Erythema annulare centrifugum (EAC) is an annular, erythematous lesion that appears as urticarial-like papules and enlarges centrifugally, then clears centrally. A fine scale is sometimes present inside the advancing edge, known as a trailing scale. Erythema annulare centrifugum is classified as a reactive erythema and has been associated with various underlying conditions, including malignancies. When erythema annulare centrifugum occurs as a paraneoplastic phenomenon, it has been designated PEACE (paraneoplastic erythema annulare centrifugum eruption). PEACE is more commonly seen in females, typically precedes the clinical diagnosis of malignancy, and may recur with subsequent relapses.[1][2][3] EAC is one of the three major figurate erythemas, with EAC being the most common. These dermatoses share the common presentation of advancing erythematous, annular lesions, but are each separated by unique clinical and histopathologic characteristics. Once the other major figurate erythemas (erythema marginatum, erythema migrans, and erythema gyratum repens) are excluded, EAC often becomes a diagnosis of exclusion.[4][5]

Neutrophilic figurate erythema (NFE) has been rarely reported. This study aimed to identify the clinical and pathological features of NFE. We retrospectively reviewed the information from diagnostic cases from 2000 to 2013. The diagnosis of NFE includes clinically annular rash, histopathologically predominant neutrophilic perivascular and interstitial infiltrate in the dermis without evidence of vasculitis, and exclusion of other known specific entities. Fifteen cases of NFE were identified, including 11 women and 4 men. The age distribution was 18-66 years (average 41). The major characteristic patterns in NFE were blistering annular erythema (5/15 patients), purpuric annular erythema with vesicles (4/15 patients), and multiple annular rash with central ring-shaped scales (4/15 patients). There was no specific predicted location and no association with a major systemic disease. Papillary dermal edema and mild-to-moderate leukocytoclasis in the upper dermis are the main histopathological features. Ten of the 15 patients had recurrent episodes. Two patients who had single episode were associated with drug reaction. Antineutrophil therapy was required to control the symptoms in 3 patients. NFE has a similar clinical course as erythema annulare centrifugum but has distinct features that can be recognized clinically. The pathologists should be aware of the entity when making the diagnosis of neutrophil-mediated inflammatory disorders. The treatment regimen for neutrophilic dermatoses may be needed to manage the skin lesions.

We report a 68-year-old Korean man presenting with asymptomatic erythematous polycyclic annular firm plaques on his back that spread to the right shoulder. Histopathologic examination showed dense, diffuse infiltrates involving the entire dermis, consisting of atypical lymphocytes with many centrocytes and a few centroblasts. Spindle-shaped cells with elongated, twisted nuclei containing dispersed chromatin were also seen. Immunohistochemical analysis showed that all of the cells were strongly positive for CD20, CD21, CD79a and CD45, while they were negative for CD3, CD5, CD10, CD23, CD35, CD43, CD45RO and CD68. The spindle cells were also negative for smooth-muscle actin, desmin, S-100 and CD34. They consistently expressed nuclear bcl-6, but did not express bcl-2, multiple myeloma-1 and p16. We diagnosed him with primary cutaneous spindle cell B-cell lymphoma (PCSBCL) and treated him with six cycles of cyclophosphamide, adriamycin, vincristine, prednisone and rituximab (R-CHOP) chemotherapy; his skin lesions disappeared completely. Immunohistochemical profiles suggest that PCSBCL is a variant of primary cutaneous follicle center lymphoma.

The pathogenesis of vitiligo is still unknown. We saw a patient with vitiligo who may support an immunological pathogenesis for this disease. A 77-year-old man developed sharply demarcated, irregularly shaped, figurate, erythematosquamous plaques on most of the areas of his body. Histologically, lymphocytic cells invaded the lower epidermis. The lesions were gradually replaced by vitiliginous macules of the same configuration. Histologically, there were no active melanocytes in the vitiliginous lesions. We believe that the present patient presents an extraordinary example of inflammatory vitiligo and may provide an indication of an immunological pathogenesis for vitiligo.