Case Report: Dermatitis Herpetiformis in an Eighteen-Months-Old Child

A case of dermatitis herpetiformis in a child of 18 months is recorded. The relevant literature is briefly reviewed.     

An Unusual Case of Dermatitis Herpetiformis

An adolescent boy whose initial presentation consisted of an asymmetric, nonvesicular rash was eventually diagnosed with dermatitis herpetiformis (DH). Certain factors, including lesions limited to the genitals, an initial biopsy revealing nonspecific findings on microscopy studies, and the absence of characteristic direct immunofluorescence findings lessened initial clinical suspicions of DH over that of scabies infestation. Classic microscopic findings of DH were identified on repeat biopsy. Serologic studies revealed immunoglobulin A (IgA) endomysial and IgA tissue transglutaminase positivity. Response to dapsone proved dramatic. Histopathologic findings, serology, and response to treatment rather than classical clinical findings and direct immunofluorescence supported the diagnosis of DH in this case.     

Pemphigus With Characteristics of Dermatitis Herpetiformis

Five patients had a form of pemphigus which in its early stages resembled dermatitis herpetiform, although the immunofluorescent findings were typical of pemphigus. Potassium iodide tests, performed for the first time in such patients, showed positive results in two patients. Follow-ups ranging from 5 to 14 years have shown a benign course with low to absent dosages of steroids.     

Palmar Purpura: An Atypical Presentation of Childhood Dermatitis Herpetiformis

Abstract: Dermatitis herpetiformis (DH) is seen most commonly as a pruritic, papulovesicular eruption in young children or adolescents. Differentiation from other bullous diseases of childhood may be difficult. We report the first case of an adolescent in whom pruritic, palmar, purpuric macules and papules were the only manifestations of DH. The patient later developed typical vesiculobullous extensor lesions and symptomatic gluten-sensitive enteropathy (GSE). All lesions and GSE symptoms resolved with dapsone and a gluten-free diet. Our purpose is to illustrate an unusual presentation of pediatric DH.     

疱疹样皮炎误诊为药疹1例

患者女,22岁。全身泛发性红斑、水疱伴瘙痒12天。临床表现为全身对称性分布多数红斑,红斑基础上见绿豆至蚕豆大水疱,呈环状排列,尼氏征阴性。皮损组织病理示:表皮下水疱,疱底及周围真皮乳头内以中性粒细胞为主的炎性浸润。诊断:疱疹样皮炎。曾因发病前有明确服药史被误诊为药疹。     

Dermatitis Herpetiformis: Clinical Presentations Are Independent of Manifestations of Celiac Disease

Background

Dermatitis herpetiformis (DH) is a skin manifestation of celiac disease (CD), and is often the presenting and only complaint. There are few data comparing those with DH who present only with DH and those with DH who present mainly due to CD.

Objective

We compared the prevalence of features usually associated with CD in those who presented with DH with patients in whom DH was part of a typical CD presentation.

Methods

A cross-sectional study of a prospectively maintained database of 1,050 patients with CD was analyzed. Only biopsy-diagnosed patients were analyzed for small bowel findings. All patients were included in the analysis of autoimmune diseases and lymphoma incidence. Small bowel biopsies were classified into mild and severe.

Results

The prevalence of villous atrophy was significantly higher in the patients who presented with CD than in those who presented with DH alone (61.8 vs. 12.5 %; p = 0.005). However, the prevalence of nutritional deficiencies, autoimmune diseases, and lymphoma occurred at a similar rate in patients with DH and patients with CD without DH.

Conclusions

Patients who present with CD and concurrent DH are more likely to have more severe pathology than those with predominantly DH, although nutritional deficiencies are similar between the two groups. It is important to screen for nutritional deficiencies in patients with DH, irrespective of the presence of typical CD manifestations.     

Contact Dermatitis in Older Adults

Contact dermatitis is a significant health problem affecting the elderly. Impaired epidermal barrier function and delayed cutaneous recovery after insult enhances susceptibility to both irritants and allergens. Exposure to more numerous potential sensitizers and for greater durations influences the rate of allergic contact dermatitis in this population.Medical co-morbidities, including stasis dermatitis and venous ulcerations, further exacerbate this clinical picture. However, while these factors tend to increase the degree of sensitization in the elderly, waning immunity can actually decrease such a propensity. This interplay of both intrinsic and extrinsic factors makes a generalization on trends for contact dermatitis in older adults challenging. The literature has varying reports on the overall incidence of allergic contact dermatitis with advancing age. Nevertheless, it does clearly show that sensitivity to topical medicaments increases with age. Irritant contact dermatitis studies are more consistent, with less reactivity (to irritants) in older compared with younger skin. Diagnosis of both irritant and allergic contact dermatitis is based on a thorough history, complete skin examination, and comprehensive patch testing. The mainstay of therapy is avoidance of the offending chemical substances and the use of topical along with systemic therapies, depending on the severity of the condition.     

IgA Endomysium Antibody in Children with Dermatitis Herpetiformis Treated with Gluten-free Diet

The IgA antibody to endomysium of smooth muscle (IgA-EmA) was measured in 32 children with confirmed dermatitis herpetiformis who were eating gluten-free diets. One patient had IgA-EmA before treatment but had a negative test one month later while on the diet. Two so treated for less than one year still had antibody, but of seven children treated for more than one year with gluten-free diet, none had detectable antibody. It was present in 13 of 20 children not adhering to the prescribed diet. The antibody was absent in 4 children with linear IgA bullous dermatosis and 43 children with various skin and intestinal diseases. These findings correspond to those in adults with dermatitis herpetiformis and indicate that IgA-EmA is also a marker for gluten-sensitive enteropathy in children.     

A Case of Pemphigus Herpetiformis with Only Immunoglobulin G Anti-Desmocollin 3 Antibodies

Pemphigus represents a group of autoimmune blistering diseases caused by autoantibodies against desmogleins (Dsgs), a class of desmosomal cadherins. Recently, several pemphigus patients only with desmocollin (Dsc) 3-specific antibodies have been reported. Here, we report a case of pemphigus herpetiformis (PH), where only anti-Dsc3-specific antibodies but not anti-Dsg antibodies were detected. A 76-year-old woman presented with a 3-year history of blister formation. Physical examination revealed pruritic erythemas with vesicles on the trunk and legs, but no lesions of the oral mucosa. A skin biopsy specimen revealed intraepidermal blister containing neutrophils, eosinophils, and lymphocytes. Direct immunofluorescence (IF) showed immunoglobulin G (IgG) and complement 3 (C3) depositions on the keratinocyte cell surfaces. Indirect IF showed IgG anti-keratinocyte cell surface antibodies. These findings hinted at a diagnosis of pemphigus. However, repeated enzyme-linked immunosorbent assays (ELISAs) for both anti-Dsg1 and 3 antibodies proved to be negative. Immunoblotting of normal human epidermal extracts revealed Dsc antibodies, and recently established ELISAs using human Dsc1-Dsc3 recombinantly expressed in mammalian cells detected anti-Dsc3 antibodies. Based on these clinical, histopathological, and immunological findings, the patient was diagnosed as PH with only anti-Dsc3 antibodies. Treatment with corticosteroid prednisolone and steroid-sparing agent dapsone accomplished complete clinical remission of the patient.