Kaposi's Varicelliform Eruption in a Patient with Darier's Disease.

Sir,

We present a 24-year-old female with a 10-year history of Darier's disease [Figure 1]. She was admitted to emergency with high fever, malaise, lymphadenopathy, and sudden vesicular eruption on the face, neck, trunk, and extremities. The eruption had begun after intense sun exposure. Physical examination revealed vesicles, pustules, and scabs on the face, neck, trunk, and genital area, with regional lymphadenopathy [Figure 2]. We suspected disseminated skin infection by the herpes simplex virus (HSV), and diagnosis was confirmed by detection of herpetic giant cells in Tzanck smear and the detection of IgM antibodies to HSV in the serum. The diagnosis of Kaposi's varicelliform eruption HSV-1 was made. The patient initiated antiviral treatment with acyclovir intravenous (IV) for a 14-day course and topically mupirocin 2% ointment. In the evolution, the patient presented secondary infection of scabby plates by Pseudomonas aeruginosa and Staphylococcus aureus methicillin-sensitive, so that, after taking culture and sensitivity, we started antibiotic therapy with vancomycin and levofloxacin [Figure 3]. The skin lesions resolved in 5 weeks with residual erythematous macules and varioliform scars.

Figure 1

Keratotic papules in seborrheic areas. Mucosal hypertrophy on hard palate

Figure 2

Vesicles, pustules, and scabs on the face, neck, trunk, and genital area

Figure 3

Secondary infection of scabby plates by Pseudomonas aeruginosa and Staphylococcus aureus methicillin-sensitive

Darier's disease is a disorder of keratinization with autosomal dominant transmission, with high penetrance and variable expression. It is caused by mutations in the gene ATP2A2, located on chromosome 12(12q23-24.1), which encodes ATPase responsible for calcium-dependent signals, and this causes alteration of intercellular junctions and cellular adhesion.[1] Clinically, it is characterized by warty papules and keratotic plaques in seborrheic areas of the scalp and trunk and intertriginous areas. Sometimes malodorous crusted plaques appear due to secondary bacterial infection. Most patients present papules resembling flat warts on the back of the hands and feet and characteristic palmar pits. Sometimes, they are affected in oral and genital mucosa and whitish papules in hard palate. The symptoms are itching and odor and they are worse with heat, sun exposure, and sweating.[2] Patients are predisposed to bacterial infections and more rarely fungal and viral, and this may occur both during activity and remission. HSV infection is a rare complication and it is thought to be associated with impaired cellular immunity.[3] But to date, it has not shown any immune deficiency. It has also been associated with defects in the skin barrier because secondary infections usually begin in skin areas most affected by the disease. The eczema herpeticum is a severe viral infection caused by cutaneous dissemination of HSV-1 (mainly), HSV-2, vaccinia virus, or coxsackievirus A16 in patients with dermatoses such as atopic dermatitis, seborrheic dermatitis, pemphigus, ichthyosis, erythrodermas, or Darier's disease.[45] The severity of the disease is mild to severe with potentially lethal forms. Secondary bacterial infection (S. aureus, Streptococcus pyogenes, Pseudomonas, and anaerobes) is common and this can evolve into sepsis. Differential diagnoses are chickenpox, impetigo and contact dermatitis.[6]

There are few reported cases of Kaposi's varicelliform eruption in Darier's disease like our patient. We highlight the importance of avoiding intense sun exposure which exacerbates this genodermatosis. In our case, herpetic infection spread to the areas with and without Darier's lesions and led to secondary bacterial infections requiring IV antibiotics.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.