Langerhan Cell Histiocytosis Face

Figure 1.244. The diagnosis of a hamartoma of hair follicle origin was established by biopsy of die midline lesions of die nose in diis infant.

Figure 1.245. Congenital self-healing reticulohis-tiocytosis (Hashimoto-Pritzker disease) is a rare disease usually present at birth or within the first few days of life. It is characterized by solitary or multiple reddish-brown, pink, or purplish papulovesicu-lar lesions mainly on the scalp, face, trunk, and extremities. They tend to break down in the center, form ulcerated craters, and involute spontaneously within 2 to 3 months leaving white atrophic scars. Although the course is benign and self-limiting, it is important to differentiate this condition from histiocytosis X and other histiocytic conditions. Diagnosis is confirmed by skin biopsy.

Figure 1.246. In juvenile xan-thogranulomatosis the lesions are present at birth. They are characterized by solitary or multiple yellow to reddish-brown papules and nodules of die face, scalp, neck, and sometimes die sublingual areas and the proximal portions of the extremities or trunk. The lesions may enlarge and become bright yellow as diey mature. Spontaneous regression usually occurs in die first year of life. Diagnosis is confirmed by biopsy which shows histiocytes of die non-Langerhans' cells and die presence of Touton giant cells. The lesions are harmless but should not be overlooked since diere is an apparent association with neurofibromatosis. (Kenny, J.)

Langerhans Cell Histiocytosis Babies
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Histiocytosis Letterer Siwe Disease

Figure 1.247. Skin lesions, present at birth in this infant with histiocytosis X, on biopsy showed the typical finding of the presence of histiocytes of the Langerhans' cell type and eosinophilia. The term "congenital histiocytosis" includes Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma. These conditions are now grouped together as histiocytosis X.

Pulmonary Histiocytosis Pic

Figure 1.249. In this infant with congenital generalized fibromatosis, on the left note the lesion on the nose and the purplish area surrounded by a pale halo above the left eyebrow. On the right is a close-up of the lesions on the back of the same infant. The infants may also have firm hard nodules in the skin and subcutaneous tissue. In congenital generalized fibromatosis it is necessary to check the long bones and lungs for systemic involvement.

Figure 1.248. Chest radiograph in an infant at the age of 3 days with histiocytosis. Note the infiltrative coin-like lesions which are typical with pulmonary involvement in histiocytosis X. The presence of pulmonary and other systemic involvement worsens the prognosis.

Figure 1.249. In this infant with congenital generalized fibromatosis, on the left note the lesion on the nose and the purplish area surrounded by a pale halo above the left eyebrow. On the right is a close-up of the lesions on the back of the same infant. The infants may also have firm hard nodules in the skin and subcutaneous tissue. In congenital generalized fibromatosis it is necessary to check the long bones and lungs for systemic involvement.

Figure 1.250. Radiograph of the long bones in another infant with congenital generalized fibromatosis showing the areas of erosion due to the presence of generalized fibromata.

Figure 1.251. Chest radiograph showing extensive pulmonary involvement in an infant with congenital generalized fibromatosis. The presence of pulmonary involvement signifies a poor prognosis.

Langerhans Cell Histiocytosis Jaw
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  • JOHAN
    Can langerhans cell histiocytosis be hereditary?
    8 years ago

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