Congenital Hemangioma with Milialike Structures: A Case Report

Pediatric Dermatology Vol. 15 No. 4 307-308, 1998

Congenital Hemangioma with Milialike Structures: A Case Report Alicia Rositto, M.D.,* Silvia Avila, M.D.,* Claudia Carames, M.D.,* and Ricardo Drut, M.D., C.C.P.M.? Departments of *Dermatology and fPathology, Hospital de Niiios ‘Superiora Sor Maria Ludovica, ’ La Plata, Argentina

Abstract:

A 4-month-old infant had two 3 cm x 4 cm hemangiomatous lesions on the scalp and back, present since birth, which contained peculiar white-yellowish small nodules. Histologically the lesions proved to have a hemangiomatous (capillary-type) component together with small keratin-containing epidermal cysts (milialike) which progressively extruded their contents. The lesions also contained embryonic-like hair structures. The hemangiomas resolved spontaneously. We were unable to find any reference in the literature describing this peculiar combination of features.

CASE REPORT A prematurely born (32 weeks gestational age; birth weight 940 g), 4-month-old male infant was referred for evaluation of two enlarging vascular tumors located on the scalp and back, respectively (Figs. 1 and 2). Each of the lesions measured 3 cm x 4 cm. Clinically they resembled common superficial hemangiomas. They presented as reddish exophytic masses with an irregular, lobulated surface (Figs. 1 and 2). However, a peculiar feature also noticed in both lesions was the presence of dilated follicular infundibula containing white to yellowish material, resembling open comedones. These small, rye seedlike structures could be appreciated through the hemangiomatous skin and proved firm on palpation. The “nodules” were noted to extrude their contents in concert with the spontaneous involution of the hemangiomas. The patient was otherwise in good health. HISTOPATHOLOGY The two biopsy specimens obtained at different times revealed hyperkeratotic epidermis and a dermis contain-

ing numerous small capillaries with plump endothelial cells surrounding cystic, milialike structures lined by keratinizing squamous epithelium. The cysts contained laminated squames in an onion-skin pattern. Also noted outside the cysts were poorly developed, embryonic-like hair structures resembling those found in the nevoid basal cell carcinoma syndrome (Fig. 3 ) .

DISCUSSION Hemangiomas are benign vascular tumors common in childhood. Most have a predictable course without complications, ending in spontaneous involution ( 1 4 ) . The most common complications which may appear during the course are ulceration, bleeding, and infection ( 1 4 ) . The peculiar combination of features found in our patient appears not to have been reported previously ( 1 4 ) . The lesions of our patient may represent the combination of dermal (hemangioma) and adnexal (abortive hair follicle) hamartomatous structures in which milialike cysts were particularly evident. The possibility that the keratin-

Address correspondence to Ricardo Drut, Servicio de Patologia, Hospital de Nifios, 1900 La Plata, Argentina, or e-mail: patologi@ netverk.com.ar.

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Figure 2. The lesion localized on the back. Figure 1. The scalp lesion has dilated infundibula and yellow-whitish small macules representing the milialike cysts.

a

b Figure 3. (a) Composite figure shows superficial epidermis with hyperkeratosis, numerous small capillaries in the dermis and a poorly developed hair structure ending in a keratin-containing, milialike infundibular cyst. (Hematoxylin and eosin; magnification 40x.) (b) Another area of one of the lesions where numerous small capillaries surround an immature hair follicle. (Hematoxylin and eosin; magnification 1OOx.)

containing cysts represented secondary milia due to the exuberant dermal capillary hemangiomatous component impinging upon the developing hair infundibula was also considered, since one of the lesions was located in the scalp. But the fact that the hemangioma located on the back showed similar clinical and histopathologic findings makes it unlikely. This patient appears to have an unusual type of hemangiomatous lesion for which we propose the name of congenital hemangioma with milialike structures.

REFERENCES 1. Mulliken JB, Young AE. Vascular birthmarks: hemangio-

mas and malformations. Philadelphia: WB Saunders, 1988: 41-75. 2. Esterly NB. Hemangiomas in infants and children: clinical observations. Pediatr Dermatol 1992;9:353-355. 3. Enjolras 0, Mulliken JB. The current management of vascular birthmarks. Pediatr Dermatol 1993;10:311-333. 4. Esterly NB. Cutaneous hemangiomas, vascular stains and malformations, and associated syndromes. Curr Probl Dermatol I995;3:69-107.