e344
Correspondence
Figure 2 Multiple, pruritic, light brown, flat seborrheic keratoses, some of which are excoriated, on the chest of an 85-year-old man
personal experience shows that dermoscopy of this initial type of SK often shows a fingerprinting1,2 of either light brown, delicate networks in a fingerprint-type pattern or an indistinct structure3 with a cobblestone-like pattern (Fig. 3). In both cases, comedo-like openings are often seen. The dermoscopic findings of thick SK, such as small milia-like cysts, irregular crypts, fissures or ridges, blue– gray globules, hairpin vessels, and fat fingers, are less common. Cryotherapy of the lesions leads in all cases to resolution of the itch. Incomprehensibly, the literature does not mention SK as a reason for general pruritus, and a search of PubMed did not reveal any papers on this issue. The standard references in Fitzpatrick’s Dermatology in General Medicine4 and Bolognia et al.5 do not mention pruritus as a symptom of SK, and the latter bring up pruritus as a sign of only the irritated type of SK. Seborrheic keratoses should be included in the list of causes of generalized pruritus. Oliver Kraigher MD Department of Dermatology Ayalon Clinic Clalit Health Services
Unilateral bullous pemphigoid in a patient with a previous ipsilateral cerebellar hemorrhage
Cases of unilateral bullous pemphigoid (BP) related to contralateral cerebral damage have been described,1 but why BP appears only on the hemiplegic side and not elsewhere remains unexplained. However, to the best of our knowledge, a case of unilateral BP associated with ipsilateral cerebellar hemorrhagic stroke has never been reported so far. International Journal of Dermatology 2014, 53, e332–e346
Figure 3 Dermoscopy of an initial type of seborrheic keratosis shows an indistinct structure with a slight cobblestone-like pattern. Scale: 1:10. (Reproduced with kind permission of Alon Scope, MD)
Tel-Aviv Israel E-mail: [email protected], [email protected] References 1 Braun RP, Rabinovitz HS, Krischer J, et al. Dermoscopy of pigmented seborrheic keratosis: a morphological study. Arch Dermatol 2002; 138: 1556–1560. 2 Argenziano G, Soyer HP, Chimenti S, et al. Dermoscopy of pigmented skin lesions: results of a consensus meeting via the Internet. J Am Acad Dermatol 2003; 48: 679–693. 3 Oakley A. Dermoscopy of seborrheic keratosis. 2008. http://dermnetnz.org/doctors/dermoscopy-course/ seborrhoeic-keratosis.html. [Accessed August 27, 2013]. 4 Silver SG, Ho VCY. Benign epithelial tumors. In: Freedberg IM, Eisen AZ, Wolff K, et al., eds. Fitzpatrick’s Dermatology in General Medicine, 6th edn. New York, NY: McGraw-Hill, 2003: 767–770. 5 Cockerell CJ, Larsen F. Benign epidermal tumors and proliferations. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology, 2nd edn. St Louis, MO: Mosby Elsevier, 2008: 1661–1664.
A 95-year-old Caucasian woman was referred to our department because of numerous itchy cutaneous bullous erosive lesions. A careful physical examination revealed the presence of erosions on an erythematous basis and tense blisters and scabs that were only located on the left side of her body (Fig. 1a,b). Serological examination demonstrated the presence of antibodies to BPAg1 and BPAg2. A biopsy specimen obtained from a fresh bullous lesion of the left leg showed a dermal–epidermal detachment ª 2014 The International Society of Dermatology
Correspondence
(a)
(b)
Figure 1 Erosive lesions, tense blisters, and scabs involving left lower limb (a) and the left upper limb (b)
(Fig. 2) and a linear deposition of IgG along the basal membrane zone on the perilesional skin (Fig. 3), which confirmed the clinically suspected diagnosis of BP. Medical history drew our attention towards a cerebellar hemorrhage of the left hemisphere (in particular involving the posterior inferior cerebellar artery) that the patient had had seven years earlier. To date, advanced pathogenic hypotheses propose that aging or disease-related neuro-autoimmunity may be involved in BP development through an autoimmune response against dystonin, a protein that shares homology with BPAg1.1 Most theories try to clarify why BP is potentially associated with some neurological diseases1 but not why the bullous lesions are circumscribed to a given cutaneous district.
Figure 2 Dermal–epidermal detachment with formation of a subepidermal bulla containing a prevalence of eosinophils trapped in a fibrin network (hematoxylin and eosin, 9 400) ª 2014 The International Society of Dermatology
Figure 3 IgG deposition along the basal membrane zone on the perilesional skin (anti-IgG serum conjugated with fluorescein, 9 200)
In our opinion, the explanation may lie in the lately proposed concept of Ruocco’s immunocompromised district (ICD).2 The term immunocompromised suggests a variation of immune response, and unnecessarily a diminution of it, while the term district highlights that this alteration is confined. The ICD represents a cutaneous site that, once it has been scarred by previous clinical events (including chronic lymphedema, herpetic infection, vaccination, thermal or mechanical injuries, amputation, paraplegia, and carpal tunnel syndrome), develops the propensity to host a secondary disease, which can appear after a variable time break (days to years).2 The acquired regional destabilization can be either defective, thus favoring opportunistic infections and tumors, or excessive, thus predisposing to immune disorders, such as pemphigus, BP, lichen planus, discoid lupus erythematosus, and drug eruptions.2 In the aforementioned case, the cerebellar hemorrhage (similarly to what can occur with paraplegia,3 hemiplegia,4 and carpal tunnel syndrome5) might have caused dysregulation in the release of neuromediators that send specific signals to cell membrane receptors of immunocompetent cells, thus altering the local immune balance. Owing to the ipsilateral anatomic connection between cerebellum and half body side, in this case the immunocompromised district took place at the same side of the cerebellar damage. It is worth noting that peripheral damage to sensory nerve fibers ensuing from amputation also favored the outbreak of BP strictly confined to the amputation stump.6 International Journal of Dermatology 2014, 53, e332–e346
e345
e346
Correspondence
This report supports the existence of a strict relationship between the skin and central nervous system hinged upon a subtle equilibrium, probably regulated by many and various factors. An alteration of this local balance, ensuing from damage to the central nervous system, may cause localized immune instability resulting in the outbreak of skin disorders confined to the involved site. The novel concept of ICD seems to be good guidance for further investigative studies aimed at clarifying opportunistic localization of several cutaneous disorders.7 Eleonora Ruocco, MD, PhD Teresa Russo, MD Vincenzo Piccolo, MD Giampiero Brunetti, MD Sonia Sangiuliano, MD Adone Baroni, MD, PhD Department of Dermatology and Venereology Second University of Naples Naples Italy Piccolo Vincenzo, MD c/o II Policlinico, Edificio 9 Primo piano Via Pansini 5 80131 Napoli Italy E-mail: [email protected]
International Journal of Dermatology 2014, 53, e332–e346
Conflicts of interest: None.
References 1 Foureur N, Descamps V, Lebrun-Vignes B, et al. Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid? Eur J Dermatol 2001; 11: 230–233. 2 Ruocco V, Brunetti G, Puca RV, et al. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol 2009; 23: 1364– 1373. 3 Baroni A, Ruocco V, Di Maio R, et al. Papillomatosis cutis arising on an immuno-compromised district due to paraplegia. Br J Dermatol 2010; 163: 646–648. 4 Piccolo V, Russo T, Baroni A. Unilateral bullous pemphigoid in hemiplegic patients: an instance of immunocompromised district. J Dermatol 2013; 40: 64–65. 5 Baroni A, Piccolo V, Russo T, et al. Recurrent blistering of the fingertips as a sign of carpal tunnel syndrome: an effect of nerve compression. Arch Dermatol 2012; 148: 545–546. 6 Brodell RT, Korman NJ. Stump pemphigoid. Cutis 1996; 57: 245–246. 7 Ruocco V, Ruocco E, Brunetti G, et al. Opportunistic localization of skin lesion on vulnerable areas. Clin Dermatol 2012; 30: 541–543.
ª 2014 The International Society of Dermatology
Correspondence
Figure 2 Multiple, pruritic, light brown, flat seborrheic keratoses, some of which are excoriated, on the chest of an 85-year-old man
personal experience shows that dermoscopy of this initial type of SK often shows a fingerprinting1,2 of either light brown, delicate networks in a fingerprint-type pattern or an indistinct structure3 with a cobblestone-like pattern (Fig. 3). In both cases, comedo-like openings are often seen. The dermoscopic findings of thick SK, such as small milia-like cysts, irregular crypts, fissures or ridges, blue– gray globules, hairpin vessels, and fat fingers, are less common. Cryotherapy of the lesions leads in all cases to resolution of the itch. Incomprehensibly, the literature does not mention SK as a reason for general pruritus, and a search of PubMed did not reveal any papers on this issue. The standard references in Fitzpatrick’s Dermatology in General Medicine4 and Bolognia et al.5 do not mention pruritus as a symptom of SK, and the latter bring up pruritus as a sign of only the irritated type of SK. Seborrheic keratoses should be included in the list of causes of generalized pruritus. Oliver Kraigher MD Department of Dermatology Ayalon Clinic Clalit Health Services
Unilateral bullous pemphigoid in a patient with a previous ipsilateral cerebellar hemorrhage
Cases of unilateral bullous pemphigoid (BP) related to contralateral cerebral damage have been described,1 but why BP appears only on the hemiplegic side and not elsewhere remains unexplained. However, to the best of our knowledge, a case of unilateral BP associated with ipsilateral cerebellar hemorrhagic stroke has never been reported so far. International Journal of Dermatology 2014, 53, e332–e346
Figure 3 Dermoscopy of an initial type of seborrheic keratosis shows an indistinct structure with a slight cobblestone-like pattern. Scale: 1:10. (Reproduced with kind permission of Alon Scope, MD)
Tel-Aviv Israel E-mail: [email protected], [email protected] References 1 Braun RP, Rabinovitz HS, Krischer J, et al. Dermoscopy of pigmented seborrheic keratosis: a morphological study. Arch Dermatol 2002; 138: 1556–1560. 2 Argenziano G, Soyer HP, Chimenti S, et al. Dermoscopy of pigmented skin lesions: results of a consensus meeting via the Internet. J Am Acad Dermatol 2003; 48: 679–693. 3 Oakley A. Dermoscopy of seborrheic keratosis. 2008. http://dermnetnz.org/doctors/dermoscopy-course/ seborrhoeic-keratosis.html. [Accessed August 27, 2013]. 4 Silver SG, Ho VCY. Benign epithelial tumors. In: Freedberg IM, Eisen AZ, Wolff K, et al., eds. Fitzpatrick’s Dermatology in General Medicine, 6th edn. New York, NY: McGraw-Hill, 2003: 767–770. 5 Cockerell CJ, Larsen F. Benign epidermal tumors and proliferations. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology, 2nd edn. St Louis, MO: Mosby Elsevier, 2008: 1661–1664.
A 95-year-old Caucasian woman was referred to our department because of numerous itchy cutaneous bullous erosive lesions. A careful physical examination revealed the presence of erosions on an erythematous basis and tense blisters and scabs that were only located on the left side of her body (Fig. 1a,b). Serological examination demonstrated the presence of antibodies to BPAg1 and BPAg2. A biopsy specimen obtained from a fresh bullous lesion of the left leg showed a dermal–epidermal detachment ª 2014 The International Society of Dermatology
Correspondence
(a)
(b)
Figure 1 Erosive lesions, tense blisters, and scabs involving left lower limb (a) and the left upper limb (b)
(Fig. 2) and a linear deposition of IgG along the basal membrane zone on the perilesional skin (Fig. 3), which confirmed the clinically suspected diagnosis of BP. Medical history drew our attention towards a cerebellar hemorrhage of the left hemisphere (in particular involving the posterior inferior cerebellar artery) that the patient had had seven years earlier. To date, advanced pathogenic hypotheses propose that aging or disease-related neuro-autoimmunity may be involved in BP development through an autoimmune response against dystonin, a protein that shares homology with BPAg1.1 Most theories try to clarify why BP is potentially associated with some neurological diseases1 but not why the bullous lesions are circumscribed to a given cutaneous district.
Figure 2 Dermal–epidermal detachment with formation of a subepidermal bulla containing a prevalence of eosinophils trapped in a fibrin network (hematoxylin and eosin, 9 400) ª 2014 The International Society of Dermatology
Figure 3 IgG deposition along the basal membrane zone on the perilesional skin (anti-IgG serum conjugated with fluorescein, 9 200)
In our opinion, the explanation may lie in the lately proposed concept of Ruocco’s immunocompromised district (ICD).2 The term immunocompromised suggests a variation of immune response, and unnecessarily a diminution of it, while the term district highlights that this alteration is confined. The ICD represents a cutaneous site that, once it has been scarred by previous clinical events (including chronic lymphedema, herpetic infection, vaccination, thermal or mechanical injuries, amputation, paraplegia, and carpal tunnel syndrome), develops the propensity to host a secondary disease, which can appear after a variable time break (days to years).2 The acquired regional destabilization can be either defective, thus favoring opportunistic infections and tumors, or excessive, thus predisposing to immune disorders, such as pemphigus, BP, lichen planus, discoid lupus erythematosus, and drug eruptions.2 In the aforementioned case, the cerebellar hemorrhage (similarly to what can occur with paraplegia,3 hemiplegia,4 and carpal tunnel syndrome5) might have caused dysregulation in the release of neuromediators that send specific signals to cell membrane receptors of immunocompetent cells, thus altering the local immune balance. Owing to the ipsilateral anatomic connection between cerebellum and half body side, in this case the immunocompromised district took place at the same side of the cerebellar damage. It is worth noting that peripheral damage to sensory nerve fibers ensuing from amputation also favored the outbreak of BP strictly confined to the amputation stump.6 International Journal of Dermatology 2014, 53, e332–e346
e345
e346
Correspondence
This report supports the existence of a strict relationship between the skin and central nervous system hinged upon a subtle equilibrium, probably regulated by many and various factors. An alteration of this local balance, ensuing from damage to the central nervous system, may cause localized immune instability resulting in the outbreak of skin disorders confined to the involved site. The novel concept of ICD seems to be good guidance for further investigative studies aimed at clarifying opportunistic localization of several cutaneous disorders.7 Eleonora Ruocco, MD, PhD Teresa Russo, MD Vincenzo Piccolo, MD Giampiero Brunetti, MD Sonia Sangiuliano, MD Adone Baroni, MD, PhD Department of Dermatology and Venereology Second University of Naples Naples Italy Piccolo Vincenzo, MD c/o II Policlinico, Edificio 9 Primo piano Via Pansini 5 80131 Napoli Italy E-mail: [email protected]
International Journal of Dermatology 2014, 53, e332–e346
Conflicts of interest: None.
References 1 Foureur N, Descamps V, Lebrun-Vignes B, et al. Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid? Eur J Dermatol 2001; 11: 230–233. 2 Ruocco V, Brunetti G, Puca RV, et al. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol 2009; 23: 1364– 1373. 3 Baroni A, Ruocco V, Di Maio R, et al. Papillomatosis cutis arising on an immuno-compromised district due to paraplegia. Br J Dermatol 2010; 163: 646–648. 4 Piccolo V, Russo T, Baroni A. Unilateral bullous pemphigoid in hemiplegic patients: an instance of immunocompromised district. J Dermatol 2013; 40: 64–65. 5 Baroni A, Piccolo V, Russo T, et al. Recurrent blistering of the fingertips as a sign of carpal tunnel syndrome: an effect of nerve compression. Arch Dermatol 2012; 148: 545–546. 6 Brodell RT, Korman NJ. Stump pemphigoid. Cutis 1996; 57: 245–246. 7 Ruocco V, Ruocco E, Brunetti G, et al. Opportunistic localization of skin lesion on vulnerable areas. Clin Dermatol 2012; 30: 541–543.
ª 2014 The International Society of Dermatology
