circles of perifollicular hyperpigmentation (figure 1E).
References
1. Duarte BMF, Pinheiro R, Cabete J. Multiple miliary osteoma cutis:
a comprehensive review and update of the literature. Eur J Dermatol
2018; 28: 435-40.
2. Novak C, Siller G, Wood D. Idiopathic multiple miliary osteomas
of the face. Australas J Dermatol 1998; 39: 109-11.
3. Chabra IS, Obagi S. Evaluation and management of multiple mil-
iary osteoma cutis: case series of 11 patients and literature review.
Dermatol Surg 2014; 40: 66-8.
4. Di Zenzo G, Della Torre R, Zambruno G, Borradori L. Bullous pem-
phigoid: from the clinic to the bench. Clin Dermatol 2012; 30: 3-16.
5. Feliciani C, Joly P, Jonkman MF, et al. Management of bullous pem-
phigoid: the European Dermatology Forum consensus in collaboration
with the European Academy of Dermatology and Venereology. Br J
Dermatol 2015; 172: 867-77.
6. Cozzani E, Gasparini G, Di Zenzo G, Parodi A. IgE and bullous
pemphigoid. Eur J Dermatol 2018; 28: 441-9.
7. Hessam S, Gambichler T, Skrygan M, et al. Reduced ten-eleven
translocation and isocitrate dehydrogenase expression in inflammatory
hidradenitis suppurativa lesions. Eur J Dermatol 2018.
8. Zhang Q, Zhao K, Shen Q, et al. Tet2 is required to resolve
inflammation by recruiting Hdac2 to specifically repress IL-6. Nature
2015; 525: 389-93.
9. Figueroa ME, Abdel-Wahab O, Lu C, et al. Leukemic IDH1
and IDH2 mutations result in a hypermethylation phenotype, disrupt
TET2 function, and impair hematopoietic differentiation. Cancer Cell
2010; 18: 553-67.
doi:10.1684/ejd.2018.3384
And next. . . Adnexa: Ulerythema ophryo-
genes and keratosis pilaris
Virgínia COELHO DE SOUSA, Rita PINHEIRO,
Nélia CUNHA, André LENCASTRE, Joana CABETE
Department of Dermatology and Venereology, Hospital de Santo
António dos Capuchos - Centro Hospitalar de Lisboa Central, Alameda
de Santo António dos Capuchos, 1169-050 Lisbon, Portugal
e-mail: virginiacoelhodesousa@gmail.com
A 61-year-old female was referred to our clinic for an eval-
uation of persistent erythema and pruritus of the eyebrows
which was present since childhood. She had been previ-
ously diagnosed with eczema and lichen planopilaris, for
which she was prescribed tacrolimus 0.1% ointment and
latanoprost, without clinical improvement. The patient was
otherwise healthy with normal cognitive development. She
reported that her grandmother had similar skin lesions.
On examination, perifollicular erythema and partial
madarosis of the external aspects of the eyebrows were
present (figure 1A). Additionally, keratosis pilaris affect-
ing the upper extremities was observed, associated with a
reduction of body hair in this location (figure 1D). There
was no scalp alopecia or reduction of hair at other body sites.
Dermoscopic examination of the eyebrows revealed back-
ground erythema with some scattered fine linear vessels
and yellow-white dots located in the follicular openings,
surrounded by brown circles of perifollicular hyperpig-
mentation (figure 1B, C). Dermoscopic examination of the
forearm revealed similar features with yellow-white dots
located in the follicular openings, surrounded by brown
566
Considering the clinical and dermoscopic findings, together
with the onset of the skin changes in childhood, ulery-
thema ophryogenes (keratosis pilaris atrophicans faciei)
was the working diagnosis and punch biopsies from skin
of the eyebrow and arm were performed. Histopathologi-
cal examination revealed follicular atrophy, perifollicular
fibrosis, hyperkeratotic plugging of follicular openings, a
mild perivascular and perifollicular lymphocytic infiltrate,
and telangiectasia (figure 1F). The findings were similar
in the two biopsy specimens. With the clinicopathological
correlation, a diagnosis of keratosis pilaris atrophicans was
made. A further genetic study was performed revealing a
normal 46 XX karyotype. No other syndromic features were
present and a sporadic form of ulerythema ophryogenes was
assumed. Treatment with tretinoin 0.05% cream every other
day was initiated, without significant clinical response.
Keratosis pilaris (KP) is a common disorder of fol-
licular keratinization, characterized by keratinous plugs
obstructing the follicular openings, with varying degrees
of perifollicular erythema, exhibiting predilection for the
extensor surfaces of the extremities and gluteal region.
The face and trunk are less commonly affected [1, 2]. It
is a benign condition and especially common in child-
hood and adolescence. KP can occur alone, in otherwise
healthy individuals, or in association with atopic dermatitis
or ichthyosis vulgaris. Noonan syndrome and other geno-
dermatoses may also be associated with KP, especially the
atrophic variants [2, 3].
Ulerythema ophryogenes (UO), also known as keratosis
pilaris atrophicans faciei, is an atrophic variant of KP
affecting the eyebrows. The other disorders within the spec-
trum of atrophic KP are atrophoderma vermiculatum and
keratosis follicularis spinulosa decalvans. These three gen-
odermatoses are rare and may be associated with other
syndromes. UO is usually present from early infancy, firstly
affecting the lateral aspects of the eyebrows with follic-
ular papules surrounded by an erythematous halo. These
changes slowly progress to affect the entire eyebrow with
ensuing scarring madarosis [2]. Less frequently, the cheeks
and forehead are also involved. UO has been associated with
Noonan and cardiofasciocutaneous syndromes, as well as
Rubenstein-Taybi syndrome, Cornelia de Lange syndrome,
isolated woolly hair, and 18p deletion [2-9]. The associa-
tion between UO, KP, and monosomy 18p has been termed
“Zouboulis syndrome” [5-7]. In our case, a sporadic form
was assumed as the karyotype was normal and no other
findings suggestive of a genetic syndrome were present.
Other genetic defects that have not yet been clarified may
be associated with the disease [1, 6-8].
The clinical diagnosis of UO may be difficult to establish in
mild cases. The main histopathological findings consist of
follicular hyperkeratosis, follicular atrophy, dermal fibrosis,
and mild inflammation. Nevertheless, as observed in this
case, dermoscopy may aid the correct diagnosis. Despite
not being previously reported, the dermoscopic findings of
UO are similar to those of KP, except for the additional
presence of coiled vellus hairs in UO. This further supports
the utility of this non-invasive technique in the evaluation
of adnexal dermatoses [10].
Treatment of UO is usually ineffective, but topical salicylic-
acid 10%, tretinoin 0.05%-0.1% cream, and hydration with
urea-containing emollients may induce a partial clinical
response [2, 3]. 
EJD, vol. 28, n◦ 4, July-August 2018
 A B C

D E

Figure 1. A) Perifollicular erythema and partial madarosis affecting predominantly the external aspects of the eyebrows. B, C)
Dermoscopy of ulerythema ophryogenes; note the yellow-white dots in the follicular openings with brown circles of perifollicular
hyperpigmentation (black circles) and areas of erythematous background with scattered fine linear vessels (arrows). D) Keratosis
pilaris affecting the upper extremities, causing a reduction of body hair at this location. E) Dermoscopy of keratosis pilaris
in the forearm; note the yellow-white dots in the follicular openings, with brown circles of perifollicular hyperpigmentation.
F) Histopathological examination reveals follicular atrophy, perifollicular fibrosis, ostial hyperkeratosis, discrete inflammatory
changes, and telangiectasia (H&E; 40×).

6. Liakou A, Esteves de Carvalho AV, Nazarenko LP. Trias of kerato-

References
1. Hwang S, Schwartz RA. Keratosis pilaris: a common follicular
hyperkeratosis. Cutis 2008; 82: 177-80.
2. Mirmirani P, Rogers M. Keratosis pilaris and other inflammatory
follicular keratotic syndromes. In: Fitzpatrick’s Dermatology in General
Medicine. 8th edition. Goldsmith LA, Katz SI, Gilchrest BA, Paller AS,
Leffell DJ, Wolff K. McGraw-Hill Medical, 2012: 973-5.
3. Chien AJ, Valentine MC, Sybert VP. Hereditary woolly hair and
keratosis pilaris. J Am Acad Dermatol 2006; 54: S35-9.
4. Neild VS, Pegum JS, Wells RS. The association of keratosis pilaris
atrophicans and wolly hair, with or without Noonan’s syndrome. Br J
Dermatol 1984; 110: 357-62.
5. Zouboulis CC, Stratakis CA, Rinck G, Wegner RD, Gollnick H,
Orfanos CE. Ulerythema ophryogenes and keratosis pilaris in a child
with monosomy 18p. Pediatr Dermatol 1994; 11: 172-5.
sis pilaris, ulerythema ophryogenes and 18p monosomy: Zouboulis
syndrome. J Dermatol 2014; 41: 371-6.
7. Nazarenko SA, Ostroverkhova NV, Vasiljeva EO, et al. Keratosis
pilaris and ulerythema ophryogenes associated with an 18p dele-
tion caused by a Y18 translocation. AM J Med Genet 1999; 85:
179-82.
8. Gómez Centeno P, Rosón E, Peteiro C, Mercedes Pereiro M, Toribio
J. Rubinstein-Taybi syndrome and ulerythema ophryogenes in a 9-year-
old boy. Pediatr Dermatol 1999; 16: 134-6.
9. Flórez A, Fernández-Redondo V, Toribio J. Ulerythema ophryo-
genes in Cornelia de Lange syndrome. Pediatr Dermatol 2002; 19:
42-5. Not cited.
10. Panchaprateep R, Tanus A, Tosti A. Clinical, dermoscopic and
histopathological features of body hair disorders. J Am Acad Dermatol
2015; 72: 890-900. Not cited.
doi:10.1684/ejd.2018.3385

EJD, vol. 28, n◦ 4, July-August 2018 567