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Mycosis fungoides presenting as a pustular eruption
908 Letters
J AM ACAD DERMATOL NOVEMBER 2009
2. Chuh AA. Diagnostic criteria for pityriasis rosea: a prospective case control study for assessment of validity. J Eur Acad Dermatol Venereol 2003;17:101-3. 3. Moss C. Cytogenetic and molecular evidence for cutaneous mosaicism: the ectodermal origin of Blaschko lines. Am J Med Genet 1999;85:330-3. 4. Grosshans EM. Acquired blaschkolinear dermatoses. Am J Med Genet 1999;85:334-7. 5. Drago F, Ranieri E, Malaguti F, Losi E, Rebora A. Human herpesvirus 7 in pityriasis rosea. Lancet 1997;349:1367-8. doi:10.1016/j.jaad.2009.01.004
Mycosis fungoides presenting as a pustular eruption A 78-year-old woman presented with a generalized and figurate erythema with marginal lamellar scaling and pustulation. In addition, scaly, erythematous, and atrophic plaques with occasional pustules were found on the trunk, arms and legs (Fig. 1). Several skin biopsy specimens showed two distinct phenomena: a) a sub- and intracorneal pustule and b) a bandlike lymphocytic infiltrate of atypical lymphocytes with cerebriform nuclei. The atypical lymphocytes showed extensive epidermotropism and intraepidermal abscess formation (Fig. 2). Immunohistochemically the atypical lymphocytes were CD45-R01, CD31, CD41 and CD81 cells. Molecular biological analysis by polymerase chain reaction showed initially no T-cellreceptor rearrangements. At follow-up, the patient was noted to have developed clinically typical plaques of mycosis fungoides (MF) and T-cell-receptor clonality could be detected. MF (T2N0M0) presenting initially with pustules was diagnosed. In the differential diagnosis, pustular psoriasis and a drug eruption were excluded histologically. Impetigo contagiosa was excluded by microbiological analysis, and autoimmune disease by serologic studies. The absence of acantholysis and negative direct immunoflourescence ruled out pemphigus foliaceous and IgA pemphigus. Acute generalized exanthematous pustulosis (AGEP) in association with MF was excluded given that our patient had no history of drugs or infection, which are the most frequent causes of this disease.1,2 Additionally, aside from subcorneal pustules, no other features of AGEP, such as papillary dermal edema, vasculitis, exocytosis of eosinophils and single-cell necrosis of keratinocytes, were observed histologically.1 Therapeutic management consisted of topical corticosteroids, bath PUVA (1 mg 8methoxalen/l for 20 minutes followed by UVA phototherapy) and dapsone 100 mg/d, with subsequent total resolution of the pustular eruption. The patient’s pustular eruption could have represented a transient expression of MF, given
Fig 1. Confluen figurate erythema with marginal lamellar scaling and pustulation in the cleavage lines of the trunk and extremities.
that the more common patch-stage MF appeared in the following years. Although the generalized rash did not recur, the patient continued to develop single recurrent erythematous plaques on the trunk and extremities for 9 years, despite continuous treatment with interferon alfa-2a (3x1,5 million units/week) and mid-potency topical corticosteroids intermittently. This case includes the longest follow-up (about 10 years) of the rare clinicopathological variant of pustular MF, which is not included in the current WHOEORTC classification for cutaneous lymphomas.3 Patients with uncommon clinical manifestations of MF often also have classic MF on other parts of the body. Although some of them have been identified as distinct entities, they are now interpreted as being merely clinicopathological variants of MF.4 Moreover, transitional periods of different clinical variants are possible, as we have seen in our patient who had a 6-month period of generalized pustulation at the onset of her MF. We believe that the generalized pustulation in MF might be an immunological phenomenon, i.e. a disturbance of interleukin release,5 although this requires confirmation. This uncommon clinic variant may also be related to overall debility. In conclusion, pustular MF should be included in the differential diagnosis when a subcorneal pustular
Letters 909
J AM ACAD DERMATOL VOLUME 61, NUMBER 5
Fig 2. A, Acanthotic epidermis with sub- and intracorneal pustules and abscesses comprised of multiple atypical lymphocytes. (Hematoxylin-eosin stain, original magnification: 340.) B, Subepidermal bandlike lymphocytic infiltrate with epidermotropism of atypical lymphocytes and subcorneal infiltration of neutrophils. (Hematoxylin-eosin stain; original magnification: 3100.)
eruption appears histologically in association with epidermotropism of atypical lymphocytes.
lymphoma (mycosis fungoides and Sezary syndrome). Br J Dermatol 2001;144(5):1016-23.
Heike Pabsch, MD,a Johannes Kunze, MD,a and Jo¨rg Schaller, MD,a,b
doi:10.1016/j.jaad.2009.01.012
Department of Dermatology and Venerologya, St. Barbara Hospital, Catholic Clinic Duisburg, Germany, Department of Dermatology and Venerologyb, Dermatobitological Unit, St. Barbara Hospital, Catholic Clinic Duisburg, Germany Funding sources: None. Conflicts of interest: None declared. Reprints not available from the authors. Correspondence to: Heike Pabsch, MD, Department of Dermatology and Venerology, St. Barbara Hospital, Catholic Clinic Duisburg, Barbarastr. 67, D-47167 Duisburg, Germany. 149-2035199595, Fax: 149-203-5199704 E-mail: [email protected] REFERENCES 1. Sidoroff A, Halevy S, Bavinck JN, Vaillant L, Roujeau JC. Acute generalized exanthematous pustulosis (AGEP)-a clinical reaction pattern. J Cutan Pathol 2001;28(3):113-9. 2. Sidoroff A, Dunant A, Viboud C, Halevy S, Bavinck JN, Naldi L, et al. Risk factors for acute generalized exanthematous pustulosis (AGEP)-results of a multinational case-control-study (EuroSCAR). Br J Dermatol 2007;157:989-96. 3. Burg G, Jaffe ES, Kempf W, et al. WHO/EORTC Classification of Cutaneous Lymphomas. In: LeBoit P, Burg G, Weedon D, Sarasin A, editors. Tumours of the Skin. WHO Pathology and Genetics. Lyon: IARC; 2006. pp. 166-7. 4. Requena L, Gonzalez-Guerra E, Angulo J, DeVore AE, Sangueza OP. Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides. Br J Dermatol 2008;158:157-62. 5. Leroy S, Dubois S, Tenaud I, Chebassier N, Godard A, Jacques Y, Dreno B. Interleukin-15 expression in cutaneous T-cell
Chemical leukoderma induced by colored strings To the Editor: In India, it is a fairly common cultural and religious practice to wear colored strings with or without various forms of amulets round the neck, arms, or waist. We report a case series of 11 patients from eastern India who developed linear leukoderma at the sites of contact with these strings. We evaluated 11 consecutive patients with linear leukoderma associated with wearing colored strings who presented to the dermatology clinic of a tertiary care hospital over a 2-year period. None of the patients had a personal or family history of vitiligo. There was no history of symptoms, such as itching, redness, or any other lesions at the sites of depigmentation in any of the patients. No signs of inflammation or any change in texture of the skin was noticed at the sites of the pigmentary changes. There were eight men and three women. Six patients were under 2 years of age. Most (6 patients) of the lesions were located over the waist (Fig 1) followed by the neck (3), groin (2), buttocks (2), and chest (1). Depigmentation in remote areas of the body (back) was noticed in only one patient. The strings were either black (7 strings) or red (5 strings) in color. One patient had worn multiple strings. The mean duration of lesions in our patients was 9.9 months. Some of our patients had metallic (3), plastic (1), or wooden (1) amulets attached to the strings. Confetti macules around the lesions were noticed in five patients. Chemical analysis of the strings was
J AM ACAD DERMATOL NOVEMBER 2009
2. Chuh AA. Diagnostic criteria for pityriasis rosea: a prospective case control study for assessment of validity. J Eur Acad Dermatol Venereol 2003;17:101-3. 3. Moss C. Cytogenetic and molecular evidence for cutaneous mosaicism: the ectodermal origin of Blaschko lines. Am J Med Genet 1999;85:330-3. 4. Grosshans EM. Acquired blaschkolinear dermatoses. Am J Med Genet 1999;85:334-7. 5. Drago F, Ranieri E, Malaguti F, Losi E, Rebora A. Human herpesvirus 7 in pityriasis rosea. Lancet 1997;349:1367-8. doi:10.1016/j.jaad.2009.01.004
Mycosis fungoides presenting as a pustular eruption A 78-year-old woman presented with a generalized and figurate erythema with marginal lamellar scaling and pustulation. In addition, scaly, erythematous, and atrophic plaques with occasional pustules were found on the trunk, arms and legs (Fig. 1). Several skin biopsy specimens showed two distinct phenomena: a) a sub- and intracorneal pustule and b) a bandlike lymphocytic infiltrate of atypical lymphocytes with cerebriform nuclei. The atypical lymphocytes showed extensive epidermotropism and intraepidermal abscess formation (Fig. 2). Immunohistochemically the atypical lymphocytes were CD45-R01, CD31, CD41 and CD81 cells. Molecular biological analysis by polymerase chain reaction showed initially no T-cellreceptor rearrangements. At follow-up, the patient was noted to have developed clinically typical plaques of mycosis fungoides (MF) and T-cell-receptor clonality could be detected. MF (T2N0M0) presenting initially with pustules was diagnosed. In the differential diagnosis, pustular psoriasis and a drug eruption were excluded histologically. Impetigo contagiosa was excluded by microbiological analysis, and autoimmune disease by serologic studies. The absence of acantholysis and negative direct immunoflourescence ruled out pemphigus foliaceous and IgA pemphigus. Acute generalized exanthematous pustulosis (AGEP) in association with MF was excluded given that our patient had no history of drugs or infection, which are the most frequent causes of this disease.1,2 Additionally, aside from subcorneal pustules, no other features of AGEP, such as papillary dermal edema, vasculitis, exocytosis of eosinophils and single-cell necrosis of keratinocytes, were observed histologically.1 Therapeutic management consisted of topical corticosteroids, bath PUVA (1 mg 8methoxalen/l for 20 minutes followed by UVA phototherapy) and dapsone 100 mg/d, with subsequent total resolution of the pustular eruption. The patient’s pustular eruption could have represented a transient expression of MF, given
Fig 1. Confluen figurate erythema with marginal lamellar scaling and pustulation in the cleavage lines of the trunk and extremities.
that the more common patch-stage MF appeared in the following years. Although the generalized rash did not recur, the patient continued to develop single recurrent erythematous plaques on the trunk and extremities for 9 years, despite continuous treatment with interferon alfa-2a (3x1,5 million units/week) and mid-potency topical corticosteroids intermittently. This case includes the longest follow-up (about 10 years) of the rare clinicopathological variant of pustular MF, which is not included in the current WHOEORTC classification for cutaneous lymphomas.3 Patients with uncommon clinical manifestations of MF often also have classic MF on other parts of the body. Although some of them have been identified as distinct entities, they are now interpreted as being merely clinicopathological variants of MF.4 Moreover, transitional periods of different clinical variants are possible, as we have seen in our patient who had a 6-month period of generalized pustulation at the onset of her MF. We believe that the generalized pustulation in MF might be an immunological phenomenon, i.e. a disturbance of interleukin release,5 although this requires confirmation. This uncommon clinic variant may also be related to overall debility. In conclusion, pustular MF should be included in the differential diagnosis when a subcorneal pustular
Letters 909
J AM ACAD DERMATOL VOLUME 61, NUMBER 5
Fig 2. A, Acanthotic epidermis with sub- and intracorneal pustules and abscesses comprised of multiple atypical lymphocytes. (Hematoxylin-eosin stain, original magnification: 340.) B, Subepidermal bandlike lymphocytic infiltrate with epidermotropism of atypical lymphocytes and subcorneal infiltration of neutrophils. (Hematoxylin-eosin stain; original magnification: 3100.)
eruption appears histologically in association with epidermotropism of atypical lymphocytes.
lymphoma (mycosis fungoides and Sezary syndrome). Br J Dermatol 2001;144(5):1016-23.
Heike Pabsch, MD,a Johannes Kunze, MD,a and Jo¨rg Schaller, MD,a,b
doi:10.1016/j.jaad.2009.01.012
Department of Dermatology and Venerologya, St. Barbara Hospital, Catholic Clinic Duisburg, Germany, Department of Dermatology and Venerologyb, Dermatobitological Unit, St. Barbara Hospital, Catholic Clinic Duisburg, Germany Funding sources: None. Conflicts of interest: None declared. Reprints not available from the authors. Correspondence to: Heike Pabsch, MD, Department of Dermatology and Venerology, St. Barbara Hospital, Catholic Clinic Duisburg, Barbarastr. 67, D-47167 Duisburg, Germany. 149-2035199595, Fax: 149-203-5199704 E-mail: [email protected] REFERENCES 1. Sidoroff A, Halevy S, Bavinck JN, Vaillant L, Roujeau JC. Acute generalized exanthematous pustulosis (AGEP)-a clinical reaction pattern. J Cutan Pathol 2001;28(3):113-9. 2. Sidoroff A, Dunant A, Viboud C, Halevy S, Bavinck JN, Naldi L, et al. Risk factors for acute generalized exanthematous pustulosis (AGEP)-results of a multinational case-control-study (EuroSCAR). Br J Dermatol 2007;157:989-96. 3. Burg G, Jaffe ES, Kempf W, et al. WHO/EORTC Classification of Cutaneous Lymphomas. In: LeBoit P, Burg G, Weedon D, Sarasin A, editors. Tumours of the Skin. WHO Pathology and Genetics. Lyon: IARC; 2006. pp. 166-7. 4. Requena L, Gonzalez-Guerra E, Angulo J, DeVore AE, Sangueza OP. Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides. Br J Dermatol 2008;158:157-62. 5. Leroy S, Dubois S, Tenaud I, Chebassier N, Godard A, Jacques Y, Dreno B. Interleukin-15 expression in cutaneous T-cell
Chemical leukoderma induced by colored strings To the Editor: In India, it is a fairly common cultural and religious practice to wear colored strings with or without various forms of amulets round the neck, arms, or waist. We report a case series of 11 patients from eastern India who developed linear leukoderma at the sites of contact with these strings. We evaluated 11 consecutive patients with linear leukoderma associated with wearing colored strings who presented to the dermatology clinic of a tertiary care hospital over a 2-year period. None of the patients had a personal or family history of vitiligo. There was no history of symptoms, such as itching, redness, or any other lesions at the sites of depigmentation in any of the patients. No signs of inflammation or any change in texture of the skin was noticed at the sites of the pigmentary changes. There were eight men and three women. Six patients were under 2 years of age. Most (6 patients) of the lesions were located over the waist (Fig 1) followed by the neck (3), groin (2), buttocks (2), and chest (1). Depigmentation in remote areas of the body (back) was noticed in only one patient. The strings were either black (7 strings) or red (5 strings) in color. One patient had worn multiple strings. The mean duration of lesions in our patients was 9.9 months. Some of our patients had metallic (3), plastic (1), or wooden (1) amulets attached to the strings. Confetti macules around the lesions were noticed in five patients. Chemical analysis of the strings was