|LETTER TO EDITOR
|Year : 2019 | Volume
| Issue : 2 | Page : 109-111
Clinicopathological correlation in diagnosis of generalized eruptive histiocytosis
Sandhya Kumari Chauhan1, Anchana Gulati2, Geeta Ram Tegta1, Mudita Gupta1
1 Department of Dermatology, IGMC, Shimla, Himachal Pradesh, India
2 Department of Pathology, IGMC, Shimla, Himachal Pradesh, India
|Date of Web Publication||28-Nov-2019|
Department of Dermatology, IGMC, Shimla, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chauhan SK, Gulati A, Tegta GR, Gupta M. Clinicopathological correlation in diagnosis of generalized eruptive histiocytosis. Indian J Dermatopathol Diagn Dermatol 2019;6:109-11
|How to cite this URL:|
Chauhan SK, Gulati A, Tegta GR, Gupta M. Clinicopathological correlation in diagnosis of generalized eruptive histiocytosis. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2020 Jun 1];6:109-11. Available from: http://www.ijdpdd.com/text.asp?2019/6/2/109/271944
Histiocytoses are a rare group of disorders resulting from reactive or malignant proliferation of mononuclear phagocyte and immunoregulatory effector system. Generalized eruptive histiocytosis (GEH) is a rare variant of histiocytosis first described by Winkelmann and Muller. There are only approximately 43 case reports of GEH till date. We are reporting a 41-year-old male who was suspected as Hansen's disease, but clinicopathological correlation confirmed GEH.
A 41-year-old male presented with multiple, discrete asymptomatic brown-to-reddish symmetrically distributed lesions all over the body for 3 months. He denied for a visit to area endemic for leishmaniasis. Cutaneous examination revealed enumerable papules, nodules, and plaques predominantly over the face, neck, trunk, and extremities with sparing of scalp, palms, soles, and mucosae [Figure 1], [Figure 2], [Figure 3]. There was no evidence of systemic or neurological involvement. Clinical and histopathological examination suggested lepromatous leprosy. Slit-skin smear examination from active lesion did not demonstrate acid-fast bacilli (AFB) or Leishman–Donovan (LD) bodies. Routine hematology, biochemistry, serum angiotensin-converting enzyme, and lipid profile were normal. Serological markers for hepatitis, HIV, and syphilis were negative. Chest X-ray, abdominal ultrasonography, computed tomography abdomen, and ophthalmological examination revealed no extracutaneous involvement. But because of slit-smear negativity and spontaneous resolution of few lesions leaving hyperpigmented macules [Figure 4], a review histopathological examination was done at our institute. There were no epidermal changes, grenz zone was present, and nodular aggregates initially thought to be granulomas were seen to be mainly composed of foamy histiocytes bearing irregular nuclei [Figure 5] and [Figure 6]. No giant cells were seen, and no AFB was demonstrated. So after clinicopathological correlation, GEH was diagnosed, which was confirmed by immunohistochemistry (positivity for CD68 and negative staining for CD34, S100, CD1a, and XIIIa factor). He was offered no treatment but was advised to come for regular follow-up.
|Figure 1: Multiple papules, nodules, and plaques over the chest and abdomen at the first presentation|
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|Figure 2: Nodules and plaque over the face at presentation and after 1 month of follow-up examination|
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|Figure 4: Self-resolution and healing of these lesions with postinflammatory erythema and hyperpigmentation after 1 month|
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|Figure 5: Normal epidermis with grenz zone and dermal nodules of histiocytes (H and E, ×40)|
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|Figure 6: Collection of histiocytes admixed with lymphocytes around eccrine glands (H and E, ×400)|
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According to Emile et al., GEH belongs to cutaneous group and xanthogranuloma (XG) family of histiocytosis. Our patient fulfilled the distribution, morphology, progression, resolution, and histopathological criteria of disease proposed by Winkelman. The atypical findings in our case were that onset of lesion was from the face and coalescence of lesions at places. Similar findings have been reported in literature.
GEH mimics other xanthoma family histiocytosis, lepromatous leprosy, sarcoidosis, secondary syphilis, diffuse cutaneous and post-kala-azar dermal leishmaniasis (PKDL), and cutaneous T-cell lymphoma. Grouped nodule and plaques are seen in progressive nodular histiocytosis with mucosal and systemic involvement without any regression. Xanthoma disseminatum has prominent flexural and mucocutaneous involvement. Benign cephalic histiocytosis is seen in children, but lesions are localized to the head and neck. Histopathologically in non-Langerhans cell histiocytosis (non-LCH) admixture of vacuolated cells, spindle-shaped, xanthomatous, and multinucleated giant cells (Touton, foreign body, or Langerhans type) cells are seen. It is the predominant type of cell which determines the type of histiocytosis. Predominance of xanthomatized cells, foam cells, and Touton giant cells within the dermis are features of XG and papular xanthoma. Indeterminate cell histiocytosis mimics GEH clinicopathologically, but immunohistochemistry shows features of both LCH and non-LCH; thus, CD68, CD1a, and S100 are positive. Histoid/lepromatous leprosy may have grenz zone with nodular aggregates of foamy histiocytes and macrophages, but Ziehl–Neelsen staining should show AFB, and also, early spontaneous regression is not a feature. The distribution of lesions, lack of residing in endemic area, progression, and spontaneous resolution excluded leishmaniasis. Furthermore, nodular lesions in PKDL histopathologically may show a grenz zone, but the infiltrate is diffuse, with plenty of plasma cells, and macrophages are loaded with LD bodies. Sarcoidal granulomas lack foamy histiocytes, and there is no grenz zone. Nodular lesions of secondary syphilis have interface dermatitis with nodular infiltrate of neutrophils and plasma cells. In cutaneous T-cell lymphoma, epidermotropism and Pautier's microabscess with pleomorphic lymphocytes are seen.
Antimalarials, methotrexate, hydroxyurea, psoralen plus ultraviolet A, thalidomide, and isotretinoin  have been tried with variable results in GEH. GEH being a self -limiting disease no active intervention is required.
We want to conclude that in rare diseases which may mimic many other common conditions, misdiagnosis is frequent. Joining together, the various clinical, histopathological, and immunohistochemistry features solves the puzzle.
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
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