Amparo Sáez

Mucoid degeneration of the anterior cruciate ligament with erosion of the lateral femoral condyle

We report a case of a mucoid degeneration of the anterior cruciate ligament (ACL) that produced osseous erosion of the medial aspect of the lateral femoral condyle. The MRI findings and differential diagnosis are discussed.

Multicentric Reticulohistiocytosis-Mimicking Dermatomyositis

Multicentric reticulohistiocytosis is a rare disorder of unknown etiology, characterized by skin and mucosal papulonodular eruptions and destructive polyarthritis. Histopathological study of these lesions shows a nodular infiltrate composed of histiocytes and multinucleated giant cells, with an eosinophilic, granular, ‘ground-glass’ cytoplasm. We report a case of multicentric reticulohistiocytosis with skin lesions mimicking dermatomyositis and we also review previously reported cases describing such a clinical situation. Our case further emphasizes that multicentric reticulohistiocytosis can mimic clinical features of dermatomyositis. A macular or patch-like erythema in a photodistributed fashion, in addition to other clinical manifestations, can be mistaken for dermatomyositis. However, skin biopsies from these areas may early differentiate both conditions with different treatment options and morbidity.

Identification of t(17;22)(q22;q13) (COL1A1/PDGFB) in dermatofibrosarcoma protuberans by fluorescence in situ hybridization in paraffin-embedded tissue microarrays

Dermatofibrosarcoma protuberans is genetically characterized by the translocation t(17;22)(q22;q13) resulting in the PDGFB/COL1A1 fusion gene. Fluorescence in situ hybridization with specific probes enables a rapid detection of this gene. In this study, the presence of the translocation t(17;22)(q22;q13) by fluorescence in situ hybridization in paraffin-embedded tissue microarrays was analyzed. Two tissue microarrays including 40 cases of dermatofibrosarcoma protuberans and 20 dermatofibromas were evaluated. Fluorescence in situ hybridization analyses were performed using a dual-color dual-fusion noncommercial probe. Clinical and histopathologic features were examined, and the association with fluorescence in situ hybridization results was assessed. A total of 29 samples of dermatofibrosarcoma protuberans and 16 of dermatofibromas were successfully evaluated. Twenty-five (86%) dermatofibrosarcoma protuberans samples were positive for the translocation, which was absent in all samples of dermatofibromas. Two of the negative dermatofibrosarcoma protuberans showed unusual, hypercellular areas with marked cytologic atypia, whereas 1 case exhibited overlap features with dermatofibroma. Tumors with fibrosarcomatous areas seemed to have a higher percentage of positive cells and the number of copies of the COL1A1/PDFGB gene. In conclusion, the COL1A1/PDGFB fusion gene was present in most of the dermatofibrosarcoma protuberans tissue samples. The detection of the translocation may be an additional diagnostic tool in cases of dermatofibrosarcoma protuberans showing nonconclusive histologic features.

Localized Xanthomas Associated with Primary Lymphedema

Abstract:  Cutaneous xanthomas arising in chronic lymphedema are rare. We present a case of verruciform xanthoma involving the left foot and toes of a 10‐year‐old boy who had developed a primary lymphedema (lymphedema praecox) in the left lower extremity. Laboratory studies demonstrated a normal lipid profile.

Cutaneous granulomatous panniculitis and sarcoidal granulomatous papular eruption in a patient with metastatic melanoma treated with a BRAF inhibitor

Dear Editor, Metastatic malignant melanoma is associated with poor outcomes. The fact that up to 80% of cases of primary melanoma harbor mutations in the BRAF gene has led to the development and recent approval of novel BRAF inhibitor drugs that, in comparison with dacarbazine, significantly improve progression-free survival. Because of limited experience with these drugs, a number of unusual side-effects have not yet been described. A 41-year-old Caucasian woman was included in a clinical trial of dabrafenib therapy (150 mg twice daily) because she had a stage IV melanoma, harboring a BRAF mutation, that failed to respond to radiotherapy and ipilimumab (CTLA-4 inhibitor). Eight months after inclusion, she presented with high fever, arthralgia, joint swelling and tender, painful, erythematous subcutaneous nodules located mainly in the upper and lower limbs (Fig. 1a). A punch biopsy revealed a lobular pattern of panniculitis with a predominantly lymphohistiocytic infiltrate forming non-necrotizing granulomas, a scarcity of neutrophils and no evidence of vasculitis (Fig. 1b). The lesions resolved completely in response to a tapered regime of corticosteroids. Five months later, the patient presented with multiple 1–1.5mm non-painful erythematous and violaceous papules and nodules, scattered bilaterally and symmetrically over the antecubital skinfolds and dorsal aspect of the hands (Fig. 1c). The patient had no systemic symptoms. Anatomopathological findings for a second punch biopsy revealed a full-thickness dermal infiltrate of numerous epithelioid histiocytes and giant multinucleated cells forming non-necrotizing granulomas. A surrounding crown of lymphocytes was present in all the granulomas, prominent in approximately half and less noticeable otherwise (Fig. 1d). No Melan-A-positive cells were found in relation to the inflammatory infiltrate in either of the biopsies, indicating an absence of cancer cells. The rash responded well to treatment with a topical steroid ointment. The main causes of non-necrotizing granulomas, namely, sarcoidosis and infections, were excluded and both the 8and 13-month reactions were attributed to treatment with dabrafenib. After 18 months, pulmonary progression of malignant melanoma was detected and dabrafenib therapy was suspended. The remaining granulomatous papules resolved completely within 2 weeks of dabrafenib suspension. Although the pathogenic mechanisms of granuloma formation have not yet been unraveled, our case reflects a previously unreported association between two infrequent granulomatous toxicities in the same patient, mild in nature and requiring no treatment discontinuation. Isolated noncaseating granulomas as reported by us were found in one of two cases of panniculitis described by Zimmer et al. for patients receiving BRAF inhibitor therapy. As for the diagnosis of granulomatous dermatitis, only two cases have been described to date, referring to three patients treated with BRAF inhibitors. In these cases, the rash consisted of multiple asymptomatic erythematous non-scaly and violaceous papules scattered bilaterally over the upper and lower (a)

Granuloma piogénico recurrente con satelitosis

Resumen —El granuloma piogenico es una tumoracion vascular benigna de la piel y las mucosas que puede aparecer espontaneamente o tras un traumatismo. Se describe el caso de un varon de 15 anos de edad que, tras la exeresis de un granuloma piogenico en la zona clavicular, desarrollo multiples lesiones alrededor de la lesion primaria. La aparicion de satelitosis es un fenomeno poco frecuente.

Dermatomyofibroma on the nape of the neck: a report of 2 pediatric cases.

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Pleomorphic mastocytoma in an adult

Cutaneous mastocytoma (CM) is a localized variant of mastocytosis, characterized by an over‐accumulation of mast cells in the skin, without extra‐cutaneous organ involvement. It is defined as the presence of up to 3 isolated mast‐cell skin lesions and commonly develops in newborns and children. We report the case of a 35‐year‐old healthy Caucasian woman presenting with a 4‐year history of a pruritic brown plaque on her left breast. Hematoxylin‐eosin staining showed a dense dermal infiltrate of atypical mast cells extending to the subcutis. The cells presented a marked nuclear pleomorphism with bilobed and multilobed nuclei. Immunohistochemical studies revealed strongly expressed KIT (CD117) and CD25 proteins. Serum tryptase levels and bone marrow biopsy were normal. The diagnosis was a solitary cutaneous pleomorphic mastocytoma. This case can be added to 17 other cases of adult mastocytoma documented in the literature, although, unlike other reported cases, and as far as we are aware, this is the first case of pleomorphic mastocytoma in an adult.