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Year : 2018  |  Volume : 5  |  Issue : 2  |  Page : 143-145

Generalized eruptive histiocytosis in a child: A rare entity

Department of Dermatology, Malda Medical College and Hospital, Malda, West Bengal, India

Date of Web Publication19-Dec-2018

Correspondence Address:
Dr. Chinmay Kar
Department of Dermatology, Malda Medical College and Hospital, Malda, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijdpdd.ijdpdd_1_18

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How to cite this article:
Kar C, Biswas R, Banerjee S, Sarkar P, Shome K, Das KD. Generalized eruptive histiocytosis in a child: A rare entity. Indian J Dermatopathol Diagn Dermatol 2018;5:143-5

How to cite this URL:
Kar C, Biswas R, Banerjee S, Sarkar P, Shome K, Das KD. Generalized eruptive histiocytosis in a child: A rare entity. Indian J Dermatopathol Diagn Dermatol [serial online] 2018 [cited 2020 Oct 31];5:143-5. Available from: https://www.ijdpdd.com/text.asp?2018/5/2/143/247862


The histiocytoses are the accumulation of reactive or neoplastic histiocytes in various tissues. Among these, non-Langerhans cell histiocytosis (non-LCH) is a broad group of disorders characterized by proliferation of histiocytes other than Langerhans cell. In non-LCH, generalized eruptive histiocytosis (GEH), first described by Winkelmann and Muller, is a rare histiocytic tumor.[1] It is mostly found in adults; childhood GEH accounts for one-fourth of the cases.[2] Here, we report a case of childhood GEH.

A 6-year-old girl presented with numerous small papules over face, axillae, and trunk in bilaterally symmetrical fashion for 3 years [Figure 1] and [Figure 2]. The lesions were asymptomatic and appeared in crops in several successions. With progression of time, a few lesions subsided with postinflammatory hyperpigmentation. The older lesions were larger in size than younger ones. The mucous membranes, palms, and soles were not involved. There was no history of any bronchial asthma, arthritis, malignancy, or drug intake of long duration. There was no family history of similar disease. Her developmental milestones were normal. On examination, the papules were discrete, 2–8 mm in size, dome shaped, smoothed surface, flesh-colored, and firm in consistency. There was no abnormality in routine blood count, lipid profile, thyroid screening, IgE level, and routine urinalysis. The abdominal ultrasonography was normal. Punch biopsy from axillary region for histopathological examination (HPE) revealed normal epidermis, Grenz zone, and collection of monomorphous histiocytes with lymphocytes in upper- and mid-dermis [Figure 3]. A few multinucleated giant cells were also found in the tumor tissue [Figure 4] and [Figure 5]. The immunohistochemistry (IHC) of tumor tissue showed strong reactivity (51%–75% cells) to CD68 [Figure 6], low reactivity (1%–25% cells) to S-100 marker [Figure 7], and nonreactivity to CD1a marker. The clinical and histopathological features and IHC suggested the diagnosis of GEH.
Figure 1: Papular lesions in face

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Figure 2: Papular lesions over the left axilla and surrounding

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Figure 3: Histopathological examination shows normal epidermis, grenz zone, and collection of cells in upper- and mid-dermis (H and E, ×8)

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Figure 4: Histopathological examination reveals monomorphous histiocytes, lymphocytes, and a few multinucleated giant cells in the tumor tissue (H and E, ×40)

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Figure 5: Histopathological examination shows monomorphous histiocytes, lymphocytes, and a few multinucleated giant cells in the tumor tissue (H and E, ×80)

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Figure 6: Strong positivity of CD68 marker (×40)

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Figure 7: Low positivity of S-100 marker (×40)

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GEH is a Type II histiocytosis. The characteristic cells of this disease are largely monomorphous vacuolated histiocytes. These vacuolated cells are the early stage of maturation. These cells tend to resolve spontaneously than more mature spindle or xanthomatized cells. In general, the IHC of GEH reveals CD68 positivity with CD1a and S-100 marker negativity.

We found that the lesional cells were strongly positive for CD68 and negative for CD1a confirmed the case as non-LCH. According to Winkelmann and Muller, the typical features of GEH include (1) widespread, symmetric, multiple papules particularly involving trunk, and proximal part of extremities; (2) distinct flesh to blue-red-colored papules without coalescing; (3) progressive development of new lesions; (4) spontaneous resolution of older lesions to hyperpigmented macules or complete clearance; and (5) a benign histology of mononuclear histiocytes.

Hence, our case fulfilled the clinical and histopathological features of GEH. Low reactivity of S-100 marker of lesional cells may be found in immature cells and likely become nonreactive with time.[3] Very few cases, multinucleated giant cells have also been reported.[4]

The differential diagnoses of GEH include papular form of sarcoidosis, eruptive xanthomas, eruptive syringomas, widespread papular granuloma annulare, indeterminate cell histiocytosis, and other variants of non-LCH. Clinical appearance, biochemical parameters, HPE, and IHC ruled out the above diseases. Hence, our case was GEH though very few features of other non-LCH were also found because of some expected overlap of features in this group. There was a report of xanthoma disseminatum evolving from GEH of a 4-year-old boy.[5] Hence, careful follow-up is needed to know the progression of disease into serious form of non-LHC.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Winkelmann RK, Muller SA. Generalized eruptive histiocytoma. A benign papular histiocytic reticulosis. Arch Dermatol 1963;88:586-96.  Back to cited text no. 1
Goodmam WT, Barrett TL. Histiocytoses. In: Bolognia JL, Jorizzo JL, Schaffer JV, editors. Dermatology. 3rd ed. Saunders: Elsevier; 2012. p. 1529-46.  Back to cited text no. 2
Yamamoto Y, Kadota M, Nishimura Y. A case of S-100-positive juvenile xanthogranuloma: A longitudinal observation. Pediatr Dermatol 2009;26:475-6.  Back to cited text no. 3
Seward JL, Malone JC, Callen JP. Generalized eruptive histiocytosis. J Am Acad Dermatol 2004;50:116-20.  Back to cited text no. 4
Repis T, Roca-Miralles M, Kanitakis J, Castells-Rodellas A. Generalized eruptive histiocytoma evolving into xanthoma disseminatum in a 4-year-old boy. Br J Dermatol 1995;132:978-82.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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