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Dermatology > PHOTO-RELATED DISEASES
Poikiloderma of Civatte
Article Last Updated: Jan 26, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: Lana H Hawayek, MD, Assistant Professor of Dermatology, University of Cincinnati, Veterans Affairs Medical Center; Consulting Staff, University Dermatology Consultants
Lana H Hawayek is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, and Women's Dermatologic Society
Coauthor(s):
Nelly Rubeiz, MD, Consulting Staff, Department of Dermatology, American University of Beirut Medical Center; Associate Professor, Department of Dermatology, American University of Beirut, Lebanon
Editors: Craig A Elmets, MD, Director of Dermatology, Departments of Dermatology, Pathology, and Environmental Health Sciences; Professor, The Kirklin Clinic, University of Alabama at Birmingham; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
Berkshire neck, mottled pigmentation on the neck, poikilodermatous lesions
Background
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.
Pathophysiology
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.
Frequency
International
The incidence of poikiloderma of Civatte is unknown; many patients may have a mild form of the disease and may not seek medical attention.
Mortality/Morbidity
In most patients, the condition is of purely cosmetic concern. Patients with the mild form do not seek medical advice.
Race
Poikiloderma of Civatte occurs most commonly in fair-skinned individuals.
Sex
Poikiloderma of Civatte occurs more commonly in females than in males.
Age
Most commonly, individuals affected are middle-aged or elderly women; however, the disease has been seen in other age groups.
History
- 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.
Physical
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
The etiology of poikiloderma of Civatte remains unknown; many factors have been implicated.
- 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. 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. The genetically determined predisposition may be expressed as an increased susceptibility of the skin to ultraviolet radiation.
Berloque Dermatitis
Bloom Syndrome (Congenital Telangiectatic Erythema)
Cutaneous T-Cell Lymphoma
Dermatomyositis
Dyskeratosis Congenita
Lupus Erythematosus, Acute
Parapsoriasis
Riehl Melanosis
Rothmund-Thomson Syndrome
Lab Studies
- Antinuclear and Anti-Ro (SS-A) antibody levels may be ordered to exclude connective tissue disease.
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.
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.
Surgical Care
- Intense pulsed light systems have been found beneficial in the treatment of poikiloderma of Civatte. 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. These light sources are reported to be effective in reducing both the pigmentation and telangiectasias seen in persons with poikiloderma of Civatte, with a low-risk profile.
- Erythema and telangiectasias in persons with poikiloderma of Civatte respond well to the flashlamp-pumped pulsed-dye laser (585 nm and 595 nm) and to the potassium-titanyl-phosphate laser. Caution must be exercised because of the higher incidence of adverse effects, such as hypopigmentation and scarring, especially on the neck and chest.
- Attempts to correct the disorder using electrosurgery, cryotherapy, and argon laser have been unsuccessful.
- Recently, use of fractional photothermolysis (laser technology that creates microthermal injury zones in skin) to treat poikiloderma of Civatte was described in one case report, with promising results. Further studies are needed to better elucidate this modality's efficacy and safety in the treatment of this condition.
Deterrence/Prevention
- Avoiding perfumes and using proper photoprotection are advocated.
Prognosis
- Poikiloderma of Civatte is a chronic skin condition, but the discomfort is solely cosmetic in most patients.
Patient Education
- Instruct patients to avoid sun exposure and to use sunscreens.
- Instruct patients to avoid perfumes and cosmetics.
Medical/Legal Pitfalls
- Failure to diagnose poikiloderma of Civatte, which may result in incorrect diagnosis of another type of poikiloderma such as mycosis fungoides or dermatomyositis. This may result in unnecessary tests or treatment.
| Media file 1:
Poikiloderma of Civatte over the neck. Notice sparing of the area under the chin. Courtesy of Dr. Shukrallah Zaynoun. |
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Media type: Photo
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Poikiloderma of Civatte excerpt Article Last Updated: Jan 26, 2007
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