doi: 10.1111/1346-8138.12481
Journal of Dermatology 2014; 41: 403–406
CONCISE COMMUNICATION
Infantile generalized pustular psoriasis: Successful disease control with intermittent etretinate Chika NAMBA,1 Masamoto MURAKAMI,1 Yasushi HANAKAWA,1 Mikiko TOHYAMA,1 Yuji SHIRAKATA,1 Hisamichi TAUCHI,2 Koji SAYAMA1 1
Departments of Dermatology, and 2Pediatrics, Ehime University Graduate School of Medicine, Ehime, Japan
ABSTRACT Infantile generalized pustular psoriasis is a rare form of psoriasis and the best treatment is controversial. We experienced a 2-year-old female with erythema on her neck and axilla starting at 3 months of age. She presented with recurrent annular and geographic scaly erythema with a few pustules on the neck, precordium and axilla, but no fever. The histopathology revealed subcorneal neutrophilic infiltration and microabscesses without Kogoj’s spongiform pustules. The initial diagnosis was subcorneal pustular dermatosis. However, she developed widespread geographic erythema and numerous pustules over her entire body with a fever when she got a cold. A second skin biopsy revealed monolocular pustules and Kogoj’s spongiform pustules in the subcorneal layer. Etretinate was administrated after a diagnosis of pustular psoriasis was made and her condition improved gradually. The choice of treatment depends on patient age, general condition and the disease severity.
Key words:
etretinate, generalized, infantile, pustular psoriasis, subcorneal pustule.
INTRODUCTION Generalized pustular psoriasis (GPP or von Zumbusch psoriasis) is an acute variant of pustular psoriasis that is characterized by a fever of several days’ duration, together with the sudden appearance of sterile pustules, 2–3 mm across, over the trunk and extremities.1 GPP is a rare type of psoriasis and infantile GPP is rarer.2–4 Usually, it involves recurrent episodes of fever followed by fresh outbreaks of pustules.1 Treatments for this disease include etretinate, methotrexate, cyclosporin, infliximab or oral corticosteroids, although the best treatment for infant GPP remains unclear.5 An early diagnosis with appropriate treatment is necessary to prevent severe complications, such as bacterial infection, dehydration and sepsis.6
CASE REPORT A 2-year-old female developed erythema on her neck and axilla at the age of 3 months. Topical steroid ointment was prescribed by a dermatologist, but her eruption did not disappear completely. She presented to our hospital in March 2012. She had no relevant history, although both of her parents had atopic dermatitis, but not psoriasis. On physical examination, she had annular and geographic scaly erythema with a few pustules on the neck, precordium and axilla, but no fever (Fig. 1a). The laboratory findings revealed that the white blood
cell count (WBC), C-reactive protein (CRP) and serum albumin were within the normal ranges. A culture of the pustules detected Neisseria and Corynebacterium species, but the bacterial numbers were very low, and no fungi were detected. A biopsy of a pustular lesion in the axilla revealed subcorneal separation containing a few neutrophils, neutrophilic infiltration in the upper epidermis and dominant spongiosis in the lower epidermis. No acantholytic cells or typical Kogoj’s spongiform pustules were found in the specimen (Fig. 1b,c). Based on these findings, the differential diagnosis included subcorneal pustular dermatosis, pustular psoriasis, immunoglobulin A pemphigus, pemphigus foliaceus, impetigo, fungal infection and necrolytic migratory erythema. Considering the physical examination, clinical course, laboratory findings and biopsy result, a diagnosis of subcorneal pustular dermatosis was suspected. Despite continued topical steroid treatment, her widespread geographic erythema and numerous pustules on the trunk, limbs and face expanded gradually. In addition, when she got a cold with a fever of 38–39°C, “lakes of pustules” appeared on the lateral malleolus and she was admitted to our hospital for further examination and treatment (Fig. 2a,b). The laboratory findings showed an increased WBC to 14 600/lL (neutrophils to 10 278/lL), increased CRP to 1.42 mg/dL, and reduced serum albumin to 3.1 g/dL and serum total protein to 6.3 g/dL. Serum aspartate aminotransferase, alanine aminotransferase, creatinine and procalcitonin were within the
Correspondence: Chika Namba, M.D., Department of Dermatology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295, Japan. Email: [email protected] This paper has been selected as a paper for the “Meet the Expert Session” at the 28th annual meeting of the Japanese Society for Psoriasis Research, Tokyo, 2013. Received 2 December 2013; accepted 24 February 2014.
© 2014 Japanese Dermatological Association
403
C. Namba et al.
(a)
(b)
(c)
Figure 1. (a) Annular and geographic scaly erythema with a few pustules on the neck, precordium and axilla. (b,c) A biopsy specimen from a pustular lesion in the axilla showing subcorneal separation with a few neutrophils, neutrophilic infiltration in the upper epidermis, and spongiosis in the lower epidermis predominantly (hematoxylin–eosin, original magnifications: [b] 9100; [c] 9400).
1 week of treatment. According to the GPP treatment guidelines in Japan,7 the severity of GPP score before the medication of etretinate was “severe” (score, 11), and improved to “mild” (score,
Journal of Dermatology 2014; 41: 403–406
CONCISE COMMUNICATION
Infantile generalized pustular psoriasis: Successful disease control with intermittent etretinate Chika NAMBA,1 Masamoto MURAKAMI,1 Yasushi HANAKAWA,1 Mikiko TOHYAMA,1 Yuji SHIRAKATA,1 Hisamichi TAUCHI,2 Koji SAYAMA1 1
Departments of Dermatology, and 2Pediatrics, Ehime University Graduate School of Medicine, Ehime, Japan
ABSTRACT Infantile generalized pustular psoriasis is a rare form of psoriasis and the best treatment is controversial. We experienced a 2-year-old female with erythema on her neck and axilla starting at 3 months of age. She presented with recurrent annular and geographic scaly erythema with a few pustules on the neck, precordium and axilla, but no fever. The histopathology revealed subcorneal neutrophilic infiltration and microabscesses without Kogoj’s spongiform pustules. The initial diagnosis was subcorneal pustular dermatosis. However, she developed widespread geographic erythema and numerous pustules over her entire body with a fever when she got a cold. A second skin biopsy revealed monolocular pustules and Kogoj’s spongiform pustules in the subcorneal layer. Etretinate was administrated after a diagnosis of pustular psoriasis was made and her condition improved gradually. The choice of treatment depends on patient age, general condition and the disease severity.
Key words:
etretinate, generalized, infantile, pustular psoriasis, subcorneal pustule.
INTRODUCTION Generalized pustular psoriasis (GPP or von Zumbusch psoriasis) is an acute variant of pustular psoriasis that is characterized by a fever of several days’ duration, together with the sudden appearance of sterile pustules, 2–3 mm across, over the trunk and extremities.1 GPP is a rare type of psoriasis and infantile GPP is rarer.2–4 Usually, it involves recurrent episodes of fever followed by fresh outbreaks of pustules.1 Treatments for this disease include etretinate, methotrexate, cyclosporin, infliximab or oral corticosteroids, although the best treatment for infant GPP remains unclear.5 An early diagnosis with appropriate treatment is necessary to prevent severe complications, such as bacterial infection, dehydration and sepsis.6
CASE REPORT A 2-year-old female developed erythema on her neck and axilla at the age of 3 months. Topical steroid ointment was prescribed by a dermatologist, but her eruption did not disappear completely. She presented to our hospital in March 2012. She had no relevant history, although both of her parents had atopic dermatitis, but not psoriasis. On physical examination, she had annular and geographic scaly erythema with a few pustules on the neck, precordium and axilla, but no fever (Fig. 1a). The laboratory findings revealed that the white blood
cell count (WBC), C-reactive protein (CRP) and serum albumin were within the normal ranges. A culture of the pustules detected Neisseria and Corynebacterium species, but the bacterial numbers were very low, and no fungi were detected. A biopsy of a pustular lesion in the axilla revealed subcorneal separation containing a few neutrophils, neutrophilic infiltration in the upper epidermis and dominant spongiosis in the lower epidermis. No acantholytic cells or typical Kogoj’s spongiform pustules were found in the specimen (Fig. 1b,c). Based on these findings, the differential diagnosis included subcorneal pustular dermatosis, pustular psoriasis, immunoglobulin A pemphigus, pemphigus foliaceus, impetigo, fungal infection and necrolytic migratory erythema. Considering the physical examination, clinical course, laboratory findings and biopsy result, a diagnosis of subcorneal pustular dermatosis was suspected. Despite continued topical steroid treatment, her widespread geographic erythema and numerous pustules on the trunk, limbs and face expanded gradually. In addition, when she got a cold with a fever of 38–39°C, “lakes of pustules” appeared on the lateral malleolus and she was admitted to our hospital for further examination and treatment (Fig. 2a,b). The laboratory findings showed an increased WBC to 14 600/lL (neutrophils to 10 278/lL), increased CRP to 1.42 mg/dL, and reduced serum albumin to 3.1 g/dL and serum total protein to 6.3 g/dL. Serum aspartate aminotransferase, alanine aminotransferase, creatinine and procalcitonin were within the
Correspondence: Chika Namba, M.D., Department of Dermatology, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0295, Japan. Email: [email protected] This paper has been selected as a paper for the “Meet the Expert Session” at the 28th annual meeting of the Japanese Society for Psoriasis Research, Tokyo, 2013. Received 2 December 2013; accepted 24 February 2014.
© 2014 Japanese Dermatological Association
403
C. Namba et al.
(a)
(b)
(c)
Figure 1. (a) Annular and geographic scaly erythema with a few pustules on the neck, precordium and axilla. (b,c) A biopsy specimen from a pustular lesion in the axilla showing subcorneal separation with a few neutrophils, neutrophilic infiltration in the upper epidermis, and spongiosis in the lower epidermis predominantly (hematoxylin–eosin, original magnifications: [b] 9100; [c] 9400).
1 week of treatment. According to the GPP treatment guidelines in Japan,7 the severity of GPP score before the medication of etretinate was “severe” (score, 11), and improved to “mild” (score,
