Yonago Acta medica 2013;56:59–61

Patient Report

Bilateral Nevus Comedonicus Syndrome
Takamichi Ito,*† Yasutaka Mitamura,† Yayoi Tsuji,† Kayo Harada† and Kazunori Urabe†*Division of Dermatology, Department of Medicine of Sensory and Motor Organs, School of Medicine, Tottori University Facultyof Medicine, Yonago 683-8503, Japan and †Department of Dermatology, National Hospital Organization Kyushu Medical Center,
Fukuoka 810-0065, Japan

fices filled with keratinous material.1 In some cases, nevus comedonicus is complicated with other conditions,such as cataracts, skeletal defects, central nervous system abnormalities or various other non-cutaneous conditions (nevus comedonicus syndrome).2 Although mostcases of nevus comedonicus occur unilaterally on theface, neck and chest, they occasionally show a bilateral
distribution (bilateral nevus comedonicus).3 We report
here an unusual case of bilaterally disseminated nevus
comedonicus with various systemic complications.

ABSTRACT
Nevus comedonicus is an uncommon skin abnormality
characterized by an aggregation of dilated follicular orifices filled with keratinous material. Nevus comedonicus
is occasionally complicated with other conditions including cataracts, skeletal defects, central nervous systemabnormalities or other extra-cutaneous diseases (nevus
comedonicus syndrome). Although most cases of nevus
comedonicus occur unilaterally on the face, neck andchest, the lesions occasionally show a bilateral distribution (bilateral nevus comedonicus). We report here an
unusual case of bilaterally disseminated nevus comedonicus with various systemic complications. A 62-yearold Japanese man presented with a 50-year history ofnumerous keratotic papules and comedo-like lesions,which gradually worsened with time. Physical examination revealed that the papules were skin-colored and 1–4mm in diameter. Some papules had dark-black keratinous materials on their surface, giving them a comedolike appearance. The lesions were located predominantly
on the face, head, neck and trunk with symmetric distribution, following Blaschko’s lines. The patient’s past
medical history was noteworthy; he had undergone surgical treatments for thyroid cancer, pneumothorax andschwannoma in the cauda equina. He also suffered fromscoliosis, cervical spondylosis and atrial fibrillation. Histopathologic examination revealed dilated and invaginated follicular structures filled with lamellar keratin,compatible with nevus comedonicus. Our patient’s case
did not fit with any previously reported diseases, andwe thought a diagnosis of “bilateral nevus comedonicus
syndrome” was the most appropriate for our patient’s
condition. He was treated with topical retinoic acid andactivated vitamin D3 ointment for 3 months each, but thelesions remained unchanged.

PATIENT REPORT

A 62-year-old Japanese man was referred to our hospitalwith a 50-year history of numerous keratotic papules
and comedo-like lesions, which gradually worsenedwith time. Physical examination revealed that the papules were skin-colored and 1–4 mm in diameter. Somepapules had dark-black keratinous materials on their surface, giving them a comedo-like appearance. The lesions
were located predominantly on the face, head, neck andtrunk with symmetric distribution, following Blaschko’slines (Fig. 1). Extremities, including hands, feet andnails, as well as mucous membranes were spared. The
patient did not complain of any symptoms. Family history and routine laboratory studies were unremarkable,
while his past medical history was noteworthy; he had
undergone surgical treatments for thyroid cancer, pneumothorax and schwannoma in the cauda equina. He has
also suffered from scoliosis, cervical spondylosis andatrial fibrillation. We performed punch biopsy of the lesion on the neck. Histopathologic examination revealed
dilated and invaginated follicular structures filled withlamellar keratin, in accordance with the features of nevus comedonicus (Fig. 2). Based on these findings, wethought a diagnosis of “bilateral nevus comedonicus
syndrome” was the most appropriate for our patient’s
condition. He was treated with topical retinoic acid andactivated vitamin D3 ointment for 3 months each, but thelesions remained unchanged.

Key words nevus comedonicus; nevus comedonicus
syndrome; schwannoma; thyroid tumor

Nevus comedonicus is an uncommon skin abnormality
characterized by an aggregation of dilated follicular oriCorresponding author: Takamichi Ito, MDtakamiti@dermatol.med.kyushu-u.ac.jp
Received 2013 February 25
Accepted 2013 March 459

DISCUSSION
Since the first description by Kofmann,1 about 200
cases of nevus comedonicus have been reported, thoughbilateral nevus comedonicus and nevus comedonicus

T. Ito et al.

syndrome have been reported only sporadically. Our patient’s case is of interest in thatit showed bilaterally disseminated comedolike lesions with various systemic complications. The skin lesions were clinically andhistopathologically compatible with bilateralnevus comedonicus. To our knowledge,
bilateral nevus comedonicus accompanied
by systemic complications as found in ourpatient has not been reported in the English
literature.
To make a diagnosis of “bilateral nevus
comedonicus syndrome”, we should rule out
other diseases showing multiple comedo- Fig. 1. Many skin-colored papules and comedo-like lesions on the neck.
like lesions or skin-colored papules such asacne vulgaris, chloroacne, Favre-Racouchot
disease, familial dyskeratotic comedones,
idiopathic disseminated comedones, disseminated congenital comedones, multiple
congenital comedones, verruca vulgaris andApert syndrome.
Acne vulgaris is a common disease thatshould be considered as a differential diagnosis. The diagnosis of acne vulgaris is usually
made from the clinical finding of a mixtureof acne lesions such as comedones, papules,pustules, nodules and hypertrophic scars.4
Fig. 2. Dilated and invaginated follicular structures filled with lamellar keratin (heIt is seen primarily in adolescents and usu- matoxylin and eosin staining). Bar = 1000 µm.
ally lacks systemic complications. Therefore,
acne vulgaris is not the perfect diagnosis forour patient. Chloracne, caused by halogenated chemicalcase report of this disease so far. Disseminated congenicompounds, is a well-known dermatosis consisting oftal comedones was described by Galvan in a 3-monthdiffuse acneiform lesions with diffuse grayish skin pigold boy who had congenital and extensive skin comedomentation,5 but our patient had no history of exposure
nes confined to the upper half of the body.9 Our case canto halogenated chemical compounds or other acnegenic
be differentiated from this disease because of the distrisubstances. Favre-Racouchot disease is a cutaneous disbution and onset age of comedones. Multiple congenital
6order with grouped comedones in sun-damaged skin.
comedones is a recently described condition showing
congenital bilateral comedones and some systemic
The lesions are usually symmetrically distributed withcomplications.10 Our case does not fit with this diseaseout developing inflammation, but the locations of thisdisease are confined to sun-exposure areas. Familial dysbecause of the onset of comedones. Verruca vulgaris
keratotic comedones is a rare abnormality characterized
sometimes occurs as multiple skin-colored lesions,11by symmetrically scattered comedo-like hyperkeratotic
but this disease can be clearly discriminated from ours
papules.7 It is inherited as an autosomal dominant condihistopathologically. Apert syndrome, characterized bycraniosynostosis, mid-facial malformations, symmetrition, which can distinguish it from our case. Idiopathic
cal syndactyly and severe acne vulgaris, does not accord
disseminated comedones is a good candidate diagnosis
with the features of our patient.12 After excluding thefor our patient. It is a rare entity clinically characterized
by symmetrically scattered comedo-like hyperkeratotic
diseases mentioned above, we concluded that “bilateral
8papules on the trunk, arms and legs. Histopathologinevus comedonicus syndrome” is the most appropriate
term reflecting our patient’s condition.
cally, crateriform cysts containing hyperkeratotic debris
and peri-infundibular inflammatory infiltrate are seen.
The authors declare no conflict of interest.
These histopathologic features, however, were not evident in our patient. Moreover, there has been only one60

Bilateral nevus comedonicus syndrome
totic comedones. Arch Dermatol 1972;105:249-51. PMID:5060870.
8 Zhang RZ, Zhu WY. Idiopathic disseminated comedones in achild. Pediatr Dermatol 2006;23:163-6. PMID: 16650228.
9 Veno-Galvan S, Hernandez-Martin A, Colmenero I, TorreloA. Disseminated congenital comedones. Pediatr Dermatol
2011;28:58-9. PMID: 21276057.
10 Ito T, Yoshida Y, Furue M, Yamamoto O. Multiple congenital
comedones, hearing impairment and intellectual disability: anew syndromic association? Eur J Dermatol 2012;22:807-8.
PMID: 23114227.
11 Ito T, Yoshida Y, Adachi K, Furue M, Yamamoto O. Wartwith depigmented halo and generalized vitiligo. Yonago Acta
Medica 2012;55:81-2.
12 Harper JI, Trembath RC. Genetics and genodermatoses. In:Burns T, Bresthnach S, Cox N, Griffiths C, editors. Rook’s
Textbook of Dermatology. Massachusetts: Blackwell Publishing; 2004. p. 12.75

References
1 Kofmann S. Ein fall von seltener localisation und verbreitungvon komedonen. Arch Dermatol Syphilol 1895;32:177.
2 Engber PB. The nevus comedonicus syndrome: a case reportwith emphasis on associated internal manifestations. Int J
Dermatol 1978;17:745-9. PMID: 730460.
3 Paige TN, Mendelson CG. Bilateral nevus comedonicus. Arch
Dermatol 1967;96:172-5. PMID: 6043616.
4 Thiboutot DM, Stauss JS. Diseases of the sebaceous glands.
In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith
LA, Katz SI, editors. Fitzpatrick’s Dermatology in GeneralMedicine. New York: McGraw-Hill; 2003. p. 672-87.
5 Passarini B, Infusino SD, Kasapi E. Chloracne: Still cause forconcern. Dermatology 2010;221:63-70. PMID: 20516651.
6 Patterson WM, Fox MD, Schwartz RA. Favre-Racouchot disease. Int J Dermatol 2004;43:167-9. PMID: 15009383.
7 Caneiro SJC, Dickson JE, Knox JM. Familial dyskera-61