Dermatologia revista edicion ingles 20110121.indd

137 Pages·2011·5.16 MB·English

ISSN 1515-8411 (Graphical version) - ISSN 1669-1636 (Online version)
Special Edition | Volume XV | 2009
Volumen XV | Noviembre-Diciembre 2009 | Número 6
Research Articles Original Articles
Retrospective study of Mucous membrane Unilateral nevoid Subcutaneous fat necrosis of
dermatomyositis. Discussion pemphigoid: our 14 years’ telangiectasia. Report of four the newborn, report of fi ve
of 40 cases consulting a experience cases cases
Dermatology Department M. del P. Beruschi, M. Bolatti, M. Arias, R. González, R. Retamar, M. Larralde, M. E. Abad, C. Corbella,
M. L. Hassan, A. Capecce, M. E. Melloni, S. Marinescu, C. Ardissone, L. López, M. C. López Santoro, M. Demarchi, C. A. Ferrari, R. Plafnik
M. Saposnik, I. Ruzzi, J. Gramajo, J. O. Zárate, E. Zeitlin, A. Kaminsky, M. C. Kien, E. Chouela
J. Lorenzo, R. Schroh, C. Corbella E. Dancziger Periorifi cial dermatitis in
HTLV-1 -associated infective children
Epidemiological study of Susceptibility of diff erent dermatitis in an adult M. Larralde, M. E. Abad, P. Luna,
basal cell carcinoma in a mouse strains to Leishmania L. Olivares, G. D’Atri, C. Martinetti, R. Plafnik, B. Pagotto
community hospital amazonensis infection L. Ciccia, O. Forero, J. Anaya
V. M. González, S. P. Busso, R. Leitner, M. A. Falú, M. F. García Bustos, Rare tumors of diffi cult initial
G. Casas, M. Larralde C. M. Parodi Ramoneda, E. Molina de Verrucous melanoma: diagnosis
Raspi, R. M. Cardozo, R. Cimino,
diff erences and similarities M. I. Sanz, D. Feinsilber, C. Corbella,
J. F. Gil, J. L. Vasvari, M. A. Basombrío
Cutaneous metastasis of between primary and R. Schröh, M. L. Hassan
internal carcinomas, our secondary varieties
Psoriasis: comorbidities in
experience in 94 cases D. Feinsilber, N. Kogan, O. M. Rosati, Vulvar squamous cell
our community
R. Azcune, M. G. Spelta, J. Moya, C. Corbella, R. Schröh, I. Calb carcinoma. Presentation of
M. M. Lustia, P. C. Luna, M. J. Nocito,
M. L. Lado Jurjo, M. I. Fontana, 28 patients
M. J. Soutelo, M. L. Castellanos Posse,
A. M. Barbarulo, S. Gavazza, M. Barrera, C. Marchesi, N. G. Cañadas, R. Garuti, Multiple miliary osteoma of V. Parra, F. Flores, C. Sánchez de
S. M. Ortega, S. Martínez, S. Vanzulli, M. Á. Mazzini the face. Three cases treated Giménez, P. Daguerre, V. García Llaver,
E. Zeitlin F. Galdeano, N. Driban
with carbon dioxide laser
Primary vasculitis in children: R. A. Retamar, M. I. Hernández, V. Battista,
Reactional leprosy D. Kaplan, G. Giavino, M. C. Kien, Hydroa vacciniforme-like
A clinical-epidemiological
L. M. Olivares, G. E. A. Pizzariello, G. Pellerano, E. N. Chouela cutaneous T/NK-cell
G. D’Atri, A. Martínez, C. Casas, study
lymphoma, nasal type
M. B. Jalo, G. Rodríguez Prados M. F. Stringa, C. Castro, A. D. Olivera,
P. Bonavía, O. J. Stringa, R. Valdez Pyoderma gangrenosum M. Dacal, E. Formentini, A. Vaccarezza,
associated with ulcerative A. Arra, L. Gagliardi
Cutaneous manifestations of
acromegaly Palmoplantar melanocytic colitis treated with
M. Dahbar, K. Danilowicz, M. Malavela, nevi: dermoscopic and Infl iximab Merkel cell carcinoma. Study
D. Velásquez, M. Allevato, H. Cabrera, histopathological correlation D. Malieni, A. C. Torre, M. C. Baztán, of fi ve cases
O. D. Bruno M. A. Barengo, M. P. Gutiérrez, C. Anselmi, R. Galimberti N. G. Cañadas, P. C. Luna, M. J. Nocito,
M. M. Lustia, M. D. Etcheverry,
E. Valente, A. Ruiz Lascano
M. L. Castellanos Posse, C. Marchesi,
Cutaneous manifestations in HLA patients with an
R. A. Garuti, G. Carabajal, M. Á. Mazzini
alcoholic patients. Relation association of familial
with cirrhosis lupus-psoriasis
Y. Murga, V. Parra, A. Aredes, B. A. Paniagua, M. E. Fiad,
M. Alasino, A. Torres, L. Santolín, A. S. Quenardelle, M. F. Giménez,
S. Salomón, J. Carena P. M. F. Motta, A. M. Habegger de
Sorrentino
www.dermatolarg.org.ar
ISSN 1515-8411 (Graphical version) - ISSN 1669-1636 (Online version)
Publication of the Sociedad Argentina de Dermatología
Editor Viviana Parra Brazil Mexico
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Myriam Alejandra Dahbar France
Cristina Echeverría Hassan, Mercedes Belaich, Stéphane
María Inés Hernández Jaimovich, León Civatte, Jean United States of America
Viviana Leiro Kaminsky, Ana Ortonne, Jean Bergfeld, Wilma
Andrea Santos Muñoz Stringa, Sergio Revuz, Jean Eaglestein, William
Viglioglia, Pablo Katz, Stephen
Sociedad Argentina Germany Kopf, Alfred
de Dermatología Australia Czarnetzki, Beate Price, Vera
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Austria
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Board of Directors Sangüeza, Martín Tosti, Antonella Macedo, Néstor
Roberto Glorio Sangüeza, Pastor Vignale, Raúl
Summary
Vol XV, January-February 2009, Nº 1 Research Article
044
Cutaneous metastasis of internal
Research Article
carcinomas, our experience in 94 cases
007 R. Azcune, M. G. Spelta, J. Moya, M. L. Lado Jurjo, M. I. Fontana,
Retrospective study of dermatomyositis.
A. M. Barbarulo, S. Gavazza, M. Barrera, S. M. Ortega, S. Martínez,
Discussion of 40 cases consulting a Dermatology
S. Vanzulli, E. Zeitlin
Department
M. L. Hassan, A. Capecce, M. E. Melloni, M. Saposnik, I. Ruzzi,
Research Article
J. Gramajo, J. Lorenzo, R. Schröh, C. Corbella
052
Reactional leprosy
Research Article
L. M. Olivares, G. E. A. Pizzariello, G. D’Atri, A. Martínez, C. Casas,
017 M. B. Jalo, G. Rodríguez Prados
Epidemiological study of basal cell carcinoma
in a community hospital
Vol XV, May-June 2009, Nº 3
V. M. González, S. P. Busso, R. Leitner, G. Casas, M. Larralde
Original Article Research Article
024 058
Unilateral nevoid telangiectasia. Report Cutaneous manifestations of acromegaly
of four cases M. Dahbar, K. Danilowicz, M. Malavela, D. Velásquez, M. Allevato,
M. Arias, R. González, R. Retamar, M. C. López Santoro, H. Cabrera, O. D. Bruno
M. Demarchi, M. C. Kien, E. Chouela
Original Article
Original Article
063
Pyoderma gangrenosum associated with
029
HTLV-1 - associated infective dermatitis ulcerative colitis treated with infl iximab
in an adult D. Malieni, A. C. Torre, M. C. Baztán, C. Anselmi, R. Galimberti
L. Olivares, G. D’Atri, C. Martinetti, L. Ciccia, O. Forero, J. Anaya
Original Article
Vol XV, March-April 2009, Nº 2
068
HLA patients with an association of familial
Original Article lupus-psoriasis
B. Á. Paniagua, M. E. Fiad, A. S. Quenardelle, M. F. Giménez,
034
Verrucous melanoma: diff erences P. M. Fabiana Motta, A. M. Habegger de Sorrentino
and similarities between primary
and secondary varieties Original Article
D. Feinsilber, N. Kogan, O. M. Rosati, C. Corbella, R. Schröh, I. Calb
072
Subcutaneous fat necrosis of the newborn,
report of fi ve cases
Original Article
M. Larralde, M. E. Abad, C. Corbella, C. A. Ferrari, R. Plafnik
039
Multiple miliary osteoma of the face. Three

cases treated with carbon dioxide laser
R. A. Retamar, M. I. Hernández, V. Battista, D. Kaplan, G. Giavino,
M. C. Kien, G. Pellerano, E. N. Chouela
6 | Sumario
Vol XV, July-August 2009, Nº 4 Original Article
109
Vulvar squamous cell carcinoma.
Research Article
Presentation of 28 patients
077 V. Parra, F. Flores, C. Sánchez de Giménez, P. Daguerre, V.
Cutaneous manifestations in alcoholic
García Llaver, F. Galdeano, N. Driban
patients. Relation with cirrhosis
Y. Murga, V. Parra, A. Aredes, M. Alasino, A. Torres, L. Santolín,
S. Salomón, J. Carena Original Article
114
Research Article Hydroa vacciniforme-like cutaneous
T/NK-cell lymphoma, nasal type
082
Mucous membrane pemphigoid: our 14 M. Dacal, E. Formentini, A. Vaccarezza, A. Arra, L. Gagliardi
years’ experience
M. del P. Beruschi, M. Bolatti, S. Marinescu, C. Ardissone, Vol XV, November-December 2009, Nº 6
L. López, J. O. Zárate, E. Zeitlin, A. Kaminsky, E. Dancziger
Research Article
Original Article
118
089 Primary vasculitis in children:
Periorifi cial dermatitis in children
A clinical-epidemiological study
M. Larralde, M. E. Abad, P. Luna, R. Plafnik, B. Pagot
M. F. Stringa, C. Castro, A. D. Olivera, P. Bonavía, O. J. Stringa,
R. Valdez
Original Article
094 Research Article
Rare tumors of diffi cult initial diagnosis
M. I. Sanz, D. Feinsilber, C. Corbella, R. Schröh, M. L. Hassan
127
Palmoplantar melanocytic nevi:
dermoscopic and histopathological
Vol XV, September-October 2009, Nº 5
correlation
M. A. Barengo, M. P. Gutiérrez, E. Valente, A. Ruiz Lascano
Research Article
Original Article
100
Susceptibility of diff erent mouse strains
135
to Leishmania amazonensis infection Merkel cell carcinoma. Study of fi ve cases
M. A. Falú, M. F. García Bustos, C. M. Parodi Ramoneda, N. G. Cañadas, P. C. Luna, M. J. Nocito, M. M. Lustia,
E. Molina de Raspi, R. Marino Cardozo, R. Cimino, M. D. Etcheverry, M. L. Castellanos Posse, C. Marchesi,
J. Fernando Gil, J. L. Vasvari, M. Á. Basombrío R. A. Garuti, G. Carabajal, M. Á. Mazzini

Research Article
106
Psoriasis: comorbidities in our community
M. M. Lustia, P. C. Luna, M. J. Nocito, M. J. Soutelo,
M. L. Castellanos Posse, C. Marchesi, N. G. Cañadas,
R. Garuti, M. Á. Mazzini
Research Article
Retrospective study of dermatomyositis. Discussion
of 40 cases consulting a Dermatology Department
Mercedes Lidia Hassan1, Ana Capecce2, María E. Melloni2, Miriam Saposnik2, Ivana Ruzzi3, Josefi na Gramajo4, Juana Lorenzo5,
Roberto Schröh6, Cristina Corbella7
Abstract
Background. Dermatomyositis (DM) is included in idiopathic infl ammatory miopathies, together with polymyositis, although clinical variants with-
out muscle involvement are recognized, exclusively showing cutaneous involvement during variable periods of time, or remaining defi nitely amyo-
pathic. Cutaneous manifestations are so characteristic that a diagnosis may be made even before any muscular involvement occurs. Association to in-
ternal malignancy, organ involvement, and evolution possibilities arouse interest of physcicians and dermatologists.
Material and methods. We retrospectively studied 40 patients (33 females) consecutively seen at the Dermatology Department of Hospital Ramos
Mejía of Buenos Aires between June 1991, and June 2007.
Objective. To analyse this population composition, cutaneous and systemic involvement characteristics, and disease evolution.
Results. The following was found: 23 percent of amyopathic forms (9 of 40), 16.6 percent associated with internal malignancy (6/36), excluding four
cases of childhood/juvenile DM. Malignancies were exclusively gynecological: breast (3), cervix (2), and ovary (1). Lapse between diagnosis of cancer
and DM was from 0 to 2 years.Infrequent skin lesions and complications of these patients were described, in addition to currently accepted cutane-
ous criteria for the diagnosis of dermatomyositis. No “fl agellated” erythema lesions or complete antisynthetase syndrome were found. Unsuspected
interstitial lung disease with simple chest X-ray was detected in two cases, both non-positive for anti-Jo1 antibodies. One case of this series appeared
three years after silicone mammary implant as amyopathic dermatomyositis (ADM). One of 4 deaths was caused by breast cancer metastasis and an-
other by aspiration pneumonia with pharyngeal muscle involvement; in the other two cases the cause was not related to DM.
Conclusions. The analysis of the observed cases enables us to understand data about this population composition (prevalence of ADM, prevalence of
internal malignancy, negative results for anti-Jo1 antibodies), to include unusual skin aspects, and to detect unsuspected respiratory diseases requiring
special studies for early diagnosis. The lack of previous original reported series of DM in our country is highlighted (Dermatol Argent 2009;15(1):27-36).
Key words: dermatomyositis, polymiositis.
Introduction
Dermatomyositis (DM) is included in idiopathic infl am-
matory myopathies, together with polymyositis (PMs),1-6
although exclusively cutaneous clinical forms without muscu-
lar involvement are recognized for a variable period of time,
and even defi nitely amyopathic (ADM) forms.7-15
It is characterized by the involvement of upper limb proxi-
mal striated muscle and neck fl exor muscles, with or without
Reception date: 12/8/08 | Approval date: 11/9/08 dysphagia, and involvement of respiratory muscles. Th e as-
sociation with neoplasias and involvement of internal organs
1. Named Professor UBA, and Head of Dermatology Department.
arouse interest in clinical physicians and dermatologists; howe-
2. Medical Dermatologist of Collagenopathy Sector.
3. Medical Resident, 2nd year, Specialist Course UBA. ver, the latter rarely see any form of PM.
4. Attending Physician, 2nd year, Specialist Course UBA.
5. Medical Course Physician, 1st year, Specialist Course UBA.
Bohan and Sontheimer classifi cations
6. Staff Physician, in charge of Pathology Sector.
7. Staff Physician, Histopathology. DM diagnosis criteria in Bohan’s classification (Table 1)
Dermatology Department. Hospital “J. M. Ramos Mejía”, Associated to Universidad de do not include patient subgroups with exclusively or predo-
Buenos Aires. Autonomous City of Buenos Aires. Argentine Republic.
minantly cutaneous lesions;8,13 therefore, a broader patho-
logical category has been suggested, designated as “idio-
Correspondence
pathic inflammatory dermatomyopathies”14,15 (Table 2).
Mercedes Lidia Hassan. Arcos 2273 1º B, (1428) Autonomous City of Buenos Aires, Ar-
gentine Republic. | E-mail: [email protected] According to Sontheimer, the adult-onset DM forms encompass:
8 | M. L. Hassan, A. Capecce, M. E. Melloni, M. Saposnik, I. Ruzzi, J. Gramajo, J. Lorenzo, R. Schröh, C. Corbella
ABBREVIATIONS TABLE 1. BOHAN’S CLASSIFICATION OF POLYMYOSITIS/DERMATOMYOSITIS.4
LAA: leukocytoclastic allergic angiitis. • Clinical subgroups
ACL: anticardiolipins. º Polymyositis.
CPK: creatine phosphokinase. º Dermatomyositis.
DM: dermatomyositis. º Polymyositis or dermatomyositis associated with malignancy.
ADM: amyopathic dermatomyositis. º Childhood dermatomyositis.
EMG: electromyogram. º Polymyositis or dermatomyositis with an associated connective tissue disorder.
FAN: antinuclear antibodies. • Diagnostic criteria5
TNF: tumor necrosis factor. º Typical skin rash.
IFN: interferon. º Symmetrical proximal muscle weakness with or without dysphagia or respi-
ILD: interstitial lung disease. ratory involvement.
LDH: lactic dehydrogenase. º EMG compatible with primary muscular involvement.
MTS: metastasis. º Alterations in muscle biopsy compatible with primary muscular involvement.
PM: polymyositis. º Elevation of muscle enzimes (creatin phosphokinase [CPK], glutamic oxala-
NMR: nuclear magnetic resonance. cetic transaminase [GOT], lactic dehydrogenase [LDH], aldolase).
Rx: X-ray. Confi dence limits for diagnosis of dermatomyositis:
CAT: computed axial tomography. Defi nite DM: rash and three of the four other diagnostic criteria.
HRCT: high resolution computed tomography. Probable DM: rash and two of the four other diagnostic criteria.
GOT: glutamic-oxalacetic transaminase. Possible DM: rash and one of the four other diagnostic criteria.
Classic DM. It shows the two cutaneous and muscular com-
TABLE 2. GLOBAL CLASSIFICATION OF IDIOPATHIC INFLAMMATORY DERMA-
ponents simultaneously or with a lapse not exceeding 6 TOMYOPATHIES (SONTHEIMER 2002).1
months between them. Th ree presentations are included: ex- 1. Dermatomyositis (DM)
clusive, as part of a collagenopathy, or associated with internal • Adult onset DM:
malignancy. Classic DM: exclusive.
Clínically amyopathic DM. It comprises amyopathic DM (sy- as part of an overlap connective tissue disorder
nonym of without myositis): distinctive classic DM skin lesions associated with internal cancer.
confi rmed by biopsy of 6 or more months evolution (provisio- Clinically amyopathic DM: amyopathic (provisional or defi nitive).
nal) to 2 years (defi nite) previous to muscular involvement; and hypomyopathic.
hypomyopathic DM: specifi c cutaneous involvement, without • Juvenile onset DM:
clinical evidence of muscular involvement, but with some alte- Classic DM.
red supplementary studies (enzymes, electromyogram [EMG] Clinically amyopathic DM: amyopathic.
and/or muscle biopsy). hypomyopathic.
Childhood/juvenile DM forms include classic DM and clini- 2. Polymyositis (PM): exclusive.
cally amyopathic DM with its two variants (amyopathic and as part of an overlap connective tissue disorder.
hypomyopathic) defi ned similarly to the adult onset DM am- associated with internal cancer.
yopathic and hypomyopathic forms. 3. Inclusion bodies myositis
4. Other clinical-pathological subgroups of myositis:
Incidence focal myositis, proliferative myositis, orbital myositis, eosinophilic myositis,
and granulomatous myositis.
Incidence in this country is unknown. In the United States,
the annual incidence estimation is 1/100,000 inhabitants;6 for
childhood/juvenile DM, 0.06 to 0.32/100,000.16 According and occasional genetic defi cit of C5 has been reported in DM.
to the Joint Committee of the American Association of Etiology also involves virus (parvovirus B19, Epstein Barr),
Dermatology, in 1998 the annual incidence was 5 cases per mi- drugs (such as cholesterol-lowering agents), D-penicillamine,
llion inhabitants.17 phenytoin, chloroquine, hydroxyurea, interferon (IFN), which
may cause myopathy and/or compatible skin lesions.18-21
Etiology Ultraviolet light has been accepted as a trigger, but no photoin-
Family-genetic risk of suffering idiopathic inflammatory duction of the lesion has been attained.
myopathies (adult and juvenile DM) related to homocygoci-
ty for HLADQA1, nucleotide polymorphism in tumor ne- Antibody-associated subgroups
crosis factor (TNF) promoter (TNF-α 308-A) was found as- A HLA DR52-related group has been identified, with hig-
sociated with chronicity, calcinosis, and high titers of TNF-α her prevalence of Jo-1 antibodies (20-30 percent of PM/
in classic juvenile DM in Caucasian population.1Association DM), characterized by lung fibrosis, arthritis, Raynaud’s
of DM with lung disease is known, more frequent in Japanese, phenomenon, mechanic’s hands, poor prognosis, higher
Retrospective study of dermatomyositis. Discussion of 40 cases consulting a Dermatology Department | 9
mortality, and poor response to treatment (antisyntheta- identified in sera of amyopathic DM related to interstitial
se syndrome); and another DR53-related group, Dw7 with lung disease or paraneoplasia, respectively.22
Mi-2 antibodies, present in 15 percent of DM, with flo-
rid skin involvement and better response to treatment. Cutaneous manifestations
PM/Scl present in 25 percent overlaps and in 8 to 12 per- Table 3 describes the distinctive cutaneous manifestations of
cent of PM is associated with myopathy plus sclerodermia. DM.14
Anti-SRP (signal recognition particle) present in 5 per- Signs of poor prognosis3,9,11,23-25 (acute, progressive, and severe
cent of PM relates to cardiac involvement without lung fi- forms) are: early and rapid respiratory, pharingeal, or esophagic
brosis, resistance to corticoid treatment, and severe myosi- involvement, resistance to early corticoid treatment, blisters or
tis.1,5 DR3, B8, and B14 have been related to juvenile DM; necrotic skin lesions in trunk, systemic vasculitis, high erythrose-
and B14 and B40, to overlap syndrome in adults. Recently, dimentation rate (more than 35 mm in the 1st hour), and old age.
antibodies of 155 kDa (Se) and 140 kDa (US) have been Mortality varies about 21 percent in adults and 3-31 per-
cent in children, according to diverse case-control studies and
TABLE 3. CLASSIC CUTANEOUS MANIFESTATIONS OF DM.14 authors.14Causes of death are respiratory failure and those re-
Pathognomonic lated to associated malignancies in adults (metastasis).1,3,9,11,25,26
1. Gottron’s papules. Incidence of internal malignancies was found in various ca-
2. Gottron’s sign: symmetric confl uent macular violaceous erythema on the dorsal se-control studies (between 11 and 43 percent).34 Relative
aspect of the interphalangeal or metacarpophalangeal joints, oleocranon, patel-
risk for internal malignancy ranges between 1.7 and 7.7, and is
lae and medial malleoli.
maximal in the term of one year.10,25,26-34
Characteristics
1. Heliotrope erithema.
Material and methods
2. Periungual telangiectasia.
3. Symmetric confl uent macular violaceous erythema on the dorsal aspect of the
hands, extensor aspect of arms and forearms, deltoid areas, posterior part of A retrospective observational analysis was carried out in 40 pa-
shoulders and neck (shawl sign), anterior neck and upper chest (V sign), central tients (7 males and 33 females) studied in the 1991-2007 pe-
aspect of the face and the forehead, and scalp.
riod in the Dermatology Department of Hospital “J. M. Ramos
4. Mechanic’s hand.
Mejía” of Buenos Aires, with confi rmed diagnosis of DM. Nine
Compatible with DM
of them were amyopathic (ADM), and 4 were childhood or ju-
1. Vascular atrophic poikilodermia.
venile cases. Excluded from computation were the cases of diffi -
2. Cutaneous calcinosis.
cult diff erential diagnosis: a mycosis fungoides (photosensitive
5%
18%
Femenino 23% Classic
Masculino Amyopathic
Hypomyopathic
82% 72%
Chart 1. Dermatomyositis per gender. Chart 3. Dermatomyositis per clinical form.
3%
12
nts10 21- 30 years 15%
e 8 31- 40 years
of pati 6 4511-- 5600 yyeeaarrss 25% MCuutsacnuelaorus
er 4 61- 70 years
mb 71- 80 years Concomitant
u 2
N Overlap
0 57%
21-30 31-40 41-50 51-60 61-70 71-80
years years years years years years
Chart 2. Dermatomyositis per age at diagnosis. Chart 4. Dermatomyositis per onset form.
10 | M. L. Hassan, A. Capecce, M. E. Melloni, M. Saposnik, I. Ruzzi, J. Gramajo, J. Lorenzo, R. Schröh, C. Corbella
lesions, facial erythema with eyelid edema, poikiloderma, high Complications not previously reported in this pathology: non-
reumatoid factor, arthralgia, and Raynaud’s phenomenon, to- tropical pyomyositis (1) (Figures 7 and 8), adult tinea (1), and
gether with neurological disease); and 3 female patients in whom episodic abdominal distension related to peritoneal adherences
the study was not completed. Also not included was a case as- evidenced by abdominal computed axial tomography (CAT)
sociated with simvastatin intake, which generated uncertainty (1) (Figure 9).
about the role of this drug in the appearance of manifestations. No case was found of antisynthetase syndrome (association
of Jo-1 antibodies, lung fibrosis o interstitial pneumonitis,
Results Raynaud’s phenomenon, and mechanic’s hands), or flage-
llated lesions. Lung involvement was found in two patients
Clincal features are summarized in Table 4, and in Charts 1, 2, with Jo-1, without mechanic’s hands in an overlap and a
3, and 4. Th irty three females and 7 males were studied (F:M classic DM.
ratio = 4.71:1). Four of them had diagnosis of childhood/juve- Association with internal malignancy was observed in 16.6
nile DM, with age of onset 5, 12, 14, and 15 years, respectively; percent (6 of 36), excluding childhood/juvenile cases.
two were females and two were males. Th e amyopathic juveni- Treatment was carried out based on oral systemic corticosteroi-
le forms, like calcinosis, were found in three cases, two females. ds, mostly meprednisone 1-2 mg/kg/day for months, with pro-
As a whole, 9 (23 percent) had diagnosis of ADM, and 31 were gressive variable dose reductions to complete 2 years. Not all
classic forms. Th e period of time lapsing from the cutaneous patients completed this regimen, or complied with it regularly.
manifestations to the onset of muscular manifestations lasted Occasionally, in special cases due to resistance, irregular intake
up to 11 years; the initial diganosis of these patients was lupus of corticoids, or concomitant contraindicated conditions, oral
erythematosus (seen at our Department by another observa- methotrexate 7.5 to 12.5 mg/week was used, or low dose intra-
tor). In addition to major and minor criteria,14 in our case-con- venous gammaglobulin (0.5 g per kg/day) for two days once a
trol study we found: rare lesions: blister lesions (in 2 patients), month, with good initial response, but they were discontinued
follicular lesions (2), extended diff use alopecia, with whitish on cost grounds. Mycophenolate mofetil was use in refractary
scales on scalp (1), gingival and lip telangiectasia (1), “milliary” myositis, 2 g/day, starting with 0.5 g/day. Th alidomide, 200
calcinosis (1), “punch-biopsy scar”-like lesions (3), cutaneous mg/day, and chloroquine phosphate, 200 mg/day, were used in
vasculitis (2) (Figures 1, 2, 3, 4, 5, and 6). other patients.
Figure 1. Poikiloderma. “Punch-biopsy” like lesions. Figure 2. Follicular lesions.
Retrospective study of dermatomyositis. Discussion of 40 cases consulting a Dermatology Department | 11
TABLE 4. CLINICAL CHARACTERISTICS AND EVOLUTION OF PATIENTS WITH DM DIAGNOSIS IN HOSPITAL “RAMOS MEJÍA”. PERIOD 1991-2007.
Systemic condition
Pat Age Sex Clinical form Onset Antibodies Neoplasia Evolution
or other feature
1 50 F Classic Muscular - Cervix Loss of weight LAA Resp. failure
2 60 F Classic Cutaneous - - Loss of weight -
3 57 F Classic Concomitant - - - -
4 37 F Classic Cutaneous - - - Resp. failure
5 35 F Amyopathic Cutaneous ACL - Follicular lesions Remission
6 58 F Classic Cutaneous - Breast LAA -
7 43 F Classic Concomitant - - - -
8 43 F Classic Cutaneous - - Blisters -
9 48 F Classic Cutaneous - Breast - Bone MTS
10 50 M Classic Cutaneous - - - Remission
11 68 F Classic Muscular - - - Resp. failure
12 54 F Amyopathic Cutaneous - - - -
13 12 F Classic Muscular - - Loss of weight Remission
14 36 F Classic Muscular - - - -
15 14 F Amyopathic Cutaneous - - Acne due to corticoids Remission
16 64 F Classic Muscular - - - -
17 15 M Amyopathic Cutaneous - - Calcinosis Irreg. Tt.
18 54 F Amyopathic Cutaneous - Breast - -
Respiratory
19 32 F Amyopathic Cutaneous - Remisión.
Post-silicone
20 56 F Classic Concomitant - - Mild resp esoph Remission
21 15 F Amyopathic Cutaneous FAN+ - Calcinosis+++ Improvement
22 5 M Classic Concomitant - - Loss of weight -
Abdominal distension
Steatosis
23 27 F Classic Cutaneous - - -
Dry Sd
Lipodystrophy
24 46 F Classic Cutaneous FAN 1/2560 Mo - - -
25 33 F Classic Concomitant FAN 1/320 Mo - Blisters Pyomyositis
Dysphagia
26 28 M Classic Cutaneous FAN 1/80 - -
Follicular lesions
27 58 F Classic Cutaneous - Ovary Dysglusia Bone MTS
28 29 F Classic Concomitant - - - -
29 34 F Classic Concomitant FAN 1/80 Mo - Tricophytic tinea -
30 78 M Amyopathic Cutaneous - - Fibromyalgia -
31 27 F Classic Muscular - - - -
32 50 F Classic Cutaneous FAN 1/1600 Mo - Erythroderma by chloroquine -
33 50 F Classic Concomitant FAN+ Cervix Vasculitis ulcers -
Lung fi brosis
34 33 M Classic Concomitant FAN 1/400 Mo - -
Pharingeal
35 75 F Hypomyopathic Cutaneous FAN 1/160 Mo - - Pneumonia.
Norwegian sca-
FAN 1/80 Ro+ ILD Cutaneous vasculitis Live-
36 40 F Classic Overlap - bies. Died due-
La+ do and acral ulcers
to neumothorax
37 72 F Hypomyopathic Cutaneous - - - -
38 32 F Classic Concomitant FAN 1/5120 - ILD -
39 40 F Amyopathic Cutaneous - - - -
40 50 M Classic Cutaneous - - - -
ACL: anticardiolipins. LAA: leukocytoclastic allergic angiitis. FAN: antinuclear antibodies. Mo: mottling pattern. MTS: metastasis. ILD: interstitial lung disease.
12 | M. L. Hassan, A. Capecce, M. E. Melloni, M. Saposnik, I. Ruzzi, J. Gramajo, J. Lorenzo, R. Schröh, C. Corbella
No satisfactory results were obtained by corticothe-
rapy or by hydroxychloroquine administration on
skin lesions, and these remained in spite of the mus-
cle involvement improvement. In facial lesions, 0.1
percent topical cream tacrolimus, or 1 percent pi-
mecrolimus were indicated in two cases, with partial
responses.
In one case of juvenile DM with calcinosis it was ad-
ministrated disodium etidronate, 20 mg/day, with
very good motor response and X-ray control aft er
three months treatment. Currently, we indicate dil-
tiazem, 10 mg/day, or alendronate.
Patient management was supplemented by kinesio-
therapy and muscular rehabilitation, indispensable
against contractures and permanent disability, espe-
cially in the childhood/juvenile form and to relieve
dysphagia.
Causes of death were: respiratory failure due to as-
piration pneumonia with pharyngeal muscle invol-
vement (2); metastasis of associated malignancy (1);
and two deaths of a cause not related to DM: pan-
creatitis (1), and traumatic pneumothorax (1).
COMMENT
Dermatomyositis appears with specifi c skin le-
sions, taken as diagnostic criteria; in addition, less
frequent lesions related to this condition exist.1,9
Th ese encompass: follicular lesions (Figure 2),
found in two cases located on presternal area and
back, having mucin deposits, perifollicular mono-
Figure 3. “Miliary” calcinosis in juvenile DM. nuclear infi ltrate, follicular hyperkeratosis, and in-
volvement of the arrector muscle of hair; diff use
alopecia with squamous lesions,16,36 which had to be
diff erenciated from adult tinea, psoriasis, anticon-
vulsant pharmacodermia, and Norwegian scabies.
Th erefore, direct mycological examination and cul-
ture of these lesions are recommended. Th ey have
also been referred to as psoriasiform scalp lesions
in juvenile DM,16 but were not exclusive to that age
range in our patients. Th e “punch-biopsy scar” ap-
pearance is noteworthy in the photosensitive areas
of poikilodermia, but is rare, and shows nonspeci-
fi c histopathology. Calcinosis in one case adopted
an uncommon “miliary” appearance in the inner
aspect of the thighs in a juvenile ADM,16 and was
not disabling. In two patients coexisted morphea-
like lesions with compatible histopathology in the
root of upper limbs. Lip and gingival telangiecta-
sia were found in the physical examination of only
one patient; they have recently been described in
this disease, and the signifi cance is yet unknown.
Figure 4. Gottron’s papules. Cutaneous vasculitis (Figures 5 and 6) was found