Poikiloderma of Civatte

 

Poikiloderma of Civatte refers to erythema associated with a mottled pigmentation seen on the sides of the neck, more commonly in women. Civatte first described the condition in 1923.

Poikiloderma of Civatte is a rather common, benign condition affecting the skin. Many consider it to be a reaction pattern of the skin and not a disease. The term poikiloderma refers to the combination of atrophy, telangiectasia, and pigmentary changes (both hypopigmentation and hyperpigmentation). Poikilodermatous lesions may be seen in certain genodermatoses (Rothmund-Thomson syndrome, Bloom syndrome, dyskeratosis congenita), in connective-tissue diseases (dermatomyositis, lupus erythematosus), in parapsoriasis/mycosis fungoides, and in radiodermatitis.

The incidence of poikiloderma of Civatte is unknown; many patients may have a mild form of the disease and may not seek medical attention.

In most patients, the condition is of purely cosmetic concern. Patients with the mild form do not seek medical advice.

Poikiloderma of Civatte occurs most commonly in fair-skinned individuals.

Poikiloderma of Civatte occurs more commonly in females than in males.¹

Most commonly, individuals affected are middle-aged or elderly women; however, the disease has been seen in other age groups.

• Patients usually complain of chronic reddish-brown discoloration on the lateral cheeks and neck.
• Lesions usually are asymptomatic, but occasionally, patients report mild burning, itching, and hyperesthesia.

Reddish-brown, reticulate pigmentation with atrophy and telangiectasia is usually present in symmetric patches on the lateral cheeks and sides of the neck.

• Lesions appear to run in line with the normal skin creases of the neck.
• The submental area shaded by the chin usually is spared.

 

 

Causes

• Chronic exposure to ultraviolet light appears to be a primary etiologic factor, which is supported by the finding that lesions occur on sun-exposed areas.^2,3 In addition, solar elastosis is a frequent histopathologic finding.
• Photosensitizing chemicals in perfumes or cosmetics have been implicated in the pathogenesis of poikiloderma of Civatte.
• Possibly, hormonal changes related to menopause or low estrogen levels may be a factor. Most patients are middle-aged or elderly women.

Genetic predisposition may exist. This is supported by the presence of the condition in successive generations, and in individuals of both sexes, consistent with an autosomal dominant inheritance with variable penetrance.⁴ The genetically determined predisposition may be expressed as an increased susceptibility of the skin to ultraviolet radiation

 

Treatment

• No specific medical treatment exists for poikiloderma of Civatte.
• Educating the patient about avoiding sun exposure and the proper use of sunscreens is most important.

• Intense pulsed light systems have been used and found to be effective in the treatment of poikiloderma of Civatte.^7,8,9,10,11 These are high-intensity light sources that emit polychromatic, noncoherent light and, thus, are different from lasers. They have a wavelength spectrum of 515-1200 nm. Several treatments may be required for complete clearing. Intense pulsed light should only be used by those experienced in the modality because persistent pigment abnormalities have been reported.¹²
• The erythema and telangiectasias in persons with poikiloderma of Civatte respond well to the flashlamp-pumped pulsed-dye laser (585 nm and 595 nm)^13,14,15 and to the potassium-titanyl-phosphate laser.^16,17 Caution must be exercised because of the higher incidence of adverse effects, such as hypopigmentation and scarring, especially when treating the neck and chest. Persistent depigmentation as a late adverse event has been reported in a series of patients with poikiloderma of Civatte after treatment with pulsed-dye laser.¹²  Patients must be informed about the possibility of temporary purpura after treatment. Several treatment sessions may be required.
• Use of fractional photothermolysis (laser technology that creates microthermal injury zones in skin) to treat poikiloderma of Civatte has also been described, with promising results. Several treatment sessions may be required.¹⁸
• Attempts to correct the disorder using electrosurgery, cryotherapy, and argon laser have been unsuccessful.