIANTS
Chilblains lupus erythematosus	Br J Dermatol 1978;98:497

 

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL	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.
VARIANTS
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

 

SPECIAL STAINS/ IMMUNOHISTO-CHEMISTRY/ OTHER

CHARACTERIZATION

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.

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSIS	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
TREATMENT	Prophylactic avoidance of cold
Nifedipine	Br Med J 1986;293:923
	Acta Derm Venereol 1989;69:320 Ultraviolet light

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Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
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Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fifth Edition. Mosby Elesevier 2008

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Last Updated February 8, 2010

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