The relationship between pulmonary Langerhans cell histiocytosis and skin rash

Pulmonary histiocytosis is one of the Langerhans cell histiocytoses. In this category of diseases, cases involving single-bone or multiple-bone lesions of the skin, or damage to organs such as the liver, spleen, lungs and hematopoietic system accompanied by bone and skin lesions, are classified as widespread; the disease may manifest as single-system or multi-system disease with systemic symptoms that vary in presentation. Generally, initial-onset or mild cases present as isolated lesions without bone pain, while severe patients develop extensive organ infiltration, fever, weight loss and other symptoms.

Pulmonary Langerhans cell histiocytosis

In Langerhans cell histiocytosis, there are more than ten series of diseases including skin rash, bone lesions, lymph node lesions, ear and mastoid lesions, bone marrow lesions, liver lesions, spleen lesions, lung lesions, gastrointestinal lesions, and central nervous system lesions. Pulmonary histiocytosis is only one type among the lung lesions of Langerhans cell histiocytosis. And in Langerhans cell histiocytosis, the most common is the skin rash type or series of diseases.

As for the skin, traditional Chinese medicine has long held the saying that "the lung governs the skin and hair." An externally invading skin rash may occur first, and it can also affect the lungs. Skin rash also occurs more often in infants and young children, mostly presenting as acute rash in children, mainly distributed on the trunk, the scalp hairline, and behind the ears. It begins as maculopapules and quickly develops exudation (similar to eczema or seborrheic dermatitis), may be accompanied by bleeding, then crusts and peels, and finally leaves pigmented white spots, which do not fade easily for a long time.

Skin rash in pulmonary Langerhans cell histiocytosis

After developing a skin rash, rashes of various stages may exist simultaneously, or one batch may fade while another batch appears. Fever often accompanies the rash; in chronic cases, the rash may be scattered over various parts of the body. It starts as pale red maculopapules or verrucous nodules, and when fading the center sinks and flattens; some are dark brown, closely resembling crusted chickenpox. Finally the local skin becomes thin, slightly sunken and somewhat glossy, or with a little scaling. The rash may appear simultaneously with other organ damage, or may exist as the only affected manifestation, and is commonly seen in male infants under 1 year of age.

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