Medical Journal, Armed Forces India

Elsevier

Congenital miliaria crystallina A

diagnostic dilemma
Sudhir Dixit, Ashish Jain, [...], and V.K. Khurana
Additional article information

Abstract
Miliaria crystallina is a transient, superficial obstruction of eccrine sweat ducts resulting in
rapidly evolving noninflammatory vesicles. The disease is observed frequently in hot, humid,
tropical climates and in the neonatal period, most likely due to lack of maturation of the
sweat duct during the first few days following birth. It is rarely present at delivery and
remains a diagnostic dilemma for the neonatologists. We report a rare case of Congenital
miliaria crystallina that was present at birth.
Keywords: Miliaria, Congenital, Vesicular lesion

Introduction
Vesicular and pustular disturbances of the neonatal period are not uncommon. Most of them
are harmless; however it is important to differentiate these from potentially life-threatening
infectious and non-infectious disorders.1 Miliaria crystallina is commonly considered in the
differential diagnosis of bullous diseases in newborns. Studies report an incidence of 1.3% in
neonates developing skin lesions within 48 h of life.2 This is a transient, superficial
obstruction of eccrine sweat ducts resulting in rapidly evolving noninflammatory vesicles.
The disease is observed frequently among neonates in hot, humid and tropical climate.
Mostly there is lack of maturation of the sweat duct during the first few days following birth,
but is rarely present at delivery.3 We report a rare case of Congenital miliaria crystallina
presenting at birth.

Case report
A female term baby, appropriate for gestational age with birth weight of 2.65 kg was born to
a primi gravida, 28-year-old mother. She was an outcome of normal vaginal delivery. The
apgar scores were 8, 9, 9 at 1, 5 and 10 min respectively. General physical and systemic
examination was within normal limits, except that the skin was covered with vesicular lesions
of variable size and distribution, containing clear fluid.
There was no associated erythema (Fig. 1).

Fig. 1
Baby with Miliaria crystallina at birth.
Antenatally, mother was a booked case and had not taken any medicine. However mother had
fever starting 3 days prior to onset of labour. On investigation her TLC was 12,000 and CRP
was positive. LFT, KFT was within normal limits and blood culture was sterile. The mother
was treated with antibiotics and antipyretics. Inspite of the above treatment, the mother's
temperature in labour room was 101 F. At birth baby's skin was covered with tiny, closely
spaced vesicles spread over face, neck, upper arm and trunk. Vesicles were clear, 13 mm in
diameter with silvery to shining surface. Tzanck test did not show any acantholytic cells or
neutrophils and gram stain found no bacteria. CBC, CRP and blood culture was negative.
TORCH screen was negative.
On the basis of typical cutaneous lesions, diagnosis of miliaria crystallina was made in
consultation with the dermatologist. Baby was moved to a cooler environment. Over next 2
days no new vesicles erupted. On 4th day vesicles dried and by 7th day skin was normal in
appearance.

Discussion
Miliaria results from retention of sweat in occluded eccrine ducts as a result of keratinous
plugs. Retrograde pressure results in rupture of duct and leakage of sweat in to the epidermis
and/or dermis. Miliaria occurs in 3 forms; miliaria crystallina, rubra, and profunda. This
classification is based on the level of blockage of eccrine sweat duct.4 In miliaria crystallina
(sudamina), the obstruction of the eccrine duct is very superficial i.e. within the stratum
corneum and commonly occurs with febrile diseases or after sunburn, most often in hot and
humid climatic conditions. Children are particularly at risk.
Clinically translucent, thin roofed vesicles of 12 mm diameter without an inflammatory halo
are observed. If opened with a needle, a clear, watery liquid is obtained. Lesions develop
preferentially on the neck and axillae. In the neonatal period, the face can be involved as
well. This was the case in our infant, too. Vesicles in this location can have a silvery sheen.
This is not based on leukocyte influx as in miliaria rubra, but caused by corneocytes in the
blister, and is known as a variant called miliaria crystallina alba. The thin roofs covering the
blisters rupture easily and the skin clears thereafter, leaving a superficial, branny
desquamation.5
The differential diagnosis of neonatal blistering includes several infectious and bullous
dermatoses. Herpes simplex and varicella show serous vesicles. Erythema toxicum
neonatorum, neonatal pustular melanosis, and acropustulosis of the child show pustules.
Staphylococcal infection causes staphylococcal scalded syndrome or large blisters which
differ clearly from minute vesicles in miliaria. The most important difference remains that, in
miliaria crystallina, the sweat in the blisters is not yellowish, but clear like water. Therefore,
the colour and the form of the blisters allow a definite clinical diagnosis.

Therapy of miliaria crystallina is simple. The only effective treatment and prevention is to
avoid further sweating. A few hours in a cool environment will bring relief. Other prevention
and treatment modalities include avoidance of excessive clothing, friction from clothing and
excessive use of soap. Topical antibacterial preparations have some role in preventing the
commonly occurring secondary infections in miliaria. Calamine lotion followed by a bland
emollient is also beneficial.
In newborns and children, the disease is rather frequent. Thus, in a retrospective study from
Japan, including 5387 infants in a newborn ward, miliaria crystallina was seen in 4.5%, with
a peak occurrence around the 6th and 7th postnatal day. In this large series, the occurrence
was classified as extremely rare before day 4.6 In an Indian study over a period of 7 months,
131 neonates were found to have miliaria crystallina within 48 h of birth.7 Most of the
articles are of the opinion that the disease does not occur at delivery.8 It is suggested that duct
disrupture is the immediate cause of miliaria.9 The hydration of corneocytes varies with the
degree of environmental humidity and temperature. In our patient, the maternal fever might
have been the trigger.
There are only 3 reports so far in the literature on congenital miliaria crystallina, two in black
newborns10,11 and only one in white new born as in our case.3 The same has not been
reported in Indian literature. According to Straka et al,10 who described the first case, the
disease most probably derives from immature sweat ducts within the first weeks of life.
Probably the occlusion of the sweat ducts had formed already in utero as in our case.

Conflicts of interest
All authors have none to declare.

Article information
Med J Armed Forces India. Oct 2012; 68(4): 386388.
Published online Jul 17, 2012. doi: 10.1016/j.mjafi.2012.01.004
PMCID: PMC3862747
Sudhir Dixit,a Ashish Jain,b, Suhas Datar,c and V.K. Khuranad
a
Senior Resident, Neonatology Division, Department of Pediatrics, Hindu Rao Hospital,
Malka Gunj, Delhi, India
b
Neonatologist, Neonatology Division, Department of Pediatrics, Hindu Rao Hospital, Malka
Gunj, Delhi, India
c
Senior Specialist, Neonatology Division, Department of Pediatrics, Hindu Rao Hospital,
Malka Gunj, Delhi, India
d
Dermatologist, Department of Skin, Hindu Rao Hospital, Malka Gunj, Delhi, India
Ashish Jain: ni.oc.oohay@0002niajhsihsard
Corresponding author. Tel.: +91 9810694789. Email: ni.oc.oohay@0002niajhsihsard
Received July 11, 2011; Accepted January 5, 2012.
Copyright 2012 Published by Elsevier B.V. on behalf of Director General, Armed Forces
Medical Services.
Articles from Medical Journal, Armed Forces India are provided here courtesy of Elsevier

References

1. Wagner A. Distinguishing vesicular and pustular disorders in the neonate. Curr Opin
Pediatr. 1997;9:396405. [PubMed]
2. Moosavi Z., Hosseini T. One-year survey of cutaneous lesions in 1000 consecutive Iranian
Newborns. Pediatr Dermatol. 2006 JanFeb;23(1):6163. [PubMed]
3. Haas Norbert, Henz Beate Maria, Weigel Heidrun. Congenital miliaria crystallina. J Am
Acad Dermatol. 2002;47(5):S270S272. [PubMed]
4. Wenzel F.G., Horn T.D. Nonneoplastic disorders of the eccrine glands. J Am Acad
Dermatol. 1998;38:117. [PubMed]
5. Hurwitz S. 2nd ed. WB Saunders; Philadelphia: 1993. Clinical Paediatric Dermatology.
278317.
6. Hidano A., Purwoko R., Jitsukawa K. Statistical survey of skin changes in Japanese
neonates. Pediatr Dermatol. 1986;3:140144. [PubMed]
7. Nanda A., Kaur S., Bhakoo O.N., Dhall K. Survey of cutaneous lesions in Indian
Newborns. Pediatr Dermatol. 1989;6:3942. [PubMed]
8. Hodgman J., Freedman R., Levan N. Neonatal dermatology. Pediatr Clin North Am.
1971;18:713756. [PubMed]
9. Shuster S. Duct disruption, a new explanation of miliaria. Acta Derm Venereol.
1997;77:13. [PubMed]
10. Straka B.F., Cooper P.H., Greer K.E. Congenital miliaria crystallina. Cutis. 1991;47:103
106. [PubMed]
11. Arpey C.J., Nagashima Whalen L.S., Chren M.M., Zaim M.T. Congenital miliaria
crystallina: case report and literature review. Pediatr Dermatol. 1992;9:283287. [PubMed]

Journal of Medical Case Reports

BioMed Central
Recurrent pyoderma gangrenosum precipitated by breast cancer: a case
report and review of the literature

Renata Duchnowska, Ewa Ziajka, [...], and Bartomiej Grala

Additional article information
Abstract
Introduction

Pyoderma gangrenosum is a rare clinical entity of poorly understood pathogenesis,

characterized by rapidly progressing skin necrosis. In around half of patients
pyoderma gangrenosum is a manifestation of underlying systemic diseases, such as
rheumatoid arthritis, inflammatory bowel disease or myeloproliferative disorders.
There have been very few reports on the association of pyoderma gangrenosum with
solid malignancies.
Case presentation

We report a case of a 68-year-old Caucasian woman in whom pyoderma gangrenosum

first appeared around 30 years earlier, at the time of exacerbation of rheumatoid
arthritis, and recurred as a manifestation of locally advanced breast cancer. The
causative role of the neoplastic process was partly confirmed by the healing of the
skin ulceration only following effective endocrine cancer therapy, whereas earlier
attempts with standard anti-inflammatory therapy were unsuccessful.
Conclusions

Pyoderma gangrenosum has a recurrent nature and may be reactivated by various

causes within several years. Therefore, a prompt and thorough diagnosis accompanied
by treatment of the underlying disease is necessary.
Keywords: Pyoderma gangrenosum, Breast cancer, Skin ulceration
Introduction

Pyoderma gangrenosum is a rare clinical condition of poorly understood pathogenesis,

characterized by rapidly progressing skin necrosis. In around half of patients, this

entity is a manifestation of underlying systemic diseases, such as rheumatoid arthritis,

inflammatory bowel disease or myeloproliferative disorders. There have been very
few reports on the association of pyoderma gangrenosum with solid malignancies. We
present a case of a 68-year-old woman in whom pyoderma gangrenosum first
appeared around 30 years earlier, at the time of exacerbation of rheumatoid arthritis,
and recurred as a manifestation of locally advanced breast cancer.
Case presentation

A 68-year-old Caucasian woman presented with an ulceration of the anterior surface

of her left leg. Thirty years earlier, at exacerbation of rheumatoid arthritis, our patient
underwent therapy for a similar lesion located on her left forearm, diagnosed as
pyoderma gangrenosum. Since then, the rheumatoid arthritis had been quiescent and
had not required any therapy, and at admission there were no signs of recurrence. A
physical examination showed an ulcerating tumor involving the lower quadrants of
her left breast. The breast lump had appeared four years earlier, progressed rapidly
within the last month and at diagnosis was clinically staged at T4aN1M0. A
histopathology examination showed invasive ductal breast carcinoma, with estrogen
receptor expression in 90 percent of the tumor cells, and no expression of
progesterone receptor and epidermal growth factor receptor type 2 (HER2). A biopsy
specimen from the edge of the leg ulceration showed pyoderma gangrenosum: an
intensive mixed inflammatory infiltrate including mainly neutrophils, with
accompanying vessel destruction and necrosis of the epithelium (Figure 1a,b). Firstline therapy for pyoderma gangrenosum included a combination of dapsone (100mg/d
orally) and prednisone (an increasing dose of 20 to 30mg/d orally), accompanied by a
topical application of 2 percent detreomycin ointment and silicon dressing. After four
months, due to further progression, cyclosporine administration was introduced at a
starting dose of 300mg/d, decreasing to 100mg/d. Therapy was stopped after three
months due to nephrotoxicity and microcytic anemia. Subsequent immunosuppressive
therapy included sulfasalazin (3g/d orally) accompanied by topical management with
potassium permanganate and 5 percent detreomycin ointment. Due to social factors,
breast cancer treatment was initiated only five months after the diagnosis of pyoderma
gangrenosum. Since our patient was diagnosed with strongly estrogen receptorpositive breast cancer with no life-threatening symptoms, a typical first-line induction

endocrine therapy with aromatase inhibitor - letrozole - at a daily dose of 2.5mg was
introduced. By this time, the leg ulceration had reached its largest dimension
(Figure 2). A partial response of the breast tumor was accompanied by continuous
healing of her leg ulceration (Figure 3) and after 12 months, the lesion completely
resolved with scarring. As our patient refused mastectomy, a wide tumor excision was
performed, however, postoperative radiotherapy was not applied due to a concern of
excessive skin toxicity and activation of pyoderma gangrenosum in the irradiated
area. After surgery, our patient finished five years of adjuvant letrozole therapy with
complete response. Her leg ulceration has not recurred.

Figure 1
Pyoderma gangrenosum. (a) Biopsy specimen from the edge of the leg
ulceration: histological pattern (hematoxylin and eosin stain (HE) staining;
200). The inset within Figure 1a refers to Figure 1b. (b) An intensive
mixed inflammatory ...

Figure 2
Ulceration of the left leg (at the time of initiation of induction endocrine
therapy).

Figure 3

A continuous healing of the leg ulceration: after eight months of dermatology

and oncology combination therapy.
Discussion

Pyoderma gangrenosum is a rare dermatosis characterized by rapidly progressive skin

ulceration. The etiology of this entity is poorly understood but neutrophil and
monocyte dysfunctions (defects in phagocytosis and chemotaxis) have been reported
[1-6]. In around half of cases, pyoderma gangrenosum is associated with various
underlying specific conditions, most frequently rheumatoid arthritis, inflammatory
bowel disease or myeloproliferative disorders. There have been only a few reports on
the association of pyoderma gangrenosum with solid malignancies including breast
cancer [7-11]. Additionally, a few cases of pyoderma gangrenosum of the breast
precipitated by breast surgery were reported [11-13]. The most common location of
pyoderma gangrenosum is the legs but the disease may also involve other regions.
Differential diagnosis includes Sweets syndrome, characterized by similar histologic
features, fungal infections and inflammatory diseases of subcutaneous tissue [2,4,6].
The recommended first-line treatment is steroids or other immunosuppressive drugs,
such as cyclosporine, azathiopirine, mycophenolate mofetil, cyclolophosfamide,
chlorambucil or thalidomide [14]. Surgery is contraindicated due to pathergy, which is
characterized by the development of new lesions at the site of even minor trauma [13].
Importantly, pyoderma gangrenosum management should be accompanied by
treatment of the underlying disease, although surgery or radiotherapy may induce
rapid development of ulceration at the site of therapeutic intervention [7,11-13].
In our patient, pyoderma gangrenosum was most likely induced by progressing breast
cancer. The causative role of the neoplastic process was partly confirmed by the
healing of the skin ulceration only following effective endocrine cancer therapy,
whereas earlier attempts with corticosteroids were unsuccessful. The lack of
improvement with corticosteroids has been atypical, as this therapy usually induces
dramatic response within the first two weeks [14]. Another interesting feature of this
case was the recurrent nature of pyoderma gangrenosum, with its first appearance 30
years earlier, during an exacerbation of rheumatoid arthritis. Thus, probably this entity
may be reactivated by various causes within several years.

Conclusions

Due to association with serious underlying conditions, rapid development and

potentially serious consequences, pyoderma gangrenosum necessitates prompt and
thorough diagnosis, including the search for coexisting disease [15,16]. If the latter is
recognized, therapy should address both pyoderma gangrenosum and its causative
factor.
Consent

Written informed consent was obtained from the patient for publication of this case
report and any accompanying images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Abbreviations

HER2: epidermal growth factor receptor type 2.

Competing interests

The authors declare that they have no competing interests.

Authors contributions

RD treated the patient and drafted the manuscript. EZ treated the patient and drafted
the manuscript. AG treated the patient and drafted the manuscript. BG carried out the
immunoassays and drafted the manuscript. All authors read and approved the final
manuscript.
Article information
J Med Case Rep. 2014; 8: 226.
Published online Jun 25, 2014. doi: 10.1186/1752-1947-8-226
PMCID: PMC4090656
Renata Duchnowska,
Grala3

Ewa Ziajka,2 Agnieszka Gralska,2 and Bartomiej

Department of Oncology, Military Institute of Medicine, Szaserw 128 Street,

04-141 Warsaw, Poland

Department of Dermatology, Military Institute of Medicine, Szaserw 128

Street, 04-141 Warsaw, Poland

Department of Pathology, Military Institute of Medicine, Szaserw 128 Street,

04-141 Warsaw, Poland
Corresponding author.

Renata Duchnowska: lp.pw@ttdr; Ewa Ziajka: lp.pw@akjaizawe; Agnieszka

Gralska: lp.pw@gajamaga; Bartomiej Grala: lp.pw@balarg
Received January 25, 2014; Accepted April 29, 2014.
Copyright 2014 Duchnowska et al.; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative
Commons Attribution License
(http://creativecommons.org/licenses/by/2.0), which permits unrestricted
use, distribution, and reproduction in any medium, provided the original
work is properly credited. The Creative Commons Public Domain
Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/)
applies to the data made available in this article, unless otherwise stated.
Articles from Journal of Medical Case Reports are provided here courtesy of
BioMed Central
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1997;8:407. [PubMed]
2. Callen JP. Pyoderma gangrenosum and related disorders. Med Clin North
Am. 1989;8:12471261. [PubMed]
3. Wines N, Wines M, Ryman W. Understanding pyoderma gangrenosum: a
review. Med Gen Med. 2001;8:6. doi: 10.1097/00125817-20010100000003. [Cross Ref]
4. Weenig RH, Davis MDP, Dahl PR, Su WP. Skin ulcers misdiagnosed as
pyoderma gangrenosum. N Engl J Med. 2002;8:14121418. doi:
10.1056/NEJMoa013383. [PubMed] [Cross Ref]

5. Callen JP. Pyoderma gangrenosum. Lancet. 1998;8:581585. doi:

10.1016/S0140-6736(97)10187-8. [PubMed] [Cross Ref]
6. Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma
gangrenosum: clinicopathologic correlation and proposed diagnostic
criteria. Int J Dermatol. 2004;8:790800. doi: 10.1111/j.13654632.2004.02128.x. [PubMed] [Cross Ref]
7. Kanno T, Ito M, Tsuji H, Kawase N, Taki Y. A case of pyoderma gangrenosum
involving the prostate gland after radiation therapy for prostate cancer.
Hinyokika Kiyo. 2002;8:565568. [PubMed]
8. Gateley CA, Foster ME. Pyoderma gangrenosum of the breast. Br J Clin
Pract. 1990;8:713714. [PubMed]
9. Simon H, Metges JP, Lucas B, Malhaire JP. Pyoderma gangrenosum and
breast cancer: a new case. Ann Med Interne. 2000;8:314315. [PubMed]
10.Sharma AK, Horgan K, Holt P. The management of acute
pyodermagangrenosum of the breast with a coincidental invasive breast
cancer. J Dermatol Treat. 1994;8:215217. doi:
10.3109/09546639409080571. [Cross Ref]
11.Davis MD, Alexander JL, Prawer SE. Pyoderma gangrenosum of the breast
precipitated by breast surgery. J Am Acad Dermatol. 2006;8:317320. doi:
10.1016/j.jaad.2006.02.066. [PubMed] [Cross Ref]
12.Rietjens M, Cuccia G, Brenelli F, Manconi A, Martella S, De Lorenzi FA.
Pyoderma gangrenosum following breast reconstruction: a rare cause of
skin necrosis. Breast J. 2009;8:200202. [PubMed]
13.Horner B, El-Muttardi N, Mercer D. Pyoderma gangrenosum complicating
bilateral breast reduction. Br J Plast Surg. 2004;8:679681. doi:
10.1016/j.bjps.2004.03.004. [PubMed] [Cross Ref]
14.Long CC, Jessop J, Young M, Holt PJ. Minimizing the risk of post-operative
pyoderma gangrenosum. Br J Dermatol. 1992;8:4548. [PubMed]
15.Gettler S, Rothe M, Grin C, Grant-Kels J. Optimal treatment of pyoderma
gangrenosum. Am J Clin Dermatol. 2003;8:597608. doi:
10.2165/00128071-200304090-00002. [PubMed] [Cross Ref]
16.Duguid CM, OLoughlin S, Otridge B, Powell FC. Paraneoplastic pyoderma
gangrenosum. Australas J Dermatol. 1993;8:1722. doi: 10.1111/j.14400960.1993.tb00841.x. [PubMed] [Cross Ref]

Journal of Medical Case Reports

BioMed Central
Berulang pioderma gangrenosum dipicu oleh kanker payudara: laporan
kasus dan kajian literatur
Renata Duchnowska, Ewa Ziajka, [...], dan Bartlomiej Grala
Informasi artikel tambahan
abstrak
pengantar
Pioderma gangrenosum adalah entitas klinis jarang kurang dipahami
patogenesis, ditandai dengan cepat berkembang nekrosis kulit. Di sekitar
setengah dari pasien pioderma gangrenosum merupakan manifestasi dari
penyakit sistemik yang mendasari, seperti rheumatoid arthritis, penyakit
inflamasi usus atau gangguan mieloproliferatif. Ada sangat sedikit laporan
tentang asosiasi pioderma gangrenosum dengan keganasan padat.
presentasi kasus
Kami melaporkan kasus seorang wanita Kaukasia 68 tahun di antaranya
pioderma gangrenosum pertama kali muncul sekitar 30 tahun
sebelumnya, pada saat eksaserbasi rheumatoid arthritis, dan muncul
kembali sebagai manifestasi dari kanker payudara stadium lanjut. Peran
penyebab dari proses neoplastik sebagian dikonfirmasi oleh penyembuhan

ulserasi kulit hanya mengikuti terapi kanker endokrin yang efektif,

sedangkan upaya sebelumnya dengan terapi anti-inflamasi standar tidak
berhasil.
kesimpulan
Pioderma gangrenosum memiliki sifat berulang dan dapat diaktifkan
kembali oleh berbagai sebab dalam beberapa tahun. Oleh karena itu,
diagnosis yang tepat dan menyeluruh disertai dengan pengobatan
penyakit yang mendasari diperlukan.
Kata kunci: Pioderma gangrenosum, Kanker payudara, ulserasi kulit
pengantar
Pioderma gangrenosum adalah kondisi klinis yang jarang kurang dipahami
patogenesis, ditandai dengan cepat berkembang nekrosis kulit. Di sekitar
setengah dari pasien, entitas ini merupakan manifestasi dari penyakit
sistemik yang mendasari, seperti rheumatoid arthritis, penyakit inflamasi
usus atau gangguan mieloproliferatif. Ada sangat sedikit laporan tentang
asosiasi pioderma gangrenosum dengan keganasan padat. Kami
menyajikan sebuah kasus seorang wanita 68 tahun di antaranya pioderma
gangrenosum pertama kali muncul sekitar 30 tahun sebelumnya, pada
saat eksaserbasi rheumatoid arthritis, dan muncul kembali sebagai
manifestasi dari kanker payudara stadium lanjut.
presentasi kasus
Seorang wanita Kaukasia 68 tahun disajikan dengan ulserasi dari
permukaan anterior kaki kirinya. Tiga puluh tahun sebelumnya, pada
eksaserbasi rheumatoid arthritis, pasien kami menjalani terapi untuk lesi
yang sama yang terletak di lengan kirinya, didiagnosis sebagai pioderma
gangrenosum. Sejak saat itu, rheumatoid arthritis telah diam dan tidak
diperlukan terapi apapun, dan saat masuk tidak ada tanda-tanda
kekambuhan. Pemeriksaan fisik menunjukkan tumor ulserasi melibatkan
kuadran bawah payudara kirinya. Benjolan payudara muncul empat tahun
sebelumnya, berkembang pesat dalam bulan lalu dan pada diagnosis
secara klinis dipentaskan di T4aN1M0. Pemeriksaan histopatologi
menunjukkan invasif duktal karsinoma payudara, dengan ekspresi
reseptor estrogen dalam 90 persen dari sel-sel tumor, dan tidak ada

ekspresi reseptor progesteron dan pertumbuhan epidermal reseptor faktor

tipe 2 (HER2). Sebuah spesimen biopsi dari tepi ulkus kaki menunjukkan
pioderma gangrenosum: peradangan menyusup campuran intensif
termasuk terutama neutrofil, dengan disertai kerusakan kapal dan
nekrosis epitel (Gambar 1a, b). Terapi lini pertama untuk pioderma
gangrenosum termasuk kombinasi dapson (100mg / d oral) dan prednison
(dosis meningkat dari 20 sampai 30mg / d oral), disertai dengan aplikasi
topikal dari 2 persen detreomycin salep dan silikon dressing. Setelah
empat bulan, karena perkembangan lebih lanjut, administrasi siklosporin
diperkenalkan dengan dosis awal 300mg / d, menurun menjadi 100mg / d.
Terapi dihentikan setelah tiga bulan karena nefrotoksisitas dan anemia
mikrositik. Terapi imunosupresif berikutnya termasuk sulfasalazin (3g / d
oral) disertai dengan manajemen topikal dengan kalium permanganat dan
5 persen detreomycin salep. Karena faktor-faktor sosial, pengobatan
kanker payudara dimulai hanya lima bulan setelah diagnosis pioderma
gangrenosum. Karena pasien kami didiagnosa menderita kanker sangat
estrogen reseptor-positif payudara tanpa gejala yang mengancam nyawa,
lini pertama terapi endokrin induksi khas dengan aromatase inhibitor letrozole - dengan dosis harian 2,5 mg diperkenalkan. Pada saat ini,
ulserasi kaki telah mencapai dimensi terbesar (Gambar 2). Sebuah respon
parsial dari tumor payudara didampingi penyembuhan terus menerus
ulserasi kakinya (Gambar 3) dan setelah 12 bulan, lesi benar-benar
diselesaikan dengan jaringan parut. Sebagai pasien kami menolak
mastektomi, eksisi tumor luas dilakukan, bagaimanapun, radioterapi pasca
operasi tidak diterapkan karena kekhawatiran toksisitas kulit yang
berlebihan dan aktivasi pioderma gangrenosum di daerah yang diradiasi.
Setelah operasi, pasien kami selesai lima tahun terapi letrozole adjuvant
dengan respon lengkap. Her ulserasi kaki tidak terulang.
Gambar 1
Gambar 1
Pioderma gangrenosum. (a) spesimen Biopsi dari tepi ulkus kaki: pola
histologis (hematoxylin dan eosin noda (HE) pewarnaan, 200 ). The inset
dalam Gambar 1a mengacu pada Gambar 1b. (b) Sebuah inflamasi
campuran intensif ...
Gambar 2
Gambar 2

Ulserasi kaki kiri (pada saat memulai terapi endokrin induksi).

Gambar 3
Gambar 3
Sebuah penyembuhan terus menerus dari ulserasi kaki: setelah delapan
bulan dermatologi dan terapi kombinasi onkologi.
diskusi
Pioderma gangrenosum adalah dermatosis langka yang ditandai dengan
ulserasi kulit progresif cepat. Etiologi entitas ini kurang dipahami tetapi
neutrofil dan monosit disfungsi (cacat pada fagositosis dan kemotaksis)
telah dilaporkan [1-6]. Di sekitar setengah dari kasus, pioderma
gangrenosum dikaitkan dengan berbagai kondisi spesifik yang mendasari,
rheumatoid arthritis yang paling sering, penyakit inflamasi usus atau
gangguan mieloproliferatif. Ada hanya beberapa laporan tentang asosiasi
pioderma gangrenosum dengan keganasan padat termasuk kanker
payudara [7-11]. Selain itu, beberapa kasus pioderma gangrenosum
payudara dipicu oleh operasi payudara dilaporkan [11-13]. Lokasi yang
paling umum dari pioderma gangrenosum adalah kaki tapi penyakit ini
juga dapat melibatkan daerah lain. Diagnosis banding meliputi Sweet
syndrome, yang ditandai dengan fitur histologis yang sama, infeksi jamur
dan penyakit inflamasi dari jaringan subkutan [2,4,6]. Dianjurkan
pengobatan lini pertama adalah steroid atau obat imunosupresif lainnya,
seperti cyclosporine, azathiopirine, mycophenolate mofetil,
cyclolophosfamide, klorambusil atau thalidomide [14]. Pembedahan
merupakan kontraindikasi karena patergi, yang ditandai dengan
perkembangan lesi baru di lokasi trauma bahkan kecil [13]. Yang penting,
manajemen gangrenosum pioderma harus disertai dengan pengobatan
penyakit yang mendasari, meskipun operasi atau radioterapi dapat
menyebabkan pesatnya perkembangan ulkus di lokasi intervensi terapi
[7,11-13].
Dalam pasien kami, pioderma gangrenosum kemungkinan besar
disebabkan oleh kemajuan kanker payudara. Peran penyebab dari proses
neoplastik sebagian dikonfirmasi oleh penyembuhan ulserasi kulit hanya
mengikuti terapi kanker endokrin yang efektif, sedangkan upaya
sebelumnya dengan kortikosteroid tidak berhasil. Kurangnya perbaikan

dengan kortikosteroid telah atipikal, karena terapi ini biasanya

menginduksi respon dramatis dalam dua minggu pertama [14]. Fitur lain
yang menarik dari kasus ini adalah sifat berulang dari pioderma
gangrenosum, dengan penampilan pertama 30 tahun sebelumnya, selama
eksaserbasi rheumatoid arthritis. Dengan demikian, mungkin entitas ini
dapat diaktifkan kembali oleh berbagai sebab dalam beberapa tahun.
kesimpulan
Karena asosiasi dengan kondisi serius yang mendasarinya, perkembangan
pesat dan konsekuensi yang berpotensi serius, pioderma gangrenosum
memerlukan cepat dan menyeluruh diagnosis, termasuk mencari hidup
bersama penyakit [15,16]. Jika yang terakhir diakui, terapi harus
mengatasi kedua gangrenosum pioderma dan faktor penyebab nya.
persetujuan
Informed consent tertulis diperoleh dari pasien untuk publikasi dari
laporan kasus ini dan setiap gambar yang menyertainya. Salinan
persetujuan tertulis tersedia untuk ditinjau oleh Editor-in-Chief pada jurnal
ini.
singkatan
HER2: tipe reseptor faktor pertumbuhan epidermal 2.
bersaing kepentingan
Para penulis menyatakan bahwa mereka tidak memiliki kepentingan
bersaing.
Penulis Kontribusi
RD diperlakukan pasien dan disusun naskah. EZ diperlakukan pasien dan
disusun naskah. AG diperlakukan pasien dan disusun naskah. BG
melaksanakan immunoassays dan disusun naskah. Semua penulis
membaca dan menyetujui naskah akhir.
informasi Pasal
J Med Kasus Rep 2014; 8: 226.
. Diterbitkan online Jun 25, 2014 doi: 10.1186/1752-1947-8-226
PMCID: PMC4090656

Renata Duchnowska, author1 sesuai Ewa Ziajka, 2 Agnieszka Goralska, 2

dan Bartlomiej Grala3
1Jurusan Onkologi, Military Institute of Medicine, Szaserw 128 Street, 04141 Warsawa, Polandia
2Department of Dermatology, Military Institute of Medicine, Szaserw 128
Street, 04-141 Warsawa, Polandia
3 Departemen Patologi, Military Institute of Medicine, Szaserw 128 Street,
04-141 Warsawa, Polandia
penulis yang sesuai authorCorresponding.
Renata Duchnowska: lp.pw @ ttdr; Ewa Ziajka: lp.pw @ akjaizawe;
Agnieszka Goralska: lp.pw @ gajamaga; Bartlomiej Grala: lp.pw @ balarg
Diterima 25 Januari 2014; Diterima April 29, 2014.
Copyright 2014 Duchnowska et al.; lisensi BioMed Central Ltd
Ini adalah artikel Open Access didistribusikan di bawah persyaratan Lisensi
Creative Commons Attribution
(http://creativecommons.org/licenses/by/2.0), yang memungkinkan
penggunaan tak terbatas, distribusi, dan reproduksi dalam media apapun,
asalkan karya asli dikreditkan dengan benar. The Creative Commons
Public Domain Dedication pengabaian
(http://creativecommons.org/publicdomain/zero/1.0/) berlaku untuk data
yang disediakan dalam artikel ini, kecuali dinyatakan lain.
Artikel dari Journal of Medical Case Reports disediakan di sini courtesy of
BioMed Central
Referensi
Takvorian T, Skarin A, Johnson R. Pioderma gangrenosum. J Clin Oncol.
1997; 8:407. [PubMed]
Callen JP. Pioderma gangrenosum dan gangguan yang terkait. Med Clin
Utara Am. 1989; 8:1247-1261. [PubMed]
Wines N, M Wines, Ryman W. Memahami pioderma gangrenosum:
review. Med Gen Med. 2001; 08:06. doi: 10.1097/00125817-20010100000003. [Palang Ref]
Weenig RH, Davis MDP, Dahl PR, Su WP. Ulkus kulit salah didiagnosis
sebagai pioderma gangrenosum. N Engl J Med. 2002; 8:1412-1418. doi:
10.1056/NEJMoa013383. [PubMed] [Palang Ref]
Callen JP. Pioderma gangrenosum. Lancet. 1998; 8:581-585. doi:

10.1016/S0140-6736 (97) 10187-8. [PubMed] [Palang Ref]

Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pioderma
gangrenosum: korelasi klinikopatologi dan kriteria diagnostik yang
diusulkan. Int J Dermatol. 2004; 8:790-800. doi: 10.1111/j.13654632.2004.02128.x. [PubMed] [Palang Ref]
Kanno T, Ito M, Tsuji H, Kawase N, Taki Y. Sebuah kasus pioderma
gangrenosum melibatkan kelenjar prostat setelah terapi radiasi untuk
kanker prostat. Hinyokika Kiyo. 2002; 8:565-568. [PubMed]
Gateley CA, Foster ME. Pioderma gangrenosum payudara. Br J Clin
Pract. 1990; 8:713-714. [PubMed]
Simon H, Metges JP, Lucas B, Malhaire JP. Pioderma gangrenosum dan
kanker payudara: kasus baru. Ann Med interne. 2000; 8:314-315.
[PubMed]
Sharma AK, Horgan K, Holt P. Manajemen pyodermagangrenosum akut
payudara dengan kanker payudara invasif kebetulan. J Dermatol Treat.
1994; 8:215-217. doi: 10.3109/09546639409080571. [Palang Ref]
Davis MD, Alexander JL, Prawer SE. Pioderma gangrenosum payudara
dipicu oleh operasi payudara. J Am Acad Dermatol. 2006; 8:317-320. doi:
10.1016/j.jaad.2006.02.066. [PubMed] [Palang Ref]
Rietjens M, Cuccia G, Brenelli F, Manconi A, Martella S, De Lorenzi FA.
Pioderma gangrenosum berikut rekonstruksi payudara: penyebab yang
jarang nekrosis kulit. Payudara J. 2009; 8:200-202. [PubMed]
Horner B, El-Muttardi N, Mercer D. Pioderma gangrenosum rumit
pengurangan payudara bilateral. Br J Plast Surg. 2004; 8:679-681. doi:
10.1016/j.bjps.2004.03.004. [PubMed] [Palang Ref]
CC lama, Jessop J, Young M, Holt PJ. Meminimalkan risiko pasca operasi
pioderma gangrenosum. Br J Dermatol. 1992; 8:45-48. [PubMed]
Gettler S, M Rothe, Grin C, pengobatan Grant-Kels J. Optimal dari
pioderma gangrenosum. Am J Clin Dermatol. 2003; 8:597-608. doi:
10.2165/00128071-200304090-00002. [PubMed] [Palang Ref]
Duguid CM, O'Loughlin S, Otridge B, Powell FC. Paraneoplastic
gangrenosum pioderma. Australas J Dermatol. 1993; 8:17-22. doi:
10.1111/j.1440-0960.1993.tb00841.x. [PubMed] [Palang Ref]

Medical Journal, Angkatan Bersenjata India

Elsevier
Bawaan miliaria crystallina - Sebuah dilema diagnostik
Sudhir Dixit, Ashish Jain, [...], dan V.K. Khurana
Informasi artikel tambahan
abstrak

Miliaria crystallina adalah sementara, obstruksi dangkal ekrin saluran

keringat sehingga berkembang pesat vesikel noninflamasinya. Penyakit ini
sering ditemukan di tempat yang panas, lembab, iklim tropis dan pada
periode neonatal, kemungkinan besar karena kurangnya pematangan
saluran keringat selama beberapa hari pertama setelah kelahiran. Hal ini
jarang hadir saat melahirkan dan tetap menjadi dilema diagnostik untuk
neonatologist. Kami melaporkan kasus langka "Bawaan miliaria
crystallina" yang hadir pada saat lahir.
Kata Kunci: miliaria, kongenital, lesi vesikular
pengantar
Vesikular dan gangguan pustular periode neonatal yang tidak biasa.
Kebanyakan dari mereka tidak berbahaya; akan tetapi penting untuk
membedakan ini dari berpotensi disorders.1 menular dan tidak menular
yang mengancam jiwa Miliaria crystallina umumnya dipertimbangkan
dalam diagnosis diferensial penyakit bulosa pada bayi baru lahir. Studi
melaporkan kejadian 1,3% pada neonatus mengembangkan lesi kulit
dalam waktu 48 jam dari life.2 ini adalah sementara, obstruksi dangkal
ekrin saluran keringat sehingga berkembang pesat vesikel
noninflamasinya. Penyakit ini sering ditemukan di antara neonatus di iklim
yang panas, lembab dan tropis. Sebagian ada kurangnya pematangan
saluran keringat selama beberapa hari pertama setelah kelahiran, tapi
jarang hadir di delivery.3 Kami melaporkan kasus langka "Bawaan miliaria
crystallina" menghadirkan saat lahir.
laporan kasus
Seorang bayi perempuan jangka, sesuai untuk usia kehamilan dengan
berat lahir 2,65 kg lahir dari primi gravida, ibu 28 tahun. Dia adalah hasil
dari persalinan pervaginam normal. Skor Apgar adalah 8, 9, 9 pada 1, 5
dan 10 menit masing-masing. Pemeriksaan fisik dan sistemik umum masih
dalam batas normal, kecuali bahwa kulit ditutupi dengan lesi vesikular
ukuran variabel dan distribusi, yang berisi cairan bening.
Tidak ada eritema terkait (Gambar 1).
Gambar. 1
Bayi dengan Miliaria crystallina saat lahir.
Antenatal, ibu adalah kasus memesan dan tidak mengambil obat apapun.
Namun ibu mengalami demam mulai 3 hari sebelum awal persalinan. Pada

penyelidikan TLC nya 12.000 dan CRP positif. LFT, KFT adalah dalam
batas-batas dan kultur darah steril normal. Sang ibu diobati dengan
antibiotik dan antipiretik. Meskipun pengobatan di atas, suhu ibu di ruang
kerja adalah 101 F. Saat lahir kulit bayi ditutupi dengan kecil, vesikel
berjarak dekat tersebar di wajah, leher, lengan atas dan batang. Vesikel
yang jelas, 1-3 mm dengan diameter keperakan ke permukaan bersinar.
Tes Tzanck tidak menunjukkan sel acantholytic atau neutrofil dan gram
stain tidak menemukan bakteri. CBC, CRP dan kultur darah negatif. Layar
TORCH negatif.
Atas dasar lesi kulit yang khas, diagnosis miliaria crystallina dibuat melalui
konsultasi dengan dokter kulit. Bayi dipindahkan ke lingkungan yang lebih
dingin. Selama 2 hari berikutnya tidak ada vesikel baru meletus. Pada
vesikel 4 hari kering dan oleh kulit hari ke-7 normal dalam penampilan.
diskusi
Miliaria hasil dari retensi keringat di saluran ekrin tersumbat sebagai
akibat dari colokan keratinous. Hasil tekanan retrograde pada pecahnya
saluran dan kebocoran keringat ke dalam epidermis dan / atau dermis.
Miliaria terjadi dalam 3 bentuk; miliaria crystallina, rubra, dan profunda.
Klasifikasi ini didasarkan pada tingkat penyumbatan keringat ekrin duct.4
Dalam Miliaria crystallina (sudamina), obstruksi saluran ekrin sangat
dangkal yaitu dalam stratum korneum dan biasanya terjadi dengan
penyakit demam atau setelah terbakar sinar matahari, paling sering di
tempat yang panas dan kondisi iklim lembab. Anak-anak sangat beresiko.
Klinis tembus, vesikel beratap tipis diameter mm 1-2 tanpa halo inflamasi
yang diamati. Jika dibuka dengan jarum, suatu cairan berair jernih. Lesi
berkembang secara istimewa pada leher dan ketiak. Pada periode
neonatal, wajah dapat terlibat juga. Hal ini terjadi pada bayi kami, juga.
Vesikel di lokasi ini dapat memiliki kemilau keperakan. Hal ini tidak
didasarkan pada leukosit masuknya seperti dalam Miliaria rubra, tetapi
disebabkan oleh corneocytes dalam blister, dan dikenal sebagai varian
yang disebut miliaria crystallina alba. Atap tipis yang menutupi lecet
mudah pecah dan kulit membersihkan sesudahnya, meninggalkan
dangkal, desquamation.5 branny
Diagnosis banding terik neonatal meliputi beberapa dermatosis menular
dan bulosa. Herpes simplex dan varicella menunjukkan vesikel serosa.
Eritema toxicum neonatorum, neonatal pustular melanosis, dan

acropustulosis acara anak pustula. Infeksi stafilokokus menyebabkan

staphylococcal scalded syndrome atau lecet besar yang berbeda dengan
jelas dari vesikel menit di miliaria. Perbedaan yang paling penting tetap
bahwa, dalam Miliaria crystallina, keringat di lepuh tidak kekuningan, tapi
jelas seperti air. Oleh karena itu, warna dan bentuk lepuh memungkinkan
diagnosis klinis pasti.
Terapi miliaria crystallina sederhana. Satu-satunya pengobatan dan
pencegahan yang efektif adalah menghindari berkeringat lebih lanjut.
Beberapa jam di lingkungan yang dingin akan membawa bantuan.
Pencegahan dan pengobatan modalitas lainnya termasuk menghindari
pakaian yang berlebihan, gesekan dari pakaian dan penggunaan
berlebihan dari sabun. Persiapan antibakteri topikal memiliki beberapa
peran dalam mencegah infeksi sekunder sering terjadi pada miliaria.
Calamine lotion diikuti dengan emolien hambar juga bermanfaat.
Pada bayi baru lahir dan anak-anak, penyakit ini lebih sering. Dengan
demikian, dalam sebuah studi retrospektif dari Jepang, termasuk 5387
bayi di bangsal bayi baru lahir, crystallina miliaria terlihat pada 4,5%,
dengan kejadian puncak sekitar 6 dan hari postnatal 7. Dalam seri besar
ini, kejadian itu tergolong sangat jarang sebelum hari 4.6 Dalam sebuah
studi India selama 7 bulan, 131 neonatus ditemukan memiliki crystallina
miliaria dalam waktu 48 jam dari birth.7 Sebagian besar artikel
berpendapat bahwa penyakit ini tidak terjadi pada delivery.8 disarankan
bahwa saluran disrupture adalah penyebab langsung dari miliaria.9 hidrasi
corneocytes bervariasi dengan tingkat kelembaban dan suhu lingkungan.
Pada pasien kami, demam ibu mungkin telah memicu.
Hanya ada 3 laporan sejauh ini dalam literatur tentang bawaan miliaria
crystallina, dua di newborns10 hitam, 11 dan hanya satu putih yang baru
lahir seperti dalam case.3 kami yang sama belum dilaporkan dalam
literatur India. Menurut Straka et al, 10 yang menggambarkan kasus
pertama, penyakit yang paling mungkin berasal dari saluran keringat
belum matang dalam minggu pertama kehidupan. Mungkin oklusi saluran
keringat telah terbentuk sudah dalam rahim seperti dalam kasus kami.
Konflik kepentingan
Semua penulis memiliki tidak ada untuk menyatakan.
informasi Pasal
Med J Angkatan Bersenjata India. Oktober 2012; 68 (4): 386-388.

. Diterbitkan online Jul 17, 2012 doi: 10.1016/j.mjafi.2012.01.004

PMCID: PMC3862747
Sudhir Dixit, seorang Ashish Jain, b, Suhas Datar, c dan VK Khuranad
aSenior Resident, Neonatologi Divisi, Departemen Ilmu Kesehatan Anak,
Rumah Sakit Hindu Rao, Malka Gunj, Delhi, India
bNeonatologist, Neonatologi Divisi, Departemen Ilmu Kesehatan Anak,
Rumah Sakit Hindu Rao, Malka Gunj, Delhi, India
cSenior Spesialis, Neonatologi Divisi, Departemen Ilmu Kesehatan Anak,
Rumah Sakit Hindu Rao, Malka Gunj, Delhi, India
dDermatologist, Departemen Kulit, Rumah Sakit Hindu Rao, Malka Gunj,
Delhi, India
Ashish Jain: ni.oc.oohay @ 0002niajhsihsard
Penulis yang sesuai. Tel:.. +91 9810694789 Email: ni.oc.oohay @
0002niajhsihsard
Diterima 11 Juli 2011; Diterima 5 Januari 2012.
Hak Cipta 2012 Diterbitkan oleh Elsevier BV atas nama Direktur
Jenderal, Angkatan Bersenjata Pelayanan Medis.
Artikel dari Medical Journal, Angkatan Bersenjata India disediakan di sini
courtesy of Elsevier
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neonatus. Curr Opin Pediatr. 1997; 9:396-405. [PubMed]
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