Pseudomonilethrix type II and pili bifurcati - Test Page for Apache

4 Beasley KL, Cooley GE, Kao GF, Lowitt MH, Burnett JW,
Aurelian L. Herpes simplex vegetans: atypical genital
herpes infection in a patient with common variable
immunodeficiency. J Am Acad Dermatol 1997; 37: 860–863.
5 Samaratunga H, Weedon D, Musgrave N, McCallum N.
Atypical presentation of herpes simplex (chronic
hypertrophic herpes) in a patient with HIV infection.
Pathology 2001; 33: 532–535.
6 Fangman WL, Rao CH, Myers SA. Hypertrophic herpes
simplex virus in HIV patients. J Drugs Dermatol 2003; 2:
198–201.
7 Fox PA, Barton SE, Francis N et al. Chronic erosive herpes
simplex virus infection of the penis, a possible immune
reconstitution disease. HIV Med 1999; 1: 10–18.
8 Fife KH, Crumpacker CS, Mertz GJ, Hill EL, Boone GS.
Recurrence and resistance patterns of herpes simplex virus
following cessation of > or = 6 years of chronic suppression
with acyclovir. Acyclovir Study Group. J Infect Dis 1994; 169:
1338–1341.
9 Pechère M, Wunderli W, Trellu-Toutous L, Harms M, Saurat
JH, Krischer J. Treatment of acyclovir-resistant herpetic
ulceration with topical foscarnet and antiviral sensitivity
analysis. Dermatology 1998; 197: 278–280.
10 Bevilacqua F, Marcello A, Toni M et al. Acyclovir resistance/
susceptibility in herpes simplex virus type 2 sequential
isolates from an AIDS patient. J Acquir Immune Defic Syndr
1991; 4: 967–969.
DOI: 10.1111/j.1468-3083.2006.01561.x
? 18 ?Letter Letters LETTERS 2006to
to the TO the Editor THE Editor EDITOR
Pseudomonilethrix type II and pili
bifurcati
Editor
We present the case of a 2-year-old boy who was brought
to our unit for evaluation of his hair problem. The hair,
especially at the occipital and temporal areas of the head,
was dry, thin and very fragile, in the context of a generalized
hypotrichosis (fig. 1), the length of the hairs was not more
than 1.5 cm. The physical and psychological development
of the boy was within normal limits. Both parents had
normal hair and the personal and family history did not
have associated diseases. We could not appreciate keratosis
pilaris at the scalp, nape of the neck, or extensor surfaces
of the upper arm and thigh. Laboratory test including
blood count test, general biochemistry, urinary test,
immunoglobulins, thyroid profile and autoantibodies
showed no abnormalities. Microscopic examination of
hair showed that the hair shaft has spindle-shaped swellings
by constricted internodes of normal appearance. In our
case pili bifurcati was associated, instead of pili torti (figs 2
and 3). Pseudomonilethrix type II was diagnosed. We
started treatment with minoxidil 2% 1 mL/day, and advised
fig. 1 Generalized hypotrichosis.
fig. 2 Microscopic examination: pseudomoniletrix.
fig. 3 Microscopic examination: pili bifurcati.
Letters to the Editor
the parents to avoid any trauma of the hair with great
improvement in 3 months.
Pseudomonilethrix is a rare dysplastic disorder of the
hair shaft that is inherited as an autosomal dominant
JEADV 2006, 20, 868–902 © 2006 European Academy of Dermatology and Venereology 889

Letters to the Editor
trait with high penetrance but variable expressivity. It was
first described by Bentley-Phillips and Bayles 1 in 1973 and
clinically affects the whole scalp as a generalized hypotrichosis
or just the occipital area. Pseudomonilethrix usually
starts in the first months of life, although, in some
cases, it does not become apparent until childhood. No
follicular papules can be seen on physical examination. 2
Pseudomonilethrix has been classiffied in three different
types: I, familiar pseudomonilethrix of Bentley-Phillips
(autosomal dominant inheritance); II, acquired pseudomonilethrix
in dysplastic disorders with hair fragility
(inheritance profile depending on the dysplastic disorder);
and III, iatrogenic pseudomonilethrix. 3
Diagnosis can be easily made by microscopic examination
of the hair shaft. Elliptical nodes form along the hair shaft
at variable intervals and separated by narrower internodes.
Differential diagnosis with monilethrix is obligated.
Monilethrix is an autosomal dominant disorder characterized
by a beaded appearance of the hair due to periodic
thinning of the shaft. 4 The phenotype results in hair fragility
and patchy dystrophic alopecia. Keratosis pilaris
is almost invariably associated with monilethrix unlike
pseudomonilethrix. Concomitant ectodermal defects
have been reported in the literature as dental abnormalities,
juvenile cataracts, and some neurologic defects that
appear as a cognitive deficiency or epilepsy. Pili torti or
partial forms of trichorrhexis nodosa have been associated
with the second type of this hair disorder. There are
no previous reports of pili bifurcati and pseudomonilethrix
in the literature, as the clinical case that we
present. The avoidance of trauma is the most effective
method of managing this anomaly. 5
R Ruiz-Villaverde,† A Villanova-Mateu,‡
R Ortega del Olmo,‡ D Sanchez-Cano§
†Dermatology Unit, Hospital de Poniente, El Ejido, Almería,
†Dermatology Unit, Hospital Clínico Universitario de Granada,
and ‡Internal Medicine, Hospital Clínico Universitario de
Granada, Granada, Spain, *Corresponding author, Lopez Font
n°10 5°A4, 18004-Granada, Spain,
E-mail: ismenios@hotmail.com
References
1 Bentley-Phillips B, Bayles MA. A previously undescribed
hereditary hair anomaly pseudo-monilethrix. Br J Dermatol
1973; 89: 159–167.
2 Rogers M. Hair shaft abnormalities: Part II. Australas J
Dermatol 1996; 37: 1–11.
3 Ferrando J, Fontarnau R, Haussman G. Is pseudomonilethrix
an artifact? Int J Dermatol 1990; 29: 380–381.
4 Camacho-Martinez F, Ferrando J. Hair shaft dysplasias. Int J
Dermatol 1988; 27: 71–80.
5 Sotiriou E, Sotiriadis D. Monilethrix. Eur J Pediatr Dermatol
2002; 12: 101–104.
DOI: 10.1111/j.1468-3083.2006.01564.x
? 18 ?Letter Letters LETTERS 2006to
to the TO the Editor THE Editor EDITOR
Adult-onset Langerhans cell
histiocytosis confined to the skin
Editor
Langerhans cell histiocytosis (LCH) is a clonal, pleomorphic
disease of unknown aetiology, with the
accumulation of local or disseminated atypical histiocytic
cells staining positively for S-100 and CD-1a, and causing
damage in the bones, lungs, mucocutaneous structures
and endocrine organs. 1 The condition is generally diagnosed
in infancy and childhood, but onset in adulthood
can occasionally occur. The eruption is usually diffuse or
manifests as part of a multisystemic disease, and up to 25–
50% of patients with LCH will present initially with a
cutaneous rash. 1–5 Cutaneous manifestations are heterogenous
and similar both in children and adults. 1,2 LCH
confined to the skin is uncommon among reported cases
of adults. 2–8 We describe a rare case of adult-onset LCH
presenting as cutaneous lesions located symmetrically on
the scalp, groin and inframammarian areas, and review the
literature for previous adult cases of isolated cutaneous
LCH.
A 38-year-old woman presented to our dermatology
department with a-two-year duration of red, painful skin
ulcerations in the inframammary and inguinal areas
located bilaterally, and severe crusting and scaling of the
scalp. Skin examination showed diffuse yellowish crusting
with erosions involving the scalp and extending beyond
the hairline onto the forehead (fig. 1) and symmetrically
distributed sharp-edged, ulcerated plaques with a small
fig. 1 Erythematous, erosive, ulcerated plaques on right inframammarian
fold.
890 JEADV 2006, 20, 868–902 © 2006 European Academy of Dermatology and Venereology