Intravenous and subcutaneous immunoglobulins-associated eczematous reactions occur with a broad range of immunoglobulin types: A French national multicenter study.

BACKGROUND: Human immunoglobulins are used for treating diverse inflammatory and autoimmune disorders. Eczema is an adverse event reported but poorly described. OBJECTIVES: To describe the clinical presentation, severity, outcome, and therapeutic management of immunoglobulin-associated eczema. METHODS: This retrospective and descriptive study included a query of the French national pharmacovigilance database, together with a national call for cases among dermatologists. RESULTS: We included 322 patients. Eczema occurred preferentially in men (78.9%) and in patients treated for neurological pathologies (76%). The clinical presentation consisted mainly of dyshidrosis (32.7%) and dry palmoplantar eczema (32.6%); 5% of cases exhibited erythroderma. Sixty-two percent of the eczema flares occurred after the first immunoglobulin course. Eczema was observed with 13 intravenous or subcutaneous immunoglobulin types and recurred in 84% of patients who maintained the same treatment and in 68% who switched the immunoglobulin type. After immunoglobulin discontinuation, 30% of patients still had persistent eczema. LIMITATIONS: Retrospective study, with possible missing data or memory bias. CONCLUSION: Immunoglobulin-associated eczema occurred with all immunoglobulin types, preferentially in patients with neurologic diseases who required prolonged immunoglobulin treatment. Recurrence was frequent, even after switching the immunoglobulin type, which can lead to a challenging therapeutic situation when immunoglobulin maintenance is required.

Dyshidrosiform Bullous Pemphigoid.

Dyshidrosiform bullous pemphigoid is a variant of bullous pemphigoid. At least 84 patients with dyshidrosiform bullous pemphigoid have been described. Dyshidrosiform bullous pemphigoid usually presents with pruritic blisters in elderly individuals; the hemorrhagic or purpuric lesions on the palms and soles can be the only manifestation of the disease. However, bullae may concurrently or subsequently appear on other areas of the patient's body. Patients typically improve after the diagnosis is established and treatment is initiated. The mainstay of therapy is systemic corticosteroids, with or without topical corticosteroids, and systemic dapsone or immunosuppressants. Drug-related or nickel-induced dyshidrosiform bullous pemphigoid improves after stopping the associated agent; however, systemic therapy has also been required to achieve resolution of the blisters. Similar to classic bullous pemphigoid, neurologic conditions and psychiatric disorders have been observed in dyshidrosiform bullous pemphigoid patients. The new onset of recurrent or persistent blisters on the palms, soles, or both of an elderly individual should prompt the clinician to consider the diagnosis of dyshidrosiform bullous pemphigoid.

Dyshidrosiform Bullous Pemphigoid: Case Reports and Review.

Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla, and groin. It is caused by circulating and tissue-bound autoantibodies directed against bullous pemphigoid antigen 1 or bullous pemphigoid antigen 2 or both. Dyshidrosiform bullous pemphigoid is a rare variant of bullous pemphigoid, and it usually presents as itchy, potentially hemorrhagic, or purpuric blisters on the palms and/or soles of elderly individuals; subsequently, typical bullous lesions of bullous pemphigoid appear on other body sites. In our study, we report the features of two men with dyshidrosiform bullous pemphigoid and review the characteristics of individuals with this rare subtype of bullous pemphigoid. Including the men whose condition is described in this paper, at least 72 patients with dyshidrosiform bullous pemphigoid have been reported so far. However, complete features of the condition have not been described for all of the individuals. Based on the cases reported so far, the condition was slightly more common in women and the onset of the disease, for most of the patients, occurred between the ages of 61 and 94 years. The patients usually presented with blisters on both their palms and soles (66%) or just their soles (31%); 77% of the patients had progression of bullous pemphigoid to other areas of their body. Whether hemorrhagic blisters or purpuric lesions are associated with dyshidrosiform bullous pemphigoid remains to be determined; these features were present in 91% of the 22 patients who were described in the case reports yet were only observed in 5% of the individuals from a single larger series of 20 patients. The mainstay of therapy for dyshidrosiform bullous pemphigoid is systemic corticosteroids, with or without topical corticosteroids, and/or systemic dapsone or immunosuppressants; nearly all of the patients showed improvement after the treatment was initiated. Similar to individuals with bullous pemphigoid, at least nine of the dyshidrosiform bullous pemphigoid patients, including both patients in this report, had either a neurologic condition (seven patients) or both a neurologic condition and a psychiatric disorder (two patients). Usually, an autoimmune bullous disease, particularly dyshidrosiform bullous pemphigoid, is not initially considered in patients who present with blisters restricted to the palms and/or soles. Indeed, the lesion morphology of dyshidrosiform bullous pemphigoid mimics several other conditions that are characterized by blisters on the hands and feet, such as allergic and irritant contact dermatitis, chronic bullous disease of childhood, cutaneous T-cell lymphoma, dermatophyte infection, dyshidrosis or pompholyx, epidermolysis bullosa acquisita, erythema multiforme, herpes gestationis, lichen planus, linear IgA disease, scabies, and systemic contact dermatitis. In conclusion, the possibility of dyshidrosiform bullous pemphigoid should be considered in elderly individuals who present with the new onset of palmar and/or plantar blisters that are either recurrent or recalcitrant to therapy or would subsequently also appear on other areas of the body.

Dyshidrosis is associated with reduced amplitudes in electrically evoked pain-related potentials in women with Fabry disease.

OBJECTIVE: To investigate A-delta fiber conduction in mild to moderate Fabry disease (FD) patients using pain-related evoked potentials (PREP). METHODS: In this case-control study we prospectively investigated 58 patients with mild to moderate FD and compared data with those of healthy controls. Small fiber function (quantitative sensory testing, QST and sympathetic skin response, SSR), morphology (intraepidermal nerve fiber density, IENFD), and electrical conduction (PREP) were assessed and correlated with sweating as major autonomic function disturbed in FD. Patients were further stratified for gender, disease severity as reflected by renal and cardiac function, and genetics. RESULTS: An- or hypohidrosis (i.e. dyshidrosis) was reported by 7/32 (22%) women and 15/26 (58%) men with FD (p < 0.01). QST showed small fiber impairment in female and male patients regardless of clinical symptoms, while SSR was obtained in all patients except one man with hypohidrosis. IENFD was reduced in 50% of FD patients, with no differences between groups with and without autonomic symptoms. However, PREP amplitudes were reduced independent of the stimulation site only in female patients with dyshidrosis (p < 0.01). Genetics had no influence on PREP parameters. CONCLUSION: A-delta fiber conduction investigated using PREP is impaired in mild to moderately affected female FD patients with clinical signs of hypohidrosis. SIGNIFICANCE: Small fiber assessment in FD is of diagnostic value already in mild to moderate stages of disease.

Clinical profile of recurrent vesicular palmoplantar dermatitis in children and adolescents.

BACKGROUND: Previously known as dyshidrosis, recurrent vesicular palmoplantar dermatitis (RVPD) is presented as severe eruption of nonerythematous, symmetrical vesicles or bullae located along the lateral sides of fingers, on the palmar or plantar areas, and developing into a chronic and recurrent condition. Although very frequently observed on the hands, there are no specific studies about such eczema in children and adolescents. OBJECTIVES: To report on the RVPD clinical profile in children and adolescents, and monitor the association of RVPD with seasonal variations, hyperhidrosis, atopy and nickel sulfate. MATERIALS AND METHODS: Eighteen patients affected by RVPD were submitted to clinical and laboratory assessment through anamnesis, physical exam, mycological exam, patch test, complete blood count and serum IgE levels. RESULTS: Seven patients (38.9%) reported increased frequency of eruptions during the summer months; 12 patients (66.7%) presented hyperhidrosis; in 14 cases (77.8%), atopic factors were determined; reaction to nickel sulfate was positive in three patients (16.7%). CONCLUSION: RVPD onset may occur at an early age, developing into a chronic and recurrent infection. Hyperhidrosis and atopic status was established in the clinical profile and no connection between nickel sulfate and RVPD was observed.

Larva migrans cutánea en un paciente viajero portador de dishidrosis: a propósito de un caso clínico / Cutaneous larva migrans in a traveling patient with dyshidrosis: a case report

Larva migrans cutánea es el resultado de una infección por nematodos y muestra una erupción serpiginosa característica. Con el aumento de los viajes a zonas tropicales, muchos pacientes pueden volver con ésta infección, que es a menudo, mal diagnosticada o tratada incorrectamente. Esta enfermedad cutánea parasitaria es causada por la migración de larvas provenientes de animales en la epidermis humana.

Cutaneous larva migrans is a result of a nematode infection and shows a characteristic creeping eruption. As travel to the tropics zone increases, many patients may be returning with this infection, which is often misdiagnosed or treated incorrectly. This parasitic skin disease is caused by the migration of animal hookworm larvae in the human epidermis.