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Three Patterns of Cutaneous Involvement in Granulomatosis with Polyangiitis.
J Adv Res. 2020 Jul; 24:311-315.JA

Abstract

Background

Skin involvement in granulomatosis with polyangiitis (GPA) is common and can appear as an initial presentation of the disease or more commonly through its course.

Case presentation

We report a case of a 24-year-old male patient, previously diagnosed as having GPA, admitted with fever, hemoptysis, generalized hemorrhagic blisters associated with arthralgia, fatigue, myalgia, nasal crusting, and vertigo. Three weeks prior to admission, he developed erythematous papules on both elbows, and purpuric papules on both lower limbs. Histopathological examination revealed: interstitial granulomatous dermatitis (elbows) and foci of dermal hemorrhage, foci of interstitial histiocytes and zones of altered necrobiotic collagen (lower limbs) consistent with cutaneous lesions of GPA. Two weeks later, his rash progressed to widespread purpura associated with hemorrhagic blisters. Another biopsy revealed leukocytoclastic vasculitis with fibrinoid necrosis of the vessel walls associated with perivascular infiltrate of neutrophils, nuclear dust and extravasated erythrocytes without an associated granulomatous inflammation or necrobiosis. The constellation of the results of the three biopsies together with clinical correlation pointed to a flare of GPA.

Conclusion

Skin involvement in GPA is quite common, and it can manifest in different forms in the same patient. Our patient developed three different skin pathologies within a short period of time.

Authors+Show Affiliations

Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Cairo, Egypt.Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Cairo, Egypt.Nephrology Unit, French Hospital, Cairo University, Cairo, Egypt.Dermatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, AP-HP, Paris Descartes University, Paris, France.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32455005

Citation

Ragab, Gaafar, et al. "Three Patterns of Cutaneous Involvement in Granulomatosis With Polyangiitis." Journal of Advanced Research, vol. 24, 2020, pp. 311-315.
Ragab G, Hegazy MT, Ali M, et al. Three Patterns of Cutaneous Involvement in Granulomatosis with Polyangiitis. J Adv Res. 2020;24:311-315.
Ragab, G., Hegazy, M. T., Ali, M., Abdel-Halim, M. R. E., & Puéchal, X. (2020). Three Patterns of Cutaneous Involvement in Granulomatosis with Polyangiitis. Journal of Advanced Research, 24, 311-315. https://doi.org/10.1016/j.jare.2020.05.009
Ragab G, et al. Three Patterns of Cutaneous Involvement in Granulomatosis With Polyangiitis. J Adv Res. 2020;24:311-315. PubMed PMID: 32455005.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Three Patterns of Cutaneous Involvement in Granulomatosis with Polyangiitis. AU - Ragab,Gaafar, AU - Hegazy,Mohamed Tharwat, AU - Ali,Mohamed, AU - Abdel-Halim,Mona R E, AU - Puéchal,Xavier, Y1 - 2020/05/07/ PY - 2020/02/12/received PY - 2020/05/05/revised PY - 2020/05/05/accepted PY - 2020/5/27/entrez PY - 2020/5/27/pubmed PY - 2020/5/27/medline KW - Granulomatosis with polyangiitis (GPA) KW - Interstitial granulomatous dermatitis KW - Leukocytoclastic vasculitis KW - Necrobiosis KW - Pathology KW - Skin manifestations SP - 311 EP - 315 JF - Journal of advanced research JO - J Adv Res VL - 24 N2 - Background: Skin involvement in granulomatosis with polyangiitis (GPA) is common and can appear as an initial presentation of the disease or more commonly through its course. Case presentation: We report a case of a 24-year-old male patient, previously diagnosed as having GPA, admitted with fever, hemoptysis, generalized hemorrhagic blisters associated with arthralgia, fatigue, myalgia, nasal crusting, and vertigo. Three weeks prior to admission, he developed erythematous papules on both elbows, and purpuric papules on both lower limbs. Histopathological examination revealed: interstitial granulomatous dermatitis (elbows) and foci of dermal hemorrhage, foci of interstitial histiocytes and zones of altered necrobiotic collagen (lower limbs) consistent with cutaneous lesions of GPA. Two weeks later, his rash progressed to widespread purpura associated with hemorrhagic blisters. Another biopsy revealed leukocytoclastic vasculitis with fibrinoid necrosis of the vessel walls associated with perivascular infiltrate of neutrophils, nuclear dust and extravasated erythrocytes without an associated granulomatous inflammation or necrobiosis. The constellation of the results of the three biopsies together with clinical correlation pointed to a flare of GPA. Conclusion: Skin involvement in GPA is quite common, and it can manifest in different forms in the same patient. Our patient developed three different skin pathologies within a short period of time. SN - 2090-1232 UR - https://www.unboundmedicine.com/medline/citation/32455005/Three_Patterns_of_Cutaneous_Involvement_in_Granulomatosis_with_Polyangiitis L2 - https://linkinghub.elsevier.com/retrieve/pii/S2090-1232(20)30092-8 DB - PRIME DP - Unbound Medicine ER -
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