Neutrophilic dermatoses

Neutrophilic dermatoses are inflammatory disorders where normal neutrophils infiltrate the skin without infection. Sweet's syndrome, pyoderma gangrenosum, subcorneal pustulosis, erythema elevatum diutinum and a few other conditions are included in the spectrum of neutrophilic dermatoses. In addition to their typical presentation, atypical and overlap forms of these disorders may be observed. According to the location of the neutrophilic infiltrate, three clinical forms are distinguished: superficial (epidermal), en plaques (dermal) and deep (dermal and hypodermal). During the disease course, other tissues can be affected by the same neutrophilic infiltrate. An association with other systemic diseases including malignant blood disorders, inflammatory bowel diseases and autoimmune disorders is frequent. The mechanisms leading to the invasion of the skin by neutrophils remains not completely elucidated. Treatment depends on the intensity and the acute or chronic form of the disease. In acute and severe forms, systemic corticosteroids are first-line therapy, whereas colchicine, dapsone, and even topical corticosteroids could be used in milder presentations of the disease.     

Vemurafenib-induced neutrophilic panniculitis

Vemurafenib is a targeted therapy, used in patients with metastatic cutaneous melanoma who carry the BRAF V600E mutation, with a relative reduction of 63% in the risk of death. Several adverse events have been described previously, such as photosensitivity or squamous-cell carcinomas. Two cases of panniculitis have been reported recently with two different selective BRAF inhibitors. We report two cases of neutrophilic panniculitis in patients treated by vemurafenib for a metastatic melanoma. Clinical and biological examinations showed no indications for an immune nor an infectious cause of neutrophilic panniculitis. Thus, we believe that vemurafenib caused this panniculitis. Treatment with vemurafenib was maintained in both patients because of the clinical and radiological tumoral responses. One patient showed spontaneous recovery, whereas the other patient presented several recurrences of panniculitis. We believe that physicians should be aware of this cutaneous side effect of vemurafenib, but it should not lead to discontinuation of this treatment.     

Captopril-induced lichen planus pemphigoides with pemphigus-like features. A case report

We report a case of a bullous lichenoid eruption due to the intake of captopril. Clinical, histological, direct immunofluorescence and ultrastructural features were consistent with the diagnosis of lichen planus pemphigoides. In addition, the in vivo immunological study also revealed an intercellular fluorescence, similar to that seen in pemphigus. Complex drug-induced cutaneous reactions have been previously reported with other drugs, especially with D-penicillamine, which bears chemical similarities with captopril. However, such a drug-induced mixed pattern of lichen planus pemphigoides with pemphigus-like features has never been reported.     

Cutaneous manifestations of neutrophilic disease. A study of seven cases.

Seven patients with a complex form of neutrophilic dermatosis are reported. Clinically, they had variable associations of four types of lesions: blisters/pustules, plaques, nodules and ulcerations. Histologically, a neutrophilic infiltrate was observed at variable levels in the epidermis, dermis and subcutis. Systemic manifestations were present in all cases (general symptoms, joint, renal, ocular and lung involvements). Three patients had an associated disease (myelodysplasia, metastatic carcinoma, IgG gammopathy). Steroids were the most efficient treatment. These observations, as well as a review of the literature, support the opinion that the neutrophilic dermatosis represents a continuous spectrum encompassing four well-defined entities: subcorneal pustular dermatosis, Sweet's syndrome, erythema elevatum diutinum and pyoderma gangrenosum. We propose that the different patterns of the neutrophilic dermatosis are the most obvious manifestations of a potentially multisystemic neutrophilic disease and allow its recognition.