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Bullous diseases of childhood
Symposium S2. Pediatric dermatology
I52-4
Neonatal
erythroderma
Christine Bodemer, Yves de Prost. Paris, Prance
Neonatal erythroderma is rare, and aetiological diagnosis is frequently difficult to make, and usually delayed, since clinical and histological signs are not very specific. The most frequent aetiologies can be divided into four main groups: erythematosqamous conditions (seborrhoeic dermatitis, atopic dermatitis, psoriasis), congenital ichthyoses (CIE, LI, neuro-ichthyoses), immunodeficiency and metabolic disorders. The most important clinical characteristics for the etiological diagnosis are: a familial history, a siblings mortality (immunodeficiency), consanguinity (ichtyoses and immunodeficiency), a congenital onset of erythroderma (ichtyoses), an infiltration of the skin with the ossociarior~ of alopecia of the scalp and alopecia of the eyebrows and eyelashes (immunodeticiency). the presence of extensive and thick squames (ichtyoses), hair shaft abnormalities, no reponse to topical corticosteroid therapy (immunodeficiency), severe infections, failure to thrive (immunodeficiency), and neurological disorders (metabolic diseases). Histology confirms the final diagnosis in only 45% of cases. However, the discovery of a significant dermal and epidermal lymphocytic infiltration, or keratinocytic necrosis, favours the diagnosis of immunodeficiency; and a marked orthokeratosis, the diagnosis of ichtyoses. The main possible laboratory abnormalities are: salt and water depletion with dehydration and hypematraemia, hypoproteinaemia with major hypoalbuminaemia. There is no correlation between the levels of IgE. or the eosinophil count, and the clinical severity or aetiology of the erythroderma. Complications are frequent due to the extent of the skin disease or the underlying pathology. The main complications are infection and growth retardation, sometimes associated with chronic diarrhoea without any malabsorption. The prognosis for early childhood erythroderma is extremely poor in our experience. In a seria of 51 patients, there was a mortality rate of 15.7%. and severe dermatosis persisted in 67% of survivors. I S2-5 Bullous diseases of childhood Fenella Wojnarowska. Oxford Radcliffe Hospital, Oxford, UK Bullous diseases in childhood are traumatic both to the child and to the entire family, Urgent diagnosis is essential. The major differential diagnoses are infections, genetic diseases, physical agents (light, heat), and the autoimmune blistering disease. This presentation will concentrate on the autoimmune blistering diseases. The diagnosis of these diseases can be difficult and the best methods of diagnosis including the newly described use of blister fluid will be discussed. Immunobullous diseases presenting in childhood are rare. Outside Europe pemphigus is one of the commonest seen but is extremely rare within Europe. Paraneoplastic pemphigus is even less common and when it does occur may be associated With Castleman’s tumour. Chronic bullous disease of childhood is the commonest sub-epidermal disease presenting in chiidhood. Mucosal involvement can be prominent and troublesome and in some cases may make differentiation between linear IgA disease and cicatricial pemphigoid impossible on clinical grounds. Cicatricial pemphigoid can occur but is very rare. Rullous pemphigoid is the second most frequent blistering
Sl
disorder presenting in children in Europe but epidermolysis bullosa acquisita also occurs. Young girls may present with vulva1 blistering and this may be a manifestation of localised vulva1 pemphigoid or the presentation of cicatricial pemphigoid with its much poorer prognosis. The clinical manifestations of these diseases will be described and related to the molecular basis of these diseases. I S2-6 Alopecia areata in children R. Hoffmann. Philipp University, Department of Dermatology, DeutschhausstraJe 9, Marburg, Germany The most common form of reversible hair loss in children and young adults is alopecia areata (AA). The differential diagnosis includes diseases such as trichotillomania, tinea capitis or the loose anagen syndrome. Careful examination of the scalp and body hair and the search for nails changes will usually be rewarded by the correct diagnosis. AA is sometimes associated with other autoirmnune diseases (e.g. vitiligo) or atopic dermatitis. The presence of nail changes and atopic eczema in a child with severe AA is of importance, because these children tend to have a chronic disease often not responding to therapy. AA is mediated by T cells targetting an unknown follicular antigen. Some phenomena in AA may be mediated by the release of cytokines in the vicinity of the hair follicle. However, the precise etiopathogenesis of AA is unclear and any kind of treatment is symptomatic still. The most effective approach for AA in children over the age of 10 is topical immunotherapy with contact sensitizers such as diphencyprone. The harmful psychological effects of AA in children should be considered. I S2-7
Gianotti-Crosti considerations
syndrome:
Diagnostic
C. Gelmetti, S. Cambiaghi, A. Coggi. Istituto di Scienze Dermatologiche, I.R.C.C.S. “Ospedale Maggiore”, Milano. Italy Gianotti-Crosti syndrome (GCS), formerly known as Papular acrodermatitis of childhood was described in 1955 as a symmetric contemporaneous eruption of erythematopapular lesions on the face and limbs. Asymmetrical Periflexural Exanthem of Childhood (APEC) was described in 1957 as a scarlatiniformeczematous eruption affecting one side of the body, starting from the axillary fold and centrifugally extending onto the thorax. However, in 113 of cases, APEC does not start around the proximal fold of the upper or lower limb but the eruption starts around a popliteal or antecubitai fold. Also in these cases of APEC, similar albeit more inconspicuous lesions appear on the contralateral side, mimicking CCS. Another differential diagnosis with GCS should be made with Hand-Foot-Mouth diseases when the lesions involve the entire limbs (but the pahnar-plantar vesicles are absent in GCS) and with anaphylactoid purpura (but the joint swelling and visceral symptoms are absent in GCS). Frictional lichenoid dermatitis when fully developed can be confused with GCS even though the lesions always spare the face. Finally erythema multiforme may recall GCS when the lesions are minute and no history of drug assumption can be recorded.
I52-4
Neonatal
erythroderma
Christine Bodemer, Yves de Prost. Paris, Prance
Neonatal erythroderma is rare, and aetiological diagnosis is frequently difficult to make, and usually delayed, since clinical and histological signs are not very specific. The most frequent aetiologies can be divided into four main groups: erythematosqamous conditions (seborrhoeic dermatitis, atopic dermatitis, psoriasis), congenital ichthyoses (CIE, LI, neuro-ichthyoses), immunodeficiency and metabolic disorders. The most important clinical characteristics for the etiological diagnosis are: a familial history, a siblings mortality (immunodeficiency), consanguinity (ichtyoses and immunodeficiency), a congenital onset of erythroderma (ichtyoses), an infiltration of the skin with the ossociarior~ of alopecia of the scalp and alopecia of the eyebrows and eyelashes (immunodeticiency). the presence of extensive and thick squames (ichtyoses), hair shaft abnormalities, no reponse to topical corticosteroid therapy (immunodeficiency), severe infections, failure to thrive (immunodeficiency), and neurological disorders (metabolic diseases). Histology confirms the final diagnosis in only 45% of cases. However, the discovery of a significant dermal and epidermal lymphocytic infiltration, or keratinocytic necrosis, favours the diagnosis of immunodeficiency; and a marked orthokeratosis, the diagnosis of ichtyoses. The main possible laboratory abnormalities are: salt and water depletion with dehydration and hypematraemia, hypoproteinaemia with major hypoalbuminaemia. There is no correlation between the levels of IgE. or the eosinophil count, and the clinical severity or aetiology of the erythroderma. Complications are frequent due to the extent of the skin disease or the underlying pathology. The main complications are infection and growth retardation, sometimes associated with chronic diarrhoea without any malabsorption. The prognosis for early childhood erythroderma is extremely poor in our experience. In a seria of 51 patients, there was a mortality rate of 15.7%. and severe dermatosis persisted in 67% of survivors. I S2-5 Bullous diseases of childhood Fenella Wojnarowska. Oxford Radcliffe Hospital, Oxford, UK Bullous diseases in childhood are traumatic both to the child and to the entire family, Urgent diagnosis is essential. The major differential diagnoses are infections, genetic diseases, physical agents (light, heat), and the autoimmune blistering disease. This presentation will concentrate on the autoimmune blistering diseases. The diagnosis of these diseases can be difficult and the best methods of diagnosis including the newly described use of blister fluid will be discussed. Immunobullous diseases presenting in childhood are rare. Outside Europe pemphigus is one of the commonest seen but is extremely rare within Europe. Paraneoplastic pemphigus is even less common and when it does occur may be associated With Castleman’s tumour. Chronic bullous disease of childhood is the commonest sub-epidermal disease presenting in chiidhood. Mucosal involvement can be prominent and troublesome and in some cases may make differentiation between linear IgA disease and cicatricial pemphigoid impossible on clinical grounds. Cicatricial pemphigoid can occur but is very rare. Rullous pemphigoid is the second most frequent blistering
Sl
disorder presenting in children in Europe but epidermolysis bullosa acquisita also occurs. Young girls may present with vulva1 blistering and this may be a manifestation of localised vulva1 pemphigoid or the presentation of cicatricial pemphigoid with its much poorer prognosis. The clinical manifestations of these diseases will be described and related to the molecular basis of these diseases. I S2-6 Alopecia areata in children R. Hoffmann. Philipp University, Department of Dermatology, DeutschhausstraJe 9, Marburg, Germany The most common form of reversible hair loss in children and young adults is alopecia areata (AA). The differential diagnosis includes diseases such as trichotillomania, tinea capitis or the loose anagen syndrome. Careful examination of the scalp and body hair and the search for nails changes will usually be rewarded by the correct diagnosis. AA is sometimes associated with other autoirmnune diseases (e.g. vitiligo) or atopic dermatitis. The presence of nail changes and atopic eczema in a child with severe AA is of importance, because these children tend to have a chronic disease often not responding to therapy. AA is mediated by T cells targetting an unknown follicular antigen. Some phenomena in AA may be mediated by the release of cytokines in the vicinity of the hair follicle. However, the precise etiopathogenesis of AA is unclear and any kind of treatment is symptomatic still. The most effective approach for AA in children over the age of 10 is topical immunotherapy with contact sensitizers such as diphencyprone. The harmful psychological effects of AA in children should be considered. I S2-7
Gianotti-Crosti considerations
syndrome:
Diagnostic
C. Gelmetti, S. Cambiaghi, A. Coggi. Istituto di Scienze Dermatologiche, I.R.C.C.S. “Ospedale Maggiore”, Milano. Italy Gianotti-Crosti syndrome (GCS), formerly known as Papular acrodermatitis of childhood was described in 1955 as a symmetric contemporaneous eruption of erythematopapular lesions on the face and limbs. Asymmetrical Periflexural Exanthem of Childhood (APEC) was described in 1957 as a scarlatiniformeczematous eruption affecting one side of the body, starting from the axillary fold and centrifugally extending onto the thorax. However, in 113 of cases, APEC does not start around the proximal fold of the upper or lower limb but the eruption starts around a popliteal or antecubitai fold. Also in these cases of APEC, similar albeit more inconspicuous lesions appear on the contralateral side, mimicking CCS. Another differential diagnosis with GCS should be made with Hand-Foot-Mouth diseases when the lesions involve the entire limbs (but the pahnar-plantar vesicles are absent in GCS) and with anaphylactoid purpura (but the joint swelling and visceral symptoms are absent in GCS). Frictional lichenoid dermatitis when fully developed can be confused with GCS even though the lesions always spare the face. Finally erythema multiforme may recall GCS when the lesions are minute and no history of drug assumption can be recorded.