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Letter

Dermoscopy of Generalized Eruptive Histiocytosis: Case Report and Brief Review of the Literature

Author Affiliation(s)

Introduction

Generalized eruptive histiocytosis (histiocytoma) (GEH) is a very rare cutaneous non-Langerhans cell histiocytosis, characterized by recurrent crops of small red to brown papules.

Case Presentation

A 58-year-old woman with an 8-month history of hundreds of symmetric yellow-brown flat-topped papules ( Figure 1, A and B ) came for dermatologist consultation. The lesions appeared in crops localized on the trunk and extremities. It was believed that the skin lesions were caused by trazodone and lithium, which were being used in the treatment of bipolar disorder. The medications were stopped, but the lesions continued to develop. Routine blood and urine analyses were unremarkable.

Figure 1 .

(A,B) Symmetric yellow-brown, flat-topped papules localized on the trunk (A) and extremities (B). (C) Dermoscopic finding: orange-yellow homogeneous pigmentation, delicate linear branching, and serpentine vessels. Solitary, red clods are present in some lesions. (D) Dermoscopic finding after 3 months: partially regressed lesions reveal yellow background and linear serpentine vessels. (E) Focally dispersed small dermal granulomas composed of histiocytes and multinuclear giant cells with peripheral arrangement of nuclei (E,F) (H&E, ×100). (F) Focal emperipolesis is noted in giant cells (arrows) (H&E, ×400). (G) Positive immunohistochemical staining of CD68 in granulomas (magnification ×200). Immunohistochemically, granulomas presented with profile CD68+ CD163+/−, and CD1a−.

Dermoscopy was performed on one representative lesion on the lower leg. The histology was consistent with the diagnosis of GEH ( Figure 1, C–G ).

After GEH diagnostics, additional investigations were done. Hyperprolactinemia, hypercorticism, and hypofunction of the thyroid gland were detected. Abdominal ultrasonography and MRI of the sella turcica and hematological studies with biopsy of the bone marrow were without pathological findings. Three years from the first onset, the skin lesions mostly resolved, leaving hyperpigmented macules.

Literature Review

Seventy-four cases (58.1% males) of GEH have been published, including 24 (32.4%) children ( Table 1 and Supplementary References, which are appended to the pdf). The average age was 30.5 years. In adults and children the average age was 43.2 years and 4.2 years, respectively. The most frequent body site was the trunk (86%), followed by the extremities (79%). The lesions resolved spontaneously from 2 weeks onward, but in rare cases persisted for 20 years. In 4 children the lesions evolved into xanthoma disseminatum; in 1 child the lesions coexisted with juvenile xanthogranuloma.

Table 1

Clinical Characteristics of the Published Cases With Generalized Eruptive Histiocytosis (Histiocytoma) (GEH)

Although GEH is a benign, self-healing eruption of non-Langerhans cell lineage, follow-up is necessary.

Two age groups of GEH patients are reported: up to 14 years and adults. Brain infiltrations and diabetes insipidus are reported in 3 (12.5%) children up to age 4 years with xanthomatous evolvement. In 5 (10.0%) of the published adult cases, hematological disorders of myeloid lineage such as acute monocytic/monoblastic leukemia (2 cases) or chronic myelomonocytic/eosinophilic leukemia (3 cases) are reported ( Table 1 ). In preschool children, diabetes insipidus should be suspected if GEH evolves into xanthoma. In adults, hematological follow-up is suggested.

Clinical differential diagnosis of GEH includes other histiocytic disorders such as Letterer-Siwe disease, juvenile xanthogranuloma (multiple), papular xanthoma, and progressive nodular histiocytosis. Exanthema due to medications and viruses, with separate entity Gianotti-Crosti syndrome and early eruption of guttate psoriasis, are main differentials as well.

A case describing dermoscopy of GEH lesions resembling molluscum contagiosum in an infant has been published [ 1 ] . A homogeneous orange-yellow pattern with an erythematous border described as “setting-sun” was recognized. Histology revealed histiocytic cells with foamy xanthomatous cytoplasm [ 1 ] . The dermoscopic finding in our case presented with orange-yellow homogeneous pigmentation, delicate linear branching, serpentine vessels, and solitary, red clods. Histology revealed histiocytic cells forming granulomas.

The dermoscopic differential diagnosis of GEH is broad and encompasses juvenile xanthogranuloma, cutaneous sarcoidosis, necrobiosis lipoidica, granuloma annulare (palisading granuloma histological subtype), elastosis perforans serpiginosa, granulomatous rosacea, annular elastolytic giant cell granuloma, and rheumatoid nodules. Among infective diseases, lupus vulgaris, cutaneous leishmaniasis, borderline tuberculoid leprosy, and Majocchi granuloma are the most important differentials [ 2 ] . Combining clinical, dermoscopic, and histological findings is of greatest importance in any of the diseases mentioned.

Conclusions

Our case emphasizes the importance of dermoscopic examination in the everyday practice of dermatologists. Further studies of skin histiocytic disorders are required in order to establish all dermoscopic criteria.

Supplementary Material: Table 1 References

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