Hashimoto Pritzer

7252019 Hashimoto Pritzer

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An Bras Dermatol 201388(6)1001-3

1001

983155

Virginia Vinha Zanuncio1 Luciana Rabelo de Carvalho1

Antocircnio Carlos Martins Guedes2 Claacuteudia Maacutercia Resende Silva3

Bernardo Gontijo2

Received on 17012013Approved by the Advisory Board and accepted for publication on 25032013 Work performed at the Hospital das Cliacutenicas of the Federal University of Minas Gerais (HC-UFMG) ndash Belo Horizonte (MG) Brazil

Conflict of interest NoneFinancial funding None

1 Medical Resident Dermatology Unit Hospital das Cliacutenicas Federal University of Minas Gerais (HC-UFMG) - Belo Horizonte (MG) Brazil2 Specialist in Dermatology and Pediatrics Master in Dermatology Federal University of Minas Gerais (UFMG) Medical Clinic of Pediatric Dermatology

Service Hospital das Cliacutenicas Federal University of Minas Gerais (HC-UFMG) ndash Belo Horizonte (MG) Brazil3 MD PHD Associate Professor of Dermatology Federal University of Minas Gerais School of Medicine (UFMG) ndash Belo Horizonte (MG) Brazil

copy2013 by Anais Brasileiros de Dermatologia

CASE REPORTA 1-month-old boy born at term after an

uneventful pregnancy with a birth weight of 3480gFirst child of healthy and unrelated parents At birthhis mother noticed a 1 x 15 cm erythematous infiltrat-ed perianal plaque that rapidly progressed to ulcera-tion (Figure 1) The patient was otherwise healthy Asecond evaluation at age two and a half monthsshowed a complete and spontaneous resolution of the

lesion (Figure 2) Serologic test for syphilis (VDRL)was negative both for the mother and the patientHistopathological exam revealed a dermal infiltratewith predominance of large round histiocytic cellswith dense eosinophilic cytoplasm with ground glassappearance and eccentric reniform nuclei (Figure3A) Immunohistochemical staining was positive forS100 and CD1a (Figures 3B and 3C) Routine laborato-ry workup and radiographs of chest skull pelvis andlong bones were within normal ranges The patientremains asymptomatic with no signs of recurrence

DISCUSSIONLangerhans cell histiocytosis (LCH) is a rare and

clinically heterogeneous condition with monoclonalproliferation of this type of histiocyte1-3 Four clinicalsubtypes which share significant clinical overlap areknown Letterer-Siwe disease Hand-Schuumlller-Christian disease eosinophilic granuloma and congen-ital self-healing reticulohistiocytosis (CSHRH) orHashimoto-Pritzker disease145 Histopathological andimmunohistochemical studies are essential for diagno-sis showing a dermal infiltrate with predominance oflarge round histiocytic cells with dense eosinophiliccytoplasm with eccentric reniform nuclei and

Langerhans cells stain positive for S100 and CD1a(Figure 3A)1-8

Case for diagnosisCaso para diagnoacutestico

WHAT IS YOUR DIAGNOSIS

DOI httpdxdoiorg101590abd1806-484120132451

FIGURE 1Erythematous infiltra-ted perianalplaque thatrapidly pro-gressed toulceration

FIGURE 2A secondevaluation attwo and ahalf monthsof age sho-wed a com-plete andspontaneous

resolution ofthe lesion



1002 Zanuncio VV Carvalho LR Guedes ACM Silva CMR Gontijo B


Birbeck granules on electron microscopy arespecific for Langerhans cells1-9 Once the diagnosis isestablished the extent of the disease must be carefullyevaluated Patients with systemic involvement mayhave a mortality rate as high as 209

CSHRH carries a good prognosis147 Its trueincidence may be underestimated since spontaneousresolution often occurs before assessment by a derma-tologist168 CSHRH classic features include 1) painlesspapules nodules or plaques present at birth or duringthe first days of life 2) spontaneous regression inmonths and 3) proliferation of histiocytes with fea-tures of Langerhans cell810

Most patients present with multiple lesionsbut solitary lesions are seen in 25 of cases and spon-taneous regression takes place in two to threemonths4-5 Development of lesions in adulthood as

well as pulmonary and ocular involvement areextremely rare9 CSHRH may eventually show multi-system recurrence with considerable increase in mor-bidity and mortality239

Routine laboratory workup should include fullblood count and ESR electrolytes urea liver functiontests C-reactive protein and radiographic study ofchest skull pelvis and long bones39 Differential diag-nosis comprises vesicular and pustular neonatal erup-tions such as congenital candidiasis herpes simplexvaricela Listeria monocytogenes infection and neonatalhemangiomatosis78

Since the differentiation between CSHRH andother forms of LCH cannot be made solely on clinical andhistopathological grounds patients must have a multi-disciplinary follow-up since recurrence and multisys-temic involvement are reported in 5-10 of all cases58

983153

Abstract Langerhans cell histiocytosis is a rare clinically heterogeneous desease Since there is considerable clini-cal overlap among the four described variants (Hand-Schuumlller-Christian eosinophilic granuloma Letterer-Siwe andHashimoto-Pritzker) the concept of spectral disease applies to this entity The Hashimoto-Pritzker variant was firstdescribed in 1973 Characteristically it is present at birth or during the first days of life impairment is limited to theskin and prognosis is favorable with spontaneous resolution We report a newborn male patient with Hashimoto-

Pritzker disease presenting as a S100 + and CD1a + single congenital perianal lesion with rapid involution in twomonthsKeywords Histiocytosis Langerhans-cell Immunohistochemistry Prognosis

Resumo A histiocitose de ceacutelulas de Langerhans eacute doenccedila rara clinicamente heterogecircnea Como haacute consideraacute-vel sobreposiccedilatildeo cliacutenica entre as quatro variantes descritas (Hand-Schuumlller-Christian granuloma eosinofiacutelicoLetterer-Siwe e Hashimoto-Pritzker) o conceito de doenccedila espectral aplica-se a esta entidade A variante deHashimoto-Pritzker foi descrita em 1973 Classicamente estaacute presente ao nascimento ou nos primeiros dias devida acomete exclusivamente a pele e o prognoacutestico eacute favoraacutevel com regressatildeo espontacircnea Relatamos caso depaciente receacutem-nascido masculino com doenccedila de Hashimoto-Pritzker que se apresenta com positividade paraS100 e CD1a observando-se lesatildeo congecircnita uacutenica perianal com involuccedilatildeo raacutepida em dois mesesPalavras-chave Histiocitose de ceacutelulas de Langerhans Imunoistoquiacutemica Prognoacutestico

FIGURE 3 A Dermal infiltrate with prredominance of large round histiocytic cells with dense eosinophilic cytoplasm with ground glassappearance and eccentric reniform nuclei B and C Immunohistochemical staining was positive for S100 and CD1a

A B C



How to cite this article Zanuncio VV Carvalho LR Guedes ACM Silva CMR Gontijo B Case for diagnosisCongenital Self-Healing Reticulohistiocytosis An Bras Dermatol 201388(6)1001-3

M AILING ADDRESSVirginia Vinha Zanuncio

Alameda Aacutelvaro Celso 55 - Santa Efigecircnia30150-260 - Belo Horizonte - MG

BrazilE-mail vivizanuncioyahoocombr

REFERENCESJensen ML Bygum A Clemmensen O Fenger-Gron J Congenital self-healing reti-1

culohistiocytosis - an impor tant diagnostic Acta Paediatr 2011100784-6

Querings K Starz H Balda BR Clinical spectrum of cutaneous Langerhans cell2

histiocytosis mimicking various diseases Acta Derm Venereol 20068639-43

Ricart J Jimenez A Marquina A Villanueva A Congenital self-healing reticulohis-3

tiocytosis repor t of a case and review of the literatu re Acta Paediatr

200493426-9Huang CY Chao SC Ho SF Lee JY Congenital self-healing reticulohistiocytosis4

mimicking diffuse neonatal hemangiomatosis Dermatology 2004208138-41

Parentin F Ventura G Pastore S Kiren V Bibalo C Pensiero S et al A singular 5

case of congenital self-healing histiocytosis with skin liver and atypical eye invol-

vement Ocul Immunol Inflamm 201119337-9

Kapur P Erickson C Rakheja D Carder KR Hoang MP Congenital self -healing reti-6

culohistiocytosis (Hashimoto-Pritzker disease) Ten-year experience at Dallas

Childrens Medical Center J Am Acad Dermatol 200756290-4

Ersoy-Evans S Gursoy T Yigit S Akcoren Z Sahin S Solitary congenital self-hea-7

ling Reticulohistiocytosis in monozygotic twins Pediatr Dermatol 200623273-5

Orle J Moacutesca AM Sousa MA Lima CM Adriano AR Rezende PM Congenital self8

healing reticulohistiocytosis in a newborn (Hashimoto Pritzker) An Bras Dermatol

201186785-8

Lau L Krafchik B Trebo MM Weitzman S Cutaneous Langerhans cell histiocyto-9

sis in children under one year Pediatr Blood Cancer 20064666-71

Nakahigashi K Ohta M Sakai R Sugimoto Y Ikoma Y Horiguchi Y Late-onset self-10

healing reticulohistiocytosis Pediatric case of Hashimoto-Pritzker typeLangerhans cell histiocytosis J Dermatol 200734205-9

Case for diagnosis 1003




1002 Zanuncio VV Carvalho LR Guedes ACM Silva CMR Gontijo B


Birbeck granules on electron microscopy arespecific for Langerhans cells1-9 Once the diagnosis isestablished the extent of the disease must be carefullyevaluated Patients with systemic involvement mayhave a mortality rate as high as 209

CSHRH carries a good prognosis147 Its trueincidence may be underestimated since spontaneousresolution often occurs before assessment by a derma-tologist168 CSHRH classic features include 1) painlesspapules nodules or plaques present at birth or duringthe first days of life 2) spontaneous regression inmonths and 3) proliferation of histiocytes with fea-tures of Langerhans cell810

Most patients present with multiple lesionsbut solitary lesions are seen in 25 of cases and spon-taneous regression takes place in two to threemonths4-5 Development of lesions in adulthood as

well as pulmonary and ocular involvement areextremely rare9 CSHRH may eventually show multi-system recurrence with considerable increase in mor-bidity and mortality239

Routine laboratory workup should include fullblood count and ESR electrolytes urea liver functiontests C-reactive protein and radiographic study ofchest skull pelvis and long bones39 Differential diag-nosis comprises vesicular and pustular neonatal erup-tions such as congenital candidiasis herpes simplexvaricela Listeria monocytogenes infection and neonatalhemangiomatosis78

Since the differentiation between CSHRH andother forms of LCH cannot be made solely on clinical andhistopathological grounds patients must have a multi-disciplinary follow-up since recurrence and multisys-temic involvement are reported in 5-10 of all cases58

983153

Abstract Langerhans cell histiocytosis is a rare clinically heterogeneous desease Since there is considerable clini-cal overlap among the four described variants (Hand-Schuumlller-Christian eosinophilic granuloma Letterer-Siwe andHashimoto-Pritzker) the concept of spectral disease applies to this entity The Hashimoto-Pritzker variant was firstdescribed in 1973 Characteristically it is present at birth or during the first days of life impairment is limited to theskin and prognosis is favorable with spontaneous resolution We report a newborn male patient with Hashimoto-

Pritzker disease presenting as a S100 + and CD1a + single congenital perianal lesion with rapid involution in twomonthsKeywords Histiocytosis Langerhans-cell Immunohistochemistry Prognosis

Resumo A histiocitose de ceacutelulas de Langerhans eacute doenccedila rara clinicamente heterogecircnea Como haacute consideraacute-vel sobreposiccedilatildeo cliacutenica entre as quatro variantes descritas (Hand-Schuumlller-Christian granuloma eosinofiacutelicoLetterer-Siwe e Hashimoto-Pritzker) o conceito de doenccedila espectral aplica-se a esta entidade A variante deHashimoto-Pritzker foi descrita em 1973 Classicamente estaacute presente ao nascimento ou nos primeiros dias devida acomete exclusivamente a pele e o prognoacutestico eacute favoraacutevel com regressatildeo espontacircnea Relatamos caso depaciente receacutem-nascido masculino com doenccedila de Hashimoto-Pritzker que se apresenta com positividade paraS100 e CD1a observando-se lesatildeo congecircnita uacutenica perianal com involuccedilatildeo raacutepida em dois mesesPalavras-chave Histiocitose de ceacutelulas de Langerhans Imunoistoquiacutemica Prognoacutestico

FIGURE 3 A Dermal infiltrate with prredominance of large round histiocytic cells with dense eosinophilic cytoplasm with ground glassappearance and eccentric reniform nuclei B and C Immunohistochemical staining was positive for S100 and CD1a

A B C

























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Hashimoto Pritzer