Poikiloderma of Civatte 

Updated: Apr 28, 2017
Author: Jeannette Rachel Jakus, MD, MBA; Chief Editor: Dirk M Elston, MD 

Overview

Background

Poikiloderma of Civatte is an acquired poikiloderma of the lateral face and neck first described by the French dermatologist Achille Civatte in 1923. 

Pathophysiology

Poikiloderma of Civatte is a commonly acquired chronic and progressive condition affecting mostly older fair-skinned individuals, and, although benign, it may result in significant cosmetic disfigurement. While the exact pathogenesis of this condition is still unknown, several causative factors have been hypothesized, including long-term ultraviolet (UV) exposure, hormonal changes of menopause, genetics, and contact hypersensitivity, most specifically to fragrances and cosmetics.[1]

Epidemiology

Frequency

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

Race

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

Sex

Poikiloderma of Civatte occurs more commonly in females than in males.[2]

Age

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

Prognosis

Poikiloderma of Civatte is a chronic and progressive skin condition and, although benign, may result in cosmetic disfigurement that can be quite concerning to some patients. Patients with the mild form typically do not seek medical advice.

Patient Education

Patients should be instructed to minimize UV exposure and to apply a broadband sunscreen of SPF 30 or more every day to all exposed areas of the face and neck. It is important to also remind patients to reapply their sunscreen every 1.5-2 hours or sooner if after exercise or water exposure.

Patients should also be instructed to avoid the application of fragrances or cosmetics to the affected areas.

 

Presentation

History

Patients usually report a chronic reddish-brown discoloration on the lateral cheeks and neck. Lesions usually are asymptomatic, but occasionally, patients may report mild burning, itching, and/or hyperesthesia.

Physical Examination

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 is classically spared (note the image below).

Poikiloderma of Civatte over the neck. Notice spar Poikiloderma of Civatte over the neck. Notice sparing of the area under the chin. Courtesy of Dr. Shukrallah Zaynoun.

Causes

Long-term exposure to UV light appears to be a primary etiologic factor, which is supported by the finding that lesions occur on sun-exposed areas.[3, 4] 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, including fragrances used in aroma therapy.[1, 5] A 2014 article cited hypersensitivity to methylchloroisothiazolinione and methylisothiazolinone (MCI/MI) as a possible contributing etiology.[1]

As this condition is seen most commonly in middle-aged and elderly women, hormonal changes related to menopause or low estrogen levels have been implicated.

Genetic predisposition may also 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.[6] The genetically determined predisposition may be expressed as an increased susceptibility of the skin to UV radiation.

A classification system has been suggested to help with the diagnosis and treatment of specific causes of poikiloderma.[7]

 

DDx

 

Workup

Laboratory Studies

While typically not necessary, antinuclear and anti-Ro (SS-A) antibody levels may be ordered to exclude connective-tissue disease if suspected.

Histologic Findings

The epidermis shows moderate thinning of the stratum malpighii, hydropic degeneration of the basal cells, and effacement of the rete ridges. In the upper dermis, a bandlike inflammatory infiltrate primarily is composed of lymphocytes with few histiocytes, in addition to pigment incontinence. In late stages, a thin flattened epidermis, edema in the upper dermis, and dilated blood vessels are demonstrated.

Katoulis et al report that poikiloderma of Civatte has distinct histological and ultrastructural features, stating these features are characteristic but not pathognomonic and that poikiloderma of Civatte should be considered a separate entity.[8]

 

Treatment

Medical Care

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. Exposure to fragrances and cosmetics should also be avoided.

Surgical Care

Intense pulsed light systems have been used and found to be effective in the treatment of poikiloderma of Civatte.[9, 10, 11, 12, 13, 14] Intense pulsed light induced a more-homogeneous distribution of melanin and increased nonfragmented elastic fibers, collagen density, and intensity, but no significant changes in vessel numbers or diameters.[15] 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.[16]

The erythema and telangiectasias in persons with poikiloderma of Civatte respond well to the flashlamp-pumped pulsed-dye laser (585 nm and 595 nm)[17, 18, 19] and to the potassium-titanyl-phosphate laser.[20, 21] 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.[16] 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 to improve both dyschromia and wrinkles but has not been shown to be effective in improving skin laxity of the neck. Several treatment sessions may be required.[22, 23, 24]

Attempts to correct the disorder using electrosurgery, cryotherapy, and argon laser have been unsuccessful.

Prevention

Avoiding perfumes and using proper photoprotection are advocated.