PMC10348830_01
Clinical presentation and diagnostic evaluations. (A) Brownish black pigmentation of the palate. (B) Tenosynovitis of extensor tendons over left wrist joint. (C) Tenosynovitis of extensor tendons over right wrist joint. (D) Tenosynovitis of flexor tendons over right ankle joint. (E) CT showing bilateral enlarged and hypodense adrenals. (F) MRI post contrast images of the ankles showing mild joint effusion with enhancing synovial thickening with tenosynovitis of extensor digitorum tendons
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There was bilateral axillary lymphadenopathy, brownish black pigmentation of the palate (Fig. 1A) and generalized hyperpigmentation of the skin. Musculoskeletal examination revealed multiple tender and swollen joints, with tenosynovitis over the wrist and dorsum of the foot (Fig. 1B-D). CT of the thorax and abdomen showed bilaterally enlarged adrenals and moderate hepatosplenomegaly, with no pulmonary or mediastinal involvement (Fig. 1E). Contrast-enhanced MRI of the ankles showed mild joint effusion, with enhancing synovial thickening and tenosynovitis of the extensor digitorum tendons (Fig. 1F).