CLINICAL HISTORY:

Signs and symptoms:  Our patient reported diffuse pruritus without pain. His complete review of systems was otherwise negative including the presence of palpable masses.

Previous Treatment:  None.

Other information:  Past Medical, Family and Social History was non contributory. He immigrated from Mexico around 10 years prior and has no significant employment history.

PHYSICAL EXAM:

Large pink plaques with a fine overlying scale interspersed with violaceous pigmentation distributed in a reticular pattern with some notable telangiectasia and atrophy.

LABORATORY TESTS:

None. Our patient lacked insurance and was unable to comply with request for blood work or radiography.

DERMATOHISTOPATHOLOGY:

Subacute spongiotic dermatitis with scattered foci of spongiosis accompanied with mildly to moderately atypical lymphocytes. In multiple foci there are histopathologic changes suggestive of Pautrier microabscesses. There is a perivascular and interstitial lichenoid inflammatory cell infiltrate. Immunohistochemical findings showed strong CD4 positivity and focal loss of CD7 positivity.

DIFFERENTIAL DIAGNOSIS:

1.   Mycosis Fungoides - Poikiloderma Atrophicans Vasculare Variant
2.   Parapsoriasis En Plaque - Large Plaque Variety
3.   Lichen Sclerosis et trophicus
4.   Sceromyxedema
5.  

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Mycosis Fungoides - Poikiloderma Atrophicans Vasculare

DISCUSSION:

Mycosis Fungoides is the most common form of Cutaneous T-cell Lymphoma. We present a patient with an uncommon variant. Poikiloderma vasculare atrophicans is a reaction pattern characterized by mottled pigmentation, atrophy and telangiectasia. The skin has been described as having a "cigarette paper" like quality. Poikilodermatous changes have been seen with other forms of cutaneous t-cell lymphoma, connective tissue disease and certain genodermatoses. However, poikilodermatous findings on non-sun exposed areas should be considered MF until proven otherwise. Though historically considered either a separate entity or a premalignant condition, poikiloderma vasculare atrophicans is now recognized as a clinical variant of patch stage MF.
Histologically, poikilodermatous patches may show epidermal atrophy with infiltration of atypical lymphocytes. Dilated dermal vessels with a perivascular lymphocytic infiltrate may also be noted. Epidermotropism may be seen with or without pautrier microabscesses. Immunohistochemical findings will help differentiate poikilodermatous findings associated with MF from other diagnoses. Our patients biopsy is more reflective of the classic mycosis histology as it was taken from a more typical plaque.

TREATMENT:

Treatment of MF in the early stages focuses on skin-directed therapies. Based on clinical exam, our patient was staged at IB(T2N0M0). Reasonable therapy at this stage includes the use of topical steroids, UVB, PUVA, nitrogen mustard, bexarotene gel or 1,3-bis(2-chloroethyl)-l-nitrosourea(carmustine). Our patient was started on combination therapy with high potency topical steroids and narrow band UVB. To date, he is less symptomatic and has some regression of his plaques. If fails to respond adequately, other therapies as above may be considered. However, the patient's financial constraints will likely preclude the use of other more expensive and less readily available treatments.

REFERENCES:

1. James, William; Berger, Timothy; Elston, Dirk. Andrews' diseases of the Skin Clinical Dermatology. Canada: Saunders Elsevier; 2006.
2. Alexander Kreuter, Klaus Hoffmamm, Peter Altmeyer. A case of poikiloderma vasculare atrophicans, a rare variant of cutaneous T-cell lymphoma, responding to extracorporeal photopheresis. Journal of the American Academy of Dermatology. 2005;52:706-708.
3. Nicola Pimpinelli, et al. Defining early mycosis fungoides. Journal of the American Academy of Dermatology. 2005;53:1053-1063.
4. Elder, David E; et al. Lever's Histopathology of the Skin. Philadelphia: Lippincott Williams & Wilkins; 2005.

Additional Comment:

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