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Peculiar distribution of tumorous xanthomas in an adult case of erdheim-chester disease complicated by atopic dermatitis.

Murakami Y, Wataya-Kaneda M, Terao M, Azukizawa H, Murota H, Nakata Y, Katayama I - Case Rep Dermatol (2011)

Bottom Line: He also had exophthalmos and diabetes insipidus.A histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells, which were positive for CD68 and CD163 and negative for S-100 and CD1a.We suggest that the complication of atopic dermatitis may have contributed to the uncommon clinical features in this case.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.

ABSTRACT
Erdheim-Chester disease is a rare non-Langerhans form of histiocytosis with multiple organ involvement. Approximately 20% of patients have xanthoma-like lesions, usually on the eyelids. We report a case of Erdheim-Chester disease in a 32-year-old male who showed peculiar xanthomatous skin lesions and also had atopic dermatitis. His skin manifestations included ring-like yellowish tumors on his periorbital regions, rope necklace-like tumors on his neck, and spindle-shaped tumors on his right preauricular region and cubital fossas. He also had exophthalmos and diabetes insipidus. Chronic eczematous lesions were present on the flexor aspect of his extremities, and his serum eosinophil numbers and immunoglobulin E levels were elevated. A histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells, which were positive for CD68 and CD163 and negative for S-100 and CD1a. We suggest that the complication of atopic dermatitis may have contributed to the uncommon clinical features in this case.

No MeSH data available.


Related in: MedlinePlus

a Ring-like yellowish tumors in the periorbital regions. b, c Rope necklace-like yellowish tumors on the patient's neck. d Spindle-shaped yellowish tumors on the patient's cubital fossas and diffuse erythema and pigmentation on the patient's forearm.
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Figure 1: a Ring-like yellowish tumors in the periorbital regions. b, c Rope necklace-like yellowish tumors on the patient's neck. d Spindle-shaped yellowish tumors on the patient's cubital fossas and diffuse erythema and pigmentation on the patient's forearm.

Mentions: A 32-year-old male presented with xanthomatous skin lesions, exophthalmos, and diabetes insipidus. His left retro-orbital mass was partially removed in childhood at another hospital, and he had been diagnosed with Hand-Schüller-Christian disease (HSCD). His past medical history included hyperlipidemia, type 2 diabetes mellitus, hypertension, and hyperuricemia, which were managed with diet and medication (nateglinide, candesartan cilexetil, and allopurinol). Cutaneous examination revealed ring-like yellowish tumors on his periorbital regions (fig. 1a), rope necklace-like tumors on his neck (fig. 1b, c), and spindle-shaped tumors on his right preauricular region and cubital fossas (fig. 1d). Histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells (fig. 2a). Immunohistochemical staining revealed that the cells were positive for CD68 (fig. 2b) and CD163 (fig. 2c) and negative for S-100 and CD1a. X-rays of the long bones of the upper and lower extremities showed no apparent osteosclerotic or osteolytic changes. Exophthalmos, diabetes insipidus, and the pathological findings confirmed the diagnosis of Erdheim-Chester disease (ECD; table 1). The patient had also been diagnosed with atopic dermatitis in childhood. He demonstrated diffuse facial erythema, pruritic chronic eczematous lesions on the trunk and flexor aspect of the extremities (fig. 1d), and multiple prurigo of the extremities. Laboratory findings related to atopic dermatitis were white blood cells: 12,640/μl, eosinophils: 19%, immunoglobulin E-radioimmunosorbent test (IgE-RIST) score: 38,500 IU/ml, IgE radioallergosorbent test (IgE-RAST) score of Dermatophagoides farinae: 99.9 IU, and thymus- and activation-regulated chemokine (TARC): 47,980 pg/ml. Therefore, our patient was diagnosed with ECD complicated by atopic dermatitis.


Peculiar distribution of tumorous xanthomas in an adult case of erdheim-chester disease complicated by atopic dermatitis.

Murakami Y, Wataya-Kaneda M, Terao M, Azukizawa H, Murota H, Nakata Y, Katayama I - Case Rep Dermatol (2011)

a Ring-like yellowish tumors in the periorbital regions. b, c Rope necklace-like yellowish tumors on the patient's neck. d Spindle-shaped yellowish tumors on the patient's cubital fossas and diffuse erythema and pigmentation on the patient's forearm.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3104866&req=5

Figure 1: a Ring-like yellowish tumors in the periorbital regions. b, c Rope necklace-like yellowish tumors on the patient's neck. d Spindle-shaped yellowish tumors on the patient's cubital fossas and diffuse erythema and pigmentation on the patient's forearm.
Mentions: A 32-year-old male presented with xanthomatous skin lesions, exophthalmos, and diabetes insipidus. His left retro-orbital mass was partially removed in childhood at another hospital, and he had been diagnosed with Hand-Schüller-Christian disease (HSCD). His past medical history included hyperlipidemia, type 2 diabetes mellitus, hypertension, and hyperuricemia, which were managed with diet and medication (nateglinide, candesartan cilexetil, and allopurinol). Cutaneous examination revealed ring-like yellowish tumors on his periorbital regions (fig. 1a), rope necklace-like tumors on his neck (fig. 1b, c), and spindle-shaped tumors on his right preauricular region and cubital fossas (fig. 1d). Histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells (fig. 2a). Immunohistochemical staining revealed that the cells were positive for CD68 (fig. 2b) and CD163 (fig. 2c) and negative for S-100 and CD1a. X-rays of the long bones of the upper and lower extremities showed no apparent osteosclerotic or osteolytic changes. Exophthalmos, diabetes insipidus, and the pathological findings confirmed the diagnosis of Erdheim-Chester disease (ECD; table 1). The patient had also been diagnosed with atopic dermatitis in childhood. He demonstrated diffuse facial erythema, pruritic chronic eczematous lesions on the trunk and flexor aspect of the extremities (fig. 1d), and multiple prurigo of the extremities. Laboratory findings related to atopic dermatitis were white blood cells: 12,640/μl, eosinophils: 19%, immunoglobulin E-radioimmunosorbent test (IgE-RIST) score: 38,500 IU/ml, IgE radioallergosorbent test (IgE-RAST) score of Dermatophagoides farinae: 99.9 IU, and thymus- and activation-regulated chemokine (TARC): 47,980 pg/ml. Therefore, our patient was diagnosed with ECD complicated by atopic dermatitis.

Bottom Line: He also had exophthalmos and diabetes insipidus.A histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells, which were positive for CD68 and CD163 and negative for S-100 and CD1a.We suggest that the complication of atopic dermatitis may have contributed to the uncommon clinical features in this case.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.

ABSTRACT
Erdheim-Chester disease is a rare non-Langerhans form of histiocytosis with multiple organ involvement. Approximately 20% of patients have xanthoma-like lesions, usually on the eyelids. We report a case of Erdheim-Chester disease in a 32-year-old male who showed peculiar xanthomatous skin lesions and also had atopic dermatitis. His skin manifestations included ring-like yellowish tumors on his periorbital regions, rope necklace-like tumors on his neck, and spindle-shaped tumors on his right preauricular region and cubital fossas. He also had exophthalmos and diabetes insipidus. Chronic eczematous lesions were present on the flexor aspect of his extremities, and his serum eosinophil numbers and immunoglobulin E levels were elevated. A histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells, which were positive for CD68 and CD163 and negative for S-100 and CD1a. We suggest that the complication of atopic dermatitis may have contributed to the uncommon clinical features in this case.

No MeSH data available.


Related in: MedlinePlus