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. 2018 Nov 1;10(1):e2018065.
doi: 10.4084/MJHID.2018.065. eCollection 2018.

Cutaneous Manifestations of Primary Immunodeficiency Diseases in Tunisian Children

Naouel Guirat Dhouib  1 , Monia Ben Khaled  1 , Monia Ouederni  1 , Imen Ben-Mustapha  2 , Ridha Kouki  1 , Habib Besbes  1 , Mohamed Ridha Barbouche  2 , Fethi Mellouli  1 , Mohamed Bejaoui  1
Affiliations

Cutaneous Manifestations of Primary Immunodeficiency Diseases in Tunisian Children

Naouel Guirat Dhouib et al. Mediterr J Hematol Infect Dis. .

Abstract

Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of PIDs among Tunisian children. We conducted a prospective study on two hundred and ninety children with immune deficiency. Demographic details (including age, sex, and consanguinity) with personal and family history were recorded. Special attention was paid to cutaneous manifestations. Dermatological involvements were grouped according to the etiology of their most prominent sign. Cutaneous manifestations were found in 164 patients (56.5%). They revealed the diagnosis of PIDs in 71 patients (24.5 %). The mean age at presentation was 21 months. Overall the most prominent cutaneous alterations were infectious. They accounted for 106 cases (36.55%). The most prevalent causes of cutaneous infections were bacterial: 93 cases (32.06%). Immuno-allergic skin diseases were among the common findings in our study. These include eczematous dermatitis found in 62 cases (21.38%). Malignancy related PIDs was seen in a boy with Wiskott Aldrich syndrome. He developed Kaposi's sarcoma at the age of 14 months. Cutaneous changes are common among children with PIDs. In pediatric patients with failure to thrive, chronic refractory systemic manifestations often present in other family members, recurrent cutaneous infections unresponsive to adequate therapy, atypical forms of eczematous dermatitis or unusual features should arouse the suspicion of PIDs and prompt specialized immunologic consultation should be made.

Keywords: Cutaneous infections; Cutaneous manifestations; Primary immunodeficiency; Wiskott Aldrich syndrome.

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Conflict of interest statement

Competing interests: The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1
Disseminated warts on oral cavity in a patient with MHC class II deficiency.
Figure 2
Figure 2
Oral candidiasis in a patient with SCID.
Figures 3 and 4
Figures 3 and 4
Erythroderma with alopecia in a patient with Omenn syndrome.
Figure 5
Figure 5
Conjunctival telangiectasias in a patient with ataxia telangiectasia syndrome.
Figure 6
Figure 6
Kaposi’s sarcoma in a patient with Wiskott-Aldrich syndrome.
See this image and copyright information in PMC

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