Papular mucinosis

a case of lichen myxedematosus by Dr/ Rania Alakad describing the clinical and histological findings of the disease.

1. Rania Alakad Assistant lecturer Dermatology,Venereology & Andrology department Zagazig University

2. 55 ♀ patient. Skin lesions, 4 months duration on the neck then progressed to involve the upper chest. Mild itching. No systemic affection Irrelevant medical history

3. Laboratory investigations CBC RFT – LFT Random blood sugar. Pelvi -abdominal US.

4. Differential diagnosis Sarcoidosis Papular mucinosis . Xanthomas Pseudoxanthoma elasticum ,

5. Papular mucinosis (Lichen myxedematosus )

6. Papular mucinosis Papular mucinosis is a primary form of cutaneous mucinosis characterized by an abnormal deposition of mucin in the skin. Divided into: Generalized ( scleromyxedema ). Localized forms.

7. Increased certain serum cytokines ( IL1, TNF α , TGF β ) and immunoglobulins were hypothesized to cause fibroblasts proliferation and increased mucin deposition in the skin. the disease is commonly associated with monoclonal gammopathy paraproteinemia .

8. a generalized form ( scleromyxedema ): Papular lesions . Sclerodermoid features. Paraproteinemia . systemic manifestations.

9. The localized form: papules , nodules and plaques that are skin colored, waxy and asymptomatic No sclerosis. No paraproteinemia No systemic involvement

10. This localized type is divided into five subtypes : 1 ) the discrete papular form. 2 ) acral persistent papular mucinosis . 3) spontaneous self healing mucinosis 4 ) cutaneous mucinosis of infancy. 5 ) the purely nodular form .

11. Serum protein electrophoresis

12. After 1 month

13. Localized papular mucinosis (self healing type) Localized papular lesions. No systemic affection. No paraproteinemia . Spontaneous self healing .

14. Treatment Topical: Tacrolimus Betamethasone (DMSO) Retinoids Systemic : Corticosteroids , Thalidomide, Interferon, Retinoids , Plasmapheresis , ECP, Immunoglobulin Melphalan chemotherapy with autologous stem cell transplantation

15. P apular mucinosis P araprotein P apules B lue