Información de la revista
Vol. 105. Núm. 1.
Páginas 85-86 (enero - febrero 2014)
Visitas
7337
Vol. 105. Núm. 1.
Páginas 85-86 (enero - febrero 2014)
Case for diagnosis
Acceso a texto completo
Nodular-Cystic Lesions on a Congenital Alopecic Plaque
Lesiones nódulo-quísticas sobre placa de alopecia congénita
Visitas
7337
V. Fuentelsaz-del Barrioa,
Autor para correspondencia
victoriafuentelsaz@hotmail.com

Corresponding author.
, V. Parra Blancob, D.E. Cieza Díaza
a Servicio de Dermatología, Hospital Gregorio Marañón, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital Gregorio Marañón, Madrid, Spain
Este artículo ha recibido
Información del artículo
Texto completo
Bibliografía
Descargar PDF
Estadísticas
Figuras (2)
Texto completo
Case Description

We present the case of a 40-year-old woman with no relevant medical history who presented with asymptomatic nodular-cystic lesions on a congenital alopecic plaque on the scalp. The lesions had developed slowly and progressively over the previous 5 years. The patient reported that her family physician had occasionally incised and drained some of the lesions. No cases of similar lesions were reported in her family history.

Physical Examination

We observed yellowish papules and skin-colored nodular-cystic lesions measuring 0.3 to 1cm in diameter that were soft on palpation and located on an alopecic plaque on the left frontotemporal region. The lesions extended to the left malar region (Fig. 1).

Figure 1
(0.12MB).
Histology

Several biopsies were taken from the area. Histologic findings revealed multilocular cystic epithelial tumors surrounded by a well-differentiated, flat epithelium with a crenulated lining that had an eosinophilic cuticle (Fig. 2, A and B). In addition, mature sebaceous glands were observed to be in close contact with the epithelium. Some of these glands opened into the lumen of the cyst.

Figure 2.

A, Hematoxylin-eosin, original magnification ×40. B, Hematoxylin-eosin, original magnification ×40.

(0.18MB).

What Is Your Diagnosis?

Diagnosis

Steatocystoma multiplex (SM) on a congenital alopecic plaque on the scalp

Clinical Course and Treatment

Simple excision was performed on several of the larger lesions, and although the esthetic outcome was favorable, the patient rejected further treatment.

Comment

SM is considered to result from hamartomatous malformations of the pilosebaceous unit. It is a rare condition that typically affects adolescents or young adults. Although it follows an autosomal dominant inheritance pattern, many sporadic cases have been described. Clinically, it consists of yellowish or skin-colored papules and nodules measuring 0.2 to 2cm in diameter that typically appear on the anterior aspect of the trunk and on the neck, axillas, thighs, and scrotum. Presentation on the scalp is rare and, unlike SM in more typical areas, it is not associated with an autosomal dominant inheritance pattern and appears in older individuals,1,2 as in our patient.

SM affecting the face and scalp has been classified into 3 types: a) a facial papular variant,3,4 b) sebocystomatosis,5 and c) lesions confined to the scalp.1,2,6,7

Only 12 cases of SM on the scalp have been described in the literature to date, and just 2 of these cases involved concurrent alopecic plaques. In 1996 Lee et al.5 reported a case of SM on the face and on a congenital alopecic plaque in the frontal region that they considered to be a clinical variant of this condition. The variant, which they called sebocystomatosis, had very similar clinical characteristics to those seen in our patient. The second published case of SM with concurrent alopecia on the scalp was described by Lee et al.8 in 2011. In that case the lesions appeared on an alopecic plaque caused by trichotillomania.

Some authors have described cases of SM associated with other disorders such as ichthyosis, koilonychia, pachyonychia congenita, acrokeratosis verruciformis of Hopf, hypertrophic lichen planus, hypohidrosis, hypothyroidism, hidradenitis suppurativa, and hypotrichosis.1 No associated conditions, however, have yet been described in patients with SM affecting the scalp only. Our patient had no other cutaneous manifestations or disorders.

Histologically, SM consists of cystic lesions that are empty, partially collapsed, folded, and covered with a wall formed by a stratified squamous epithelium lacking a granular layer and lined with a crenulated cuticle. Sebaceous gland lobules embedded in the cyst wall are also a very characteristic finding.

The clinical differential diagnosis typically includes eruptive vellus hair cysts, the histology of which shows cysts containing keratin and vellus hair surrounded by a thin wall comprised of several layers of squamous cells. As in SM, the sebaceous glands may form part of the cyst wall.

Other diagnoses to consider are epidermal cysts, milia cysts, neurofibromas, and xanthomas.

Although most SM lesions are asymptomatic, they may cause serious esthetic concern. Treatment with simple excision or drainage of cyst content may produce satisfactory results. In cases of inflamed lesions, treatment with locally injected corticosteroids, laser treatment, oral retinoids, and cryotherapy may be effective.

Conflicts of Interest

The authors declare that they have no conflicts of interest.

References
[1]
S.J. Kim, H.J. Park, S.T. Oh, J.Y. Lee, B.K. Cho.
A case of steatocystoma multiplex limited to the scalp.
Ann Dermatol, 21 (2009), pp. 106-109
[2]
S.Y. Jeong, J.H. Kim, S.H. Seo, S.W. Son, I.H. Kim.
Giant steatocystoma multiplex limited to the scalp.
Clin Exp Dermatol, 34 (2009), pp. 318-319
[3]
M. Nishimura, H. Kohda, A. Urabe.
Steatocystoma multiplex. A facial papular variant.
Arch Dermatol, 122 (1986), pp. 205-207
[4]
L. Requena, L. Martin, G. Renedo, D. Arias, M.L. Espinel, A. de Castro.
A facial variant of steatocystoma multiplex.
Cutis, 51 (1993), pp. 449-452
[5]
Y.J. Lee, S.H. Lee, S.K. Ahn.
Sebocystomatosis: a clinical variant of steatocystoma multiplex.
Int J Dermatol, 35 (1996), pp. 734-735
[6]
P. Ambrojo Antunez, A. Aguilar Martínez, E.F. Cogolludo, E. Sanchez Yus, F. Sanchez de Paz.
Esteatocistoma múltiple limitado a cuero cabelludo.
Actas Dermosifiliogr, 79 (1988), pp. 215-216
[7]
I. Belinchón, M.J. Mayol, J.A. Onrubia.
Steatocystoma multiplex confined to the scalp.
Int J Dermatol, 34 (1995), pp. 429-430
[8]
D. Lee, J.S. Chun, S.K. Hong, J.K. Seo, J.H. Choi, J.K. Koh, et al.
Steatocystoma multiplex confined to the scalp with concurrent alopecia.
Ann Dermatol, 23 (2011), pp. 258-260

Please cite this article as: Fuentelsaz-del Barrio V, Parra Blanco V, Cieza Díaz D. Lesiones nódulo-quísticas sobre placa de alopecia congénita. Actas Dermosifiliogr. 2014;105:85–86.

Copyright © 2013. Elsevier España, S.L. and AEDV
Descargar PDF
Idiomas
Actas Dermo-Sifiliográficas
Opciones de artículo
Herramientas
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?