Correspondence
An immunocompromised district in an immunocompromised patient
Editor, We read with interest the letter by Niedermeier et al.1 reporting a persistent granulomatous plaque caused by zoster infection of the left T11–L1 dermatomes in a patient immunocompromised by HIV infection. The report is of interest because it describes a concrete example of an immunocompromised district (ICD)2 in a systemically immunocompromised patient. The recently coined concept of the ICD refers to a cutaneous site that has been damaged and immunologically ‘‘marked’’ by a variety of clinical events, such as ionizing radiation, thermal or mechanical injury, chronic lymph stasis, and herpetic infection (Wolf’s post-herpetic isotopic response). An ICD becomes a weak, vulnerable site (an Achilles’ heel) that is more susceptible to subsequent outbreaks of opportunistic infections, tumors, and dysimmune reactions, often of the granulomatous type.2,3 A regional hold-up of immune cell traffic and/or a locally altered signaling of neuromediators are thought to be responsible for the destabilization of immune control in the damaged area.2,3 Niedermeier et al.1 do not consider their case to represent an instance of Wolf’s isotopic response because they noted a continuous transition from the zoster lesions to the formation of the granulomatous plaque and, by definition, an isotopic response is the occurrence of a new skin disorder exactly at the site of another, unrelated and already healed skin disease (herpes zoster in most cases).4 However, this definition applies to an individual with a normally functioning immune system. In the patient reported by Niedermeier et al.,1 as the authors themselves noticed, the pre-existing HIV infection caused an impairment of the cellular immune response that may not have been able to clear the skin of the varicella zoster virus (VZV), and thus the persistence of VZV in the affected dermatomes may have led to the formation of the granulomatous plaque. In other words, the patient’s immunocompromised
Bullous formation in a patient with familial amyloid polyneuropathy type I
1398
Editor, Familial amyloid polyneuropathy type I (FAP I) is an autosomal dominant amyloidosis characterized by the systemic deposition of amyloid with particular peripheral nerve involvement.1 Subjects with FAP I carry a heterozyInternational Journal of Dermatology 2013, 52, 1398–1461
background prevented the VZV-infected dermatomes from healing, and a dysimmune granulomatous reaction immediately took place in the involved areas. This unique report from Niedermeier et al.1 confirms the existence of zoster-related ICDs and proves that an ICD may retain its raison d’eˆtre even in an immunocompromised patient, thus introducing a more vulnerable site in an already vulnerable subject. Vincenzo Ruocco, MD Eleonora Ruocco, MD, PhD Giampiero Brunetti, MD Department of Dermatology Second University of Naples Naples Italy Ronni Wolf, MD Dermatology Unit Kaplan Medical Center Rechovot Israel E-mail: [email protected]
References 1 Niedermeier A, Flaig MJ, Rupec RA, Ruzicka T. Persistent granulomatous plaque type zoster in a patient with HIV infection. Int J Dermatol 2011; 50: 368–370. 2 Ruocco V, Brunetti G, Puca RV, Ruocco E. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol 2009; 23: 1364– 1373. 3 Ruocco V, Ruocco E, Brunetti G, Wolf R. Achilles’ heel in dermatology. J Eur Acad Dermatol Venereol 2010; 24: 1119–1120. 4 Wolf R, Brenner S, Ruocco V, Grimaldi Filioli F. Isotopic response. Int J Dermatol 1995; 34: 341–348.
gous germline mutation in the transthyretin gene, which encodes a plasma transport protein for thyroxine and retinol.2 Transthyretin is mainly synthesized in the liver, and mutant transthyretin forms an aggregate of misfolded protein, which results in extracellular amyloid deposits. Cutaneous manifestations of FAP include xerosis, seborrheic dermatitis, traumatic and burn lesions, acne, ª 2013 The International Society of Dermatology
An immunocompromised district in an immunocompromised patient
Editor, We read with interest the letter by Niedermeier et al.1 reporting a persistent granulomatous plaque caused by zoster infection of the left T11–L1 dermatomes in a patient immunocompromised by HIV infection. The report is of interest because it describes a concrete example of an immunocompromised district (ICD)2 in a systemically immunocompromised patient. The recently coined concept of the ICD refers to a cutaneous site that has been damaged and immunologically ‘‘marked’’ by a variety of clinical events, such as ionizing radiation, thermal or mechanical injury, chronic lymph stasis, and herpetic infection (Wolf’s post-herpetic isotopic response). An ICD becomes a weak, vulnerable site (an Achilles’ heel) that is more susceptible to subsequent outbreaks of opportunistic infections, tumors, and dysimmune reactions, often of the granulomatous type.2,3 A regional hold-up of immune cell traffic and/or a locally altered signaling of neuromediators are thought to be responsible for the destabilization of immune control in the damaged area.2,3 Niedermeier et al.1 do not consider their case to represent an instance of Wolf’s isotopic response because they noted a continuous transition from the zoster lesions to the formation of the granulomatous plaque and, by definition, an isotopic response is the occurrence of a new skin disorder exactly at the site of another, unrelated and already healed skin disease (herpes zoster in most cases).4 However, this definition applies to an individual with a normally functioning immune system. In the patient reported by Niedermeier et al.,1 as the authors themselves noticed, the pre-existing HIV infection caused an impairment of the cellular immune response that may not have been able to clear the skin of the varicella zoster virus (VZV), and thus the persistence of VZV in the affected dermatomes may have led to the formation of the granulomatous plaque. In other words, the patient’s immunocompromised
Bullous formation in a patient with familial amyloid polyneuropathy type I
1398
Editor, Familial amyloid polyneuropathy type I (FAP I) is an autosomal dominant amyloidosis characterized by the systemic deposition of amyloid with particular peripheral nerve involvement.1 Subjects with FAP I carry a heterozyInternational Journal of Dermatology 2013, 52, 1398–1461
background prevented the VZV-infected dermatomes from healing, and a dysimmune granulomatous reaction immediately took place in the involved areas. This unique report from Niedermeier et al.1 confirms the existence of zoster-related ICDs and proves that an ICD may retain its raison d’eˆtre even in an immunocompromised patient, thus introducing a more vulnerable site in an already vulnerable subject. Vincenzo Ruocco, MD Eleonora Ruocco, MD, PhD Giampiero Brunetti, MD Department of Dermatology Second University of Naples Naples Italy Ronni Wolf, MD Dermatology Unit Kaplan Medical Center Rechovot Israel E-mail: [email protected]
References 1 Niedermeier A, Flaig MJ, Rupec RA, Ruzicka T. Persistent granulomatous plaque type zoster in a patient with HIV infection. Int J Dermatol 2011; 50: 368–370. 2 Ruocco V, Brunetti G, Puca RV, Ruocco E. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol 2009; 23: 1364– 1373. 3 Ruocco V, Ruocco E, Brunetti G, Wolf R. Achilles’ heel in dermatology. J Eur Acad Dermatol Venereol 2010; 24: 1119–1120. 4 Wolf R, Brenner S, Ruocco V, Grimaldi Filioli F. Isotopic response. Int J Dermatol 1995; 34: 341–348.
gous germline mutation in the transthyretin gene, which encodes a plasma transport protein for thyroxine and retinol.2 Transthyretin is mainly synthesized in the liver, and mutant transthyretin forms an aggregate of misfolded protein, which results in extracellular amyloid deposits. Cutaneous manifestations of FAP include xerosis, seborrheic dermatitis, traumatic and burn lesions, acne, ª 2013 The International Society of Dermatology
