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Fifty-year-old twin sisters are seen for what appears to be the same problem: changes in the color and texture of their neck skin, recently noted by a family member. Both deny ever noticing it before and further deny experiencing any associated symptoms.
The sisters are accompanying their mother, who is being treated for basal cell carcinoma. All three women grew up living and working on the family farm, spending a great deal of time in the sun from an early age.
EXAMINATION
Both patients have type III skin, meaning that they seldom burn, tan fairly easily, and are able to keep that tan. The patients have identical, obvious hypo- and hyperpigmentation covering the sides of their faces and necks, sparing a well-defined oval area on the submental anterior neck.
The affected areas appear hyperemic, displaying a reddish brown tinge, but fail to blanch with digital pressure. The changes are completely macular.
What is the diagnosis?
DISCUSSION
The changes seen with poikiloderma of Civatte (PC) are obvious to the observer (see photograph) but appear so gradually that the patient often fails to notice them until they are pointed out. Both patients in this case almost certainly have had the condition for years.
PC was first described by Achille Civatte in France in 1923, around the same time that sunbathing became popular. Prior to that time, most women carefully protected their skin from the sun.
Civatte and others found that virtually all PC patients had a history of chronic overexposure to the sun, and that the clinical features of the condition—especially the characteristic sparing of the anterior neck skin (due to shading by the chin)—supported this hypothesis. They were also able to detect histologic changes (eg, solar elastosis) on biopsy, which further corroborated this theory.
There is little doubt that chronic overexposure to UV radiation is the cause, though other factors appear to be involved, as this case illustrates. For example, PC affects far more women than men, which suggests a possible role for hormones. It also appears that, in some cases, the tendency to develop PC is due to the genetic inheritance of an increased susceptibility to UV light. The mother of the two women in this case also had PC.
The range of presentations of PC includes involvement of the chest, face, and posterior neck. It should also be noted that somewhat similar patterns can be seen in other diseases, such as lupus, dermatomyositis, and early cutaneous T-cell lymphoma. Careful history-taking and biopsy, when indicated, will serve to distinguish between these diagnoses.
PC is usually left untreated, although lasers have been successfully used on motivated patients.
TAKE-HOME LEARNING POINTS
• Poikiloderma of Civatte (PC) is quite common and is seen more in women than men.
• Chronic overexposure to UV light is the primary cause of PC, but other factors—such as gender and heredity—appear to be involved.
• The term poikiloderma refers to the variability of the red, brown, and white color changes, as well as the solar atrophy seen with this condition.
• The differential for PC includes lupus, dermatomyositis, and early cutaneous T-cell lymphoma.
Fifty-year-old twin sisters are seen for what appears to be the same problem: changes in the color and texture of their neck skin, recently noted by a family member. Both deny ever noticing it before and further deny experiencing any associated symptoms.
The sisters are accompanying their mother, who is being treated for basal cell carcinoma. All three women grew up living and working on the family farm, spending a great deal of time in the sun from an early age.
EXAMINATION
Both patients have type III skin, meaning that they seldom burn, tan fairly easily, and are able to keep that tan. The patients have identical, obvious hypo- and hyperpigmentation covering the sides of their faces and necks, sparing a well-defined oval area on the submental anterior neck.
The affected areas appear hyperemic, displaying a reddish brown tinge, but fail to blanch with digital pressure. The changes are completely macular.
What is the diagnosis?
DISCUSSION
The changes seen with poikiloderma of Civatte (PC) are obvious to the observer (see photograph) but appear so gradually that the patient often fails to notice them until they are pointed out. Both patients in this case almost certainly have had the condition for years.
PC was first described by Achille Civatte in France in 1923, around the same time that sunbathing became popular. Prior to that time, most women carefully protected their skin from the sun.
Civatte and others found that virtually all PC patients had a history of chronic overexposure to the sun, and that the clinical features of the condition—especially the characteristic sparing of the anterior neck skin (due to shading by the chin)—supported this hypothesis. They were also able to detect histologic changes (eg, solar elastosis) on biopsy, which further corroborated this theory.
There is little doubt that chronic overexposure to UV radiation is the cause, though other factors appear to be involved, as this case illustrates. For example, PC affects far more women than men, which suggests a possible role for hormones. It also appears that, in some cases, the tendency to develop PC is due to the genetic inheritance of an increased susceptibility to UV light. The mother of the two women in this case also had PC.
The range of presentations of PC includes involvement of the chest, face, and posterior neck. It should also be noted that somewhat similar patterns can be seen in other diseases, such as lupus, dermatomyositis, and early cutaneous T-cell lymphoma. Careful history-taking and biopsy, when indicated, will serve to distinguish between these diagnoses.
PC is usually left untreated, although lasers have been successfully used on motivated patients.
TAKE-HOME LEARNING POINTS
• Poikiloderma of Civatte (PC) is quite common and is seen more in women than men.
• Chronic overexposure to UV light is the primary cause of PC, but other factors—such as gender and heredity—appear to be involved.
• The term poikiloderma refers to the variability of the red, brown, and white color changes, as well as the solar atrophy seen with this condition.
• The differential for PC includes lupus, dermatomyositis, and early cutaneous T-cell lymphoma.
Fifty-year-old twin sisters are seen for what appears to be the same problem: changes in the color and texture of their neck skin, recently noted by a family member. Both deny ever noticing it before and further deny experiencing any associated symptoms.
The sisters are accompanying their mother, who is being treated for basal cell carcinoma. All three women grew up living and working on the family farm, spending a great deal of time in the sun from an early age.
EXAMINATION
Both patients have type III skin, meaning that they seldom burn, tan fairly easily, and are able to keep that tan. The patients have identical, obvious hypo- and hyperpigmentation covering the sides of their faces and necks, sparing a well-defined oval area on the submental anterior neck.
The affected areas appear hyperemic, displaying a reddish brown tinge, but fail to blanch with digital pressure. The changes are completely macular.
What is the diagnosis?
DISCUSSION
The changes seen with poikiloderma of Civatte (PC) are obvious to the observer (see photograph) but appear so gradually that the patient often fails to notice them until they are pointed out. Both patients in this case almost certainly have had the condition for years.
PC was first described by Achille Civatte in France in 1923, around the same time that sunbathing became popular. Prior to that time, most women carefully protected their skin from the sun.
Civatte and others found that virtually all PC patients had a history of chronic overexposure to the sun, and that the clinical features of the condition—especially the characteristic sparing of the anterior neck skin (due to shading by the chin)—supported this hypothesis. They were also able to detect histologic changes (eg, solar elastosis) on biopsy, which further corroborated this theory.
There is little doubt that chronic overexposure to UV radiation is the cause, though other factors appear to be involved, as this case illustrates. For example, PC affects far more women than men, which suggests a possible role for hormones. It also appears that, in some cases, the tendency to develop PC is due to the genetic inheritance of an increased susceptibility to UV light. The mother of the two women in this case also had PC.
The range of presentations of PC includes involvement of the chest, face, and posterior neck. It should also be noted that somewhat similar patterns can be seen in other diseases, such as lupus, dermatomyositis, and early cutaneous T-cell lymphoma. Careful history-taking and biopsy, when indicated, will serve to distinguish between these diagnoses.
PC is usually left untreated, although lasers have been successfully used on motivated patients.
TAKE-HOME LEARNING POINTS
• Poikiloderma of Civatte (PC) is quite common and is seen more in women than men.
• Chronic overexposure to UV light is the primary cause of PC, but other factors—such as gender and heredity—appear to be involved.
• The term poikiloderma refers to the variability of the red, brown, and white color changes, as well as the solar atrophy seen with this condition.
• The differential for PC includes lupus, dermatomyositis, and early cutaneous T-cell lymphoma.