Poikiloderma of Civatte

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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.

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).



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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]

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]

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.

Author

Jeannette Rachel Jakus, MD, MBA, Clinical Assistant Professor, Director of Clinical Research, Assistant Program Director, Department of Dermatology, SUNY Downstate Medical Center; Dermatologist, Brody Dermatology

Disclosure: Nothing to disclose.

Specialty Editors

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD, Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Craig A Elmets, MD, Professor and Chair, Department of Dermatology, Director, Chemoprevention Program Director, Comprehensive Cancer Center, UAB Skin Diseases Research Center, University of Alabama at Birmingham School of Medicine

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: University of Alabama at Birmingham; University of Alabama Health Services Foundation<br/>Serve(d) as a speaker or a member of a speakers bureau for: Ferndale Laboratories<br/>Received research grant from: NIH, Veterans Administration, California Grape Assn<br/>Received consulting fee from Astellas for review panel membership; Received salary from Massachusetts Medical Society for employment; Received salary from UpToDate for employment. for: Astellas.

Lana H Hawayek, MD, Assistant Professor of Dermatology, Cosmetic Dermatologic Surgery and Laser Specialist, University of Cincinnati, Veterans Affairs Medical Center; Consulting Staff, University Dermatology Consultants

Disclosure: Nothing to disclose.

Nelly Rubeiz, MD, Consulting Staff, Department of Dermatology, American University of Beirut Medical Center; Professor, Department of Dermatology, American University of Beirut, Lebanon

Disclosure: Nothing to disclose.

References

  1. Khunkhet S, Wattanakrai P. The possible role of contact sensitization to fragrances and preservatives in poikiloderma of civatte. Case Rep Dermatol. 2014 Sep. 6 (3):258-63. [View Abstract]
  2. Graham R. What is poikiloderma of Civatte?. Practitioner. 1989 Sep 22. 233(1475):1210. [View Abstract]
  3. Goldberg LH, Altman A. Benign skin changes associated with chronic sunlight exposure. Cutis. 1984 Jul. 34(1):33-8, 40. [View Abstract]
  4. Lautenschlager S, Itin PH. Reticulate, patchy and mottled pigmentation of the neck. Acquired forms. Dermatology. 1998. 197(3):291-6. [View Abstract]
  5. Katoulis A, Makris M, Gregoriou S, Rallis E, Kanelleas A, Stavrianeas N, et al. Poikilodermatous changes on the forearms of a woman practicing aroma-therapy: extracervical poikiloderma of Civatte?. An Bras Dermatol. 2014 Jul. 89(4):655-6. [View Abstract]
  6. Katoulis AC, Stavrianeas NG, Georgala S, et al. Familial cases of poikiloderma of Civatte: genetic implications in its pathogenesis?. Clin Exp Dermatol. 1999 Sep. 24(5):385-7. [View Abstract]
  7. Nofal A, Salah E. Acquired poikiloderma: proposed classification and diagnostic approach. J Am Acad Dermatol. 2013 Sep. 69(3):e129-40. [View Abstract]
  8. Katoulis AC, Stavrianeas NG, Panayiotides JG, et al. Poikiloderma of Civatte: a histopathological and ultrastructural study. Dermatology. 2007. 214(2):177-82. [View Abstract]
  9. Goldman MP, Weiss RA. Treatment of poikiloderma of Civatte on the neck with an intense pulsed light source. Plast Reconstr Surg. 2001 May. 107(6):1376-81. [View Abstract]
  10. Raulin C, Greve B, Grema H. IPL technology: a review. Lasers Surg Med. 2003. 32(2):78-87. [View Abstract]
  11. Weiss RA, Goldman MP, Weiss MA. Treatment of poikiloderma of Civatte with an intense pulsed light source. Dermatol Surg. 2000 Sep. 26(9):823-7; discussion 828. [View Abstract]
  12. Wheeland RG, Applebaum J. Flashlamp-pumped pulsed dye laser therapy for poikiloderma of Civatte. J Dermatol Surg Oncol. 1990 Jan. 16(1):12-6. [View Abstract]
  13. Rusciani A, Motta A, Fino P, Menichini G. Treatment of poikiloderma of Civatte using intense pulsed light source: 7 years of experience. Dermatol Surg. 2008 Mar. 34(3):314-9; discussion 319. [View Abstract]
  14. Campolmi P, Bonan P, Cannarozzo G, Bruscino N, Troiano M, Prignano F, et al. Intense pulsed light in the treatment of non-aesthetic facial and neck vascular lesions: report of 85 cases. J Eur Acad Dermatol Venereol. 2011 Jan. 25(1):68-73. [View Abstract]
  15. Scattone L, de Avelar Alchorne MM, Michalany N, Miot HA, Higashi VS. Histopathologic changes induced by intense pulsed light in the treatment of poikiloderma of Civatte. Dermatol Surg. 2012 Jul. 38(7 Pt 1):1010-6. [View Abstract]
  16. Meijs MM, Blok FA, de Rie MA. Treatment of poikiloderma of Civatte with the pulsed dye laser: a series of patients with severe depigmentation. J Eur Acad Dermatol Venereol. 2006 Nov. 20(10):1248-51. [View Abstract]
  17. Clark RE, Jimenez-Acosta F. Poikiloderma of Civatte. Resolution after treatment with the pulsed dye laser. N C Med J. 1994 Jun. 55(6):234-5. [View Abstract]
  18. Geronemus R. Poikiloderma of civatte. Arch Dermatol. 1990 Apr. 126(4):547-8. [View Abstract]
  19. Haywood RM, Monk BE. Treatment of poikiloderma of Civatte with the pulsed dye laser: a series of seven cases. J Cutan Laser Ther. 1999 Jan. 1(1):45-8. [View Abstract]
  20. Batta K, Hindson C, Cotterill JA, Foulds IS. Treatment of poikiloderma of Civatte with the potassium titanyl phosphate (KTP) laser. Br J Dermatol. 1999 Jun. 140(6):1191-2. [View Abstract]
  21. Ross BS, Levine VJ, Ashinoff R. Laser treatment of acquired vascular lesions. Dermatol Clin. 1997 Jul. 15(3):385-96. [View Abstract]
  22. Behroozan DS, Goldberg LH, Glaich AS, Dai T, Friedman PM. Fractional photothermolysis for treatment of poikiloderma of civatte. Dermatol Surg. 2006 Feb. 32(2):298-301. [View Abstract]
  23. Tierney EP, Hanke CW. Review of the literature: Treatment of dyspigmentation with fractionated resurfacing. Dermatol Surg. 2010 Oct. 36(10):1499-508. [View Abstract]
  24. Tierney EP, Hanke CW. Treatment of Poikiloderma of Civatte with ablative fractional laser resurfacing: prospective study and review of the literature. J Drugs Dermatol. 2009 Jun. 8 (6):527-34. [View Abstract]

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

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