Postgrad Med J: first published as 10.1136/pgmj.51.597.463 on 1 July 1975. Downloaded from

Postgraduate Medical Journal (July 1975) 51, 463-467.

Poikiloderma atrophicans vasculare as a pointer to reticulosis of the ROBERT S. CHAPMAN C. JOSE PAUL M.B., M.R.C.P. M.B., B.S. Department ofDermatology, Stobhill General Hospital, Glasgow G21 3 UW

Summary Initially, these healed with simple dressings. A case of reticulosis of the skin ( fungoides) Later ulcers were more stubborn, leading him to seek terminating in reticulum cell sarcoma is described advice. where the features of poikiloderma atrophicans vasculare had been present for 40 years. The likelihood Examination of reticulosis eventually developing after such a change This showed striking pigmentation of skin on in the skin is believed to be around 50°/. trunk, face and arms, at the sites of previous ulceration (Fig. 1) and infiltration of deeper layers AN important location of reticulo-endothelial tissue of the skin. Closer examination (Fig. 2) showed the is the of the skin (Robb-Smith, 1944). As in features of poikiloderma atrophicans vasculare in- other sites, this tissue can undergo proliferation, cluding the thin which could easily be ultimately to give a lymphoma, the clinical presenta- picked up between finger and thumb. A large tion being termed by dermato- over the right scapula (Fig. 3) showed a yellow logists. Evolution is slow, 20 years or more usually indolent base, very slightly pouting edges and firmly by copyright. elapsing before frank malignancy occurs. At this adherent black . Examination was otherwise stage the fungating skin tumours which Alibert unremarkable, there being no hepatosplenomegaly first described in the early 19th century may and no lymphadenopathy of note except for small, appear. discrete nodes in the axillae. These were of the size Poikiloderma atrophicans vasculare of the skin, often found in patients with extensive skin disease defined as pigmentation, , light scaling and resulting in what is termed dermopathic lympha- and epidermal giving so-called cigarette- denopathy. Pruritus was not experienced at this time paper skin, was reported by Lane in 1923 to precede nor during the rest of his illness. Clinically, we con-

Hodgkin's disease. Another case mentioned byhim in sidered the patient to show poikiloderma atrophi- http://pmj.bmj.com/ 1921, also with poikiloderma, died eventually from cans vasculare with underlying skin reticulosis. lymphosarcoma. Increasing evidence would suggest that while poikiloderma may be found in one or two Investigations inherited disorders, in dermatomyositis and as a ESR 36 mm/hr, Hb 14'6 g/100 ml, WBC 7500/mm3 consequence of radiotherapy or of actinic damage, with 81%Y neutrophils, 2%Y eosinophils, 14% its main importance is as an indicator of present or lymphocytes and 3/% monocytes. Sternal marrow future disorder of the reticulo-endothelial system of showed no abnormality, erythropoiesis being normo- the skin. Samman (1964) found that of twenty-five blastic. Plasma cells and reticulum cells were within on September 25, 2021 by guest. Protected cases with poikiloderma, twelve were showing pro- normal limits. Urea, electrolytes, serum proteins, gression towards a reticulosis. In 1968, he reported strip electrophoresis and liver function tests were that fourteen of the twenty-five were showing such normal. Serum IgA was slightly raised at 323 mg%. progress while of thirty-five additional cases eighteen but IgG and IgM were within normal limits. Coli- were following a similar course towards reticulosis. forms, staphylococci and streptococci wererepeatedly We wish to report a case where poikiloderma grown on culture from skin ulcers. atrophicans vasculare was present for 40 years, to be Histological examination of skin showed followed bymycosis fungoides and terminal reticulum atrophy of epidermis with liquefaction degeneration cell sarcoma. of the basal layer. There was marked sub-epidermal oedema and marked telangiectasia of dermal blood Case report vessels. In the upper dermis there was a dense A man of 63 presented in 1972 with a history of inflammatory infiltrate composed of histiocytes, progressively extending areas of redness and scaling lymphocytes and a few granulocytes. There were also on his trunk from his early twenties. In 1970, ulcers many pigment-laden macrophages. Mycosis fun- appeared on his skin, mainly at sites of friction. goides or lymphoma was strongly suggested. Postgrad Med J: first published as 10.1136/pgmj.51.597.463 on 1 July 1975. Downloaded from

464 Case reports

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Case reports 465

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FIG. 3. Indolent ulcer with black eschar and slightly pouting margin. by copyright. Progress one on the upper chest which responded well to In view of the lengthy course of the patient's superficial radiotherapy. The patient was now ex- illness, radiotherapy and electron beam therapy were periencing in the right hip. Orthopaedic considered but rejected temporarily. Conservative examination indicated disease of the right sacro-iliac management failed to give healing of the ulcers, . X-ray showed erosion of the joint margins but however, and prednisone in a dose of 20 mg daily no other abnormality of the pelvis. An isotope scan was begun. Healing was rapid at all sites. Steroid indicated a considerable increase in uptake by the therapy was continued from then on, dosage being entire right ilium. Histology of a from increased to a maximum of 60 mg prednisone daily the ilium showed the marrow to be replaced by http://pmj.bmj.com/ during episodes of ulceration. Hospital admission histiocyte-like cells with pink or clear cytoplasm with was necessary at 4-6 month intervals to ensure many fine reticulin fibres between the cells, giving a adequate care of ulcerated areas. There was never diagnosis of diffuse histiocytic malignant lymphoma more than a minimal degree of pouting of the or reticulum cell sarcoma. Sternal marrow was still edges of such ulcers and no frank tumour forma- entirely normal. tion. Death occurred suddenly 3 weeks later from

Skin biopsies taken at intervals during the 18- pulmonary embolism. Autopsy revealed a large mass on September 25, 2021 by guest. Protected month period of the patient's attendance showed in the pelvis arising from the right iliac fossa with features similar to those in the initial biopsy, the destruction ofbone and invasion ofthe psoas muscle. dermal infiltrate being composed mainly of lymphoid Histological examination showed the psoas muscle cells with smaller numbers of eosinophils and (Fig. 5) to be partly replaced and partly infiltrated histiocytes or reticulum cells, some of which were by histiocyte-like cells of uniform size and shape atypical. A biopsy taken after the patient's final arranged in continuous sheets. The spleen contained admission in October 1973 showed a considerable nodules composed of cells of varying size and shape increase of the infiltrate with many histiocytes and separated by reticulin fibres. Thyroid gland and lymphocytes often with pyknotic nuclei (Fig. 4). abdominal lymph nodes showed similar features. Extension of the infiltrate to deeper layers of the Axillary and inguinal nodes showed no evidence of dermis was also prominent. neoplasm. The liver showed some fatty change. No Since there was now little evidence of the steroid other organs were involved. The final pathological being of benefit, prednisone was reduced and finally diagnosis was pleomorphic reticulum cell sarcoma discontinued early in November 1973. In spite of or malignant diffuse nodular pleomorphic histio- this, all ulcers were healing satisfactorily apart from cytic lymphoma. Postgrad Med J: first published as 10.1136/pgmj.51.597.463 on 1 July 1975. Downloaded from

466 Case reports

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FIG. 5. Psoas muscle (x 224). Muscle fibres separated by sheets of uniform histiocyte-like cells. Postgrad Med J: first published as 10.1136/pgmj.51.597.463 on 1 July 1975. Downloaded from

Case reports 467

Discussion experience is poikiloderma atrophicans vasculare in Much of the confusion over skin reticulosis is due which he places lichenoides, atrophic to the terminology employed. Mycosis fungoides can parapsoriasis and the large plaque type of parap- be considered (Samman, 1972) to be a clinical but soriasis en plaques. There may be small or large probably not a distinct pathological entity. It is patches of skin involvement as in our patient, often logical to expect such a reticulosis, at least in a per- symmetrically involving chest, buttocks and flexures. centage of cases, to become progressively more Definite infiltration indicates the onset of mycosis primitive giving Hodgkin's disease, lymphosarcoma fungoides or other reticulosis. The upper dermis on or reticulum cell sarcoma, sometimes with terminal histology will show a predominantly lymphocytic leukaemia. The earlier polymorphous dermal infil- infiltrate with excess of reticulum cells and histio- trate becomes simplified (Lever, 1967) as host cytes-unlike the appearances in a benign . resistance diminishes with the lymphoma cell becom- In conclusion, our patient would appear to have ing the dominant cell type. While some (Milne, 1972) had progressive involvement of head, arms and would restrict the term mycosis fungoides to a trunk by poikiloderma atrophicans vasculare over reticulosis confined clinically and pathologically to the course of 40 years. Transition to mycosis fun- the skin, another point of view (Clendenning, 1971) goides probably occurred with the appearance of would prefer the term mycosis fungoides to include ulceration. Although fungating tumours were never every malignant reticulosis originating in the skin present, the features were in keeping with those of and to be retained even when spread occurs to other the tumour stage of mycosis fungoides. With pro- reticulo-endothelial structures such as lymph nodes gressive increase in malignancy, systemic spread and bone marrow or to other organs. Such a holistic occurred after 3 years as reticulum cell sarcoma. approach is supported by studies of the outcome of mycosis fungoides. Visceral dissemination was found Acknowledgments (Block et al., 1963) in seventeen patients at autopsy. We wish to thank Dr R. R. Wilson, Consultant Patholo- Three other patients with cutaneous changes had no gist, for his help and guidance, Mr G. Illingworth, Consultant evidence of visceral involvement at post-mortem Orthopaedic Surgeon, for help in management, and Mr by copyright. suggesting that mycosis fungoides is not preceded by Fergus Colman, Department of Medical Illustration, for visceral lymphoma. It seems likely that mycosis producing the photographs and photomicrographs. fungoides arises primarily in the skin, not coinci- dentally with visceral lymphoma. Of 106 cases with References biopsy-proved BLOCK, J.B., EDGCOMB, J., EisEN, A. & VAN Scorr, E.J. (1963) mycosis fungoides (Cyr, Geokas and Mycosis fungoides. Natural history and aspects of its Worsley, 1966), fourteen showed terminal reticulum relationship to other malignant lymphomas. American cell sarcoma with five having monocytic leukaemia. Journal of Medicine, 34, 228. Fifteen others had lymphosarcoma, six of them also CLENDENNING, W.E. (1971) Mycosis fungoides. In: Modern Trends in. Dermatology-4 (Ed. by P. Bornie), p. 214. http://pmj.bmj.com/ having terminal lymphoblastic leukaemia. Another Butterworth & Co., London. seven patients had Hodgkin's disease terminally. CYR, D.P., GEOKAS, M.C. & WORSLEY, G.H. (1966) Mycosis However, in twenty-three of the forty-two patients fungoides. Haematologic findings and terminal course. from the series who came to autopsy the disease Archives of Dermatology, 94, 558. remained as mycosis fungoides LANE, J.E. (1921) Poikilodermia atrophicans vascularis. throughout its course. Archives ofDermatology and Syphilology, 4, 563. The skin in classical mycosis fungoides of the LANE, J.E. (1923) Poikiloderma atrophicans vasculare. Con- Alibert type are usually non-specific eczematous or clusion of previously reported case. Archives of Dermato- and psoriasiform plaques of varying colour and bizarre logy Syphilology, 8, 373. on September 25, 2021 by guest. Protected shape which fail to respond to therapy and progress LEVER, W.F. (1967) Mycosis fungoides. In: Histopathology of the Skin, 4th edn. p. 757. Pitman Medical Publishing to infiltration and tumours, often with severepruritus. Co., London. Diagnostic difficulties centre on the question of what MILNE, J.A. (1972) Lymphoid neoplasms of the skin. In: An skin lesions indicate a pre-reticulotic state. In the Introduction to the Diagnostic Histopathology of the Skin, light of experience, a greatly simplified classification Edward Arnold, London. Ist edn, p. 230. ROBB-SMITH, A.H.T. (1944) The reticular tissue and the skin. has been put forward (Samman, 1972). Conditions British Journal ofDermatology, 56, 151. which rarely, if ever, progress to reticulosis he would SAMMAN, P.D. (1964) Survey of reticuloses and pre-mycotic include under the heading ofparapsoriasis en plaques eruptions. A preliminary report. British Journal of (benign type) including such entities as xantho- Dermatology, 76, 1. SAMMAN, P.D. (1968) St. John's Hospital reticulosis erythroderma perstans, chronic superficial dermatitis register. Transactions of the St. John's Hospital Dermato- and the small, discrete type of parapsoriasis en logical Society, 54, 200. plaques. Histology will show only the features of SAMMAN, P.D. (1972) Mycosis fungoides. In: Textbook of eczema. Dermatology (Ed. by A. Rook, D. S. Wilkinson and F. J. G. Ebling), 2nd edn, p. 1396. Blackwell Scientific The one significant pre-mycotic eruption in his Publications, Oxford.