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Histiocytoses COVER FOCUS Histiocytoses: A Review of the Most Recent Classification System A look at the proposed new classification system, grouping the histiocytoses into five categories, with tabular summaries for differential diagnosis. BY JOLIE KROOKS, BS, MILEN MINKOV, MD, PHD, AND KLEMENS RAPPERSBERGER, MD istiocytes are immune cells found in various tissues ior” and malignant, which included further subdivision to throughout the body whose functions are diverse and dendritic cell-related, macrophage-related, and monocyte- include housekeeping via phagocytosis, activating the related. “Disorders of varied biological behavior” represented immune system via antigen presentation to T cells, non-malignant disorders of diverse clinical severities.14 Hand promoting peripheral tolerance via proliferation of regula- Since then, there have been breakthroughs regarding the tory T cells.1,2 Histiocytes of the skin include the CD14+ dendrit- specific molecular mechanisms involved in the pathogenesis of ic cells and macrophages of the dermis and CD14- Langerhans many of the histiocytoses, especially those pertaining to LCH cells of the epidermis and mucosa.3,4 Langerhans cells share and Erdheim-Chester disease. The identification of specific unique properties with both macrophages and dendritic cells. mutations in the MAPK pathway, shared by both LCH and Like dendritic cells, macrophages and Langerhans cells may be Erdheim-Chester disease, has shaped the way researchers regard long-standing byproducts of fetal hematopoiesis in the yolk sac these disorders in relation to each other, and there has been a and liver or may be renewed by bone marrow myeloid precur- shift in considering these disorders as neoplastic, rather than sor cells during severe inflammatory conditions. However, in as simply inflammatory, conditions.15-20 Considering these new contrast to dendritic cells, macrophages and Langerhans cells insights, Emile et al recently presented a new classification of the self-renew during stable conditions.5-7 Nevertheless, Langerhans histiocytoses.9 Specifically, in this new classification, the histio- cells more closely resemble dendritic cells than macrophages in cytic disorders are grouped into five categories based on clinical, their ability to migrate to lymph nodes in order to present anti- genetic, and (immuno)-histologic features: Langerhans (L); cuta- gens to and stimulate T lymphocytes.8 neous and mucocutaneous (C); malignant (M); Rosai-Dorfman The pathologic propagation of histiocytic cells encompasses disease (R); and hemophagocytic lymphohistiocytosis (H).9 over 100 different subtypes of rare disorders collectively referred Indicative of the relevance of the histiocytoses to derma- to as the histiocytoses.9 Of the histiocytoses, Langerhans cell his- tology is the fact that there is a distinct category for histio- tiocytoses (LCH) is the most common, affecting an estimated cytoses with predominantly cutaneous and mucocutaneous four to five per million children per year (age 0-15).10,11 LCH manifestations. Additionally, many of the systemic clinical affects children in >90 percent of cases.12 forms (including the malignant entities) can also present The first classification of the histiocytoses, established by with cutaneous manifestations. Other histiocytoses classifi- the Working Group of the Histiocyte Society in 1987, classi- cation systems have also emphasized the dominating role of fied the histiocytoses as Langerhans cell-related, non-Lang- the skin.21 Accordingly, correct diagnosis may depend on the erhans cell-related, or malignant.13 The original classification expertise of the dermatologist and dermatopathologist. was updated in 1997 by a joint effort of the Histiocyte This review includes a discussion of the proposed classifica- Society and the WHO Committee on Histiocytic/Reticulum tion system from Emile et al and the pathogenesis of the dif- Cell Proliferations to “disorders of varied biological behav- ferent histiocytic disorders, and provides tabular summaries APRIL 2018 PRACTICAL DERMATOLOGY 41 COVER FOCUS TABLE 1. IMMUNOHISTOCHEMICAL AND CLINICAL FINDINGS; L GROUP* Disease Histology Clinical presentation Cutaneous manifestations LCH LCH cells with “coffee-bean” Highest incidence before age 1, decreases thereafter Most commonly presents as cleaved nuclei, benign, round- Systemic involvement, with potential for multi- a seborrheic dermatitis-like or ed morphology, and eosino- organ failure, likely when patients present with eczematous rash on the trunk, philic cytoplasm cutaneous signs (87-93%) head, and/or face, though vari- CD1a+, CD207+, HLA-DR+ Bone (80%), skin (33%), pituitary (25%), liver (15%), ous lesions and sites of involve- cytoplasmic dot, Birbeck gran- spleen (15%), hematopoietic system (15%), lungs ment have been observed ules present (15%), lymph nodes (5–10%), and CNS excluding the pituitary (2–4%) are most commonly affected ECD This entity represents an extra- Highest incidence in men age 40-70, Periorbital xanthelasmas cutaneous systemic form of Predominantly affects long bones, bilateral cortical increasing in size (33%); other JXG immunohistochemically. osteosclerosis (90%) skin lesions resemble either See XG family (Table 2) Cardiovascular (45%), especially aortic coating, those of JXG, diffuse xanthoma, However, Emile et al empha- CNS (50%), pulmonary (20%), and endocrine (5%) reticulohistiocytoma, or LCH; size ECD’s molecular similarity involvement, hairy kidney (30%), exopthamos, dip- most commonly involves upper with LCH and co-occurrence lopia, and/or vision impairment (25%), and cutane- trunk and extremities in the same patients. ous lesions (30%) are common Most common initial findings are diabetes insipi- dus (25%) and bone pain (17%) Typically involves >1 organ system (90%) 60% mortality Mixed Mimics LCH, ECD, or both In a report of 23 patients, average age of diagnosis LCH-like lesions more prevalent LCH/ECD was 43 years (range 2-75), 1 males:1.1 females (61%), though ECD-like lesions ECD either followed (52%, median interval also common (35%) between diagnoses 13.5 years) or presented at the same time as LCH (48%); ECD preceding LCH has rarely been reported in the literature Most prevalent lesions: LCH-type: bone (70%), skin (61%) • Nonspecific: CNS (48%), pituitary (57%) • ECD-type: bone lesions (91%), large vessel (65%), retroperitoneal (56%) • Equivalent mortal- ity rate as ECD, but significantly higher in patients diagnosed with both diseases concurrently ICH Dermal inflammatory infiltrate Most commonly an isolated cutaneous disease in Presents with pleomorphic and foamy histiocytes with otherwise healthy adults, though bone and corneal pink-red papules or nodules liposomes involvement and cases in children have also been that are either single, multiple S100+, CD1a+ but CD207-, reported unifocal, or disseminated with- CD68+, Birbeck granules Typically has a benign course out accompanying pain, pruritis, absent or mucosal findings Langerhans cell histiocytosis LCH, Erdheim-Chester disease ECD, Xanthogranuloma XG, Juvenile xanthogranuloma JXG, Indeterminate cell histiocytosis ICH *Data from multiple sources.10,18,22,30,89-105 42 PRACTICAL DERMATOLOGY APRIL 2018 COVER FOCUS TABLE 2. IMMUNOHISTOCHEMICAL AND CLINICAL FINDINGS; C GROUP; XG FAMILY* Histology: Different histiocytic cell morphologies observed on histology include: vacuolated, spindle-shaped, xanthomatized, scal- loped, and/or oncocytic mononuclear cells or Touton, ground-glass appearing, Langhans, and/or foreign body multinuclear cells. CD68+, factor VIIIa+, CD163+, fascin+, S100-, CD1a-, CD207-, Birbeck granules absent Disease Clinical presentation Cutaneous manifestations JXG Typically, benign and self-limited in otherwise healthy children <2 years Most commonly a solitary (66%), asymptomatic 5% of patients present with an isolated extracutaneous lesion pink/yellow rubbery papule or nodule that progress- 4% have systemic involvement with multiple cutaneous lesions es to a yellow/brown color on the head and neck Eye involvement rare (0.3-0.5%) in patients with cutaneous disease, how- (42%) or trunk (20-40%) ever it is still the most common of the extracutaneous manifestations Other areas of involvement less common AXG Extracutaneous disease is rare, but has been reported to affect the Compared to JXG, lesions are more often solitary spine, CNS, orbit, and heart. and larger. Spontaneous resolution is less common than in JXG Lesions are typically the same color as JXG and most Multiple lesions are associated with a higher rate of spontaneous commonly involve the head and neck resolution (50%) than solitary lesions, but with a greater prevalence Rare instances of multiple lesions have been of hematologic malignancy reported If BRAF+, must exclude ECD by clinical and imaging studies SRH May occur in children but more commonly in adults (mean age 35 Solitary, asymptotic, cutaneous or mucosal nodule years) typically not larger than 1cm Benign with spontaneous resolution in otherwise healthy individuals Most commonly on the trunk (36%), lower extrem- ity (27%), head/neck (18%), upper extremity (14%) BCH Isolated cutaneous disease in patients 2-66 months with spontane- Yellow and red-brown asymptotic macules and pap- ous resolution ules (1-8 mm) that first appear on the face and neck and may then spread caudally GEH Benign Multiple self-limited, non-coalescing, red-brown or More prevalent in adults, but may also affect children blue-red symmetric papular lesions on the trunk and proximal extremities with sparing of the
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