Tuesday, 25 October 2011


Non-Langerhans cell histiocytosis represents a broad group of different disorders characterized by the proliferation of histiocytes other than Langerhans cells. One of which, is multicentric reticulohistiocytosis shown here in this middle-aged woman with yellow-pink papules and nodules (with scleral involvement). Multicentric reticulohistiocytosis has subtype (multiple reticulohistiocytomas) that is restricted to skin. It is possible that the diffuse, purely cutaneous form is an early stage of multicentric reticulohistiocytosis before the appearance of joint and other lesions. In multicentric reticulohistiocytosis the onset of arthropathy, may precede, follow, or accompany the onset of skin lesions. No treatment is of consistent value for this disease.

The characteristic pathological picture in multicentric reticulohistiocytosis is of infiltration by mononucleated and multinucleated giant cells with voluminous eosinophilic ground-glass cytoplasm (finely granular). In early lesions, the predominant infiltrating cells are histiocytes, lymphocytes and eosinophils, with few giant cells, but the giant cell infiltrate quickly follows.
The multinucleate giant cells (the hallmark of the disease) have 3-10 or more nuclei, which may be placed haphazardly, or along the periphery, or clustered in the centre of the giant cell. They contain variable amounts of lipid and free or esterified cholesterol.

Severe involvement of the face may lead to a leonine facies. Does this disease show Köbner (Koebner) response? What are the diseases that may lead to a leonine facies?

1 comment:

  1. Yes it does*.

    *Aldridge RD, Main RA, Daly BM. The Köbner’s response in multicentric reticulohistiocytosis. Cutis 1984; 34: 78–80.

    Examples of diseases that may lead to a leonine facies

    Carcinoid syndrome
    Chronic actinic dermatitis/actinic reticuloid
    Focal facial dermal dysplasia
    KID syndrome (keratitis, ichthyosis, deafness)
    Lepromatous Leprosy
    Lipoid proteinosis
    Lymphoma, leukaemia
    Mycosis fungoides
    Multicentric reticulohistiocytosis
    Multiple keratoacanthoma syndrome
    Pachydermoperiostosis/cutis verticis gyrata
    Paget's disease of bone (the patient may first complain that hats no longer fit properly due to cranial enlargement)
    Progressive nodular histiocytosis
    Pseudophotodermatitis (when chronic, airborne pattern of allergic contact dermatitis induced by Compositae plants)
    Systemic mastocytosis

    Note that:

    1) The histiocyte is a somewhat controversial cell which still lacks precise definition.

    The various types of histiocyte express different antigens that assist in their distinction.

    a) Macrophage CD45 positive, LYOSOZYME positive, S100 negative, factor XIIIa negative

    b) Dermal dendrocyte CD45 positive, factor XIIIa POSITIVE, S100 negative

    c) Indeterminate cell CD45 positive, S100 positive, factor XIIIa negative

    d) Langerhans cell (antigen processing cells in the epidermis) CD45 positive, BIRBECK granules (Langerhans bodies) present, S100 positive
    factor XIIIa negative

    Most cells possess a single nucleus. Cells with more than one nucleus are called multinucleate cells e.g fused macrophages.

    2) There is a condition called fibroblastic rheumatism that mimics multicentric reticulohistiocytosis clinically (cutaneous nodules +/- polyarthritis) but not pathologically (proliferation of plump spindle cells [myofibroblasts]).


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Main Works of Reference List (The first eight are my top favourites)

  • British National Formulary
  • British National Formulary for Children
  • Guidelines (BAD - BASHH - BHIVA - Uroweb)
  • Oxford Handbook of Genitourinary Medicine, HIV, and Sexual Health
  • Oxford Handbook of Medical Dermatology
  • Rook's Textbook of Dermatology
  • Simple Skin Surgery
  • Weedon's Skin Pathology
  • A Concise Atlas of Dermatopathology (P Mckee)
  • Andrews' Diseases of the Skin
  • Andrology (Nieschlag E FRCP, Behre M and Nieschlag S)
  • Bailey and Love's Short Practice of Surgery
  • Davidson's Essentials of Medicine
  • Davidson's Principles and Practice of Medicine
  • Fitzpatrick's Colour Atlas and Synopsis of Clinical Dermatology (Klaus Wolff FRCP and Richard Allen Johnson)
  • Fitzpatrick’s Dermatology in General Medicine
  • Ganong's Review of Medical Physiology
  • Gray's Anatomy
  • Hamilton Bailey's Demonstrations of Physical Signs in Clinical Surgery
  • Hutchison's Clinical Methods
  • Lever's Histopathology of the Skin
  • Lever's Histopathology of the Skin (Atlas and Synopsis)
  • Macleod's Clinical Examination
  • Martindale: The Complete Drug Reference
  • Oxford Handbook of Clinical Examination and Practical Skills
  • Oxford Textbook of Medicine
  • Practical Dermatopathology (R Rapini)
  • Sexually Transmitted Diseases (Holmes K et al)
  • Statistics in Clinical Practice (D Coggon FRCP)
  • Stockley's Drug Interactions
  • Treatment of Skin Disease: Comprehensive Therapeutic Strategies
  • Yen & Jaffe's Reproductive Endocrinology