British Journal of Dermatology {1992) 127, 289-291.
Pemphigus vegetans in a child J.H.SILLEVIS SMITT. T.J.MULDER.* F.W.ALBEDAt:!: AND J.C.VAN NIEROP* Departments of Dermatology. 'Paediatrics, and -^Pathology. University of Amsterdam. Academisch Medisch Centrum. Emma Kinderziekenhuis/Het Kinder AMC. Meibergdreef 9. HfH AZ Amsterdam. The Nether!ands Accepted for publication 16 April 1992
Summary
Pemphigus vegetans was diagnosed in a 12-year-old boy based on clinical, histopathological and immunohistological findings. To our knowledge, this is the first case of juvenile pemphigus vegetans to be published in the literature. Suggested treatment with prednisone and azathioprine was refused, and the patient was treated with a decoction of herbs prescribed by a practitioner of traditional Chinese herbal medicine. This treatment gave excellent results. Possible active components of the treatment are discussed.
Pemphigus is very rare in children, although endemic pemphigus, fogo selvagem. occurs quite frequently in children in endemic areas.' Pemphigus foiiaceus^ and pemphigus vulgaris' are known to occur in childhood, and 35 children with pemphigus vulgaris have been reported in the literature. The clinical picture and the prognosis in children are comparable with the disease in adults.' Although pemphigus vegetans is mentioned in textbooks of paediatric dermatology,^' no reference is made in these books to published case reports. We present a ease of juvenile pemphigus vegetans and note the surprisingly good result from treatment with a herb decoction prescribed by a practitioner of traditional Chinese medicine.
Case report A 12-year-old Chinese boy was referred to our hospital with a 5-month history of stomatitis. Initially, painful blisters appeared on his tongue and on the inside of his lips, and subsequently he gradually developed a severe stomatitis. Although there were some episodes of fever. his general condition remained good. Treatment with analgesics and amoxyciliin was unsuccessful and the boy was referred to our hospital. On presentation the inflammation had spread to the perioral skin, and skin lesions had also appeared on other parts ofthe body. The Correspondence: Dr |.H.Sillevis Smitt. Department of Dermatology. Academisch Medisch Centrum, MeibergdreefJ. 1105 AZ Amsterdam, Tbe Netherlands, :f Deceased, This case was presented, in part, at the 6th International Congress of Pediatric Dermatology. 8-11 June 1992, Toronto. Canada.
patient complained ofan extremely painful mouth and throat, and of chest pain. In spite ofa good appetite, his food intake had diminished. There was a loss of weight of 4 kg in 2 weeks. Physical examination on admission showed a slender boy. height 144 em. weight 32 kg. temperature 37-4°C. who could hardly speak due to painful erosions and swelling of the lips and tongue. There were erythematous papular lesions with crusts and some small blisters on the nares (Fig. 1). the perioral skin, the axillae, the groins and the umbilical and perianal regions. In addition there was a balanitis. There was no lymphadenopathy. Investigations showed an ESR of 5 mm/h; white eell eount 8-5 x l()''/l. with a normal differential; eosinophils 490 X lO'yi. Staphylococcus aureus was isolated from the skin lesions. Histopathology ofthe first skin biopsy (Fig. 2) showed erosion of the epidermis, with a purulent exudate. Some necrosis of keratinocytes was seen, while the nuclei ofthe basal cell layer showed hypochromasia and eosinophilic nucleoli. There was minimal focal suprabasal acantholysis. Because an extensive mixed cellular infiltrate was seen in the dermis, with neutrophils in the epidermis, the lesions were interpreted as being of infectious origin. Serological investigations suggested a recent herpes simplex virus {HSV) infection, which was compatible with the histopathology showing cytonuclear changes suggestive of a viral infection. He was treated with oral acyclovir (200 mg 5 times/ day) for 3 weeks. During this period the HSV-antibody titre decreased. There was no serological evidence of other viral or bacterial infections. Immunological investigations showed no evidence of immunodeficiency. The skin lesions, especially those in the axillae and groins. 289
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J.H.SILLEVIS SMITT et al.
Figure 1. Some small blisters on the nares and haemorrhagic crusts on skin and mucou.s membrane of the nose.
Figure 3. Large vegetating, erosive plaques in combination with some small blisters in the groins.
Figure 2. First skin biopsy showing partial erosion of tbe epidermis, witb a purulent exudate and keratinocyte changes consisting of nuclear hypochromasia. large eosinopbilic nucleoli and focal multinucleate keratinocytes (haematoxylin and eosin. x 200),
Figure 4. Second skin biopsy showing a hyperplastic epidermis with suprabasal acantbolytic cleft formation and extensive infiltration by eosinophils (haematoxylin and eosin x 125).
PKMPHIGUS VEGETANS IN A CHILD
grew into large, very painful, vegetating plaques (Fig. 3). Histopathology of one of these lesions showed a papillomatous. hyperplastic epidermis with suprabasal acantholysis. Some of the suprabiisa! clefts were filled with eosinophils. The histological picture was diagnostic oi pemphigus vegetans (Fig. 4). Direct immunofluorescence of the biopsy showed the presence of intercellular IgG antibodies, characteristic of pemphigus. The serum titre of the antibodies against intercellular epidermal antigen was 1:240. A diagnosis of pemphigus vegetans was made, and treatment with prednisone in combination with azathioprine was proposed. However, the boy's parents refused this treatment and turned to a practitioner of traditional Chinese herbal medicine, who treated him with a decoction consisting of a mixture of herbs (containing Taraxacum mon()oUctmi [Hand.-Mazz.]. Viola yezoensis [Maxim,]. Cortex rhizoma. Paeonia albiflora [Pall.], Paeonia Moutan [Sims.]. Glycyrrhiza uralensis |Fisch.]. Polyporus cocos [Schw.]. Scutellaria rivularis [Wall.]. Oldenlandia diffusa [Roxb.] and Smilax glabra [Roxb.]. as its constant components). This treatment resulted in complete clearance of skin lesions, leaving only post-infiammatory hyperpigmentation. The oral lesions did not disappear completely. A fasting morning serum cortisol taken during treatment with this decoction was within normal limits. At present. 1 year after commencing treatment with the herb decoction, the patient is still in remission, although the antibody titre against intercellular epidermal antigen is unchanged.
291
prescribed by a practitioner in traditional Chinese medicine. To obtain an indication of the possible presence of corticosteroids in this decoction, a fasting serum cortisol was performed, and showed a value within normal limits. This suggests that no. or at least no considerable amount of, corticosteroids was present in the decoction. Recently the effectiveness of Chinese herbs in the treatment of atopic eczema has been reported.*" It has been suggested that paeonol. present in various plants including those of the genus Paeonia. was an active component of this preparation in the treatment of eczema.' ^' Our patient's herb decoction contained two plants of the genus Paeonia. Whether paeonol is the effective component in the treatment given to our patient, remains to be determined.
Acknowledgments We would like to thank Mr Fong Chi Kwan and Mrs Yvonne Lau Shuet Ming, for making the composition of the decoction available, and Ing W.N.J.Ursem. Hortus Botanicus, Amsterdam, for botanical advice.
Addendum To our deepest regret our colleague F.W.AIbeda died on 27 February 1992.
References Discussion Pemphigus vegetans is considered to be a pemphigus variant, ultimately often evolving into pemphigus vulgaris. At first presentation most patients show stomatitis, as did our patient. The herpes simplex infection, leading to blisters showing acantholysis. caused delay in diagnosis in our case. The question arises as to whether the herpes infection provoked the onset of the pemphigus. Good therapeutic results are obtained in the treatment of pemphigus with prednisone and azathioprine. but these agents may give rise to significant side-effects. Our patient was by chance treated with a decoction of herbs.
1 Diaz LA. Sampaio SAP, Rivitti EA et al Endemic pemphigus foiiaceus (Fogo Selvagem): II. Current and historic epidemiologic studies. / Invesl Dermato! 19K9; 92: 4-12. 2 Ahmed AR.SalmM. )uvenlle pemphigus.//^wMcadDprmiHo/ 19HJ: 8: 799-807, 5 Sillevis Smitt )H, Pemphigus vulgaris in childhood: clinical features, treatment and prognosis, i'ediatr Dermato! 1985; 2; 185-90, 4 Hurwitz S, Bullous disorders of childhood. In: CUnica! Pediatric I>mifllo%i/, Philadelphia: WB Saunders Company, 1981; J25-4t, 5 Schachner IA.. Press S. Vesicular, bullous and pustular disorders. In: Pediatric Dermatology (Schachner LA. Hansen RC, eds), Vol 1. New York: Churchill Livingstone, 1988: 77S-835. ft Harper JI. Yimg S-L, Evans AT el al. Cbinese herbs for eczema. Lancet 1990; iiS: 795. 7 Galloway JH, Marsh II). Bittiner SB et al Cbinese herbs for eczema, the acUvf compound,-LwHccI 1991; J37: 5ft(i, 8 fiirirper |l, Chinese herbs for eczema,/^mcl 1990; 3ih: 177.
Pemphigus vegetans in a child J.H.SILLEVIS SMITT. T.J.MULDER.* F.W.ALBEDAt:!: AND J.C.VAN NIEROP* Departments of Dermatology. 'Paediatrics, and -^Pathology. University of Amsterdam. Academisch Medisch Centrum. Emma Kinderziekenhuis/Het Kinder AMC. Meibergdreef 9. HfH AZ Amsterdam. The Nether!ands Accepted for publication 16 April 1992
Summary
Pemphigus vegetans was diagnosed in a 12-year-old boy based on clinical, histopathological and immunohistological findings. To our knowledge, this is the first case of juvenile pemphigus vegetans to be published in the literature. Suggested treatment with prednisone and azathioprine was refused, and the patient was treated with a decoction of herbs prescribed by a practitioner of traditional Chinese herbal medicine. This treatment gave excellent results. Possible active components of the treatment are discussed.
Pemphigus is very rare in children, although endemic pemphigus, fogo selvagem. occurs quite frequently in children in endemic areas.' Pemphigus foiiaceus^ and pemphigus vulgaris' are known to occur in childhood, and 35 children with pemphigus vulgaris have been reported in the literature. The clinical picture and the prognosis in children are comparable with the disease in adults.' Although pemphigus vegetans is mentioned in textbooks of paediatric dermatology,^' no reference is made in these books to published case reports. We present a ease of juvenile pemphigus vegetans and note the surprisingly good result from treatment with a herb decoction prescribed by a practitioner of traditional Chinese medicine.
Case report A 12-year-old Chinese boy was referred to our hospital with a 5-month history of stomatitis. Initially, painful blisters appeared on his tongue and on the inside of his lips, and subsequently he gradually developed a severe stomatitis. Although there were some episodes of fever. his general condition remained good. Treatment with analgesics and amoxyciliin was unsuccessful and the boy was referred to our hospital. On presentation the inflammation had spread to the perioral skin, and skin lesions had also appeared on other parts ofthe body. The Correspondence: Dr |.H.Sillevis Smitt. Department of Dermatology. Academisch Medisch Centrum, MeibergdreefJ. 1105 AZ Amsterdam, Tbe Netherlands, :f Deceased, This case was presented, in part, at the 6th International Congress of Pediatric Dermatology. 8-11 June 1992, Toronto. Canada.
patient complained ofan extremely painful mouth and throat, and of chest pain. In spite ofa good appetite, his food intake had diminished. There was a loss of weight of 4 kg in 2 weeks. Physical examination on admission showed a slender boy. height 144 em. weight 32 kg. temperature 37-4°C. who could hardly speak due to painful erosions and swelling of the lips and tongue. There were erythematous papular lesions with crusts and some small blisters on the nares (Fig. 1). the perioral skin, the axillae, the groins and the umbilical and perianal regions. In addition there was a balanitis. There was no lymphadenopathy. Investigations showed an ESR of 5 mm/h; white eell eount 8-5 x l()''/l. with a normal differential; eosinophils 490 X lO'yi. Staphylococcus aureus was isolated from the skin lesions. Histopathology ofthe first skin biopsy (Fig. 2) showed erosion of the epidermis, with a purulent exudate. Some necrosis of keratinocytes was seen, while the nuclei ofthe basal cell layer showed hypochromasia and eosinophilic nucleoli. There was minimal focal suprabasal acantholysis. Because an extensive mixed cellular infiltrate was seen in the dermis, with neutrophils in the epidermis, the lesions were interpreted as being of infectious origin. Serological investigations suggested a recent herpes simplex virus {HSV) infection, which was compatible with the histopathology showing cytonuclear changes suggestive of a viral infection. He was treated with oral acyclovir (200 mg 5 times/ day) for 3 weeks. During this period the HSV-antibody titre decreased. There was no serological evidence of other viral or bacterial infections. Immunological investigations showed no evidence of immunodeficiency. The skin lesions, especially those in the axillae and groins. 289
290
J.H.SILLEVIS SMITT et al.
Figure 1. Some small blisters on the nares and haemorrhagic crusts on skin and mucou.s membrane of the nose.
Figure 3. Large vegetating, erosive plaques in combination with some small blisters in the groins.
Figure 2. First skin biopsy showing partial erosion of tbe epidermis, witb a purulent exudate and keratinocyte changes consisting of nuclear hypochromasia. large eosinopbilic nucleoli and focal multinucleate keratinocytes (haematoxylin and eosin. x 200),
Figure 4. Second skin biopsy showing a hyperplastic epidermis with suprabasal acantbolytic cleft formation and extensive infiltration by eosinophils (haematoxylin and eosin x 125).
PKMPHIGUS VEGETANS IN A CHILD
grew into large, very painful, vegetating plaques (Fig. 3). Histopathology of one of these lesions showed a papillomatous. hyperplastic epidermis with suprabasal acantholysis. Some of the suprabiisa! clefts were filled with eosinophils. The histological picture was diagnostic oi pemphigus vegetans (Fig. 4). Direct immunofluorescence of the biopsy showed the presence of intercellular IgG antibodies, characteristic of pemphigus. The serum titre of the antibodies against intercellular epidermal antigen was 1:240. A diagnosis of pemphigus vegetans was made, and treatment with prednisone in combination with azathioprine was proposed. However, the boy's parents refused this treatment and turned to a practitioner of traditional Chinese herbal medicine, who treated him with a decoction consisting of a mixture of herbs (containing Taraxacum mon()oUctmi [Hand.-Mazz.]. Viola yezoensis [Maxim,]. Cortex rhizoma. Paeonia albiflora [Pall.], Paeonia Moutan [Sims.]. Glycyrrhiza uralensis |Fisch.]. Polyporus cocos [Schw.]. Scutellaria rivularis [Wall.]. Oldenlandia diffusa [Roxb.] and Smilax glabra [Roxb.]. as its constant components). This treatment resulted in complete clearance of skin lesions, leaving only post-infiammatory hyperpigmentation. The oral lesions did not disappear completely. A fasting morning serum cortisol taken during treatment with this decoction was within normal limits. At present. 1 year after commencing treatment with the herb decoction, the patient is still in remission, although the antibody titre against intercellular epidermal antigen is unchanged.
291
prescribed by a practitioner in traditional Chinese medicine. To obtain an indication of the possible presence of corticosteroids in this decoction, a fasting serum cortisol was performed, and showed a value within normal limits. This suggests that no. or at least no considerable amount of, corticosteroids was present in the decoction. Recently the effectiveness of Chinese herbs in the treatment of atopic eczema has been reported.*" It has been suggested that paeonol. present in various plants including those of the genus Paeonia. was an active component of this preparation in the treatment of eczema.' ^' Our patient's herb decoction contained two plants of the genus Paeonia. Whether paeonol is the effective component in the treatment given to our patient, remains to be determined.
Acknowledgments We would like to thank Mr Fong Chi Kwan and Mrs Yvonne Lau Shuet Ming, for making the composition of the decoction available, and Ing W.N.J.Ursem. Hortus Botanicus, Amsterdam, for botanical advice.
Addendum To our deepest regret our colleague F.W.AIbeda died on 27 February 1992.
References Discussion Pemphigus vegetans is considered to be a pemphigus variant, ultimately often evolving into pemphigus vulgaris. At first presentation most patients show stomatitis, as did our patient. The herpes simplex infection, leading to blisters showing acantholysis. caused delay in diagnosis in our case. The question arises as to whether the herpes infection provoked the onset of the pemphigus. Good therapeutic results are obtained in the treatment of pemphigus with prednisone and azathioprine. but these agents may give rise to significant side-effects. Our patient was by chance treated with a decoction of herbs.
1 Diaz LA. Sampaio SAP, Rivitti EA et al Endemic pemphigus foiiaceus (Fogo Selvagem): II. Current and historic epidemiologic studies. / Invesl Dermato! 19K9; 92: 4-12. 2 Ahmed AR.SalmM. )uvenlle pemphigus.//^wMcadDprmiHo/ 19HJ: 8: 799-807, 5 Sillevis Smitt )H, Pemphigus vulgaris in childhood: clinical features, treatment and prognosis, i'ediatr Dermato! 1985; 2; 185-90, 4 Hurwitz S, Bullous disorders of childhood. In: CUnica! Pediatric I>mifllo%i/, Philadelphia: WB Saunders Company, 1981; J25-4t, 5 Schachner IA.. Press S. Vesicular, bullous and pustular disorders. In: Pediatric Dermatology (Schachner LA. Hansen RC, eds), Vol 1. New York: Churchill Livingstone, 1988: 77S-835. ft Harper JI. Yimg S-L, Evans AT el al. Cbinese herbs for eczema. Lancet 1990; iiS: 795. 7 Galloway JH, Marsh II). Bittiner SB et al Cbinese herbs for eczema, the acUvf compound,-LwHccI 1991; J37: 5ft(i, 8 fiirirper |l, Chinese herbs for eczema,/^mcl 1990; 3ih: 177.
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