Dermatitis herpetiformis in a child with Wiskott‐Aldrich syndrome: A rare occurrence

This article presents a case of clinical observation of a 5-year-old child with herpetiformis dermatitis (Duhrings). This rare dermatosis is characterized by a chronic relapsing course, the presence of itching polymorphic rashes, typical histological and immunomorphological signs. The diagnosis was made on the basis of the clinical picture, histological and immunohistochemical studies of skin biopsy, as well as the results of HLA typing by PCR. Clinical observation of this case is of interest to practicing physicians-dermatologists due to the rare occurrence of Duhrings herpetiformis in children, the complexity of differential diagnostic search, which requires further generalization of experience using histological, immunohistochemical and molecular genetic research methods. The disease is clearly differentiated from other rashes with the formation of subepidermal blisters according to histological, immunological and gastrointestinal criteria. The prevalence of dermatosis in various populations of the Europian race ranges from 10 to 39 cases per 100,000 population. Duhrings dermatitis herpetiformis can develop at any age (cases of the childhood form of Dhrings dermatitis herpetiformis have been reported), but most often the disease occurs at the age of 4050 years. Dermatitis herpetiformis persists indefinitely with variable severity. In patients with Duhrings dermatitis, associated gluten-sensitive enteropathy is often noted, which in most cases is asymptomatic.

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Disymptomatic Wiskott Aldrich Syndrome: Overcoming a Diagnostic Challenge.

10.46940/scmcrj.02.1006 ◽

2020 ◽

pp. 1-6

Keyword(s):

Liver Disease ◽

Skin Disorder ◽

Severe Form ◽

Rare Occurrence ◽

Rare Disorders ◽

Diagnostic Challenge ◽

Wiskott Aldrich Syndrome ◽

Allergic Skin ◽

Medical Reports ◽

Apparently Healthy

Abstract Background: There are three main forms of the Wiskott Aldrich syndrome, the classic severe form, the disymptomatic form without cutaneous signs (Harzheim and colleagues, 1965) and a milder variant X-linked thrombocytopenia and neutropenia. The aim of this paper is to describe the very rare occurrence of disymptomatic form of Wiskott Aldrich syndrome in two Iraqi brothers and the diagnostic challenge associated with such cases. Patients and methods: The case of two Yezidis brothers referred because each boy had two different medical reports from Hevi Teaching Children Hospital in Dohuk, Kurdistan Iraq. Two of these medical reports recommended sending them outside Kurdistan or outside Iraq for the diagnosis and management of their illnesses. Each of these medical reports was signed by a committee that included four consultant doctors. Results: The two brothers had chronic illness of more than two years duration characterized by thrombocytopenia, leucopenia, splenomegaly, draining ear (more prominent in the older brother) and chronic liver disease (more prominent in the younger bladder). The parents were consanguineous. The father was apparently healthy, but the mother was having allergic skin disorder. The boys had an older brother who died at the age of thirteen. The two patients have four healthy sisters aged 18, 15, 10 and 8 years respectively. Conclusion: This paper demonstrates that when it comes to the diagnostic challenges associated with rare disorders, obscurity, uncertainty and complexities can be transformed to a crystal-clear diagnosis in the hands of the expert.

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Disymptomatic Wiskott Aldrich Syndrome: Overcoming a Diagnostic Challenge.

10.46940/scmcrj.01.1006 ◽

2020 ◽

pp. 1-6

Keyword(s):

Liver Disease ◽

Skin Disorder ◽

Severe Form ◽

Rare Occurrence ◽

Rare Disorders ◽

Diagnostic Challenge ◽

Wiskott Aldrich Syndrome ◽

Allergic Skin ◽

Medical Reports ◽

Apparently Healthy

Abstract Background: There are three main forms of the Wiskott Aldrich syndrome, the classic severe form, the disymptomatic form without cutaneous signs (Harzheim and colleagues, 1965) and a milder variant X-linked thrombocytopenia and neutropenia. The aim of this paper is to describe the very rare occurrence of disymptomatic form of Wiskott Aldrich syndrome in two Iraqi brothers and the diagnostic challenge associated with such cases. Patients and methods: The case of two Yezidis brothers referred because each boy had two different medical reports from Hevi Teaching Children Hospital in Dohuk, Kurdistan Iraq. Two of these medical reports recommended sending them outside Kurdistan or outside Iraq for the diagnosis and management of their illnesses. Each of these medical reports was signed by a committee that included four consultant doctors. Results: The two brothers had chronic illness of more than two years duration characterized by thrombocytopenia, leucopenia, splenomegaly, draining ear (more prominent in the older brother) and chronic liver disease (more prominent in the younger bladder). The parents were consanguineous. The father was apparently healthy, but the mother was having allergic skin disorder. The boys had an older brother who died at the age of thirteen. The two patients have four healthy sisters aged 18, 15, 10 and 8 years respectively. Conclusion: This paper demonstrates that when it comes to the diagnostic challenges associated with rare disorders, obscurity, uncertainty and complexities can be transformed to a crystal-clear diagnosis in the hands of the expert.

Download Full-text