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  • Presentation

S010. Clinicopathologic Self-Assessment

Description

In this clinicopathologic self-assessment, Dr. Antonella Kalami presents a series of dermatological case studies to engage and educate attendees. The first case involves a 67-year-old woman with a growing plaque on her scalp, diagnosed as sarcoidal alopecia, showcasing granulomatous inflammation. Dr. Kalami emphasizes the importance of systemic evaluations in these patients. The second case concerns a 39-year-old with widespread scaly papules, initially thought to be generalized eruption, which is ultimately diagnosed as eruptive xanthomas linked to familial hypertriglyceridemia; histological findings indicate lipidized histiocytes and local nutritional deficiency-like changes. The last case features a 53-year-old female who presents with a painful abdominal plaque after exposure to contaminated water, leading to the diagnosis of cutaneous Rosai-Dorfman disease. This case highlights the significance of thorough investigations in revealing unrelated serious conditions, such as pancreatic carcinoma, discovered during diagnostic imaging. Throughout the presentation, Dr. Kalami engages the audience with quizzes and encourages discussion on the clinical presentations, differential diagnoses, and histopathological insights relevant to dermatological practice.

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Conclusions

  • Sarcoidal alopecia is a rare condition that can present as a granulomatous inflammation affecting the scalp and is crucial to diagnose due to its association with systemic diseases.
  • Scalp sarcoidosis often manifests as scarring alopecia and may rarely appear without scarring, requiring thorough systemic evaluation if diagnosed.
  • Familial hypertriglyceridemia can cause eruptive xanthomas, which typically presents with reddish lesions rather than the expected yellowish appearance.
  • Eruptive xanthomas resolve with effective treatment of the underlying hypertriglyceridemia, but lesions may not resolve in some cases, highlighting the need for accurate diagnosis.
  • Cutaneous Rosai-Dorfman disease is characterized by dermal infiltration of histiocytes and may present similarly to other dermatoses, necessitating careful histological examination for diagnosis due to its rare occurrence.
  • Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathologic entity. Arch Pathol 1969; 87: 63-70.
  • Kong YY, Kong JC, Shi DR, et al. Cutaneous Rosai-Dorfman disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol. 2007;31:341-350.
  • Chu P, LeBoit PE. Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement. J Cutan Pathol 1992; 19: 201-206.
  • Brenn T, Calonje E, Granter SR et al. Cutaneous Rosai-Dorfman disease is a distinct clinical entity. Am J Dermatopathol 2002; 24: 385-391.
  • Frater JL, Maddox JS, Obadiah JM, Hurley MY. Cutaneous Rosai-Dorfman disease: comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case. J Cutan Med Surg. 2006;10:281-90.
  • Scheel MM, Rady PL, Tyring SK, Pandya AG. Sinus histiocytosis with massive lymphadenopathy: presentation as giant granuloma annulare and detection of human herpesvirus 6. J Am Acad Dermatol. 1997. 37(4):643-646.
  • Chan CC and Chu CY. Dapsone as a potential treatment for cutaneous Rosai-Dorfman disease with neutrophilic predominance. Arch Dermatol. 2006. 142: 428-430.
  • Kutlubay Z, Bairamov O, Sevim A, Demirkesen C, Cem Mat M. Rosai-Dorfman Disease: A Case Report With Nodal and Cutaneous Involvement and Review of the Literature. 2014. Am J Dermatopathol. 36:4.
  • Mebazaa A, Trabelsi S, Denguezli M, et al. Extensive purely cutaneous Rosai-Dorfman disease responsive to acitretin. Int J Dermatol. 2007;46:1208-1210.