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AlexanderSalava

Temperature-Induced Skin Disorders

See also articles on Frostbite injuries Frostbite Injuries and Raynaud's phenomenon or white finger disease Raynaud's Phenomenon (RP) or White Finger Disease.

Essentials

  • Temperature changes may provoke skin changes.
  • The underlying cause may be a characteristic reaction of the peripheral circulation or an inflammatory reaction to the temperature.
  • The tendency may be hereditary.
  • Treatment is mostly symptomatic, avoiding triggering factors is paramount.
  • In rare cases, the symptoms may be associated with an autoimmune or internal disease.

General information

  • Temperature changes cause physiological reactions in the peripheral circulation that are controlled by the autonomic nervous system.
  • In cold weather, the aim of these reactions is to reduce peripheral blood flow (shutting off arteriovenous anastomoses), and in hot weather to increase it (heat loss).
  • In some patients, temperature changes provoke visible skin manifestations. In some cases, temperature changes cause an inflammatory reaction (such as local vasculitis in chilblains).
  • Most such skin manifestations are benign and not associated with any disease. The appearance of symptoms tends to be individual and is not usually associated with any absolute external temperatures.

Cold-related symptoms

Chilblains (perniosis)

  • Cold exposure causes painful, erythematous nodules on the skin (pictures ).
  • Typical sites include the dorsal aspects and sides of fingers and toes; the heels, lower legs and feet, more rarely the wrists, nose and earlobes.
  • They may appear with a delay of 1-2 days from cold exposure and normally persist for 2-3 weeks.
  • They are more common in children, elderly people and patients with either Raynaud's phenomenon or acrocyanosis Raynaud's Phenomenon (RP) or White Finger Disease.
  • In children and adolescents, chilblains may occur a few winters in succession and then normally subside.
  • In adults and elderly people, the problem is usually more chronic and may get worse if cold exposure is not avoided.
  • A particular subtype (cold panniculitis) may occur in wintertime on the cheeks or forehead in children, on the lateral thighs in horse riders (e.g. equestrian chilblain) or on the lower abdomen, buttocks or thighs in overweight patients, for example.

Primary chilblains

  • Occur in individually susceptible people; also affected by hereditary factors.
  • Predisposing factors include smoking, arteriosclerosis obliterans, diabetes, eating disorders and undernutrition (e.g. anorexia), obesity and vasoconstrictive medication, for example.

Secondary chilblains

  • Less common than primary chilblains
  • There may be an underlying rheumatic connective tissue disorder, notably lupus erythematosus Systemic Lupus Erythematosus (Sle), chilblain lupus http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=90280 or systemic sclerosis Systemic Sclerosis (including Raynaud's phenomenon).
  • Secondary chilblains may also be triggered by infections.
  • Treatment
  • Prevention
    • Protection from cold, good protective gloves, smoking cessation
    • In severe cases, calcium channel blockers, such as nifedipine prolonged-release tablets, 20-60 mg once daily, or topical nifedipine or diltiazem ointment (extemporaneous prescription) once or twice daily (during the cold season or in courses)

Acrocyanosis (discolouration, of various shades of blue, of the distal extremities)

  • Functional disturbance of cutaneous circulation
  • The skin on hands and feet, fingers and toes in particular, shows an evenly purplish colour or purplish spots . Acrocyanosis may also occur on the face. Excessive sweating (cold, damp skin) is often simultaneously present Excessive Sweating (Hyperhidrosis).
  • Treatment
    • Usually no need for treatment
    • Protection from cold
    • Calcium channel blockers are normally not helpful.
    • In the secondary form of the disease, treatment of the underlying disease

Primary acrocyanosis

  • More common in women
  • The tendency usually appears in childhood or adolescence. The patient may also have chilblains or Raynaud's phenomenon.
  • Aggravating factors are the same as for chilblains.

Secondary acrocyanosis

Erythrocyanosis

  • Erythrocyanosis is a mottled blue discolouration of more extensive skin areas.
  • It can be seen at the back of the ankles or thighs in young people, for example. The symptoms may be associated with chilblains.

Livedo reticularis (mottled skin)

  • The term livedo reticularis denotes a lace-like pattern of reddish-blue discoloured skin.
    • The skin may appear purplish (cyanotic), and there are usually light pale spots surrounded by reticular lesions.
    • Livedo reticularis appears most commonly on the limbs (thighs, legs, upper arms) and is associated with changes in superficial cutaneous circulation.
  • Physiological livedo
    • Occurs particularly in newborn babies, children and young adults (cutis marmorata)
  • Congenital livedo reticularis
  • Primary livedo reticularis
    • Appears without any underlying cause; often also found in near relatives.
  • Secondary livedo (livedo racemosa)
    • Persistent, sometimes extensive livedo of acute onset
    • No association with temperature changes
    • The reticular erythema is often asymmetric and there are irregular circular lesions.
    • There may be pain and ulceration of the skin.
    • There may be various underlying disorders that often also cause other symptoms apart from the livedo pattern on the skin.
    • Possible causes
      • Thrombophilia (acquired or hereditary, such as antiphospholipid-antibody syndrome, Sneddon syndrome, livedoid vasculopathy)
      • Vascular diseases (atherosclerosis, vasculitis, heart failure)
      • Haematological diseases (such as polycythaemia, myeloma, lymphomas)
      • Autoimmune diseases (such as SLE, rheumatoid arthritis)
      • Infections (such as hepatitis C, erythema infectiosum)
      • Medication (such as amantadine, sympathomimetics, cytostatic drugs)
    • Causal teatment should be used, as far as possible; protection from cold.

White spots on palms and soles (pseudoleukoderma angiospasticum)

  • A common finding
  • With varying temperatures, reticular erythema and white spots appear on palms and/or soles.
  • There may also be white spots on arms and legs (Bier spots).
  • No association with any disease

Cold urticaria (urticaria e frigore)

  • A subtype of physical urticaria (chronic inducible urticaria) where itchy wheals, erythema and oedema appear shortly after exposure to cold (cold air, water or objects)
  • Symptoms may persist for a few hours. Anaphylactic reactions may also occur.
  • Often associated with other chronic urticarias, particularly physical subtypes (dermographism, cholinergic urticaria)
  • May occur as a secondary symptom triggered by an infection or, rarely, in association with haematological malignancies.
  • Rarely a symptom of a familial autoinflammatory disease (such as familial cold autoinflammatory syndrome, FCAS http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=10608)
  • Treatment the same as for other chronic urticarias Hives (Urticaria)

Heat-related symptoms

Erythromelalgia (burning pain in lower or upper limbs)

  • Episodes of burning pain, erythema and heat in hands or feet.
  • Occurs typically in soles, toes or fingers. May also occur on the face or ears.
  • Symptoms are provoked by heat or by the limb hanging downward.
  • Cooling and elevating the limb alleviate the symptoms.
  • The primary form of the disease is familial. It is more common in women and often begins in teenage. Between episodes, there may be acrocyanosis, chilblains or Raynaud's phenomenon.
  • The secondary type occurs as a new symptom. It is often asymmetric. It may be associated with a myeloproliferative disease (such as polycythaemia vera Polycythaemia Vera (PV), essential thrombocythaemia Essential Thrombocythaemia (Et)) or medication, rarely with a neuropathy, rheumatologic connective tissue disorder (such as rheumatoid arthritis) or an infection.
  • Treatment
    • Cooling, avoidance of provoking factors
    • Cooling creams or ointments, as necessary
      • Capsaicin cream or ointment (extemporaneous or special permit product)
    • Topical analgesic creams or ointments (such as lidocaine and/or prilocaine creams)
    • Oral NSAIDs, such as aspirin 100-250(-500) mg/day
    • Calcium channel blockers, such as nifedipine prolonged-release tablets, 20-60 mg once daily
    • In severe cases, drugs indicated in the treatment of neuropathic pain, such as amitriptyline, can be used starting with 10-25 mg/day (in the evening) and increasing the dose as necessary (see article Chronic pain Chronic Pain).

Cholinergic urticaria (urticaria cholinergica, heat bumps)

  • Wheals appear particularly on the chest, trunk and neck in hot weather, when taking a shower, after a bath or during sweaty physical exercise. The symptoms usually persist for a few tens of minutes.
  • The treatment is the same as for chronic urticaria. Antihistamines have also been used prophylactically before exertion Hives (Urticaria).

Excessive sweating (hyperhidrosis) and flushing

Erythema ab igne

  • A net-like pattern of erythema caused by local exposure to heat (such as a heat pad, radiator, laptop computer, fireplace).
  • Usually heals leaving reticular hyperpigmentation that will slowly become lighter over several weeks or months.

Heat rash (miliaria)

  • A quite common complaint resulting from obstructed sweat glands
  • Usually caused by humid, warm weather, sauna bathing, clothing or warm bed clothes
  • Most commonly seen in babies and small children and in the elderly (particularly patients in hospital or institutional care)
  • Extensive eruption of pinhead-sized erythematous papules that are often itchy (miliaria rubra; picture )
  • Some patients may also have tiny vesicles (miliaria crystallina) or pustules (miliaria pustulosa).
  • Typical sites include the neck, armpits, groin and upper body. The papules often mostly occur in macerated skin areas rubbed by clothing.
  • Treatment: cooling and light emollient lotions, short courses of weak or moderately potent glucocorticoid ointments

Workup

  • Clinical picture and association of symptoms with the temperature
    • To make the diagnosis, it is essential to examine the skin thoroughly, perform a clinical examination and take a thorough history of symptoms.
  • Targeted further investigations are necessary only if the skin symptom is suspected to be associated with an underlying systemic disease (secondary disease types). These practically always cause other symptoms apart from just skin symptoms.
  • If there are chilblains, acrocyanosis or livedo, basic blood count with platelet count, ESR, antinuclear antibodies, anti-ENA antibodies can be checked.
  • Other targeted investigations can be performed as necessary. Specialized care should be consulted if an underlying systemic disease (such as a rheumatic connective tissue disorder or haematological disease) is suspected. See also article Raynaud's phenomenon Raynaud's Phenomenon (RP) or White Finger Disease.
  • It is important to identify patients with secondary livedo and thromboembolic events (thrombophilia etc.) or skin ulceration (vasculitis).

Treatment

  • Symptomatic treatment, as far as possible (see above)
  • Symptoms can usually be alleviated by avoiding aggravating factors (avoiding exposure to cold and humidity, protecting the skin, cooling), and by stopping smoking.
  • In secondary types of disease, the underlying cause should be treated.

Specialist consultation

  • In severe or unclear cases, a dermatologist should be consulted, as necessary (to confirm the diagnosis).
  • If there is a strong suspicion of an underlying systemic disease, an internist, rheumatologist or haematologist should be consulted based on clinical findings and examination results, as necessary.

References

  • Nyssen A, Benhadou F, Magnée M et al. Chilblains. Vasa 2020;49(2):133-140. [PubMed]
  • Sachdeva M, Mufti A, Maliyar K et al. A Review of COVID-19 Chilblains-like Lesions and Their Differential Diagnoses. Adv Skin Wound Care 2021;34(7):348-354. [PubMed]
  • Wollina U, Koch A, Langner D et al. Acrocyanosis - A Symptom with Many Facettes. Open Access Maced J Med Sci 2018;6(1):208-212. [PubMed]
  • Klein-Weigel PF, Volz TS, Richter JG. Erythromelalgia. Vasa 2018;47(2):91-97. [PubMed]
  • Souwer IH, Bor JH, Smits P et al. Nifedipine vs Placebo for Treatment of Chronic Chilblains: A Randomized Controlled Trial. Ann Fam Med 2016;14(5):453-9. [PubMed]
  • Sánchez-Borges M, González-Aveledo L, Caballero-Fonseca F et al. Review of Physical Urticarias and Testing Methods. Curr Allergy Asthma Rep 2017;17(8):51. [PubMed]
  • Medlin JL, Hansen KE, Fitz SR et al. A systematic review and meta-analysis of cutaneous manifestations in late- versus early-onset systemic lupus erythematosus. Semin Arthritis Rheum 2016;45(6):691-7. [PubMed]
  • Sajjan VV, Lunge S, Swamy MB et al. Livedo reticularis: A review of the literature. Indian Dermatol Online J 2015;6(5):315-21. [PubMed]
  • Parsi K, Partsch H, Rabe E et al. Reticulate eruptions. Part 1: Vascular networks and physiology. Australas J Dermatol 2011;52(3):159-66. [PubMed]
  • Mann N, King T, Murphy R. Review of primary and secondary erythromelalgia. Clin Exp Dermatol 2019;44(5):477-482. [PubMed]