 Abstract | | |
Poikilodermic parapsoriasis is a rare varaint of poikiloderma where the chances of going into mycoses fungoides exists.But not all cases do go to mycoses. In the early stage of poikiloderma atrophicans vasculare, without respect to its course, shows moderate thinning of stratum malpigji, effacement of rete ridges, hydropic degeneration of basal cells. In the upper dermis-there is band like infiltrate which in places invades the epidermis. The infiltrate consists of a few histiocytes-so called initial 'lichenoid pattern'. In addition there is abundance of melanophages with pigment incontinence. In late stages there is thinning of is with hydropic degeneration of basal cells. In MF inflammatory infiltrate increases rather than decreases in time. Large hyperchromatic nuclei, so called 'mycoses cells' are likely to be present in this variant and often there is moderate to marked epidermotropism. It may result in Pautrier microabscess. formation.The diagnosis of Mycoses Fungoides is supported by presence of an aberrant phenotype, for example T cell lacking one or more pan T cell antigens. Most commonly absent antigen is CD-7 followed by CD-2, CD-3 and CD-5 3 . Making a differential diagnosis between early MF and parapsoriasis is often difficult at clinical and histological level. We report two cases of poikilodermic parapsoriasis, both showing some degree of epidermotropism ,but one had panT cell marker CD 3, CD5, CD7 present and other absent.The lady with Pan T cell markers did not progress to frank mycoses fungoides while the patient with absent markers progressed rapidly and finally succumbed.
Keywords: Pokiloderma parapsoriasis, review of literature, lichenoid pattern
How to cite this article:
Das S, Giri PP. Poikilodermatous parapsoriasis - two cases with review of literature. Indian J Dermatol 2009;54, Suppl S1:32-6 |
How to cite this URL:
Das S, Giri PP. Poikilodermatous parapsoriasis - two cases with review of literature. Indian J Dermatol [serial online] 2009 [cited 2021 Mar 9];54, Suppl S1:32-6. Available from: https://www.e-ijd.org/text.asp?2009/54/5/32/45440 |
| Introduction | |  |
Poikiloderma like lesions are features of early mycoses fungoides that may be seen in any of the two clinical forms- either as large plaque like or parapsoriasis en plaque also known as poikilodermatous parapsoriasis or as parapsoriasis variegata which in early stage manifests as a net like pattern. Though these two types of parapsoriasis represent an early stage of mycoses fungoides - not all cases progress clinically into fully developed mycoses fungoides. Cases in which there is no progression to mycoses fungoides has been described as idiopathic poikiloderma atrophicans vasculare. [1]
Clinically, the form poikiloderma atrophicans vasculare is applied to lesions that in the early stage, show erythema and telangiectasia. In the late stage the skin appears atrophic and the erythema is less pronounced and the telangiectasias are more pronounced.
In the early stage poikiloderma atrophicans vasculare, without respect to its course, shows moderate thinning of stratum malpigji, effacement of rete ridges, hydropic degeneration of basal cells. In the upper dermis, there is band like infiltrate which in places invades the epidermis. The infiltrate consists of a few histiocytes - so called initial 'lichenoid pattern'. In addition, there is abundance of melanophages with pigment incontinence. In late stages, there is thinning of epidermis with hydropic degeneration of basal cells. [2]
In poikiloderma atrophicans vasculare associated with genodermatoses, the mononuclear cells are mild to absent whereas in those associated with dermatomyositis or SLE there is only slight dermal infiltration. In contrast in MF inflammatory infiltrate increases rather than decreases in time. Large hyperchromatic nuclei, so called 'mycoses cells' are likely to be present in this variant and often there is moderate to marked epidermotropism. It may result in Pautrier microabscess More Details formation.
The histologic hallmark of mycoses fungoides is epidermotropism, which is defined as presence of atypical Tcells in epidermis. Mycoses fungoides usually is a neoplastic proliferation of CD-4 positive, CD-8 negative Tcells [Table 1].
The diagnosis of Mycoses Fungoides is supported by presence of an aberrant phenotype, for example T cell lacking one or more pan T cell antigens. Most commonly absent antigen is CD-7 followed by CD-2, CD-3 and CD-5. [3] Making a differential diagnosis between early MF and parapsoriasis is often difficult at clinical and histological level. Expression of MY17(CD-13) antigen which is lacking in cutaneous T cell lymphomas (butnot in intermediate lesions), and rearrangment of T cell receptor gene were analyzed and they observed that MY-7 antigen (CD-13) combined with T cell clone can help the dermatologists to confirm the diagnosis of early MF. [4] However further studies are required to corroborate it.
| Case History | | |
Patient 1: A 40-year-old female patient presented with erythematous macules and plaques on forearm, bilaterally gradually spreading to trunk, limbs and face for two to three years [Figure 1],[Figure 2]. She also had rippled pigmentation on head and neck for 4 years.
Other sites are asymptomatic
Reports of serial skin biopsies with serial evolution of the diseases: INITIAL ONE - Saving lichenoid pattern, no features of mycoses fungoides [Figure 3].
Lymph node biopsy-Dermatopathic lymphadenitis. Improved spontaneously for first two to three years, but no improvement for the last two years. After one year, lesions have extended into forearm with marked photosensitivity.
No plaque or tumor was found at this stage.
CT of chest done on admission shows- Mild hepatosplenomegaly.
Imprint smear shows no atypical cells.
Bone marrow - No abnormality detected.
Serum electrophoresis - WNL.
Patient was put on Narrow band UVB with Acitretin therapy - 70-80% success after three month therapy.
Skin biopsy
Moderate hyperkeratosis and focal parakeratoses with focal acrokeratoses. Flattened epidermis with lichenoid pattern of interface dermatitis was present. Exocytoses was seen [Figure 4]. Stain for Toludine Blue was negative.
Lymph node biopsy
Lymphoid follicle with intact germinal centre was present. Cortical area was expanded; infiltrate of lymphocytes, plasma cells and histiocytes were present. No granuloma or no evidence of lymphoma was present.
Impression - Dermatopathic lymphadenitis.
Sections done on 04.12.2003 show mild hyperkeratosis and focal acrokeratoses of epidermis, Rete ridges were not effaced. Occasional microabscess were seen in keratin layer in left axillary skin biopsy. A spongioform pustule was found in cubital fossa biopsy. Papillary dermis did not show fibrosis. A moderate infiltrate of lymphocytes and histiocytes were present. Cells in upper dermis in a lichenoid pattern was seen. Exocytoses of these cells in an occasional focus but neither there was any suprabasal linear distribution of lymphocytes. None of these cells were atypical. Mild perivascular and periappendageal inflammatory infiltrate consistent with poikilodermic parapsoriasis.
Serum ASO Titre - Not elevated
Serum ANA by Hep-2 method - Negative.
Skin biopsy done subsequently shows -
Thinned out epidermis with loss of rete ridges focal basal spongiosis. Moderate lymphangitic infiltrate in the subjacent dermis with focal intraepidermal extension was noted.
Some histiocytes admixtures with many macrophages are present within the infiltrate.
Dermis showed capillary proliferation pericapillary lymphangitic infiltrate.
Those intraepidermal lymphocytes are mostly small and appeared mature on morphology. A few had larger size slight convulated nuclei. Dermal collections were present but not in adequate amount. Features were consistent with poikilodermic parapsoriasis.
Serum B2 Microglobulin= 2-36 micro gm/dl.
Serum protein Electrophoresis= Normal pattern with mild hypergummaglobulinemia
Skin biopsy done on 09.01.2007 shows -
Section shows atrophic epidermis, upper dermis showed lichenoid infiltrate of lymphomononuclear cells, some of which are atypical. Epidermotropism with prominent lymphoid cells in cluster of rows closed to basal layers
CD-3, CD-5 CD-7 were positive.
CD-30 was negative.
Skin biopsy done subsequently shows -
Thinned out epidermis with loss of rete ridges focal basal spongiosis. Moderate lymphangitic infiltrate in the subjacent dermis with focal intraepidermal extension was noted.
Some histiocytes admixtures with many macrophages are present within the infiltrate.
Dermis showed capillary proliferation and pericapillary lymphangitic infiltrate.
Those intraepidermal lymphocytes are mostly small and appeared mature on morphology. A few had larger size slight convulated nuclei. Dermal collections were present not in adequate amount. Features were consistent with poikilodermic parapsoriasis.
Serum B2 microglobulin= 2-36 micro gm/dl.
Serum protein electrophoresis= Normal pattern with mild hypergammaglobulinemia.
She was put on Narrow band UVB and acitretin along with emollients and sunscreen. After receiving nearly 70 UV B sessions andacitretin therapy she had clearance of eczematous lesions by 80% anderythema by 60%. No plaque or nodule was found [Figure 4] and [Figure 5]. There was significant histological alteration also [Figure 8]. Pavlotsky et al , [5] in a study from Turkey reported 83.3% clearing in patients of early stage mycoses fungoides with narrow band UVB.
Second patient
A 9-year-old girl was admitted in the Medicine department with severe anemia. On examination she had few ill defined plaques in addition to easy bruisability [Figure 6],[Figure 7]. She had pancytopenia, bone marrow showed involvement in the form of pan Tcell markers CD-3 CD-5 CD-7 were negative on immunophenotyping studies. On next visit in 10 days time she had infiltrated plaques on face, back and thighs. She deteriorated very quickly and developed hepatosplenomegaly and lymphoadenopathy in the next 15 days time. She was put on chemotherapy by hemato-oncolgists but she succumbed in 14days time. Skin biopsy done showed epidermotropism and intraepidermal atypical T lymphocytes. Because the patient succumbed very quickly and also hails from poor economic background, no further investigation was possible.
| Discussion | | |
Poikilodermic parapsoriasis is a problematic area to handle even by best clinicians in the field of dermatology. It is a premalignant condition but not all cases progress to mycoses fungoides but which case will progress is difficult to predict. Mycoses fungoides is usually a neoplastic proliferation of CD-4 positive, CD-8 negative T cells. The diagnosis of lymphoma is supported by presence of an aberrant phenotype, for e.g. T cells lacking one or more pan T cell markers. Loss of more than one pan T cell antigen is particularly indicative of progression of T cell lymphoma.
The early lesions of mycoses fungoides with minimal cytologic atypia and late tumors without epidermotropism are the most challenging to diagnose. In these cases, repeated skin biopsies and Immunophenotype and molecular studies with a consideration of other subclass of cutaneous T cell lymphoma will aid in arriving in diagnosis.
| References | | |
| 1. | Saman PD. The natural history of parapsoriasis en plaques (chronic superficial dermatitis) and prereticulotic poikiloderma. Br J Dermatol 1972;87:405-11.  |
| 2. | Jaworsky C. Connective tissue diseases. In : Elder D, editor. LEVERS Histopathology of skin. 9 th Indian ed. New Delhi: Lippincot Williams and Wilkins; p. 294-314. |
| 3. | Bardach H, Raff M. Poikilodermic parapsoriasis: Case report and discussion of nomenclature. Hautarzt 1977;28:542-6. [PUBMED] |
| 4. | Wood GS. Benign and malignant cutaneous lymphoproliferative disorders including mycoses fungoides. In : Knowles DM, editor. 4 th ed. Neoplastic haematopathology. Baltimore: Williams and Wilkins; 1992. p. 1117-51. |
| 5. | Pavlotsky F, Barzilai A, Kasem R, Shpiro D, Trau H. UVB in the management of early stage mycosis fungoides. Eur Acad Dermatol Venerol 2006;20:565-72. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
[Table 1] |
