Answer of Dermatopathology Case 81

Immunohistochemistry: S100 protein is strongly positive.

Immunohistochemistry: CD1a is stongly positive

Langerhans Cell Histiocytosis

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Abstract:

Langerhans cell hyperplasia of the skin mimicking Langerhans cell histiocytosis: a report of two cases in children not associated with scabies. Fetal Pediatr Pathol. 2010;29(4):231-8.

Langerhans cells histiocytosis (LCH) affecting the skin most commonly has clinical and histopathologic diagnostic features. We are reporting two examples of Langerhans cell (LC) hyperplasia recognized in the skin biopsies of two children initially interpreted as LCH. The first was an 8-year-old boy finally interpreted as having an atypical type of contact dermatitis, while the second, an 8-year-old girl, was assumed to have Pytiriasis lichenoides et varioliformis acuta. None showed evidences of scabies. Both presented spongiotic dermatitis with numerous CD1a+ cells. As more cases of LC hyperplasia are recognized, new details emerge helping in the differential diagnosis. Strict clinical-pathologic correlation is suggested in order to avoid misdiagnosis.

Cutaneous Langerhans cell histiocytosis in an elderly woman. Dermatol Online J. 2010 Oct 15;16(10):6.

Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells typically seen in infants and children. Rare adult cases usually have systemic involvement. We report an uncommon case of skin-limited LCH in an elderly woman, who is to our knowledge one of the oldest patients reported with this condition.

Skin-limited Langerhans' cell histiocytosis in children. Cancer Invest. 2009 Jun;27(5):504-11.

Langerhans' cells are dendritic cells derived from precursors in the bone marrow. They constitute 2-4% of the resident epidermal cells and are found within the epidermis above the basal layer. They function as immunologic cells by recognizing antigens and presenting them to T cell lymphocytes. Langerhans' cell histiocytosis is a rare pathology characterized by an abnormal clonal proliferation of Langerhans' cells that infiltrates different organs of the human body. The proliferating Langerhans' cells appears to be primarily responsible for the clinical manifestations. The stimulus for their proliferation is unknown. Among different organs, cutaneous involvement is encountered in 40% of cases. The aim of this investigation is to review the clinicopathologic, immunologic and ultrastructural features of skin-confined Langerhans' cell histiocytosis in children through seven case series. Four boys and two girls with age range of 1 year to 8 years presented with scaling, crusted papules, nodules and papulonodular lesions (two cases each). The locations included the face (three cases), scalp, trunk and vulva (one case each). The histological features included histiocytic reaction (one case), granulomatous reactions (three cases) and both granulomatous and histiocytic reactions (two cases). The diagnosis was confirmed by histochemical (S-100 + CD1a +) and ultrastructural studies (Birbeck granules). Langerhans' cell histiocytosis is a rare disease with pleomorphic cutaneous clinical expressions. Three types of skin lesions usually occur: nodules (common), scaling, or crusted papules (next in frequency) and finally soft, yellow papular xanthomas (rare). Three types of histological pictures are seen: histiocytic, granulomatous (common) and xanthomatous (rare) in children.

Langerhans cell histiocytosis in a premature baby presenting with skin-isolated disease: case report and literature review. Acta Paediatr. 2008 Dec;97(12):1751-4. Epub 2008 Aug 27.

Langerhans cell histiocytosis (LCH) in premature babies is extremely rare as is a vesicular skin rash, while gastrointestinal involvement is associated with a poor outcome. We report a case of LCH in a premature baby presented with isolated vesiculo-papulo-macular skin lesions and insidiously developed gastrointestinal symptoms, haematological and severe pulmonary involvement. We also reviewed a few cases of LCH in premature babies in the English language medical literature. LCH in preterm babies appears to be a severe systemic disease, usually lethal in-utero or post delivery. CONCLUSION: Careful observation should be applied to newborns with skin-only Langerhans cell histiocytosis in order to identify in time progression to potentially fatal systemic disease.