Autoimmune Progesterone Dermatitis

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Autoimmune progesterone dermatitis (APD) is a rare skin disorder that is marked by relapsing cyclic eruptions during the luteal phase of the menstrual cycle when the serum progesterone level increases (40). Pathogenetically, an autoallergic reaction to endogenous progesterone is involved in APD, which can be demonstrated by a positive intracutaneous test reaction to progesterone. An allergic genesis of the skin disorder is also corroborated by a positive basophilic degranulation test following provocation with progesterone (41). Cutaneous manifestations can also be provoked by the intramuscular or oral administration of progesterone. Indirect immunofluorescence can detect progesterone antibodies in the serum of some women affected with APD. Ovulation-inhibiting drugs can suppress the clinical symptoms of APD (40).

There are several hypotheses concerning the mechanism of autosensitiza-tion. One is based on the assumption that the previous use of exogenous progesterone leads to the formation of antibodies, which, as a result of cross-reactivity with endogenous progesterone, subsequently leads to premenstrual cutaneous manifestations (4,42). However, not all women with APD have taken synthetic progesterone preparations previously. Alternatively, a cross-reactivity to steroids has been proposed as the mechanism of sensitization (4). The clinical morphological picture of APD is extremely variable (Figs. 2 and 3).

The cutaneous manifestations of APD include:

2. Erythema multiforme (42,43)

3. Urticaria (42,44)

4. Angioedema, anaphylaxis (1)

5. Pompholyx (42)

6. Stomatitis (45)

7. Dermatitis herpetiformis (39)

8. Erythema annulare centrifugum (46)

9. Prurigo simplex subacuta (41)

10. Nonspecific maculopapulous exanthemas (40)

However, the cutaneous manifestations differ neither morphologically nor histologically from the cycle-independent variants. One characteristic feature, however, is that they occur in the premenstrual phase. As a rule, the different manifestations of APD do not respond to conventional therapeutic regimens of the individual disorders. Treatment options include the use of conjugated estrogen-containing preparations, the ovulation-inhibiting antiestrogen

Figure 2 Autoimmune progesterone dermatitis, eczema type. (See color insert p. 7.)

tamoxifen, the androgen danazole (4,40), or—in severe cases—an elimination of the ovaries by bilateral oophorectomy or by administering buserelin, an analog of the GnRH (4,47).

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