A 32‐year‐old white man with no previous medical past was admitted to our department with an 8‐month intermittent history of acute spiking fever with axillary, inguinal, and neck lymphadenopathy, arthritis, and cutaneous eruption. Cutaneous examination showed nonpruriginous, indurated erythematous papules affecting the face, ears, and trunk, suggestive of subacute lupus erythematosus ( Fig. 1a,b). On general physical examination, no abnormalities were observed except for arthritis of the right ankle and, a few months later, arthritis of the left knee. Biological investigations revealed a raised erythrocyte sedimentation rate with a serum ferritin level at 800 ng/mL (normal < 200 ng/mL), a white blood cell count of 12,500/mm3 (with 80% neutrophils, 16% lymphocytes, and 4% monocytes), a hematocrit of 40%, and a hemoglobin level of 13.1 g/dL. Electrolytes and liver function tests were normal. Antinuclear antibodies and antibodies to deoxyribonucleic acid (DNA) were negative. Rheumatoid factor was absent. No evidence of the following infections/organisms was found on serologic screening: syphilis, Streptococcus, Toxoplasma, Mycoplasma, Brucella, Borrelia burgdorgferi, spotted fever, leptospirosis, hepatitis B and C virus, parvovirus, human immunodeficiency virus (HIV) 1 and 2, human T‐cell lymphotropic virus (HTLV) 1, Epstein–Barr virus (EBV), cytomegalovirus (CMV), herpes simplex virus (HSV) 6. Immunoglobulin M (IgM) HSV(1+2) by enzyme‐linked immunosorbent assay (ELISA) analysis with 1 year follow‐up was still detected. The articular synovial fluid of the involved ankle and knee was inflammatory but sterile. A bone marrow biopsy specimen revealed no lymphomatous involvement. Abdominal and chest scans were normal. Skin biopsy of a facial nodule showed, in the dermis and in the subcutaneous fat tissue, a dense perivascular and periadnexal infiltrate composed of numerous plasmacytoid histiocytes and large‐ or medium‐sized lymphocytes ( Fig. 2a,b). No epidermal changes, edema in the papillary dermis, or vasculitis were found. Immunofluorescence staining of frozen tissue sections of a facial nodule showed granular deposits of C3. Inguinal lymph node biopsy revealed, in the paracortical area, focal necrosis with a proliferation of transformed lymphocytes, histiocytes, and numerous nuclear debris typical of histiocytic necrotizing lymphadenitis ( Fig. 3a,b). On immunohistochemical staining examination, CD68 was positive in pathologic paracortical areas. Few mononuclear cells were positive for CD3, CD8, and L‐26 ( Fig. 4). Polymerase chain reaction (PCR) amplification did not show human herpes simplex viruses in the skin biopsy specimen, and culture was also negative. 1 (a) Clinical aspect: erythematous papulonodules on the face. (b) Clinical aspect: erythematous papulonodules of the upper part of the trunk 2 (a) Perivascular and periadnexal dense lympho‐ histiocytic infiltrate involving the dermis (hematoxylin and eosin, × 10). (b) Plasmacytoid histiocytic infiltrate in the subcutaneous fat tissue (hem...

We report on a 32-year-old female with a 3-year history of an asymptomatic erythema of the forehead. The lesion had a linear distribution following the lines of Blaschko. Histopathological findings and direct immunofluorescence allowed to establish the diagnosis of cutaneous lupus erythematosus. Treatment with local corticosteroid and antimalarial agents given for 2 months resulted in a complete remission.

We report a 53-year-old woman who presented with multiple painful red cutaneous papules that had been growing slowly for 13 years. Histopathology showed typical features of neuroma. Biological, morphological and genetic investigations were negative and excluded the diagnosis of multiple endocrine neoplasia type 2b. After reviewing the literature, we concluded that our patient has an extremely unusual acquired disease, which must be considered as a distinct entity in the spectrum of cutaneous neurological disorders.

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