Print version ISSN 0365-0596
An. Bras. Dermatol. vol.86 no.4 Rio de Janeiro July/Aug. 2011
Joana OrleI; Ana Maria MóscaII; Maria Auxiliadora Jeunon SousaIII; Cíntia Maria Oliveira LimaIV; André Ricardo AdrianoV; Patrícia Makino RezendeV
IGraduate work in Dermatology at the General Polyclinic of Rio de Janeiro - "Carlos Chagas" Medical Graduate School Institute (Policlinica Geral do Rio de Janeiro - Instituto de Pos Graduacao Medica Carlos Chagas - PGRJ - IPGMCC) - Rio de Janeiro (RJ), Brazil
IIDermatologist and Pediatrician; Assistant Professor of Graduate Course in Dermatology of the General Polyclinic of Rio de Janeiro - "Carlos Chagas" Medical Graduate School Institute(Policlinica Geral do Rio de Janeiro - Instituto de Pos Graduacao Medica Carlos Chagas - PGRJ - IPGMCC) - Rio de Janeiro (RJ), Brazil
IIIDermatologist and Dermatopathologist; Retired Professor of the Dermatology Service of the Rio de Janeiro State University (Universidade do Estado do Rio de Janeiro - UERJ) - Rio de Janeiro (RJ), Brazil
IVStudent of the Specialization Course in Dermatology, School of Medicine and Teaching Hospital "Clementino Fraga Filho (Faculdade de Medicina e Hospital Universitario Clementino Fraga Filho - Universidade Federal do Rio de Janeiro (HUCFF - UFRJ) - Rio de Janeiro (RJ), Brazil
VGraduate student of the Institute of Dermatology "Prof. Rubem David Azulay (Instituto de Dermatotologia Prof. Rubem David Azulay) - Rio de Janeiro (RJ), Brazil
Congenital self-healing reticulohistiocytosis is the benign spectrum of Langerhans Cell Histiocytosis, characterized by cutaneous lesions at birth or in the neonatal period, absence of systemic manifestations and spontaneous resolution of clinical status. Despite the benign and often self-resolving course in most patients, studies show that in some cases there may be metastasis or recurrence of the disease, emphasizing that the clinical course is variable, requiring long-term follow-up. The monitoring of the patient for a long period is important to detect possible systemic involvement, as there is a report of recurrence involving the skin, mucosa, bone and pituitary gland.
Keywords: Histiocytosis; Histiocytosis, Langerhans-Cell; Histiocytosis, Non-Langerhans-Cell
Langerhans cell histiocytosis (LCH) is a generic term that encompasses clinically distinct diseases, but that present as a point in common clonal proliferation of Langerhans cells with positive Birbeck granules and S100/CD1A in immunohistochemistry tests. 1 It is considered a rare disease, with an annual incidence rate of three to four cases per million in pediatric patients. 2
The most common clinical manifestations are osteolytic lesions, lymphadenomegaly and skin lesions. The skin lesions are frequently the first manifestation of LCH and cutaneous involvement is observed as the only disease site in approximately 10% of cases. 3,4
Congenital self-healing reticulohistiocytosis of Hashimoto-Pritzker is the benign spectrum of Langerhans cell histiocytosis, characterized by the presence of skin lesions at birth or in the neonatal period, absence of systemic manifestations and spontaneous resolution of the clinical picture. 5
A full-term female infant, delivered by cesarean section, presented papulonodular reddish-brown erythematous lesions and crusts of different sizes located predominantly on the face, distributed on the trunk, upper and lower limbs (Figures 1 and 2). The physical examination did not otherwise reveal any abnormalities. Routine laboratory tests did not show alterations. The serological tests done for the TORCH group (toxoplasmosis, rubella, cytomegalovirus, herpes and syphilis) were negative. Skull, chest and long bone X-rays and abdominal ultrasound were normal. The skin biopsy revealed histiocyte aggregates with granulomatous formation, surrounding vases and annexes with a great quantity of infiltrated erythrocytes. The aggregates are disposed in the middle and deep reticular dermis, quite distant from the papillary dermis and without any epidermis involvement (Figure 3). Some of the histiocytes have reniform nuclei, while others have small granules in the cytoplasm. FITE and GROCOTT staining were negative for microorganisms. Immunohistochemical tests revealed positivity for CD1A/S100 (Figure 4). The lesions completely regressed in five weeks (Figure 5). With these findings the diagnosis of congenital self-healing reticulohistiocytosis of Hashimoto Pritzker was completed. The patient has been under follow-up for two years and up to the present moment has not shown signs of recidivation of the disease.
Congenital self-healing reticulohistiocytosis was described in 1973 by Hashimoto-Pritzker. 6 The disease has as characteristic the presence of cutaneous lesions such as papules, nodules and vesicles, usually at birth or in the neonatal period, without systemic involvement. Histopathology presents infiltrate of Langerhans cells, with a positive immunohistochemical profile for S100/CD1A and spontaneous resolution in the first year of life. Spontaneous involution and absence of systemic symptoms are marked characteristics for differentiation with the other clinical spectra of Langerhans cell histiocytoses.7
Most cases are limited to the skin, but there are reports of cutaneous symptom complex accompanied by pulmonary and ophthalmic involvement.8,9
Despite the benign course and frequent spontaneous resolution in most of the patients with this form of LCH, studies show that in some cases there may be dissemination or relapse of the illness, demonstrating that the clinical course is variable and that a long-term follow-up is required.4,10,11
Patients with LCH in a single compromised system present excellent survival rate prognosis and no reports of death have been found in the literature concerning this group of patients. 12
There is no specific treatment for congenital self-healing reticulohistiocytosis. The conduct recommended by the Histiocyte Society consists in following the clinical picture and awaiting spontaneous regression. If the lesions persist, topical corticosteroids or topical nitrogen mustard may be effective. In case of cutaneous recurrence, the adopted conduct is the same: await spontaneous resolution of the symptoms, which presents favorable results. In cases of systemic recurrence, chemotherapy with vinblastine or etoposide, with or without corticosteroids is recommended.13,14,15
It is important to follow the patient for long periods to detect possible systemic involvement, since there are reports of recurrence involving skin, mucosa, bones and pituitary gland.4
Differential diagnosis should be made comparing it with cytomegalovirosis, candidiasis, varicella, herpes simplex, neonatal toxic erythema, infantile acropustulosis, pigmentary incontinence, eosinophilic pustular folliculitis, neonatal erythropoiesis, disseminated neonatal hemangiomatosis and congenital leukemia cutis.15
As this is a rare disease with very variable clinical behavior, it is of the essence to establish uniform clinical and histopathological criteria for diagnosis and patient stratification. According to the Histiocyte Society, the definitive diagnosis requires the demonstration of Birbeck granules in the histiocytes by means of electronic microscopy or surface antigen CD1a positivity by immunohistochemistry. 13, 3
Actual incidence should be higher than observed in literature, which is justified by the fast spontaneous resolution.5
1. Satter E, High W. Langerhans cell histiocytosis: a review of the current recommendations of the Histiocyte Society. Pediatr Dermatol. 2008;25:291-5. [ Links ]
2. Hamre M, Hedberg J, Buckley J, Bhatia S, Finlay J, Meadows A, et al. Langerhans Cell Histiocytosis: an exploratory epidemiologic study of 177 cases. Med Pediatr Oncol. 1997;28:92-7. [ Links ]
3. Egeler RM, D'Angio GJ. Langerhans cell histiocytosis. J Pediatr. 1995;127:1-11. [ Links ]
4. Longaker MA, Frieden IJ, Leboit, PE, Sherertz EF. Congenital "self-healing" Langerhans cell histiocytosis: the need for long-term follow-up. J Am Acad Dermatol. 1994;31:910-6. [ Links ]
5. Larralde M, Rositto A, Giardelli M, Carlos F. Gatti, Munõz A. Congenital self-healing histiocytosis (Hashimoto-Pritzker). Int J Dermatol. 1999;38:693-6. [ Links ]
6. Hashimoto K, Pritzker MS. Electron microscopic study of reticulohistiocytoma: an unusual case of congenital, self-healing reticulohistiocytosis. Arch Dermatol. 1973;107:263-70. [ Links ]
7. 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 histiocytosis. J Am Acad Dermatol. 2005;53:838-44. [ Links ]
8. Chunharas A, Pabunruang W, Hongeng S. Congenital self-healing Langerhans cell histiocytosis with pulmonary involvement: spontaneous regression. J Med Assoc Thai. 2002;85(Suppl 4):S1309-13. [ Links ]
9. Zaenglein AL, Steele MA, Kamino H, Chang MW. Congenital self-healing reticulohistiocytosis with eye involvement. Pediatr Dermatol. 2001;18:135-7. [ Links ]
10. Titgemeyer C, Grois N, Minkov M, Flucher-Wolfram B, Gatterer-Menz I, Gadner H. Pattern and course of single-system disease in Langerhans Cell Histiocytosis data from the DAL-HX 83- and 90-study. Med Pediatr Oncol. 2001;37:108-14. [ Links ]
11. Munn S, Chu AC. Langerhans Cell Histiocytosis of the Skin. Hematol Oncol Clin North Am. 1998;12:269-86. [ Links ]
12. Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, et al. A randomized Trial of treatment for multisystem Langerhans' Cell Histiocytosis. J Pediatr. 2001;138:728-34. [ Links ]
13. Histiocyte Society. org [Internet]. HLCH Evaluation and treatment Guidelines, april 2009. [cited 2010 May 22].Available from: http://www.histiocytesociety.org/site/c.mqISL2PIJrH/b.4442715/k.A339/Treatment_Plans.htm. [ Links ]
14. Whitehead B, Michaels M, Sahni R, Strobel S, Harper JI. Congenital selfhealing Langerhans cell histiocytosis with persistent cellular immunological abnormalities. Br J Dermatol. 1990;122:563-8. [ Links ]
15. Willman CL. Detection of clonal histiocytes in Langerhans cell histiocytosis: biology and clinical significance. Br J Câncer. 1994;70(Suppl.):S29-33. [ Links ]
Mailing address: Received on 21.06.2010. * Study carried out at Jesus City Hospital (Hospital Municipal Jesus - Rio de Janeiro (RJ), Brazil.
Joana Orle Coutinho de Azevedo
Rua Júlio Moura, 66, Centro
88020-150 Florianópolis (SC) - Brazil
Phone.: (48) 3322-2533
Approved by the Advisory Board and accepted for publication on 21.07.2010.
Conflict of interest: None
Financial funding: None
Received on 21.06.2010.
* Study carried out at Jesus City Hospital (Hospital Municipal Jesus - Rio de Janeiro (RJ), Brazil.