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Acrokeratoelastoidosis treated with etretinate S. Handfield-Jones, M.R.C.P. , and C. T. C. Kennedy, F.R.C.P. Bristol, England

Acrokeratoelastoidosis is a chronic condition manifested by acral hyperkeratotic papules. There have been no reports of effective treatment. We report a case that responded to etretinate. (J AM ACAD DERMATOL 1987;17:881-2.)

Acrokeratoelastoidosis was first described by Costa at the Brazilian Society of Dermatology in 1952 and reported in 1953. l Since then, three clin- icopathologic entities have been described. Costa's cases fit in a group of familial early-onset acral papules that histologically show fragmentation of dermal elastin fibers. A second group is of late onset and is associated with solar damage and trauma. The third group, into which our patient falls, is clinically identical with the first group but

From the Department of Dermatology, Bristol Royal Infirmary.

Reprint requests to: Dr. S. Handfield-Jov.es, Registrar in Dermatol- ogy, Bristol Royal Infirmary, Bristol 1352 8HW, England.

lacks histologic features of elastorrhexis. 2'3 An al- ternative name, "focal acral hyperkeratosis," has been suggested. 4

There are no reports of response to treatment in the literature.

CASE REPORT

A 47-year-old Jamaican woman developed hyper- pigmented, umbilicated papules on the pa]mar-dorsal borders of her hands and fingers (Fig. I), the edges of her feet, and the anterior surface of her wrists. She first noticed them in her teens. In addition, she had increas- ing thickening and pigmentation over the knuckles and dorsa of the feet. Palms and soles were normal.

General health was good, and there was no known

Fig. 1. Before treatment, keratotic papules and pigmentation are evident.

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American Academy of Dermatology

Fig. 2. Improvement after 6 months of treatment with etretinate.

family history. She found the appearance distressing and before referral had visited many practitioners. She had received treatment, including parenteral methyl- prednisolone and homeopathic remedies, with no effect.

Histologic examination of a palmar papule showed marked orthokeratotic hyperkeratosis with a normal dermis. Elastin stains showed uniform fibers with no evidence of elastorrhexis.

The patient was postmenopausal, and plasma lipids were normal. She was started on a regimen of 50 mg etretinate daily. She noticed improvement after a month, and by 6 months there was definite flattening of the papules and a decrease in abnormal pigmentation (Fig. 2).

When the dose of etretinate was reduced to 25 mg daily, the skin worsened slightly. The patient stopped treatment 2 months ago, and the lesions have recurred, although they remain less prominent than before etre- tinate was started.

DISCUSSION

Acrokeratoelas toidosis withou.t elastorrhexis ap- pears to be a distinct clinical entity. Dowd 4 re- ported 15 patients in whom histologic examinat ion showed no evidence of elastorrhexis. In common with our patient, all these patients were nonwhi te and showed onset in chi ldhood or early adult life.

Autosomal dominant inheritance was suggested by one family group, but 12 patients had no family history.

Our patient responded well to etretinate. Ob- viously for the majority of patients the condition is only a minor nuisance, but for those who are sufficiently distressed, systemic retinoids can be helpful.

A D D E N D U M

Since submission of this article, we have treated an additional patient. She is a 49-year-old white woman with a 10-year history of skin-colored papules on her hands and feet. Histologic examination of a lesion from the hand showed results similar to those obtained with the reported patient: hyperkeratosis without elastor- rhexis. Treatment with etretinate, 25 mg daily, led to a marked improvement after 3 months.

REFERENCES

1. Costa OG. Acrokeratoelastoidosis. Dermatologica 1953;107:164-8.

2. Highet AS, Rook A, Anderson JR. Acrokeratoelas- toidosis. Br J Dermatol 1982;106:337-44.

3. Korc A, Hansen RC, Lynch pJ. Acrokeratoelastoidosis of Costa in North America. J AM ACAD DERMATOL 1985; 12:832-6.

4. Dowd PM, Harman RRM, Black MM. Focal acral hy- perkeratosis. Br J Dermatol 1983;109:97-103.