Derm Dx: Pink flat papules on the face - Clinical Advisor

Derm Dx: Pink flat papules on the face

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  • Flat wart 1_0413 Derm Dx

  • Flat wart 2_0413 Derm Dx

  • Flat wart 3_0413 Derm Dx

A 20-year-old man with severe combined immunodeficiency syndrome (SCIDS) presents to the dermatology clinic complaining of lesions in his beard area for several years. He has tried multiple treatments but they will not go away.

The patient underwent hematopoietic stem cell transplantation during infancy and has had very few health problems in recent years.

This is a case of an immunosuppressed man with severe combined immunodeficiency syndrome (SCIDS) who is suffering from extensive flat warts in the beard region.  SCIDS is a group of disorders in which genetic defects lead severe deficits in both...

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This is a case of an immunosuppressed man with severe combined immunodeficiency syndrome (SCIDS) who is suffering from extensive flat warts in the beard region. 

SCIDS is a group of disorders in which genetic defects lead severe deficits in both cell-mediated and humoral immunity. Patients with SCIDS have extremely weak immune systems and will die in infancy from infection unless treated.  The treatment of choice is hematopoietic stem cell transplantation during infancy.  Long-term survival is excellent if patients receive the transplant from an HLA-identical sibling donor. 

The classic triad of infections in an infant with SCIDS prior to stem cell transplantation consists of cutaneous candidal infections, severe diarrhea and pneumonia.  Multiple other bacterial and viral infections may occur. Infants may develop severe eczematous or seborrheic dermatitis like eruptions.

The most common type of SCIDS is X-linked recessive due to mutations in the gamma-chain of the IL-2 receptor. The other variants of SCIDS are autosomal recessive. The most common autosomal recessive subtype is due to mutations in the adenosine deaminase gene. Other subtypes are due to mutations in JAK3, CD45, RAG1 and multiple other genes.

Patients with mutations in gamma-chain and JAK3 may develop severe human papilloma-virus (HPV) infections with flat warts or common warts. The average age of wart onset in SCIDS is 8 years.

Flat warts are most commonly due to HPV subtypes 3 and 10. They appear as pink flat papules. Autoinoculation is common, and the patient can spread the warts on their own skin by scratching or shaving. For this reason, flat warts are often seen on the legs of females who shave. In the case presented above, the flat warts appear in the beard area, likely spread by shaving.

In general, flat warts have the highest rate of spontaneous remission compared with other forms of warts. However, in immunocompromised patients, such as SCIDS patients, the warts may be refractory to treatment.

Multiple treatments for flat warts have been proposed including (but not limited to) cryotherapy, laser therapy, topical immunotherapy, topical imiquimod, topical and systemic retinoids, topical cidofovir, photodynamic therapy, electrodessication, topical 5-fluorouracil and photodynamic therapy.

In immunocompetent patients, the lesions may be self-limited and require minimal treatment. Unfortunately in immunosuppressed patients, the lesions can be quite persistent and may not resolve despite multiple treatment approaches. 

Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine in Houston.

References

  1. Bolognia J, Jorizzo JL, Rapini RP. “Chapter 59: Primary Immunodeficiencies.” Dermatology. St. Louis: Mosby/Elsevier, 2008.
  2. James WD, Berger TD, Elston DM et al. “Chapter 19: Viral Diseases.” Andrews’ Diseases of the Skin: Clinical Dermatology. Philadelphia: Saunders Elsevier, 2006.
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