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[Hailey-Hailey disease: A case report]
Jesús Sebastián Rodríguez-Gutiérrez1, Antonio Tirado-Motel1, Jesús Leonel Sarabia-Esquerra1
1Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Hospital Regional "Dr. Manuel Cárdenas de la Vega", Servicio de Dermatología. Culiacán, Sinaloa, México.
Revista Medica Del Instituto Mexicano Del Seguro Social
|August 8, 2024
Summary
Hailey-Hailey disease, a rare genetic disorder caused by ATP2C1 mutations, presents as easily ruptured vesicles. Diagnosis involves histopathology showing a "dilapidated brick wall" appearance.
Area of Science:
- Dermatology
- Genetics
Background:
- Hailey-Hailey disease is a rare autosomal dominant genodermatosis.
- It stems from mutations in the ATP2C1 gene, affecting approximately 1 in 50,000 individuals.
- The condition manifests as easily ruptured, grouped flaccid vesicles.
Observation:
- A male patient presented with disseminated dermatosis affecting the neck, trunk, axillary, inguinal, and intergluteal folds.
- The lesions were unilateral, asymmetric, and polymorphous, including exulceration, erythema, pustules, and flaccid vesicles coalescing into eczematous and hypertrophic plaques.
- The chronic condition was accompanied by pruritus.
Findings:
- Histopathological examination reveals a characteristic "dilapidated brick wall" appearance.
- Dyskeratosis, presenting as round bodies and pimples, is a key diagnostic feature.
- Topical corticosteroids are frequently employed in treatment.
Implications:
- Recognizing Hailey-Hailey disease is crucial for accurate differential diagnosis in clinical practice.
- Understanding the genetic basis and clinical presentation aids in patient management.
- Further research into therapeutic options for this rare genodermatosis is warranted.
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