Pseudoatrophoderma Colli 

Updated: Aug 31, 2018
Author: Anna Choczaj-Kukula, MD, PhD; Chief Editor: Dirk M Elston, MD 

Overview

Background

Pseudoatrophoderma colli is an unusual persistent dermatosis characterized by the presence of pigmented macules and plaques with a wrinkled atrophic appearance, involving the neck and the upper part of the trunk.[1]

Becker and Muir[2] reported the first case of pseudoatrophoderma colli in 1934. Since then, only single cases of this condition have been described; however, pseudoatrophoderma colli is likely more common than has been reported.[3, 4]

Pathophysiology

The nature of the disease remains uncertain. Some authors suggest that vitamin A deficiency may be a contributing factor to the cause of this condition. In the past, pseudoatrophoderma colli was considered a subvariety or a transitional form of confluent and reticulate papillomatosis of Gougerot and Carteaud,[5, 6] or a variant of parapsoriasis. Reports of familial cases of pseudoatrophoderma colli support the theory that it is an autosomal dominant dermatosis and a single disease entity.[7, 8]

Etiology

The cause of the condition is unknown.

Vitamin A deficiency has been believed to be a possible factor contributing to the cause of pseudoatrophoderma colli.

Some authors previously regarded it as a form of parapsoriasis or an epidermal nevus.

Pseudoatrophoderma colli was also considered a variant of papillomatosis of Gougerot and Carteaud.

Epidemiology

Frequency

Pseudoatrophoderma colli is rare, with only 10-20 cases reported.

Race

No clear racial predilection is described.

Sex

All patients described in the literature, except one,[9] are females.

Age

The reported cases of pseudoatrophoderma colli are in patients aged 14-45 years.

Prognosis

Pseudoatrophoderma colli is a benign disease with no associated mortality or morbidity. The disease tends to spread gradually with partial regression and intervals, persisting for years or decades. Spontaneous clearing was reported in one patient.

 

Presentation

History

The atrophic-appearing macules and plaques usually appear insidiously and asymptomatically on the back of the neck. The appearance of the initial lesions, which tend to be hypopigmented and depressed, may resemble vitiligo.

The eruption slowly encircles the neck and gradually spreads to the scapular and chest region, involving the intermammary area. In some instances, pseudoatrophic plaques continue to spread over the trunk and occasionally appear in other regions, such as the buttocks, the arms, and the lower part of the abdomen.

The disease tends to spread gradually with partial regression and intervals, persisting for years or decades.

Spontaneous regression has been described in one patient.

Usually asymptomatic, it occasionally causes mild pruritus.

Physical Examination

Individual atrophic-appearing plaques and macules are 3-7 mm in diameter, oval or irregular, white or pink, and some coalesce in arcuate patches. The surface of the plaques may be shiny or wrinkled and may show some scaling. The lesions may form a dappled pattern of hypopigmented and hyperpigmented areas.

The eruption is ill defined, and the pseudoatrophic plaques tend to be arranged in a vertical direction. The pseudoatrophic plaques are most often situated on the neck and the upper part of the thorax.

 

DDx

 

Workup

Laboratory Studies

Laboratory test results are within the reference range.

Histologic Findings

Histologic examination may be helpful to exclude the possibility of atrophic lichen planus, lichen sclerosus, poikiloderma, or porokeratosis of Mibelli.

The histologic findings in pseudoatrophoderma colli include hyperkeratosis of the epidermis with occasional parakeratosis and thinning of the granular layer, acanthosis of some areas with vacuolation and pyknosis of prickle cells, dilatation of superficial blood vessels, and minimal perivascular lymphocytic infiltration in the dermis. Fragmentation of the elastic tissue has also been described in some patients. Mild atrophy of the epidermis was noted only in single cases.

 

Treatment

Medical Care

In the past, therapy for pseudoatrophoderma colli included the use of a quartz lamp, keratolytics, topical corticosteroids, superficial radiation, and oral vitamin A. Spontaneous clearing was reported in 1 patient. Kauh et al treated a patient with 5% lactic acid in hydrophilic ointment, with relief of itching and scaliness and a decrease in pigmentation.[8]

 

Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Keratolytics

Class Summary

These agents are used for symptomatic relief of dry scaling skin and for exfoliation.

Ammonium lactate 12% (Lac-Hydrin, AmLactin)

This is an alpha-hydroxy acid and a normal constituent of tissues and blood. It is believed to act as a humectant when applied to the skin. This may influence hydration of the stratum corneum. In addition, when applied to the skin, it may act to decrease corneocyte cohesion. The mechanisms by which this is accomplished are not yet known. It relieves itching and aids healing of skin in mild eczemas and dermatoses, itching skin, minor wounds, and minor skin irritations. It is found in a variety of topical emollient lotions. Ammonium lactate 5% lotion is available over the counter, and lactic acid 12% cream and lotion are available by prescription.