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Solitary Type of Congenital Self-healing Reticulohistiocytosis.
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PMID:  22028569     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Congenital self-healing reticulohistiocytosis is a rare, congenital, benign, self-healing variant of Langerhans cell histiocytosis. It usually appears as multiple papules or nodules; however, occurrence of the solitary type is very rare. We report on a case of solitary congenital self-healing reticulohistiocytosis in a 29-day-old girl who presented with a papule on her sole. Two months later, the lesion regressed with a slight scar. Based upon clinical and histologic findings, we made a diagnosis of solitary congenital self-healing reticulohistiocytosis. In this report, we summarized reported cases of solitary congenital self-healing retioculohistiocytosis in Korea with a review of the literature.
Gantsetseg Dorjsuren; Hee Jung Kim; Jin Young Jung; Byung Gi Bae; Ju Hee Lee
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Publication Detail:
Type:  Journal Article     Date:  2011-09-30
Journal Detail:
Title:  Annals of dermatology     Volume:  23 Suppl 1     ISSN:  2005-3894     ISO Abbreviation:  Ann Dermatol     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-10-26     Completed Date:  2011-11-10     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  8916577     Medline TA:  Ann Dermatol     Country:  Korea (South)    
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Languages:  eng     Pagination:  S4-7     Citation Subset:  -    
Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
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Journal Information
Journal ID (nlm-ta): Ann Dermatol
Journal ID (publisher-id): AD
ISSN: 1013-9087
ISSN: 2005-3894
Publisher: Korean Dermatological Association; The Korean Society for Investigative Dermatology
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Copyright © 2011 Korean Dermatological Association; The Korean Society for Investigative Dermatology
Received Day: 07 Month: 4 Year: 2010
Revision Received Day: 10 Month: 6 Year: 2010
Accepted Day: 10 Month: 6 Year: 2010
Print publication date: Month: 9 Year: 2011
Electronic publication date: Day: 30 Month: 9 Year: 2011
Volume: 23 Issue: Suppl 1
First Page: S4 Last Page: S7
ID: 3199419
PubMed Id: 22028569
DOI: 10.5021/ad.2011.23.S1.S4

Solitary Type of Congenital Self-healing Reticulohistiocytosis
Gantsetseg Dorjsuren, M.D.A1
Hee Jung Kim, M.D.A1
Jin Young Jung, M.D.A1
Byung Gi Bae, M.D.A1
Ju Hee Lee, M.D., Ph.D.A1
Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
Correspondence: Corresponding author: Ju Hee Lee, M.D., Ph.D., Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemoon-gu, Seoul 120-752, Korea. Tel: 82-2-2228-2080, Fax: 82-2-393-9157,


Langerhans cell histiocytosis (LCH) is a disease of dysregulated proliferation of Langerhans cells with subsequent organ involvement. Congenital self-healing reticulohistiocytosis (CSHRH) is a congenital variant of LCH, which does not show systemic involvement1. Clinical presentation typically consists of multiple papules or nodules, which show spontaneous involution within a few months. Due to its poorly specific clinical features and self-limiting character, solitary CSHRH is much rarer and difficult to diagnose. Since it is prone to be confused with other dermatologic tumor conditions, dermatologists should have an understanding of the clinical and histologic findings.

In Korea, just two cases of solitary CSHRH have been reported2,3. Herein, we report on an additional case of solitary CSHRH.


A 29-day-old girl presented with a solitary skin colored papule with crust on her left sole since birth. She was delivered at term by vaginal delivery following a normal pregnancy to a primipara primigravida mother. Overall, she was healthy, without perinatal problems. There was no Ed-highlight-Please review.

Family history. Physical examination revealed a domeshaped, skin colored papule measuring 7 mm, with crust, on her left sole (Fig. 1A). However, no organomegaly or superficial palpable lymph nodes were observed. Clinical diagnoses, including hemangioma, juvenile xanthogranuloma, and congenital self-healing reticulohistiocytosis were made and skin biopsy was performed on the sole. Atrophic epidermis and effacement of rete ridges were observed (Fig. 2A). A dense infiltrate of histiocytic cells admixed with numerous eosinophils and some scattered lymphocytes were observed. No multinucleated giant cells were observed in our histologic section (Fig. 2B). Histiocytes had a large, vesicular, and some kidney-shaped nuclei with prominent nucleoli. On immunohistiochemical staining with CD1a and S-100, the majority of tumor cells showed strong reactivity (Fig. 3). Ultrastructural studies were not performed. Laboratory studies, including complete blood count, coagulation profile, serum protein electrophoresis, chemical battery, urinalysis, VDRL, congenital infection studies (TORCH), and chest radiography were normal. Two months later, the lesion had completely regressed, leaving a scar (Fig. 1B). At 1 year after diagnosis, no recurrence or systemic involvement has been observed. Based upon clinical, histopathologic, and laboratory findings, a diagnosis of congenital self-healing reticulohistiocytosis was made.


CSHRH, first described by Hashimoto-Pritzker in 1973, is a rare and self-limited form of LCH1. The disease is characterized by multiple papules or nodules, which have a tendency to show spontaneous regression. Lesions are usually multiple, but, more rarely, solitary. The first case of solitary CSHRH was reported by Berger et al.4 in 1986. Since then, several cases of a solitary form have been reported. Bernstein et al.5 reported that solitary CSHRH appeared to contribute to approximately 25% of CSHRH cases. However, this disease shows poorly specific clinical features and spontaneous regression within a few months; therefore, the real incidence of solitary CSHRH might be underreported6. Two cases of solitary CSHRH have been reported in Korea2,3. Reported cases are summarized in Table 1.

Clinical manifestations of CSHRH are polymorphic, presenting as papules, nodules, crusts, vesicles, and, rarely, hemorrhagic bullae, which can sometimes become ulcerated, necrotic, and crusted7-9. Lack of systemic involvement, as well as spontaneous resolution of cutaneous lesions, are essential for a diagnosis of CSHRH. The lesions regress within a mean period of 15 weeks and recurrence has never been reported10. Histopathologically, there are dense intradermal infiltrations of histiocytes showing abundant eosinophilic cytoplasm and kidney-shaped or indented nuclei associated with lymphocytes and eosinophils. These histiocytes show strong expression of S-100 protein and CD1a, markers of Langerhans cells (LC)11,12.

Differential diagnosis includes hemangioma, juvenile xanthogranuloma, Spitz nevi, mastocytoma, and histiocytic disorders. Those diseases can be differentiated by histopathologic findings. In cases of juvenile xanthogranuloma, histiocytes can be differentiated with immunohistochemical staining, such as S-100 protein and CD1a. Among histiocytic disorders, differentiation of indeterminate cell histiocytoma (ICH) from CSHRH is difficult. ICH is characterized by positive immunohistochemical staining for S-100 protein and CD1a, but can be differentiated from CSHRH by absence of Birbeck granules13. Electron microscopic examination of CSHRH reveals Birbeck granules and laminated dense bodies in 10 to 25% of histiocytes14. In our case, we did not perform an electron microscopic examination, but made a diagnosis of CSHRH, since, ICH is much more rare than CSHRH, has tendency to occur in adults, and only a few cases have been reported as a solitary variant7,15.

The pathogenesis of the self-limiting nature of CSHRH remains to be elucidated. Weiss et al.16 have studied various dendritic cell markers in CSHRH. With immunohistochemistry, tumor cells showed positive staining for S-100 protein, CD1a, and HLA-DR. However, tumor cells showed negative staining for Langerin and CD68. They speculated that CSHRH should be comprised of activated mature LCs, because CD68 expression is lost and Langerin staining is decreased in the process of LCs and dendritic cell maturation17,18. The authors explained the self-regressing character of CSHRH as the tumor cells of CSHRH eventually becoming apoptotic on terminal maturation, which is the natural course of LC activation16.

No treatment is required for CSHRH; however, clinical and laboratory monitoring is mandatory. To date, there have been no reports of systemic involvement or complications in solitary CSHRH. However, there has been one case of a girl with CSHRH, who had sparse skin lesions resolving at 1 and 1/2 years of age, but developed diabetes insipidus at the age of 4 years19. Although, there is no evidence of an association of CSHRH with diabetes insipidus in that case, regular follow up will be important for management. Zunino-Goutorbe et al.11 recommended regular physical examination for at least 2 years. They proposed performance of simple laboratory tests and radiographs initially, with repetition of laboratory analyses, including inflammation, hepatic, and differential blood counts at 6 months, and imaging studies only if required by clinical manifestations. We are now following up the patient for one year; however, there is no evidence of systemic involvement and recurrence.

Herein, we report on an additional case of solitary CSHRH with a review of literature.

1. Hashimoto K,Pritzker MS. Electron microscopic study of reticulohistiocytoma. An unusual case of congenital, self-healing reticulohistiocytosisArch DermatolYear: 19731072632704346639
2. Chun SI,Song MS. Congenital self-healing reticulohistiocytosis-report of a case of the solitary type and review of the literatureYonsei Med JYear: 1992331941981413898
3. Shin MS,Park HJ,Choi YJ,Park MY. A case of solitary type of congenital self-healing reticulohistiocytosisKorean J DermatolYear: 200846411413
4. Berger TG,Lane AT,Headington JT,Hartmann K,Burrish G,Levin MW,et al. A solitary variant of congenital self-healing reticulohistiocytosis: solitary Hashimoto-Pritzker diseasePediatr DermatolYear: 198632302363523469
5. Bernstein EF,Resnik KS,Loose JH,Halcin C,Kauh YC. Solitary congenital self-healing reticulohistiocytosisBr J DermatolYear: 19931294494548217762
6. Kapur P,Erickson C,Rakheja D,Carder KR,Hoang MP. Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): ten-year experience at Dallas children's medical centerJ Am Acad DermatolYear: 20075629029417224372
7. Jang KA,Ahn SJ,Choi JH,Sung KJ,Moon KC,Koh JK. Histiocytic disorders with spontaneous regression in infancyPediatr DermatolYear: 20001736436811085663
8. Higgins CR,Tatnall FM,Leigh IM. Vesicular Langerhans cell histiocytosis-an uncommon variantClin Exp DermatolYear: 1994193503527955483
9. Inuzuka M,Tomita K,Tokura Y,Takigawa M. Congenital self-healing reticulohistiocytosis presenting with hemorrhagic bullaeJ Am Acad DermatolYear: 2003485 SupplS75S7712734483
10. Kanitakis J,Zambruno G,Schmitt D,Cambazard F,Jacquemier D,Thivolet J. Congenital self-healing histiocytosis (Hashimoto-Pritzker). An ultrastructural and immunohistochemical studyCancerYear: 1988615085163276380
11. Zunino-Goutorbe C,Eschard C,Durlach A,Bernard P. Congenital solitary histiocytoma: a variant of Hashimoto-Pritzker histiocytosis. A retrospective study of 8 casesDermatologyYear: 200821611812418216473
12. Zwerdling T,Konia T,Silverstein M. Congenital, single system, single site, Langerhans cell histiocytosis: a new case, observations from the literature, and management considerationsPediatr DermatolYear: 20092612112619250436
13. Saijo S,Hara M,Kuramoto Y,Tagami H. Generalized eruptive histiocytoma: a report of a variant case showing the presence of dermal indeterminate cellsJ Cutan PatholYear: 1991181341361856341
14. Willman CL,Busque L,Griffith BB,Favara BE,McClain KL,Duncan MH,et al. Langerhans'-cell histiocytosis (histiocytosis X)-a clonal proliferative diseaseN Engl J MedYear: 19943311541608008029
15. Sidoroff A,Zelger B,Steiner H,Smith N. Indeterminate cell histiocytosis--a clinicopathological entity with features of both X- and non-X histiocytosisBr J DermatolYear: 19961345255328731682
16. Weiss T,Weber L,Scharffetter-Kochanek K,Weiss JM. Solitary cutaneous dendritic cell tumor in a child: role of dendritic cell markers for the diagnosis of skin Langerhans cell histiocytosisJ Am Acad DermatolYear: 20055383884416243135
17. Valladeau J,Duvert-Frances V,Pin JJ,Dezutter-Dambuyant C,Vincent C,Massacrier C,et al. The monoclonal antibody DCGM4 recognizes Langerin, a protein specific of Langerhans cells, and is rapidly internalized from the cell surfaceEur J ImmunolYear: 1999292695270410508244
18. Geissmann F,Lepelletier Y,Fraitag S,Valladeau J,Bodemer C,Debré M,et al. Differentiation of Langerhans cells in Langerhans cell histiocytosisBloodYear: 2001971241124811222366
19. Esterly NB,Maurer HS,Gonzalez-Crussi F. Histiocytosis X: a seven-year experience at a children's hospitalJ Am Acad DermatolYear: 1985134814963877082

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Keywords: Congenital self-healing reticulohistiocytosis, Solitary.

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