Figure 1-Low power magnification showing a lichenoid infiltrate of Langerhans cells effacing the dermal-epidermal junction.



Figure 2-Higher power magnification of intraepithelial and dermal Langerhans cells.



Figure 3-Langerhans cells with characteristic reniform nuclei.

Figure 4-Langerhans cells are strongly positive for CD1a. Note the benign non-staining squamous epithelial cells.



Figure 5-Birbeck granules identified by electron microscopy (not from this case), diagnostic for Langerhans cell histiocytosis.

Case Study

This is a 3 1/2 month old boy with a one month history of a scaly papular rash on the scalp and trunk.

Diagnosis:

Langerhans Cell Histiocytosis

This is an uncommon hematopoietic neoplasm of the skin. For many years, these diseases were known by such terms as Histiocytosis X, Hand-Schuller-Christian disease, Eosinophilic granuloma, and Letterer-Siwe disease. Currently, all of these previous designations are considered clinical variants, best categorized under the appellation cited above. The unifying histopathologic feature is the Langerhans cell, a cell normally involved in antigen processing within the skin. These cells have a characteristic immunohistochemical profile for CD1a (Figure 4) and for S100. If one can avail of an electron microscope, a diligent search will reveal the Birbeck granule (Figure 5), pathognomonic for a Langerhans cell.

The clinical presentation of this patient is rather classic but as we all know, sometimes tumors do not read the books. The recent dermatology literature is replete with unusual clinical presentations, including a blueberry muffin baby (see reference below).

The management is controversial and is dependent upon the extent and clinical stage of the disease. The references cited below are two of the larger studies following a total of 427 patients.

Clinical photograph and information submitted by Elizabeth Lener, M.D.
Ladera Ranch Dermatology, Southern California

References:

The Doctor's Doctor-Langerhans Cell Histiocytosis

Shaffer MP, Walling HW, Stone MS.Langerhans cell histiocytosis presenting as blueberry muffin baby. J Am Acad Dermatol. 2005 Aug;53(2 Suppl 1):S143-6.

Braier J, Chantada G, Rosso D, Bernaldez P, Amaral D, Latella A, Balancini B, Masautis A, Goldberg J. Langerhans cell histiocytosis: retrospective evaluation of 123 patients at a single institution. Pediatr Hematol Oncol 1999 Sep-Oct;16(5):377-85.

Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer 1999 May 15;85(10):2278-90.

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