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Annular atrophic plaques of skin (Christianson's Disease)

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Annular atrophic plaques of skin (Christianson's Disease)
Rajeev Sharma MD1, Mithilesh Chandra MD2
Dermatology Online Journal 9 (1): 11

Consultant 1. Dermatologist, Bishen Skin Centre, Aligarh India, 2. Consultant Histopathologist, Tissue Path, Noida India

Abstract

Atrophic plaques with white borders are occasionally seen on sun-exposed areas of the skin. These patients are usually elderly and have solar elastosis. This condition is referred to as annular atrophic plaques of skin and we describe a typical case.



Introduction

Annular atrophic plaques of the skin is an uncommon condition described in 1967 by Christianson [1, 2]. The initial lesions, which generally occur in sun-exposed areas, are small atrophic macules that develop insidiously and gradually enlarge to become annular with either atrophic or normal looking skin in the center. The advancing margins remain white and become sclerotic with the passage of time. Large plaques are formed by coalescence of the smaller lesions. Most of the cases are reported from the western world [3-9]. We report a case of Christianson¹s disease in a sixty- eight-year-old Indian farmer.


Case report


Figure 1Figure 2
Figure 1. Annular atrophic plaque on the dorsum of the foot with raised borders.
Figure 2. Close up of a lesion on foot.

A 68-year-old farmer presented with multiple, asymptotic, gradually enlarging lesions on the limbs, which had been present for about 10 years. Initially they were small but gradually increased in size over the last 4 years. He was otherwise healthy, and review of the systems was not suggestive of any underlying systemic disease.


Figure 3Figure 4
Figure 3. Annular lesions on the hands.
Figure 4. Similar lesion on the knee and hand.

Cutaneous examination revealed lesions on hands, forearms, knees, legs, and dorsa of the feet. The lesions varied from 1.2 to 4 cm in size, had thickened margins, and exhibited central atrophic or normal looking skin. All lesions were present on the sun-exposed areas only.


Figure 5Figure 6
Figure 5. Sclerotic appearance of the superficial and mid-dermal collagen (H & E x 40).
Figure 6. Broken wavy elastic fibers (H & E x 120).

Skin biopsies were taken from the edge and the center of the lesion. Histologic examination revealed hyperkeratosis at the edges and mild atrophy of the epidermis in the center. There was edema of the upper dermis. Fragmentation of the elastic fibers in the upper dermis, collagenization of the dermis, and atrophy of the appendages were present. Hydropic degeneration of the basal cell layer was also present. A mild periadenexal mononuclear infiltrate was observed. Direct immunofluorescence was negative.

The patient was treated with clobetasol propionate ointment (0.05 percent) under occlusion without any benefit. Similar results were obtained with intralesional injections of Triamcinalone acetonide.


Discussion

Christianson [1, 2] described annular, atrophic plaques in two farmers who initially developed small, white, atrophic macules. The onset was insidious and the lesions gradually progressed, enlarged, and became annular, exhibiting centers with either atrophic or normal appearing skin. Despite progression of the lesions, the advancing edge remained white and became more sclerotic with the passage of time. Most of the lesions were found on sun-exposed skin and larger plaques were formed by the coalescence of smaller lesions. Clinical features of morphoea and/or lichen sclerosus were seen in some of the the lesions. There were no signs of systemic disease in these patients and there was no response to various treatment modalities, as we also observed.[2]

Histology varies with the duration of the lesion.[2] The degree of sclerosis at the edge becomes prominent with the passage of time. The melanin content of the basal cell layer in the sclerotic zone is decreased and the central area shows atrophy of the epidermis and appendages. Most biopsies display elastosis.

Similar patients have been described earlier by others.[3-8] Most of the cases described had lesions limited to the face and hence the condition was referred to as annular, atrophic plaques of the face. We agree with the contention of Ramos-Caro et al. that the lesions are not limited to the face and can involve any sun-exposed area, as in our case.[9] The sites of involvement will vary according to the dress code of the region. Farmers in the northern part of India wear dhoti, a three-meter long cloth that is usually white in color. It is tied around the waist, passed between the legs, and tucked in at the back. It leaves the legs exposed up to the lower third of the thighs. This type of dress explains the occurrence of the lesions on the knees in our case.

Some authors have proposed that the condition is a variant of either lichen sclerosus, [3,7] discoid lupus erythematosus,[6] lichen planus,[7] or actinic granuloma.[8] It is possible that in some patients, annular, atrophic plaques may be an end-stage of one of these conditions.

References

1. Christianson HB, Pasarell R. Unusual manifestations of scleroderma. Cutis 1967;3:855-60.

2. Christianson HB, Mitchell WT. Annular atrophic plaques of the face. A clinical and histologic study. Arch Dermatol 1969;100:703-16.

3. Stevanovic DV. Annular, en bande, and plaque, lichen sclerosus. Arch Dermatol 1971;103:226-7.

4. Kalsbeck GL. Annular atrophic plaques of the face. Dermatologica 1971;143:246-7.

5. Jablonska S, Chorezelski T, MazurKiewiez W. Annular atrophic plaques of the face. An entity or an annular atrophic discoid lupus erythematosus. Dermatologica 1974;149:379-84.

6. Chorzelski TP, Jablonska S, Blaszyczyk M, Fabjanska L. Annular atrophic plaques of the face. A variety of atrophic discoid lupus erythematosus? Arch Dermatol 1976;112(8):1143-5. PubMed

7. Patel RI, Reed WB. Annular atrophic plaques of the face and upper body. An unusual variant of lichen sclerosus et atrophicus or lichen planus. Cutis 1979;24(1):90-3. PubMed

8. Ramos-Caro FA, Podnos S, Ford M, Mullins D, Flowers FP. Annular atrophic plaques of the skin (Christianson's disease). Int J Dermatol 1997;36(7):518-21. PubMed

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