A specific cutaneous lesion revealing myelodysplastic syndrome

ARTICLE

Myelodysplastic syndrome (MDS) is characterized by ineffective and dysplastic hematopoiesis in one or more cell lines in the bone marrow. Cutaneous lesions of MDS are usually separated into specific lesions and nonspecific lesions, and whether atypical hematopoietic cells infiltrate the skin or not. Early diagnosis of specific cutaneous lesions of MDS is important, because these lesions can be the only clue to the diagnosis of MDS and may precede acute transformation.

We report here on a patient with MDS, which was revealed by a specific cutaneous lesion in the form of an ulcerated nodule.

Case report

An 86-year-old man visited our clinic in December 1991, because of an ulcerated nodule on his left leg, with an erythematous border and fibrinous exudate (Fig. 1). Histological findings from this ulcerated nodule showed a dense infiltration of atypical cells in the upper dermis through the subcutaneous tissue (Fig. 2). Neoplastic cells varied in size and were pleomorphic. Most of the infiltrate was composed of large cells with round nuclei and prominent nucleoli (Fig. 3). Immunohistochemical studies were performed on paraffin sections. The atypical cells were negative for CD3, L26, CD68, lysozyme, and glycopholin A. Periodic acid-Schiff (PAS) stain did not demonstrate any micro-organisms. The blood count disclosed pancytopenia (8.3 x 10⁸ white blood cells/l with 8% blasts, 28 x 10¹¹ red blood cells/l, 17 x 10⁶ platlets/l). A bone marrow smear showed hypercellularity with 25.7% blasts. These findings were compatible with the refractory anemia with an excess of blasts in transformation (RAEB-T), typical of MDS. The patient was then transferred to the hematology department to receive chemotherapy. The ulcerated nodule decreased in size in response to combination chemotherapy (methyl predonisolone, cytosine arabinoside, aclarubicine, etoposide), but two months after visiting our out-patient clinic, acute transformation occurred and the patient died.

Discussion

MDS comprises five pathological entities based on cytological features and blast-cell numbers in bone marrow smears and peripheral blood; (i) refractory anemia (RA), (ii) RA with ring sideroblasts (RARS), (iii) RA with an excess of blasts (RAEB), (iv) chronic myelomonocytic leukemia (CMML), (v) RAEB in transformation (RAEB-T) [1].

Transformation into acute myelogenous leukemia develops in 6 to 37% of patients with MDS [2].

Various types of skin manifestations may occur during the course of MDS. The non-specific lesions, which resemble Sweet's disease or pyoderma gangrenosum, are characterized by an infiltration of neutrophils and are known as neutrophilic dermatosis of MDS.

Specific lesions result from the dermal invasion of malignant hematopoietic cells. Specific cutaneous lesions of MDS are very rare [3]. Aractingi reported 18 out of 36 patients died in less than 3 months after the specific lesions developed [4]. The presentation of the specific cutaneous lesion in MDS is closely related to acute transformation and rapid deterioration.

The clinical appearance of specific lesions in MDS consists mainly of papules, nodules, and tumors [4]. Our case was characterized by an ulcerated nodule which mimicked pyoderma gangrenosum. A skin biopsy demonstrated dense infiltrates of atypical hematopoietic cells, and immunohistochemical studies showed that almost all infiltrated cells were negative for lymphocytic, monocytic and erythrocytic markers. These findings supported the notion that cells were myeloid origin.

Occasionally only a subjective symptom such as a skin lesion precedes the diagnosis of MDS, as in our case. Peripheral blood examination before biopsy was very helpful for the diagnosis.

REFERENCES

1. Bennet JM, Catvsky D, Daniel MT. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 1982; 51: 189-99.

2. Sanz GF, Sanz MA, Vallespi T, Canizo MC, Torrabadella M, Garcia S et al. Two regression models and scoring system for predicting survival and planing treatment in myeloproliferative syndromes: investigation of prognostic factors in 370 patients. Blood 1989; 74: 395-408.

3. Jacobs RH, Cornbleet MA, Vardiman JW, Larson RA, Le Beau MM, Rowley JD. Prognostic implications of morphology and karyotype in primary myelodysplastic syndromes. Blood 1986; 67(6): 1765-72.

4. Aractingi S, Bachmeyer C, Miclea JM, Verola O, Rousselot P, Dubertret L, et al. Unusual specific cutaneous lesions in myelodysplastic syndromes. J Am Acad Dermatol 1995; 33: 187-91.