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January 1994

Cutaneous Manifestations and Histologic Findings in the Hyperimmunoglobulinemia D Syndrome

Author Affiliations

and the International Hyper IgD Study Group

From the Department of Medicine, Division of General Internal Medicine, University Hospital St Radboud, Nijmegen (Drs Drenth and Van der Meer); Department of Dermatology, University Hospital Leiden (Dr Boom); and Department of Dermatology, University Hospital Utrecht (Dr Toonstra), the Netherlands.

Arch Dermatol. 1994;130(1):59-65. doi:10.1001/archderm.1994.01690010063008

Background and Design:  The hyperimmunoglobulinemia D (hyper-IgD) syndrome is characterized by recurrent febrile attacks with abdominal distress, headache, and arthralgias. Physical examination reveals cervical lymphadenopathy in most cases and, sometimes, splenomegaly. Skin lesions have been observed in isolated cases during attacks. We summarize the features of skin lesions and the histopathologic findings in biopsy specimens in the hyper-IgD syndrome.

Results:  A total of 44 patients with the hyper-IgD syndrome were studied. Thirty-five (79%) of them, 19 males and 16 females, had skin lesions during febrile attacks. Erythematous macules were the most common cutaneous manifestation (15 cases), followed by erythematous papules (12 cases), urticarial lesions (nine cases) and erythematous nodules (seven cases). Skin biopsy specimens of 10 patients with the hyper-IgD syndrome were available for review. The findings varied considerably. Most biopsy specimens showed mild features of vasculitis. Nonspecific findings were noted in five biopsy specimens; Sweet-like features in two, cellulitislike findings in one, and deep vasculitis characteristics in one.

Conclusions:  Skin lesions are common in the hyper-IgD syndrome suggesting that they are a true manifestation of the disease.(Arch Dermatol. 1994;130:59-65)