Interface dermatitis in skin lesions of Kikuchi-Fujimoto's disease: a histopathological marker of evolution into systemic lupus erythematosus?

Kikuchi's disease (KD) is a self-limiting histiocytic necrotizing lymphadenitis (HNL). Cutaneous manifestations are frequent and usually show histopathological findings similar to those observed in the involved lymph nodes. HNL with superposed histological features to KD has been described in patients with lupus erythematosus (LE), and a group of healthy patients previously reported as having HNL may evolve into LE after several months. Up to date, features to predict which HNL patients will have a self-limiting disease and which could develop LE have been not identified. In order to clarify the characteristics of skin lesions associated with KD, we report a case of HNL with evolution into systemic lupus erythematosus (SLE) and a review of previous reports of KD with cutaneous manifestations. A 17-year-old woman presented with a 4-month history of fever and generalised lymphadenopathy. A diagnosis of HNL was established based on a lymph node biopsy. One month later, she developed an erythematoedematous rash on her upper body, with histopathological findings of interface dermatitis. After 8 months, anti-nuclear antibodies (ANA) at titre of 1/320, anti-DNA-ds antibodies and marked decrease of complement levels were detected. During the following 2 years, she developed diagnostic criteria for SLE, with arthralgias, pleuritis, aseptic meningitis, haemolytic anaemia and lupus nephritis. To our knowledge, 27 cases of nodal and cutaneous KD have been reported, 9 of which later developed LE. In all these patients, the skin biopsy revealed interface dermatitis. Skin biopsy revealed a pattern of interface dermatitis in all reviewed KD cases, which evolved into LE. Even this histopathological finding was not previously considered significant; it might be a marker of evolution into LE.

Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus.

The term "Acute Syndrome of Apoptotic Pan-Epidermolysis" (ASAP) designs clinical entities characterized by massive cleavage of the epidermis resulting from hyperacute epidermal basal cell apoptotic injury. It can be seen typically in classic toxic epidermal necrolysis (TEN), but occasionally occurs in non-drug-induced entities called "TEN-like" diseases (e.g., lupus erythematosus (LE), acute graft versus host disease and pseudoporphyria). We would like to highlight the difficulties of establishing differential diagnoses between "TEN-like" LE and drug reactions, especially when LE has not been previously diagnosed. We report a patient with fulminate pattern of epidermal cell injury resulting in a clinical presentation having combined features of drug-induced TEN and acute cutaneous LE with laboratory findings of systemic LE (SLE) and without systemic symptoms or high-risk drug ingestion. Although most cases of ASAP in the setting of LE are drug-induced TEN, there are reported cases of "TEN-like" LE with subacute progression, absence of systemic involvement and lack of drug ingestion. Such cases usually have a previous history of SLE and positive serologic markers. Although some authors observed that these lesions could be related to systemic severity of SLE, this is the first patient reported who progresses to discoid LE and we think it could be a marker of good prognosis.

Hypocomplementemic urticarial vasculitic syndrome, asthma and anaphylactic reaction with ampicillin.

A patient with hypocomplementemic urticarial vasculitic syndrome (HUV) is presented. This is an immunological pathology, limited to skin or multisystemic, that requires a differential diagnosis with erythematosus systemic lupus on the same occasions. The ever-present symptom is skin participation, such as urticaria-angioedema or fixed exanthema; biopsy shows necrotizing venulitis with polymorphonuclear infiltration and leukocytoclastic powder. Typical laboratory data are: diminished C3, C4 and C1q; C1 inhibition can be low or normal; the more characteristic finding is the presence of C1q associated immunocomplexes. Leukocytoclastic necrotizing vasculitis was found in the skin biopsy. During the course of illness (three years) the patient presented moderate cutaneous symptoms and asthma, without other systemic participation. During this period, antihistamines and, occasionally, corticoids were administered with improvement. Moreover, the patient presented urticaria related to ampicillin ingestion, and furthermore, the presence of anaphylaxis to beta-lactam was diagnosed in vivo and specific IgE was found in the laboratory study. This feature was previously observed by other authors; however, we cannot determine why the IgE-mediated allergy to beta-lactam and a complement pathology like HUV are related.

[Progressive symmetric erythrokeratodermia. Report of a case with delayed onset treated by etretinate]. / Eritroqueratodermia simétrica y progresiva. Aportación de un caso de inicio tardío tratado con etretinato.

A case of delayed onset (17 years) progressive symmetric erythrokeratodermia is presented. The treatment with etretinate initially improved the clinical picture, but it had to be discontinued because of personality changes, particularly depressive crisis. In addition, clinical manifestations, histology, kinetics and ultrastructural changes in this condition are also commented on.