Spontaneous Regression of a Plaque-Type Striated Muscle Hamartoma.

Striated muscle hamartoma (SMH) is a rare, congenital or acquired, benign tumor that predominantly affects children. Therapeutic management has classically been surgical intervention. We present a pediatric case of a facial plaque-type SMH with spontaneous regression that highlights the importance of clinical observation for a conservative approach.

The expression of podoplanin is associated with poor outcome in cutaneous squamous cell carcinoma.

BACKGROUND: Cutaneous squamous cell carcinoma (CSCC) is the second most frequent cancer in humans and can be both locally invasive and metastatic at distant sites. While research efforts have been made to predict poor outcome of CSCC, there is a lack of knowledge regarding molecular markers. Podoplanin has been associated with poor outcome in several types of cancer including CSCC, but this is controversial and only a few studies have evaluated the prognostic implications of podoplanin in the development of this tumor. METHODS: We evaluated podoplanin expression in a series of 94 CSCCs, and searched for associations between podoplanin expression and histopathological characteristics and with events of poor clinical evolution of the disease. RESULTS: Podoplanin expression was observed in 48.9% of the cases and the expression was considered moderate to intense in 19 of the cases. Moderate/intense podoplanin was associated with infiltrative growth pattern, desmoplasia, lymphovascular invasion, higher risk of nodal progression (NP) and short disease-free survival, specifically with a short latency to NP. CONCLUSIONS: This article provides evidence supporting the implication of podoplanin expression as a marker of bad prognosis of CSCC.

Multiple primary cutaneous plasmacytoma a decade after a nasal solitary extramedullary plasmacytoma: a puzzling case.

Primary cutaneous plasmacytoma should be in the differential diagnosis in case of solitary or multiple erythematous-violaceous nodules or papules. The diagnosis relies on clinical, histological, and immunochemical findings, without underlying evidence of multiple myeloma. Treatment should be individualized, and agents such as bortezomib or lenalidomide have shown to be effective.

Genome-Wide Pathway Analysis Identifies Genetic Pathways Associated with Psoriasis.

Psoriasis is a chronic inflammatory disease with a complex genetic architecture. To date, the psoriasis heritability is only partially explained. However, there is increasing evidence that the missing heritability in psoriasis could be explained by multiple genetic variants of low effect size from common genetic pathways. The objective of this study was to identify new genetic variation associated with psoriasis risk at the pathway level. We genotyped 598,258 single nucleotide polymorphisms in a discovery cohort of 2,281 case-control individuals from Spain. We performed a genome-wide pathway analysis using 1,053 reference biological pathways. A total of 14 genetic pathways (PFDR ≤ 2.55 × 10(-2)) were found to be significantly associated with psoriasis risk. Using an independent validation cohort of 7,353 individuals from the UK, a total of 6 genetic pathways were significantly replicated (PFDR ≤ 3.46 × 10(-2)). We found genetic pathways that had not been previously associated with psoriasis risk such as retinol metabolism (Pcombined = 1.84 × 10(-4)), the transport of inorganic ions and amino acids (Pcombined = 1.57 × 10(-7)), and post-translational protein modification (Pcombined = 1.57 × 10(-7)). In the latter pathway, MGAT5 showed a strong network centrality, and its association with psoriasis risk was further validated in an additional case-control cohort of 3,429 individuals (P < 0.05). These findings provide insights into the biological mechanisms associated with psoriasis susceptibility.

Cutaneous annular lesions as the first sign of transformation of follicular lymphoma into diffuse large B-cell lymphoma.

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma with diverse clinical, pathological and genetic features. An 80-year-old woman was diagnosed with a stage IV-X-A (Ann Arbor staging system) low grade systemic follicular lymphoma (FL). Four months after the diagnosis, she developed asymptomatic, indurated, annular erythematous plaques with centrifugal growth on the abdomen, arms and neck. The skin biopsy revealed a dermal infiltration compatible with diffuse large B-cell lymphoma. Light chain restriction by flow cytometry was demonstrated. The variable, diverse and joining genes of immunoglobulin G heavy chains were sequenced and cloned, and showed the same pattern for both the initial follicular lymphoma and the skin infiltration. Translocation t (14;18) was present in both samples. Based on these findings, a diagnosis of transformation of follicular lymphoma into diffuse large B cell lymphoma was made. Although other hematological disorders such as primary cutaneous diffuse large B cell lymphoma, mycosis fungoides and the cutaneous infiltration of chronic juvenile myeloid leukemia can present as annular lesions, we were unable to find any previous reports of these as a manifestation of cutaneous infiltration by systemic non-Hodgkin lymphoma.

Follicular mucinosis associated with nonlymphoid skin conditions.

BACKGROUND: Follicular mucinosis coexisting with lymphoproliferative disorders has been thoroughly debated. However, it has been rarely reported in association with inflammatory disorders. METHODS: Thirteen cases have been retrieved, and those with cutaneous lymphoma or alopecia mucinosa were excluded. RESULTS: Follicular mucinosis was found in the setting of squamous cell carcinoma, seborrheic keratosis, simple prurigo, acne vulgaris, dextrometorphan-induced phototoxicity, polymorphous light eruption (2 cases), insect bite (2 cases), tick bite, discoid lupus erythematosus, drug-related vasculitis, and demodecidosis. Unexpectedly, our observations revealed a preponderating accumulation of mucin related to photo-exposed areas, sun-associated dermatoses, and histopathologic solar elastosis. The amount of mucin filling the follicles apparently correlated with the intensity of perifollicular inflammatory infiltrate, which was present in all cases. The concurrence of dermal interstitial mucin was found in 7 cases (54%). CONCLUSIONS: The concurrence of interstitial dermal mucinosis or the potential role of both ultraviolet radiation and the perifollicular inflammatory infiltrates in its pathogenesis deserves further investigations. Precise recognition and understanding of this distinctive, reactive histological pattern may prevent our patients from unnecessary diagnostic and therapeutic strategies.

Photoletter to the editor: Localized pyoderma gangrenosum after interferon-alpha2b injections.

We present a male patient with polycythemia vera (PV) in whom pyoderma gangrenosum (PG) was induced by subcutaneous injections of interferon-α2beta (IFN-α2b).The patient presented with a 6 cm wide necrotic ulcer on the external aspect of his left thigh, which was surrounded by an erythematous and indurated plaque. He also had a simetrical but smaller 2 cm of size ulcer on the external aspect of the right thigh. Histopathological examination showed a massive perivascular and interstitial inflammatory infiltrate. It was vastly composed of neutrophils and secondary formation of interstitial neutrophilic microabscesses was also observed.To our knowledge only two cases of PG secondary to IFN-α2b injections have been reported, none of them in a patient with PV. Physicians should be aware of these IFN-α2b-related local adverse effects as they might become extremely severe. Immediate local discontinuation of drug administration is mandatory. In order to avoid these complications, alternating injection sites is highly advisable.

Gemcitabine-associated livedoid thrombotic microangiopathy with associated sclerema neonatorum-like microscopic changes.

Gemcitabine is a deoxycytidine analog antimetabolite that is now accepted as first-line treatment for advanced and metastatic pancreatic carcinoma. Gemcitabine-related thrombotic microangiopathy associated with systemic hemolytic-uremic syndrome or thrombotic thrombocytopenia purpura has rarely been described. Herein, we report a patient who developed a livedoid thrombotic microangiopathy with no signs of associated hemolytic-uremic syndrome. Cutaneous thrombotic microangiopathy occurred after the administration of his 17th cycle and a cumulative dose of 53.65 g/m(2) of gemcitabine. Some authors have suggested that this toxicity may be dose-related, and a 10th cycle or a cumulative dose of 9-56 g/m(2) have been proposed as the prothrombotic threshold. Interestingly, dermatopathologic findings were limited to the subcutis and they consisted of small-vessel occlusion by intravascular fibrin and leukocytes, vessel wall thickening and endothelial cell swelling. Surprisingly, we observed some structures arranged radially with needle-shaped clefts resembling those of sclerema neonatorum. Awareness of this potential cutaneous toxicity by dermatologists and dermatopathologists is extremely important.