A structured approach on when to suspect an immunodeficiency and the initial investigations pathway is given in the publication by Grammatikos et al. Immunodeficiency and autoimmunity
There are a large number of immunodeficiency syndromes that present clinical and laboratory characteristics of autoimmunity. The decreased ability of the immune system to clear infections in these patients may be responsible for causing autoimmunity through perpetual immune system activation. One example is common variable immunodeficiency (CVID) where multiple autoimmune diseases are seen, e.g., inflammatory bowel disease, autoimmune thrombocytopenia, and autoimmune thyroid disease. Familial hemophagocytic lymphohistiocytosis, an autosomal recessive primary immunodeficiency, is another example. Low blood levels of red blood cells, white blood cells, and platelets, rashes, lymph node enlargement, and enlargement of the liver and spleen are commonly seen in these patients. Presence of multiple uncleared viral infections due to lack of perforin are thought to be responsible. In addition to chronic and/or recurrent infections many autoimmune diseases including arthritis, autoimmune hemolytic anemia, scleroderma and type 1 diabetes are also seen in X-linked agammaglobulinemia (XLA). Recurrent bacterial and fungal infections and chronic inflammation of the gut and lungs are seen in chronic granulomatous disease (CGD) as well. CGD is caused by a decreased production of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase by neutrophils. Hypomorphic RAG mutations are seen in patients with midline granulomatous disease; an autoimmune disorder that is commonly seen in patients with granulomatosis with polyangiitis and NK/T cell lymphomas. Wiskott–Aldrich syndrome (WAS) patients also present with eczema, autoimmune manifestations, recurrent bacterial infections and lymphoma.
Pigmented purpuric dermatosis refers to one of the three major classes of skin conditions characterized by purpuric skin eruptions. Pigmented purpuric dermatosis are distinguished from other purpura by size (0.3–1 cm) and are most often seen in the lower extremities. : 829   Pigmentary purpuric eruptions may present with one of several clinical patterns. There may be overlapping characteristics among pigmented purpuric dermatosis and between their signs and those of other purpuric eruptions. : 829   Examples of the pigmented purpuric dermatosis group include:: 829–30 
Schambergs disease
Majocchis disease (Purpura annularis telangiectodes)
Gougerot-Blum syndrome (Pigmented purpuric lichenoid dermatitis)
Ducas and Kapetanakis pigmented purpura
Lichen aureusAlthough vascular damage may be present, it is insufficient for these conditions to be considered forms of vasculitis.A few very small non-blinded studies of treatment with narrow-band ultraviolet light have been reported as promising. See also
Purpura
Skin lesion
List of cutaneous conditions

References


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