THE OCCURRENCE OF DISCOID LUPUS ERYTHROMATOSUS PROGRESSING TO SYSTEMIC LUPUS ERYTHEMATOSUS, WITH OVERLAPPING ANCA-ASSOCIATED VASCULITIS, AND RHEUMATOID ARTHRITIS PRESENTING AS PAPULAR-PURPURIC GLOVE AND SOCK SYNDROME

PV285 / #617 Case Report Poster Topic: AS07 - Cutaneous Lupus Introduction Chronic cutaneous lupus erythematosus (CCLE), particularly discoid lupus erythematosus (DLE), is usually the most disfiguring of the lupus erythematosus (LE) – specific skin lesions. It manifests with sharply-demarcated skin lesions that are photosensitive. While it physically causes scarring, systemic involvement is rare. In certain cases, however, DLE may be part of, or may progress to systemic lupus erythematosus (SLE). The occurrence of concomitant DLE and Rheumatoid Arthritis (RA), DLE and ANCA-Associated Vasculitis (AAV) have only been reported in a few cases. There are also more cases of overlap syndromes occurring in subacute and acute LE, rather than in the discoid subtype. The paucity of local data in the Philippines and Southeast Asia also adds to the diagnostic dilemma. Finally, the role of a viral infection prompting papular-purpuric glove and sock syndrome (PPGSS) in heralding a CTD remains to be determined. Case Presentation With Investigation We present the case of 28-year-old female, with known history of papular-purpuric glove and sock syndrome attributed to systemic viral infection occurring 2 years prior. She presented to the emergency room with a chief complaint of fever and rash. Accompanying symptoms included unintentional weight loss, joint pain, muscle pain, alopecia, with marked headache upon sun exposure, and photosensitive discoid rashes. PE showed scarring alopecia and generalized discoid rashes with raised, erythematosus plaques and adherent scales on the scalp, face, neck, cheeks, nose, ears, and upper lip. Both hands showed signs of active synovitis. Despite the presence of discoid lesions, and absence of the traditional acute cutaneous lesions, patient progressed to SLE. She presented with the following criteria: fever, hemolytic anemia with direct Coomb’s positivity, oral ulcers, arthritis, and hypocomplementemia, satisfying the ACR/EULAR 2019 Criteria for SLE. Morning stiffness and hand joint deformities typical of RA prompted RF testing, which turned out to be seropositive. c-ANCA was likewise seropositive at 1:80. Infection and malignancy were ruled out. Patient was started on corticosteroids and conventional synthetic DMARDs which prompted resolution of fever, rash, and arthritis. While discoid skin lesions remain permanent, her disease activity is presently quiescent. Literature Review CCLE encompasses wide range of dermatologic manifestations which may or may not include systemic disease, and some cutaneous manifestations, these includes discoid lupus erythromatosus, lupus erythematosus profundus, chilblain cutaneous lupus, and lupus tumidus, which may incidentally include systemic disease that would provide clinical clues, signs, and symptoms for MCTD or an overlap syndrome. Early signs and symptoms may involve hands, fingers, fingertips. Although dermatologic manifestations are common and may occur at initial presentation, skin lesions vary in morphology like cutaneous nodules, macular eruptions, and ecchymoses. SLE and RA overlap, termed “Rhupus Syndrome” is rare, and is estimated to be prevalent in 1% of patients with RA, the occurrence of DLE and AAV also affects a range of internal organs which are managed with high-dose glucocorticoids, immunosuppressants and targeted biologic medications. Discussion While CCLE lesions usually remain limited to cutaneous involvement, vigilance should always be exercised due to its possible progression to SLE. Likewise, confirmation of SLE, if with concomitant symptoms typical of another CTD, should prompt further workup. In this case, our patient presented with an overlap syndrome, comprised of SLE, RA, and AAV. The role of a prior PPGSS remains unknown. Our case highlights the need to have high index of suspicion for overlapping syndromes, especially in The Philippines, which is developing and is resource-limited.

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