CAMEO
CUTANEOUS LYMPHOMA SOSHAMMA GEORGE, M.D., M.N.A.M.S., SUSY KURIAN, M.D., AND MARY JACOB, M.N.A.M.S.
A 36-year-old man was admitted with a history of recurrent skin lesions of 25 years duration and a large nonhealing ulcer on the abdominal wall of 2 years duration. His symptoms began as recurrent, itchy raised erythematous lesions on the extremities with no associated constitutional symptoms. Some of the lesions bad ulcerated and healed, while others had resolved spontaneously. The nonhaaling ulcer on the abdominal wall started as an erythematous lesion, which gradually ulcerated and then expanded rapidly over a period of 4 months. Physical examination revealed a large ulcer on the abdominal wall, which measured 30 x 25 cm. Tbe floor was covered by unhealthy granulation tissue, slough, and purulent discharge. The edges were rolled out and raised. There was induration and erythema of the surrounding skin (Eig. 1). A well-defined hyperpigmented plaque was present on the left cheek, and there were numerous depressed scars all over the body. There was no significant lymphadenopatby, and systemic examination revealed no abnormality. A biopsy from the plaque on the left cheek showed the appearances of discoid lupus erythematosus. The epidermis in tbis biopsy, however, showed focal disarray due to the presence of cells with nuclear enlargement and mitotic activity. In order to rule out the possibility of a malignant process, another biopsy was considered. As there were no other active skin lesions, biopsies were taken from the edges of the large ulcer on the anterior abdominal wall. The initial two biopsies showed nonspecific chronic inflammation. A third deeper biopsy showed in the dermis, beneath a layer of chronic inflammatory cells, a sheet-like infiltrate of moderately large pleomorphic cells (Eig. 2). Under higher power these ploemorphic cells showed vesicular nuclei and a central prominent nucleolus (Eig. 3). There was increased mitotic activity. Immunostaining showed positive staining for leukocyte common antigen. A diagnosis of malignant lymphoma of the large cell immunoblastic type was made.^ No further tests were available to establish B- or T-cell phenotype.
Figure 1. Large ulcer on anterior abdominal wall with raised rolled out edges.
Chest x-ray, bone marrow examination, ultrasound of abdomen, and pelvic scan did not reveal any evidence of extracutaneous visceral or lymph nodal involvement. Hiv antibody test was negative. A staging laparotomy could not be performed because of the extensive ulceration of the abdominal wall. The patient was given chemotherapy using cyclophos.phamide adriamycin, prednisolone, and vincristine. The patient, however, deteriorated and died 2 weeks later of septicemia.
DISCUSSION
Primary cutaneous lymphomas, excluding mycosis fungoides, are often difficult to identify botb clinically and histologically. The overall skin involvement in nonHodgkin's lymphoma is approximately 20%, and the skin is tbe second most common extralymphnodal site of involvement.' Men over 50 years of age are generally more affected. A young age of onset of lymphoma, as in our patient, has been reported by Shelley,-' with disease occurring as early as 10 years and 24 years. Although the head and neck are the commonest sites of involvement,"* lesions involving the abdominal wall have also been reported.^ The presentation can be either as a solitary or as multiple cutaneous lesions.''
Laboratory investigations showed a raised ESR as the only abnormal test. Tests for collagen disease were negative. Smears from the ulcer for Leishmania donovani bodies and for Entamoeba histolytica were negative. Tissue cultures for fungal organisms were negative. Mantoux and dinitrochlorobenzene tests were negative.
From the Departments of Dermatology and Pathology, Christian Medical College & Hospital, Vellore, India. Address for correspondence: Soshammn George, M.D., Department of Dermatology, Christian Medical College & Hospital, Vellore-632004, India. 789
Intcrnntional loiirnal of t^crtnatology Vol. i i . No. I t, November 1992
Figure 3 High-power photomicrograph showing large cells with vesicular nuclei and a .single inclusion like nucleolus. (hematoxylin and eosin stain, original magnification x 620)
phoma, the physical well being despite tbe protracted course of illness, the presence of extensive ulceration, and the apparent spontaneous resolution of sotne of the lesions. Multiple biopsies were necessary to establish tbe diagnosis as has been observed by other workers.'
Figure 2. Low power photomicrograph showing a bandlike infiltrate of cells in mid dermis (arrow showing rete peg). (hematoxylin and eosin stain, original magnification x 160)
As noticed by others,' in our case also, the disease may have been active for many months or even years before the histologic diagnosis was made. Some of the lesions, we presume, healed spontaneously, as reported by Kim and Winkelrnann"" in 3 of their 16 cases. Two of these three cases had relatively long survival periods of 4 and 7 years respectively. Shelley' reports an apparent remission for 20 years in one of his cases. Because several benign cutaneous lymphoid infiltrates simulate lymphoma of the skin, there is considerable uncertainty regarding the histologic reliability of the diagnosis of malignant lymphotna based on a skin biopsy alone.-''' The tnost significant cytologic feature of malignant lyrnphoma is the cellular monomorphistn and cytologic atypia''"** with the infiltrate usually involving the mid and deep dermis.^-^ Cutaneous ulceration is rare in non-Hodgkins lymphoma as seen in only 3 out of 25 cases reviewed by Long'' and in 6 out of .SO cases reported by Burke et al.^ The prognosis is worsened by initial extent of skin and extracutaneous involvement, the presence of ulceration' and an unfavorable histologic subtype.^•'' Some feel that malignant lytnphoma with skin lesions as a primary manifestation almost invariably disseminates and usually has a fatal outcome.^•'' This case is reported to highlight the following unusual features: the young age of onset of the lyrn-
Acknowledgniciit: Mrs. Linda Robert typed the manitscript. REFERENCES
1.
2. 3. 4. 5. 6.
7. 8.
9.
790
The Non-Llodgkins Lymphoma Pathologic Classification Project, National Cancer Institute Sponsored Study of C:lassificati()ns of Non Llodgkin's Lymphoma: Sutntnary and description of a workitig fortiuilation for clinical usage. Cancer 1982; 49:21 12-213.5. Wilkel CS, Grant-kels j . Cutaneous B-cell lymphoma. Arch Dermatol 1987; 123:1362-1367. Long |C, Mihtii MC, Quazi R. Malignant lyiiiplioma of the skin. Cancer 1976; 38:1282-1296. Kim R, Winkeltnann RK, Dockerty M. Reticulum cell sarcoma of the skin. Cancer 1963; 16:646-65.5. Burke JS, Hoppe RT, Cibull ML, Dorfman RF. Cutaneous malignant lytiiphotna. Cancer 198 1; 47:300-310. Shelley WB, Graywood M. Observations on occult malignant lymphomas in the skin. Cancer 1976; 38:1757-1770. Caro WA, Helwig EB. Ckitaneous lymphoid hyperplasia. Caticer 1969; 24:487-502. Fisher ER, Park EJ, Wechsler HL. Histologic identification of malignant lymphoma cutis. Arn J Cliti Pathol 1976; 65:149-158. Evans HL, Winkeltnann RK, Banks PM. Differential diagnosis of rnalignant and benign cutaneous lymphoid infiltrates. Cancer 1979; 44:699-717.
CUTANEOUS LYMPHOMA SOSHAMMA GEORGE, M.D., M.N.A.M.S., SUSY KURIAN, M.D., AND MARY JACOB, M.N.A.M.S.
A 36-year-old man was admitted with a history of recurrent skin lesions of 25 years duration and a large nonhealing ulcer on the abdominal wall of 2 years duration. His symptoms began as recurrent, itchy raised erythematous lesions on the extremities with no associated constitutional symptoms. Some of the lesions bad ulcerated and healed, while others had resolved spontaneously. The nonhaaling ulcer on the abdominal wall started as an erythematous lesion, which gradually ulcerated and then expanded rapidly over a period of 4 months. Physical examination revealed a large ulcer on the abdominal wall, which measured 30 x 25 cm. Tbe floor was covered by unhealthy granulation tissue, slough, and purulent discharge. The edges were rolled out and raised. There was induration and erythema of the surrounding skin (Eig. 1). A well-defined hyperpigmented plaque was present on the left cheek, and there were numerous depressed scars all over the body. There was no significant lymphadenopatby, and systemic examination revealed no abnormality. A biopsy from the plaque on the left cheek showed the appearances of discoid lupus erythematosus. The epidermis in tbis biopsy, however, showed focal disarray due to the presence of cells with nuclear enlargement and mitotic activity. In order to rule out the possibility of a malignant process, another biopsy was considered. As there were no other active skin lesions, biopsies were taken from the edges of the large ulcer on the anterior abdominal wall. The initial two biopsies showed nonspecific chronic inflammation. A third deeper biopsy showed in the dermis, beneath a layer of chronic inflammatory cells, a sheet-like infiltrate of moderately large pleomorphic cells (Eig. 2). Under higher power these ploemorphic cells showed vesicular nuclei and a central prominent nucleolus (Eig. 3). There was increased mitotic activity. Immunostaining showed positive staining for leukocyte common antigen. A diagnosis of malignant lymphoma of the large cell immunoblastic type was made.^ No further tests were available to establish B- or T-cell phenotype.
Figure 1. Large ulcer on anterior abdominal wall with raised rolled out edges.
Chest x-ray, bone marrow examination, ultrasound of abdomen, and pelvic scan did not reveal any evidence of extracutaneous visceral or lymph nodal involvement. Hiv antibody test was negative. A staging laparotomy could not be performed because of the extensive ulceration of the abdominal wall. The patient was given chemotherapy using cyclophos.phamide adriamycin, prednisolone, and vincristine. The patient, however, deteriorated and died 2 weeks later of septicemia.
DISCUSSION
Primary cutaneous lymphomas, excluding mycosis fungoides, are often difficult to identify botb clinically and histologically. The overall skin involvement in nonHodgkin's lymphoma is approximately 20%, and the skin is tbe second most common extralymphnodal site of involvement.' Men over 50 years of age are generally more affected. A young age of onset of lymphoma, as in our patient, has been reported by Shelley,-' with disease occurring as early as 10 years and 24 years. Although the head and neck are the commonest sites of involvement,"* lesions involving the abdominal wall have also been reported.^ The presentation can be either as a solitary or as multiple cutaneous lesions.''
Laboratory investigations showed a raised ESR as the only abnormal test. Tests for collagen disease were negative. Smears from the ulcer for Leishmania donovani bodies and for Entamoeba histolytica were negative. Tissue cultures for fungal organisms were negative. Mantoux and dinitrochlorobenzene tests were negative.
From the Departments of Dermatology and Pathology, Christian Medical College & Hospital, Vellore, India. Address for correspondence: Soshammn George, M.D., Department of Dermatology, Christian Medical College & Hospital, Vellore-632004, India. 789
Intcrnntional loiirnal of t^crtnatology Vol. i i . No. I t, November 1992
Figure 3 High-power photomicrograph showing large cells with vesicular nuclei and a .single inclusion like nucleolus. (hematoxylin and eosin stain, original magnification x 620)
phoma, the physical well being despite tbe protracted course of illness, the presence of extensive ulceration, and the apparent spontaneous resolution of sotne of the lesions. Multiple biopsies were necessary to establish tbe diagnosis as has been observed by other workers.'
Figure 2. Low power photomicrograph showing a bandlike infiltrate of cells in mid dermis (arrow showing rete peg). (hematoxylin and eosin stain, original magnification x 160)
As noticed by others,' in our case also, the disease may have been active for many months or even years before the histologic diagnosis was made. Some of the lesions, we presume, healed spontaneously, as reported by Kim and Winkelrnann"" in 3 of their 16 cases. Two of these three cases had relatively long survival periods of 4 and 7 years respectively. Shelley' reports an apparent remission for 20 years in one of his cases. Because several benign cutaneous lymphoid infiltrates simulate lymphoma of the skin, there is considerable uncertainty regarding the histologic reliability of the diagnosis of malignant lymphotna based on a skin biopsy alone.-''' The tnost significant cytologic feature of malignant lyrnphoma is the cellular monomorphistn and cytologic atypia''"** with the infiltrate usually involving the mid and deep dermis.^-^ Cutaneous ulceration is rare in non-Hodgkins lymphoma as seen in only 3 out of 25 cases reviewed by Long'' and in 6 out of .SO cases reported by Burke et al.^ The prognosis is worsened by initial extent of skin and extracutaneous involvement, the presence of ulceration' and an unfavorable histologic subtype.^•'' Some feel that malignant lytnphoma with skin lesions as a primary manifestation almost invariably disseminates and usually has a fatal outcome.^•'' This case is reported to highlight the following unusual features: the young age of onset of the lyrn-
Acknowledgniciit: Mrs. Linda Robert typed the manitscript. REFERENCES
1.
2. 3. 4. 5. 6.
7. 8.
9.
790
The Non-Llodgkins Lymphoma Pathologic Classification Project, National Cancer Institute Sponsored Study of C:lassificati()ns of Non Llodgkin's Lymphoma: Sutntnary and description of a workitig fortiuilation for clinical usage. Cancer 1982; 49:21 12-213.5. Wilkel CS, Grant-kels j . Cutaneous B-cell lymphoma. Arch Dermatol 1987; 123:1362-1367. Long |C, Mihtii MC, Quazi R. Malignant lyiiiplioma of the skin. Cancer 1976; 38:1282-1296. Kim R, Winkeltnann RK, Dockerty M. Reticulum cell sarcoma of the skin. Cancer 1963; 16:646-65.5. Burke JS, Hoppe RT, Cibull ML, Dorfman RF. Cutaneous malignant lytiiphotna. Cancer 198 1; 47:300-310. Shelley WB, Graywood M. Observations on occult malignant lymphomas in the skin. Cancer 1976; 38:1757-1770. Caro WA, Helwig EB. Ckitaneous lymphoid hyperplasia. Caticer 1969; 24:487-502. Fisher ER, Park EJ, Wechsler HL. Histologic identification of malignant lymphoma cutis. Arn J Cliti Pathol 1976; 65:149-158. Evans HL, Winkeltnann RK, Banks PM. Differential diagnosis of rnalignant and benign cutaneous lymphoid infiltrates. Cancer 1979; 44:699-717.
