Erythematous-violaceous Plaques Research Articles

Cutaneous vascular calcification. Perieccrine calcification as a diagnostic key for calciphylaxis.

Attempts have been made to establish discriminative criteria between classic calciphylaxis (CPX) and those cases in which cutaneous vascular calcification (CVC) represents an incidental finding (epiphenomenon). Retrospective, observational cohort study of patients with CVC to distinguish clinicopathological features between CVC as classic CPX (CVC in cutaneous lesions with erythematous-violaceous plaques with or without ulceration) or as an epiphenomenon (CVC in cutaneous lesions with known diagnosis). Different clinicopathological parameters and the presence of perieccrine calcification and pseudoxanthoma elasticum (PXE)-like changes were evaluated. Sixty-six patients were studied. The CPX group showed a significantly higher percentage of renal failure, hypertension, altered laboratory parameters, painful lesions, and mortality rate. Histopathologically, the CPX group was associated with more than one vessel per field involved with subintimal concentric calcification and perieccrine calcification (observed exclusively in the CPX group), while PXE-like changes, although more frequent in the CPX group, were also observed in the epiphenomenon group. Perieccrine calcification and the presence of more than one vessel per field involved by concentric pattern calcification could be used as a diagnostic marker of CPX. Although PXE-like changes are not an exclusive marker, they could suggest CPX diagnosis.

PLASMA CELL CHEILITIS SUCCESSFULLY TREATED WITH TOPICAL CORTICOSTEROID AND CRYOTHERAPY

Plasma cell cheilitis (PCC) is an unusual benign plasma cell proliferative disease of an unknown etiology that clinically presents as erythematous-violaceous plaques, ulcerations, or eruptions often affecting the lower lip. PCC is known to respond poorly to corticosteroid treatment. A 43-year-old male presented with multiple painful hemorrhagic crusts on the lower lip that had developed over 1 month. An incisional biopsy was performed and microscopy showed a bandlike inflammatory cell infiltration composed mainly of plasma cells in the connective tissue. Immunohistochemistry confirmed a reactive nature of plasma cells. Thus, the diagnosis of PCC was established. Initially, the patient was treated with topical corticosteroids over 1 month, but had a relapse 3 months after complete remission. Then, we proposed the use of topical corticosteroids with cryotherapy. No recurrence was observed after 8 months of follow-up. In conclusion, cryotherapy associated with topical corticosteroids appears to be a successful option to control the relapses of PCC.

Enfermedad de Rosai-Dorfman con presentación cutánea y ausencia de mutaciones BRAF-V600, KRAS y NRAS: ¿trastorno neoplásico o reactivo

Non-Langerhans cell histiocytosis, including Rosai-Dorfman disease (RDD) and xanthogranuloma are rare disorders with occasional overlapping in the histopathological and immunohistochemical (IHC) findings. We report the case of a 53-year-old woman with erythematous-violaceous plaques on the cheeks and edema in the auricular pavilions. A biopsy was performed and the histopathological examination revealed a histiocytic proliferation with emperipolesis characteristic of RDD and lymphoplasmocitic infiltrate. IHC analysis showed S100 and CD68 positivity in the histiocytes but was negative for CD1a, supporting the diagnosis of RDD. Molecular analysis failed to detect BRAF-V600, NRAS or KRAS mutation. We discuss the differential diagnosis of cutaneous non-Langerhans cell histiocytosis. Pathologist must be aware of unusual presentations of RDD and further treatment options must be explored for patients with unresectable lesions and/or resistance to the classical management of RDD.

Necrolytic Acral Erythema

Background: Necrolytic acral erythema (NAE) is a rare dermatosis which has been regarded as an early cutaneous marker of hepatitis C virus infection. The clinical manifestasion of NAE is similar to necrolytic migratory erythema, psoriasis and tinea corporis. The difference is that the patients with NAE also suffer from hepatitis C virus infection. Case: A 59 year old woman came and complained about itchy erythematous-violaceous plaques since a year ago. The patient has a history of hepatitis C infection since 2 years ago. On the superior and inferior extremities region, there were erythematous-violaceous plaques witch is partially hyperpigmentation with well-demarcated border, multiple discretes with thin scales and lichenification. Histopathological examination of the lesion obtained psoriasiform, hyperkeratosis, neutophylic microabscess, epidermal necrosis, spongiosis and infiltration of inflammatory cells in the epidermis. Discussion: Necrolytic acral erythema has been reported exclusively in patients with hepatitis C and is thought to be pathognomonic of this infection. Acute lesions often show erythema with vesicles and flaccid bullae. Chronic lesions appear as erythematous to violaceous plaques with thick scales, erosions and crust. Acral sites are predominantly involved. The histopathological examination shows psoriasiform hyperplasia epidermal, neutrophylic microabscesses, dilatation of small vessels, parakeratosis and infiltration of inflammatory cells. In this case, the physical and histopathological examination support the diagnosis of NAE.

Localized Post-Radiation Kaposi Sarcoma in a Renal Transplant Immunosuppressed Patient

Organ transplant recipients are at high risk to develop secondary cutaneous neoplasms because of immunosuppression. However, little is known about secondary neoplasms developing within a skin area exposed to radiation therapy in these patients. We report a case of a 45-year-old man with history of kidney transplantation in 2005 and rectal adenocarcinoma in 2006 for which he underwent 2 cycles of chemotherapy and a cycle of radiotherapy. In February 2010, he presented with clustered erythematous-violaceous plaques and nodules of 2-month duration, located on the left buttock in the area previously exposed to radiations. Histological examination revealed a poorly demarcated dermal and subcutaneous proliferation of spindle and partly pleomorphic cells, associated with irregularly shaped vessels that dissected through dermal collagen. Immunohistochemistry showed expression of CD31 and podoplanin. Although a moderate expression of the c-Myc protein was found by immunostaining, no amplification of c-myc gene was detected by fluorescence in situ hybridization. Human herpes virus 8 was positive both on immunohistochemistry and PCR. Based on clinicopathologic findings a diagnosis of iatrogenic Kaposi sarcoma localized in the area treated with radiotherapy was made. Clinical and histopathological features of vascular neoplasms may be overlapping, and correct diagnosis may be difficult, particularly in organ transplant recipients. Only the combination of all available information, including histopathological, immunohistochemical, fluorescence in situ hybridization, and PCR data, permit to achieve a correct diagnosis in particularly difficult setting.

Cutaneous thrombosis related to paroxysmal nocturnal hemoglobinuria: Clinical report

Paroxysmal nocturnal hemoglobinuria is a clonal stem cell disorder typically characterized by hemolysis, bone marrow failure, and venous thrombosis. The latter can affect up to 40 percent of patients and is a significant cause of mortality. Despite being recognized as a possible complication, cutaneous thrombosis is quite uncommon and a variety of findings have been reported. Owing to the rarity of this complication and the importance of its correct and timely diagnosis, we report the case of a 64-year-old man with paroxysmal nocturnal hemoglobinuria who presented with isolated and well-demarcated erythematous-violaceous plaques, mainly located on the neck and trunk. Given the history and the histological features, the diagnosis of cutaneous thrombosis related to paroxysmal nocturnal hemoglobinuria was assumed and the patient was successfully treated with therapeutic anticoagulation. The early identification of these patients is essential for necessary surveillance because they could carry a higher probability of recurrence compared with those with no prior complications.

Splenic Marginal Zone B-Cell Lymphoma with Epidermotropic Skin Involvement

Marginal zone B-cell lymphoma (MZL) is subclassified into extranodal MZL of mucosa-associated lymphoid tissue (including cutaneous lymphomas), splenic MZL, and nodal MZL. We report the case of a 68-year-old man with erythematous-violaceous plaques and nodules. Skin biopsy showed an epidermotropic lymphocytic infiltration and cytology and immunohistochemistry were consistent with MZL. The workup revealed disease in the peripheral blood and bone marrow and massive splenomegaly. Splenectomy confirmed the diagnosis of splenic MZL and led to resolution of the skin lesions. Cutaneous recurrence was treated successfully with chemotherapy and rituximab but caused fatal hepatitis due to hepatitis B virus reactivation. Skin involvement by splenic MZL is uncommon; this form of the disease can present epidermotropism, a very rare finding in primary cutaneous MZL. Treatment consists of splenectomy, which may be associated with chemotherapy and/or rituximab; this treatment may lead to reactivation of latent hepatitis B infection and screening for hepatitis should therefore be performed prior to starting therapy.

Chilblain lupus induced by TNF-α antagonists: a case report and literature review

We report the case of a 72-year-old man with history of ankylosing spondylitis, who, during the treatment with infliximab, developed painful, erythematous-violaceous plaques with later development of ulcers on his feet associated with cold exposure. Concomitantly with the appearance of these lesions, he presented increased antinuclear antibodies (ANA) titers, positivity for anti-DNA and IgM anticardiolipin antibodies, low complement levels, polyclonal hypergammaglobulinemia, and lymphopenia. He was diagnosed of chilblain lupus induced by infliximab, this agent was withdrawn and initiated treatment for chilblains with improvement of lesions. On reviewing of the literature, we found seven reported cases of tumor necrosis factor α (TNF-α) antagonists-induced chilblain lupus, all in rheumatoid arthritis patients and four of them with clinical and immunological characteristics available are presented and compared with our case. Although it is infrequent, chilblain lupus forms part of the spectrum of TNF-α antagonists-induced lupus erythematosus; usually is limited to skin without progression to systemic lupus erythematosus; presents ANA, anti-DNA, and antinucleosome antibodies positivity as more frequent immunological alterations; and responds appropriately to the specific treatment, TNF-α antagonists withdrawal being not necessary in almost all cases.

Linfoma B esplénico de la zona marginal con afectación cutánea epidermotropa

Marginal zone B-cell lymphoma (MZL) is subclassified into extranodal MZL of mucosa-associated lymphoid tissue (including cutaneous lymphomas), splenic MZL, and nodal MZL. We report the case of a 68-year-old man with erythematous-violaceous plaques and nodules. Skin biopsy showed an epidermotropic lymphocytic infiltration and cytology and immunohistochemistry were consistent with MZL. The workup revealed disease in the peripheral blood and bone marrow and massive splenomegaly. Splenectomy confirmed the diagnosis of splenic MZL and led to resolution of the skin lesions. Cutaneous recurrence was treated successfully with chemotherapy and rituximab but caused fatal hepatitis due to hepatitis B virus reactivation. Skin involvement by splenic MZL is uncommon; this form of the disease can present epidermotropism, a very rare finding in primary cutaneous MZL. Treatment consists of splenectomy, which may be associated with chemotherapy and/or rituximab; this treatment may lead to reactivation of latent hepatitis B infection and screening for hepatitis should therefore be performed prior to starting therapy.

Acral Psoriasiform Lesions

The patient presented symmetrical, erythematousviolaceous plaques on the distal phalanges of the fingers and toes; the plaques had a hyperkeratotic and desquamative surface (Figures 1 and 2). There was also slight reddening and desquamation of the lobes of the ears. In addition, the patient was asthenic, with mild jaundice of the skin and mucosas, and presented hepatomegaly and palpable right lateral cervical lymph nodes that formed a mass of approximately 4 by 3 cm (Figure 3). Additional Tests