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The Diagnosis and Management of Perniosis Chilblains
The Diagnosis and Management of Perniosis Chilblains
To cite this article: HF Jordaan MBChB, MMed (Derm) (2007) The diagnosis and
management of perniosis (chilblains), South African Family Practice, 49:6, 28-29, DOI:
10.1080/20786204.2007.10873574
Abstract
Perniosis (chilblains) is an abnormal reaction to cold that occurs most frequently in women, children, and the elderly. Chilblains
may be idiopathic and self-limited, or associated with systemic diseases. This article discusses the approach to the diagnosis
and treatment perniosis and explains the concepts of acrocyanosis and erythrocyanosis.
SA Fam Pract 2007;49(6): 28-29
palms are often cold and clammy. The perniosis of the hands, gloves are rec- and cryoproteins. Pediatr Dermatol
condition is caused by arteriolar con- ommended. Clothing should be loose- 2000;17:97-99.
striction, dilatation of the subpapillary fitting. The ambient temperature should 4. Viguier M, Pinquier L, Cavelier-Balloy
venous plexus, and increases in blood be warm. These environmental chang- B, et al. Clinical and histopathologic
features and immunologic variables in
viscosity induced by the cold tempera- es are most critical in preventing recur-
patients with severe chilblains: a study
tures. rences. Feet should always be kept dry of the relationship to lupus erythemato-
as moisture enhances cold injury. sus. Medicine 2001;80:180-188.
Erythrocyanosis 5. Cribier B, Djeridi N, Peltre B, Grosshans
Erythrocyanosis occurs in obese, often Alternative Steps E. A histologic and immunohistochemi-
young, women. Purple-red mottled 1. Nicotinamide (500 mg three times cal study of chilblains. J Am Acad Der-
discoloration is seen on the buttocks, daily) may be useful alone or in ad- matol 2001;45:924-929.
thighs and lower legs. Cold provokes dition to calcium channel blockers. 6. Rustin MHA, Newton JA, Smith NP,
the condition and causes an unpleasant Flushing and palpitation are prob- Dowd PM. The treatment of chilblains
burning sensation. lematic. with nifedipine: the results of a pilot
study, a double-blind placebo-con-
2. Hexylnicotinate (2% cream applied
trolled randomized study and a long-
Diagnosis three times daily) may be useful for term open trial. Br J Dermatol 1989;120:
The diagnosis of perniosis is usually patients who are, intolerant of, or un- 267-275.
based on clinical grounds. A skin biopsy willing to take oral medication.
may be helpful in cases where the diag- 3. Amlodipine, (2.5-5 mg once dai-
nosis is in doubt. The four characteristic ly) may be used as an alternative to
findings are scattered necrosis of indi- calcium channel blockers. The long
vidual keratinocytes, marked subepider- half-life and consequent once dai-
mal oedema, perivascular lymphocytes ly dosing of this drug are beneficial
and lymphocytic vasculitis. Perniosis when chronic therapy is required.
may be difficult to distinguish from lupus 4. Erythema doses of ultraviolet light
erythematosus. Spongiosis and periec- (UVB) to affected areas 2 to 3 times
crine lymphocytes are more common a week at the start of winter may pre-
in perniosis. In lupus erythematosus vent the development of lesions.
vacuolation of the basal layer is more 5. Sympathectomy may be advised in
common, mucin is usually increased in severe cases.
the dermis and the lupus band test (that
is IgG at the dermo-epidermal junction) Pitfalls
is positive. The presence of antinuclear 1. Pernio-like lesions occur in both
antibodies favours a diagnosis of lupus discoid and systemic lupus erythe-
erythematosus. matosus, as well as in sarcoidosis.
The possibility of lupus should be
Treatment excluded by appropriate laboratory
First Steps tests and biopsy. Lupus pernio is a
The therapeutic strategy comprises confusing term; it is related to neither
avoiding chronic exposure to cold tem- pernio nor to lupus erythematosus,
peratures and employing therapeutic but is a particular manifestation of
agents that increase peripheral circu- sarcoidosis.
lation. 2. Cryoglobulinaemia, cryofibrinogenae-
The calcium channel blocker, nifedip- mia and other hypercoagulable states
ine (10 mg three times daily or 20 mg should be considered, especially in
twice daily) is very effective both in in- atypical or refractory cases. Most
creasing the rate of resolution of pernio cases of classic perniosis are not as-
lesions and in preventing their appear- sociated with such conditions but are
ance. This efficacy is due to the vaso- environmentally triggered.
dilatory effect of this medication. While
mild symptoms of peripheral oedema, See CPD Questionnaire, page 43
flushing, headaches and hypotension
may occur, these symptoms rarely re-
P This article has been peer reviewed
quire discontinuation of the medication
when used in low doses. Blood pres-
sure should be monitored at the start of References
treatment and at return visits. 1. St. Clair NE, Kim CC, Semrin G, et al.
Celiac disease presenting with chil-
Ancillary Steps blains in an adolescent girl. Pediatr
Dermatol 2006;23:451-454.
Keeping both the affected extremities
2. Weedon D. Skin Pathology.2nd. ed. St
and the core body dry and warm are Louis, Mo: Churchill Livingstone Inc;
essential in preventing pernio. Patients 2002; 250-251.
should wear thick socks and shoes. For 3. Weston WL, Morelli J. Childhood pernio