British Journal of Dermatology (1975} 92, 711.

Milia occurring in lichen sclerosus et atrophicus BARBARA LEPPARD* AND I.B.SNEDDON Rupert Hallam Department of Dermatology, Sheffield Accepted for publication 20 September 1974


Large numbers of milia were seen in areas of skin affected with buUae in a 68-year-old woman who had extensive lichen sclerosus et atrophicus. Although milia arc frequently seen in other sub-epidermal bullous disorders, they have not previously been reported in lichen sclerosus et atrophicus.

Lichen sclerosus et atrophicus is an uncommon condition of the skin which predominantly affects the genital region of middle-aged and elderly women. The skin of the trunk and limbs may also be affected with small white macular or atrophic plaques. Occasionally the lesions may spread rapidly to involve up to half the body surface. Bullous lesions are often seen histologically, but less frequently on clinical examination. A patient was seen with extensive lichen sclerosus et atrophicus and is being reported because there were several unusual features, including milia formation. CASE REPORT

A 68-year-old Caucasian female first noticed white patches on her wrists at the age of 63 years. They were symptomless, but gradually spread to involve the skin of her neck, trunk (Fig. i), upper arms and thighs. She was not aware of any abnormality in the perineum and had never had pruritus vulvae or ani. On examination the individual lesions were characteristic of lichen sclerosus et atrophicus with tiny white porcelain-coloured macules and larger plaques which, in places, coalesced to produce large semitranslucent areas. Over the whole of her lower back the skin appeared to be thickened and could be moved over the underlying structures. In two areas on her back, examination showed a large number of milia within the area affected by the bullae (Fig. 2). Elsewhere there was no evidence of hyperkeratosis or of follicular plugging. The vulva and perianal skin showed the characteristic white pallor of lichen sclerosus et atrophicus with some telangiectasia and atrophy. Two biopsies were taken from the patient's back. The first was through the edge of a typical lesion of lichen sclerosus et atrophicus and showed an atrophic epidermis with some liquefaction degeneration of the basal cells, homogenization of the collagen in the upper dermis and sub-epidermal bulla formation. There were a few inflammatory cells below the homogeneous band. The second biopsy, taken from an area of milia formation, showed several small keratinizing cysts in the upper dermis, * Present address and address for reprints: St John's Hospital for Diseases of the Skin, Lisle Street, Leicester Square, London WC2H 7BJ. 711


Barbara Leppard and I.B.Sneddon

FIGURE I. Patient's back showing extent of lichen sclerosus et atrophicus.

FIGURE 2. Close up of patient's back showing bullous area and large numbers of milia (arrowed).

MHia with lichen sclerostis et atrophicus






FIGURE 3. Histological features. Hyjicrkeratosis, sub-epidermal bulla formation and small keratinizing cyst in the upper dermis (H & E, x 100).

unconnected to any adnexal structure or the overlying epidermis (Fig. 3), in addition to the other histological changes of lichen sclerosus et atrophicus. further investigation After this patient had been seen, histology sections from all the patients diagnosed as having lichen sclerosus et atrophicus and attending St John's Hospital for Diseases of the Skin during the last 10 years were examined. Eighty-eight sections from thirty-three patients were reviewed and those of five patients (15%) showed milia formation. DISCUSSION

The usual sites to be involved with lichen sclerosus et atrophicus are the neck, upper trunk, flexor aspects of wrists and the ano-genital area (Kindler, 1953), and this patient showed extensive changes in these areas. The unusual features were, first, the rapid spread of the lesions to involve more than half the body surface in less than 5 years; second, the large areas of bulla formation on her back; and of third, the presence of milia. In his long review, Wallace (1971) reported rapid spread of the lesions in only six (I'S/'o) of his 396 patients. The appearance of sub-epidermal bullae is a well recognized histological feature, but is less often observed on clinical examination. However, the patient reported by Myers & Dowling {1936) showed a very similar clinical picture to that seen in our patient, because he had a type of flat bulla with a thick horny roof One of the characteristic histological findings in lichen sclerosus et atrophicus is diffuse hyperkeratosis with plugging of the hair follicles and sweat duct orifices^ but milia formation has not been described. Because milia are known to occur as secondary phenomena in other skin disorders which produce sub-epidermal bullae, such as pemphigoid, dystrophic cpidermolysis bullosa and porphyria cutanea tarda, it would be unusual if they did not occur in lichen sclerosus et atrophicus. A milium may develop


Barbara Leppard and LB.Sneddon

from any epithelial structure and on serial sections can usually be seen 10 be connected by a cord of undifferentiated epithelial cells to the parent structure whether this be hair follicle, sweat duct, sebaceous duct or epidermis (Epstein & Kligman, 1956). Any condition which produces damage to the adnexal structures may invoke milium formation. In long-standing lichen sclerosus et atrophicus there may be atrophy of the hair follicles and sebaceous glands, although the sweat glands often remain intact. It is probable that the milia in this case were derived from the eccrine sweat ducts, for example, if these were ruptured when blistering occurred. Alternatively they could be produced by a mechanism similar to that which occurs in the type of hairless mice which have disappearing hair follicles. Montagna, Chase & Melaragno (1952) described a breed of homogeneous autosomal recessive hairless mice which grow a normal first pelage, but lose this by spontaneous epilation after the first few weeks of life. Why this happens is not known but once the hair is lost spherical epithelial cysts are formed in the dermis, and become larger and more numerous so that they eventually obliterate the dermis and adipose layer. Whatever the cause, it is surprising that the occurrence of milia has not been described before in lichen sclerosus et atrophicus. It may be that they have been present but not noted in the past and that a careful clinical and histological examination will show that they are not infrequently present. REFERENCES EPSTEIN, W.& KLIGMAN, A.M. (1956) The pathogenesis of milia and benign tumours of the skin.7'o«ma/o//MoejM"garivc Dermatology, 26, i.

KINDLER, T . (1953) Lichen sclerosus et atrophicus in young subjects. British Jmtrtial of Dermaiolagy, 65, 269. MONTAGNA, W., CHASE, H.B.& MELAR/XGNO. H. (1952) The skin of hairless mice. I. The formation of cysts and the distribution of lipids. Journal of Invesiigative Dermawlogy, 19, S3. MYERS, W.K.& DOWLING, G.B. (1936) Lichen sclerosus. British Journal of Dermalology, 48, 658. WALLACE, HJ. (1971J Lichen sclerosus et atrophicus. Transactiom of Si. Johi's Hospiiat Dermaiological Society 57.9.

Milia occurring in lichen sclerosus et atrophicus.

Large numbers of milia were seen in areas of skin affected with bullae in a 68-year-old woman who had extensive lichen sclerosus et atrophicus. Althou...
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