Case report
Gianotti–Crosti syndrome in a postpartum adult Ann C. Brewer1, BS, Jason D. Michaels2, MD, MS, Dan Hobohm3, MD, David J. DiCaudo2,4, MD, and Xuan Nguyen5,6, MD
1 University of Arizona College of Medicine, Phoenix, AZ, USA, 2Department of Dermatology, Mayo Clinic, Scottsdale, AZ, USA, 3Department of Laboratory Medicine and Pathology, Maricopa Integrated Health Services, Phoenix, AZ, USA, 4Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA, 5 Department of Dermatology, Mayo Clinic, Scottsdale, Arizona and 6Department of Dermatology, Maricopa Integrated Health Services, Phoenix, AZ, USA
Correspondence Jason D. Michaels, MD Department of Dermatology Mayo Clinic 13400 E Shea Blvd Scottsdale AZ 85259 USA E-mail: [email protected]
Report of a case
84
A 28-year-old Hispanic woman who was three weeks postpartum presented with a 5-day history of pruritic rash and a 2-day history of arthralgia and sore throat. Her pregnancy was complicated by pre-eclampsia at term, and she had a Cesarean delivery. The rash first appeared on the shoulders and spread centrifugally to the arms, thighs, and legs. Arthralgia began in the small joints of the hands and progressed to involve the wrists and shoulders. At presentation, the rash involved the extensor arms and legs with monomorphous, edematous, pink papules (3– 4 mm) in a linear configuration (Fig. 1). The trunk was spared. Biopsy specimens were obtained from lesional and perilesional skin on the left arm. Direct immunofluorescence performed on the perilesional specimen was negative for autoimmune blistering disorders. Microscopic examination of a representative lesion showed prominent epidermal spongiosis, papillary dermal edema, and perivascular lymphocytic inflammation involving the superficial and deep dermis (Fig. 2). The clinical and histopathologic features supported a diagnosis of Gianotti–Crosti syndrome (GCS). Serologic testing was negative for Epstein–Barr virus, parvovirus B19, hepatitis B or C, Coccidioides spp., MycoInternational Journal of Dermatology 2015, 54, 84–85
Figure 1 Monomorphous, edematous, pink papules are arranged in a linear configuration, most prominently on the extensor aspects of the bilateral arms
plasma spp., rapid plasma reagin, Neisseria gonorrhoeae, Chlamydia trachomatis, and Streptococcus spp. An autoimmune evaluation showed normal levels of antinuclear antibody, cyclic citrullinated peptide, C-reactive protein, anti-SSA/Ro antibodies, anti-SSB/La antibodies, and double-stranded DNA. ª 2014 The International Society of Dermatology
Brewer et al.
(a)
(b)
Figure 2 Histopathologic findings. (A) Papillary dermal edema and perivascular, lymphocytic, dermal inflammation (hematoxylin–eosin, original magnification 920). (B) Prominent epidermal spongiosis (hematoxylin–eosin, original magnification 9200)
Given the rarity of GCS in adults, we considered other entities in the differential diagnosis, including drug eruption, molluscum contagiosum infection, and papular urticaria. However, we ultimately concluded that GCS best explained the clinical and histopathologic features: symmetrically distributed monomorphous papules affecting the extremities and sparing the trunk, duration of the rash for more than 10 days, and absence of molluscum bodies in microscopic analysis. The patient was treated with a 10-day course of oral prednisone. At the 3-week follow-up appointment, her rash had subsided, leaving only postinflammatory hyperpigmentation. Comment GCS, a papular acrodermatitis of childhood, is an uncommon dermatosis with peak incidence between 1 and 6 years of age. Clinically, GCS is characterized by a symmetric papular eruption in an acral distribution and has been reported most frequently in association with Epstein–Barr virus.1 Although GCS is generally consid-
ª 2014 The International Society of Dermatology
Gianotti-Crosti syndrome in a postpartum adult
Case report
ered a childhood dermatosis, it has been observed on rare occasions in adults. Because most patients with GCS are women, some have posited a role for the hormonal shifts of pregnancy and menstruation in disease pathogenesis.2,3 Theoretically, a temporary hormonal imbalance causes immune dysregulation akin to that seen in the relatively immature immune system of a child. This immune dysregulation would allow for the largely CD4-mediated hypersensitivity reaction observed in GCS. Immune suppression, mediated primarily by excess progesterone, is a well-known physiologic effect of normal pregnancy.4 It is possible that the immune suppression of pregnancy temporarily mimics the impaired immunity seen in a disproportionally high number of cases of pediatric GCS.5 We suggest that for our patient, postpartum immune dysregulation may have had a role in the development of GCS and possibly allowed infection with an undetected virus or bacterium. It is possible that women and atopic children with GCS share a common pathogenesis of immune dysregulation. Exactly how this immune dysregulation may lead to GCS merits further study. Acknowledgments J.D.M. and X.N. had full access to all of the data in the study and take responsibility for the integrity of the data and accuracy of the data analysis. Study concept and design was done by A.C.B., J.D.M., and X.N., acquisition of data by A.C.B. and J.D.M., analysis and interpretation of data by A.C.B., J.D.M., and X.N., drafting of the manuscript by A.C.B. and J.D.M., critical revision of the manuscript for important intellectual content by J.D.M. and X.N., and study supervision by X.N. References 1 Hurwitz S. Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence, 2nd edn. Philadelphia: Saunders, 1993. 2 Brandt O, Abeck D, Gianotti R, et al. Gianotti-Crosti syndrome. J Am Acad Dermatol 2006; 54: 136–145. 3 Claudy AL, Ortonne JP, Trepo C, et al. [Adult papular acrodermatitis (Gianottis disease). Report of 3 cases]. Ann Dermatol Venereol 1977; 104: 190–194. [In French]. 4 Weinberg ED. Pregnancy-associated immune suppression: risks and mechanisms. Microb Pathog 1987; 3: 393–397. 5 Ricci G, Patrizi A, Neri I, et al. Gianotti-Crosti syndrome and allergic background. Acta Derm Venereol 2003; 83: 202–205.
International Journal of Dermatology 2015, 54, 84–85
85
Gianotti–Crosti syndrome in a postpartum adult Ann C. Brewer1, BS, Jason D. Michaels2, MD, MS, Dan Hobohm3, MD, David J. DiCaudo2,4, MD, and Xuan Nguyen5,6, MD
1 University of Arizona College of Medicine, Phoenix, AZ, USA, 2Department of Dermatology, Mayo Clinic, Scottsdale, AZ, USA, 3Department of Laboratory Medicine and Pathology, Maricopa Integrated Health Services, Phoenix, AZ, USA, 4Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA, 5 Department of Dermatology, Mayo Clinic, Scottsdale, Arizona and 6Department of Dermatology, Maricopa Integrated Health Services, Phoenix, AZ, USA
Correspondence Jason D. Michaels, MD Department of Dermatology Mayo Clinic 13400 E Shea Blvd Scottsdale AZ 85259 USA E-mail: [email protected]
Report of a case
84
A 28-year-old Hispanic woman who was three weeks postpartum presented with a 5-day history of pruritic rash and a 2-day history of arthralgia and sore throat. Her pregnancy was complicated by pre-eclampsia at term, and she had a Cesarean delivery. The rash first appeared on the shoulders and spread centrifugally to the arms, thighs, and legs. Arthralgia began in the small joints of the hands and progressed to involve the wrists and shoulders. At presentation, the rash involved the extensor arms and legs with monomorphous, edematous, pink papules (3– 4 mm) in a linear configuration (Fig. 1). The trunk was spared. Biopsy specimens were obtained from lesional and perilesional skin on the left arm. Direct immunofluorescence performed on the perilesional specimen was negative for autoimmune blistering disorders. Microscopic examination of a representative lesion showed prominent epidermal spongiosis, papillary dermal edema, and perivascular lymphocytic inflammation involving the superficial and deep dermis (Fig. 2). The clinical and histopathologic features supported a diagnosis of Gianotti–Crosti syndrome (GCS). Serologic testing was negative for Epstein–Barr virus, parvovirus B19, hepatitis B or C, Coccidioides spp., MycoInternational Journal of Dermatology 2015, 54, 84–85
Figure 1 Monomorphous, edematous, pink papules are arranged in a linear configuration, most prominently on the extensor aspects of the bilateral arms
plasma spp., rapid plasma reagin, Neisseria gonorrhoeae, Chlamydia trachomatis, and Streptococcus spp. An autoimmune evaluation showed normal levels of antinuclear antibody, cyclic citrullinated peptide, C-reactive protein, anti-SSA/Ro antibodies, anti-SSB/La antibodies, and double-stranded DNA. ª 2014 The International Society of Dermatology
Brewer et al.
(a)
(b)
Figure 2 Histopathologic findings. (A) Papillary dermal edema and perivascular, lymphocytic, dermal inflammation (hematoxylin–eosin, original magnification 920). (B) Prominent epidermal spongiosis (hematoxylin–eosin, original magnification 9200)
Given the rarity of GCS in adults, we considered other entities in the differential diagnosis, including drug eruption, molluscum contagiosum infection, and papular urticaria. However, we ultimately concluded that GCS best explained the clinical and histopathologic features: symmetrically distributed monomorphous papules affecting the extremities and sparing the trunk, duration of the rash for more than 10 days, and absence of molluscum bodies in microscopic analysis. The patient was treated with a 10-day course of oral prednisone. At the 3-week follow-up appointment, her rash had subsided, leaving only postinflammatory hyperpigmentation. Comment GCS, a papular acrodermatitis of childhood, is an uncommon dermatosis with peak incidence between 1 and 6 years of age. Clinically, GCS is characterized by a symmetric papular eruption in an acral distribution and has been reported most frequently in association with Epstein–Barr virus.1 Although GCS is generally consid-
ª 2014 The International Society of Dermatology
Gianotti-Crosti syndrome in a postpartum adult
Case report
ered a childhood dermatosis, it has been observed on rare occasions in adults. Because most patients with GCS are women, some have posited a role for the hormonal shifts of pregnancy and menstruation in disease pathogenesis.2,3 Theoretically, a temporary hormonal imbalance causes immune dysregulation akin to that seen in the relatively immature immune system of a child. This immune dysregulation would allow for the largely CD4-mediated hypersensitivity reaction observed in GCS. Immune suppression, mediated primarily by excess progesterone, is a well-known physiologic effect of normal pregnancy.4 It is possible that the immune suppression of pregnancy temporarily mimics the impaired immunity seen in a disproportionally high number of cases of pediatric GCS.5 We suggest that for our patient, postpartum immune dysregulation may have had a role in the development of GCS and possibly allowed infection with an undetected virus or bacterium. It is possible that women and atopic children with GCS share a common pathogenesis of immune dysregulation. Exactly how this immune dysregulation may lead to GCS merits further study. Acknowledgments J.D.M. and X.N. had full access to all of the data in the study and take responsibility for the integrity of the data and accuracy of the data analysis. Study concept and design was done by A.C.B., J.D.M., and X.N., acquisition of data by A.C.B. and J.D.M., analysis and interpretation of data by A.C.B., J.D.M., and X.N., drafting of the manuscript by A.C.B. and J.D.M., critical revision of the manuscript for important intellectual content by J.D.M. and X.N., and study supervision by X.N. References 1 Hurwitz S. Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence, 2nd edn. Philadelphia: Saunders, 1993. 2 Brandt O, Abeck D, Gianotti R, et al. Gianotti-Crosti syndrome. J Am Acad Dermatol 2006; 54: 136–145. 3 Claudy AL, Ortonne JP, Trepo C, et al. [Adult papular acrodermatitis (Gianottis disease). Report of 3 cases]. Ann Dermatol Venereol 1977; 104: 190–194. [In French]. 4 Weinberg ED. Pregnancy-associated immune suppression: risks and mechanisms. Microb Pathog 1987; 3: 393–397. 5 Ricci G, Patrizi A, Neri I, et al. Gianotti-Crosti syndrome and allergic background. Acta Derm Venereol 2003; 83: 202–205.
International Journal of Dermatology 2015, 54, 84–85
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