Eur. J. Pediat. Dermatol.                                                                                      Contents vol. 9, 1999
9, 89-92, 1999

Congenital self-healing histiocytosis (Hashimoto-Pritzker).
A study of eleven cases*.

Larralde M., RosittoA.*, Giardelli M.**, Santos Muñoz A.
Pediatric Dermatology Div., Ramos Mejía Hospital, Buenos Aires
*Pediatric Dermatology Div., La Plata Children's Hospital, La Plata, Buenos Aires
**Pediatric Dermatology Div., Italian Hospital, Buenos Aires

*Presented in the International Congress of Neonatal Dermatology
Bari (Italy), September 24-27, 1998

Summary Congenital self-healing Langerhans cell histiocytosis is a rare disorder in the neonatal period. It manifests itself with papules, vesicles or nodules and does not affect other organs besides the skin. The cutaneous lesions tend to regress spontaneously within weeks or months. This report includes the clinical features and laboratoy findings of eleven infants with congenital self-healing Langerhans cell histiocytosis, which were observed in Buenos Aires in the period between January, 1989 and May, 1998. In these eleven cases are evaluated the sex, age of onset, type of cutaneous lesions, color, presence of erosion, affected sites and time of healing. The Authors believe that the disorder is more frequent than reported, because of its rate of rapid spontaneous resolution. They propose that in all patients with this disorder a systemic evaluation must be done to exclude any possible extracutaneous involvement. The Authors also suggest a long term follow up to put in evidence relapse or progression of the disease.

Key words Langerhans, histiocytosis, Hashimoto-Pritzker disease.

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is an uncommon disease, initially seen at birth or in the neonatal period with generalized papules, vesicles or nodules on the skin without involving other organs (6). The skin lesions tend to involute spontaneously within weeks to months (8). We describe eleven patients with widespread CSHLCH, that we have seen from 1989 up to 1998. We want to emphasize the need of a careful evaluation for systemic involvement and long term follow-up for evidence of relapse or progression of the disease.

Cases reported

In this study we included eleven patients, nine of whom hospitalized in the Pediatric Dermatology Department of the Ramos Mejia Hospital and the others in the Italian Hospital and La Plata Children Hospital, all of them located in Buenos Aires, Argentina, between January 1989 and May 1998, with definite diagnosis of congenital self-healing Langerhans cell histiocytosis.
A total of eleven patients were studied (Table 1), seven females and four males. All of them presented at birth the lesions, which disappeared one to three months later. The skin lesions consisted of multiple red-brownish papules, vesicles, crusts and nodules, distributed in face, scalp, trunk (Fig. 1, 2), limbs, palms and soles. Two patients had oral mucosal lesions.

Fig. 1: Case n. 4. Crusted and eroded lesions.

The laboratory examinations were normal in all the patients and none of them had extracutaneous involvement. On light microscopy, the sections stained with hematoxylin and eosin showed a lichenoid infiltrate composed of many histiocytes and few neutrophils and lymphocytes in the papillary dermis.
Immunomarking with S-100 protein was positive in all the eleven patients. Moreover, immunomarking with CD1 was positive in the four patients tested.
Two cases were studied by electron microscopy and the Langerhans cells showed a lobulated nucleus, Birbeck granules and laminated bodies as cytoplasmatic markers.
No relapses were observed in the eleven patients of this study.

Discussion

Langerhans cells histiocytosis (LCH) is a generic term including several clinical diseases with proliferation of distinctive histiocytes containing Birbeck granules in their cytoplasm (3).
Self-healing histiocytosis, is also included among LCH (14).
The characteristical features of CSHLCH are:
1- congenital skin lesions;
2- in an otherwise healthy infant with no or mild systemic symptoms;
3- histopathology demonstrating Langerhans cell infiltrate;
4- spontaneous involution of the skin lesions (8).
The clinical features include a cutaneous eruption of red, violaceous or brownish firm painless nodules, vesicles, papules and crusts scattered all over the face, scalp, trunk, limbs, palms and soles. Some patients with solitary lesions that fit criteria of CSHLCH have been reported (1, 10, 12). In some cases of CSHLCH there might be lesions in the oral mucosa.
The patients with CSHLCH have no or mild systemic involvement and the skin lesions tend to regress spontaneously within one to three months, being followed occasionally by residual hypo- (Fig. 1, 2) or hyperpigmented macules (7) or scarring.

Esterly et Al. (4) and other Authors (11, 13) reported several cases in which relapses of the disease appeared until four years after resolving congenital lesions. Histologically, CSHLCH demonstrates massive dermal infiltration of pleomorphic histiocytes with abundant eosinophilic ground-glass cytoplasm (9), and kidney-shaped nuclei with irregular border (5). Multinucleated giant cells may also exist. The epidermis can show the same infiltrate as the dermis, and is often ulcerated. Lymphocytes, eosinophils, neutrophils, and xanthomatous features are common. The histiocytes are S-100 and CD1 positive by immunomarking. Electron microscopy demonstrates Birbeck granules or laminated dense bodies inside a 10 to 30% of histiocytes. We propose that in all patients with a CSHLCH diagnosis a systemic evaluation must be done to exclude any possible extracutaneous involvement.

Fig. 2: Case n. 4. Crusted and eroded lesions wich resolved with hypochromic macules.

This evaluation includes complete physical examination, full blood count, serum chemistry, liver function test, skull, chest and long bones X-rays and abdominal ultrasound (2). We also suggest a long term follow-up to evidence relapse or progression of the disease. This is essential when facing this kind of patients.
Finally, we think that the real incidence of CSHLCH might be higher than reported, because of its rate of rapid spontaneous resolution.


Address to: Prof. Dr. Margarita Larralde
Pediatric Dermatology Division
Ramos Mejía Hospital
Buenos Aires (Argentina)

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