Saturday, August 06, 2011

Congenital Hypopigmented Macules

Presented by Henry Foong, Ipoh, Malaysia
A healthy 16 year old girl complains of asymptomatic 1-2 mm in diameter hypopigmented macules on both shins since birth. There are similar, but to a lesser extent, macules on the arms. Her elder sister has similar lesions. In an older individual with later onset I would have thought of idiopathic guttate hypomelanosis. However, these lesions were congenital and her sister is similarly affected. It does not look like a form of dyschromia but plain hypopigmentation. so unlikely to be dyschromatosis symmetrical hereditaria? or dyschromia cutis amyloidosis? Does not look like pigmentary mosaicism either. Any suggestions? Click images to enlarge.

Impression: Congenital Hypopigmented Macules. Has anyone seen a similar case?

1. Fukai K, Monozygotic twins with congenital guttate leukoderma. Osaka City Med J. 2005 Jun;51(1):33-6.
Abstract: We report here two cases of congenital guttate hypomelanotic macules observed in monozygotic twins. They both have had discrete leukoderma regions in the axillae, inguinal region and lower abdomen since birth. The size and the shape did not change until at least the age of nine. Development of both patients was otherwise normal. The split-DOPA reaction revealed no DOPA-positive melanocytes in the hypomelanotic skin, but electron microscopy revealed melanocytes that were regular but decreased in number. Cytogenetic analysis of the peripheral leukocytes revealed normal female karyotype in both cases. Considering the unique pattern of the leukoderma lesions which occurred in both monozygotic twins, this might be a new clinical entity.

2. Grosshans E, Sengel D, Heid E. White lentiginosis [ in French] Ann Dermatol Venereol. 1994;121(1):7-10.
INTRODUCTION: A congenital guttate hypomelanosis is an unusual feature not yet mentioned in the dermatologic literature.
CASE REPORT: We observed 1982 in a 28 y. female patient numerous guttate lesions, which were flat and pigmented on the light-exposed areas of her limbs, flat or papulokeratotic and depigmented on her trunk. These lesions disclosed a particular histological aspect characterized by a lentiginous hyperplasia of the epidermis, with elongated club-shaped rete ridges, and an unusual loss of pigmentation without disturbance of the keratinization. Further electronmicroscopical and immunohistochemical data were not available. The patient emphasized the congenital occurrence of these lesions, whose fixity could be assessed during a 4 year-follow up time.
COMMENTS: The unusual histological aspect allows the differentiation of these depigmented spots and other known similar conditions: macular leucoderma as sequellae of previous inflammatory diseases, hypomelanotic macules associated with genodermatoses, idiopathic guttate hypomelanoses.
CONCLUSION: This seems to be a not yet described entity which we propose to denominate "white lentiginosis".

1 comment:

  1. From Amit Pandya: "The lesions look like idiopathic guttate hypomelanosis to me. I have seen extensive cases in teenagers and patients in their 20’s occasionally, especially those who have had a lot of sun damage. These patients sometimes have a family history of such lesions, however, I haven’t seen it in a child. Any history of exposure to arsenic? Perhaps living in an area where arsenic is present in the drinking water? Treatment is very difficult. I have had success with light cryotherapy for patients with a few lesions but this would not be practical for this patient. I have heard of success with light dermabrasion but have not tried it myself."


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