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- Presentation
Four Common Immune-Mediated Diseases in Asia: Diagnostic Clues and Treatment
Description
The speaker reviews four immune-mediated diseases that are relatively common in Asia and highlights their key clinical clues, pathology, and treatments. Anti-P200 pemphigoid is a rare autoimmune blistering disease associated with antibodies to laminin gamma-1 or sometimes laminin beta-4; it often affects the extremities, face, and mucosa, shows subepidermal blisters with neutrophils, has negative routine bullous pemphigoid and collagen VII tests, and is confirmed by immunoblotting. Localized disease may respond to potent topical steroids, while extensive disease may need systemic corticosteroids or other combined therapy. Eosinophilic pustular folliculitis is described as a noninfectious eosinophilic follicular disorder common in Asians, presenting with pruritic red or skin-colored papules and pustules on the face and neck that are often mistaken for acne or fungal infection; biopsy is needed, and treatment options include indomethacin, dapsone, tetracyclines, retinoids, and newer JAK inhibitors. Hydroa vacciniforme-like lymphoproliferative disorder is an EBV-associated cutaneous condition in children, marked by papulovesicles, ulceration, necrotic crusts, fever, facial edema, and mucosal involvement; pathology shows epidermal necrosis with angiocentric/angiodestructive lymphocytic infiltrates, CD8-predominant T cells, and EBV confirmation by in situ hybridization, with close long-term monitoring because some cases progress. Kimura disease is presented as a benign lymphoid proliferative disorder in Asian males causing head and neck subcutaneous nodules or lymphadenopathy with eosinophilia and high IgE; biopsy shows lymphoid follicular hyperplasia with eosinophils and vascular proliferation, and treatment may include surgery, steroids, immunosuppressants, radiation, and possibly dupilumab.
View moreConclusions
- Anti-p200 pemphigoid should be suspected in patients with annular or widespread blistering lesions on the extremities or face, especially when mucosal involvement is present and common pemphigoid antibodies are negative.
- For anti-p200 pemphigoid, diagnosis is strengthened by subepidermal blistering with neutrophils, linear C3/IgG at the basement membrane, and immunoblot reactivity to laminin gamma 1 or occasionally laminin beta 4.
- Anti-p200 pemphigoid can respond well to potent topical steroids for localized disease and systemic corticosteroids or combination therapy for more extensive disease, with dapsone often being effective.
- Eosinophilic pustular folliculitis is an important Asian-predominant dermatosis that is often mistaken for acne or fungal infection, so biopsy is needed when pruritic facial papules and pustules do not respond to standard therapy.
- Eosinophilic pustular folliculitis is supported by eosinophilic infiltration around hair follicles and sebaceous glands and may improve with indomethacin, topical calcineurin inhibitors, or other anti-inflammatory treatments.
- Hydroa vacciniforme-like lymphoproliferative disorder is a chronic active EBV-driven disease of children in Asia that presents with vesiculonecrotic or hemorrhagic lesions and depressed scars.
- Because hydroa vacciniforme-like lymphoproliferative disorder can progress to aggressive EBV-positive T-cell lymphoma or leukemia, EBV confirmation and long-term monitoring are essential.
- Kimura disease should be considered in Asian males with head-and-neck subcutaneous nodules, lymphadenopathy, eosinophilia, and elevated IgE levels.
- Kimura disease is characterized pathologically by lymphoid follicular hyperplasia with marked eosinophilic infiltration.
- Kimura disease is often managed with surgery, steroids, immunosuppressants, or radiation, and dupilumab appears to be a promising newer treatment option.
- Goletz et al. — cited on the Anti-p200 pemphigoid slide (reference text at bottom; exact citation not fully legible).#10.1007/s00105-019-4376-x
- Hashimoto et al. — cited on the Anti-p200 pemphigoid slide (reference text at bottom; exact citation not fully legible).#10.26226/m.63185c4d7e215f5e7f3850eb
- Zillikens et al. — cited on the Anti-p200 pemphigoid slide (reference text at bottom; exact citation not fully legible).#10.1007/s00105-019-4376-x
- Schmidt et al. — cited on the Anti-p200 pemphigoid slide (reference text at bottom; exact citation not fully legible).#10.1055/f-0009-0009-b000000424
- Kasperskiewicz et al. — cited on the Anti-p200 pemphigoid slide (reference text at bottom; exact citation not fully legible).#10.26226/m.63185c4d7e215f5e7f3850eb
- Pruritic annular papules and plaques on the face and back — reproduced journal figure cited on a Treatment slide (likely a figure title rather than full reference).#10.1016/j.jaad.2016.05.016