Biomaterial Database

Good's syndrome (hypogammaglobulinemia with thymoma) presenting intractable opportunistic infections and hyperkeratotic lichen planus. 2010

Takaaki Hanafusa, and Noriko Umegaki, and Yuji Yamaguchi, and Ichiro Katayama

Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.

Good's syndrome (GS) is a rare acquired combined T- and B-cell immunodeficiency accompanying thymoma. This report concerns a case of a 57-year-old man with GS manifesting intractable opportunistic infections and hyperkeratotic lichen planus. He had a past history of extended thymectomy for removal of thymoma. He consulted us about scaly and exudative intractable erythematous plaque on his right forearm. The histology was compatible with phlegmon coexisting with lichen planus. Laboratory examination results indicated hypogammaglobulinemia accompanied by complete absence of B cells, which is consistent with GS. Combined treatment with immunoglobulin replacement and administration of antibiotics and antifungal drugs was effective for the phlegmon and overlying fungal infection. The patient also presented with hyperkeratotic lichen planus on both knees and the right elbow, suggesting that intractable opportunistic infection and lichen planus may be associated with GS.

UI	MeSH Term	Description	Entries
D007136	Immunoglobulins	Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.	Globulins, Immune,Immune Globulin,Immune Globulins,Immunoglobulin,Globulin, Immune
D007717	Knee	A region of the lower extremity immediately surrounding and including the KNEE JOINT.
D008010	Lichen Planus	An inflammatory, pruritic disease of the skin and mucous membranes, which can be either generalized or localized. It is characterized by distinctive purplish, flat-topped papules having a predilection for the trunk and flexor surfaces. The lesions may be discrete or coalesce to form plaques. Histologically, there is a "saw-tooth" pattern of epidermal hyperplasia and vacuolar alteration of the basal layer of the epidermis along with an intense upper dermal inflammatory infiltrate composed predominantly of T-cells. Etiology is unknown.	Cutaneous Lichen Planus,Lichen Planopilaris,Lichen Ruber Planus,Mucosal Lichen Planus,Lichen Rubra Planus,Lichen Planus, Cutaneous,Lichen Planus, Mucosal,Planopilaris, Lichen
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D009894	Opportunistic Infections	An infection caused by an organism which becomes pathogenic under certain conditions, e.g., during immunosuppression.	Infection, Opportunistic,Infections, Opportunistic,Opportunistic Infection
D002481	Cellulitis	An acute, diffuse, and suppurative inflammation of loose connective tissue, particularly the deep subcutaneous tissues, and sometimes muscle, which is most commonly seen as a result of infection of a wound, ulcer, or other skin lesions.	Phlegmon
D004550	Elbow	Region of the body immediately surrounding and including the ELBOW JOINT.
D005542	Forearm	Part of the upper extremity in humans and primates extending from the ELBOW to the WRIST.	Antebrachium,Antebrachiums,Forearms
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man