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Adult Onset of BRAF(V600E)-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining.

Tono H, Fujimura T, Kakizaki A, Furudate S, Ishibashi M, Aiba S - Case Rep Dermatol (2015)

Bottom Line: Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement.Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH.In this report, we describe a 28-year-old Japanese man with adult onset of BRAF(V600E)-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining.

View Article: PubMed Central - PubMed

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

ABSTRACT
Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAF(V600E)-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAF(V600E) gene could be a diagnostic tool to determine the clinical type of LCH.

No MeSH data available.


Related in: MedlinePlus

Multiple crusted papules on the dorsal region of the left foot.
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Figure 1: Multiple crusted papules on the dorsal region of the left foot.

Mentions: A 28-year-old Japanese man visited our outpatient clinic with a 1-year history of pruritic papules on his extremities. He had noticed a slow-growing subcutaneous nodule on his right chest 2 years before, and half a year before this nodule had been diagnosed as adult-onset LCH and irradiated locally. On his initial visit, physical examination revealed multiple crusted papules on the trunk, lower legs and dorsal region of the foot (fig. 1). A biopsy specimen showed atypical large lymphocytes infiltrated mainly in the perivascular region of the upper dermis with involvement of the overlying epidermis (fig. 2a). Immunohistochemical staining revealed that these atypical lymphocytes were positive for CD1a (fig. 2b). Since the infiltrating cells were limited to the perivascular areas and it was difficult to diagnose these eruptions as cutaneous involvement of LCH or dermadromes of LCH, we employed immunohistochemical staining for BRAFV600E mutation. We detected p.V600E-mutated cells in the perivascular region of the upper dermis (fig. 2c, d). From the above findings, we diagnosed this patient as having adult-onset BRAFV600E-mutated LCH with cutaneous involvement. He was treated for LCH with a regimen of the Japan LCH Study Group protocol C (vinblastine, prednisolone, methotrexate, mercaptopurine) as described previously [2]. One month after the administration of this regimen, in parallel with the induction of complete remission of LCH, his pruritic eruption disappeared.


Adult Onset of BRAF(V600E)-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining.

Tono H, Fujimura T, Kakizaki A, Furudate S, Ishibashi M, Aiba S - Case Rep Dermatol (2015)

Multiple crusted papules on the dorsal region of the left foot.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4608616&req=5

Figure 1: Multiple crusted papules on the dorsal region of the left foot.
Mentions: A 28-year-old Japanese man visited our outpatient clinic with a 1-year history of pruritic papules on his extremities. He had noticed a slow-growing subcutaneous nodule on his right chest 2 years before, and half a year before this nodule had been diagnosed as adult-onset LCH and irradiated locally. On his initial visit, physical examination revealed multiple crusted papules on the trunk, lower legs and dorsal region of the foot (fig. 1). A biopsy specimen showed atypical large lymphocytes infiltrated mainly in the perivascular region of the upper dermis with involvement of the overlying epidermis (fig. 2a). Immunohistochemical staining revealed that these atypical lymphocytes were positive for CD1a (fig. 2b). Since the infiltrating cells were limited to the perivascular areas and it was difficult to diagnose these eruptions as cutaneous involvement of LCH or dermadromes of LCH, we employed immunohistochemical staining for BRAFV600E mutation. We detected p.V600E-mutated cells in the perivascular region of the upper dermis (fig. 2c, d). From the above findings, we diagnosed this patient as having adult-onset BRAFV600E-mutated LCH with cutaneous involvement. He was treated for LCH with a regimen of the Japan LCH Study Group protocol C (vinblastine, prednisolone, methotrexate, mercaptopurine) as described previously [2]. One month after the administration of this regimen, in parallel with the induction of complete remission of LCH, his pruritic eruption disappeared.

Bottom Line: Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement.Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH.In this report, we describe a 28-year-old Japanese man with adult onset of BRAF(V600E)-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining.

View Article: PubMed Central - PubMed

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

ABSTRACT
Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAF(V600E)-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAF(V600E) gene could be a diagnostic tool to determine the clinical type of LCH.

No MeSH data available.


Related in: MedlinePlus